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__NOTOC__
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{{SI}}
{{SI}}
{{CMG}}
{{CMG}}; {{AE}} {{Hudakarman}}


{{SK}} Gammel's disease.
{{SK}} [[Gammel's disease]]




==Overview==
==Overview==
Erythema gyratum repens (EGR) is a rare dermatologic disorder associated with a variety of internal malignancies (paraneoplastic cutaneous syndrome). The most common malignancies EGR associated with are Lung, esophageal, and breast cancers. EGR is a systemic disease and it has a characteristic generalized rash.  
   
Erythema gyratum repens is a rare highly specific and characteristic [[Paraneoplastic Syndromes|paraneoplastic s]][[syndrome|yndrome]] that usually affect older people. It is characterized by [[wood]]-[[grain]] scaly skin [[eruption]] with intense [[pruritus]]. The cause of erythema gyratum repens is unknown but many theories suggest [[Immunology|immunologic]] etiology or [[Toxicology|toxicologic]] products that are released by the associated [[tumor]]. The first case of erythema gyratum repens was described by a [[dermatologist]] named Gammel in the year 1952. For many years after erythema gyratum repens original description, there was little progress in defining the pathogenesis of erythema gyratum repens. [[Erythema]] gyratum repens has no specific [[classification]] but we can classify it based on its association with an internal [[malignancy]] into [[Paraneoplastic Syndromes|para-neoplastic]] and [[Para-|non-para-neoplastic]] erythema gyratum repens. The most common [[malignancies]] associated with erythema gyratum repens are [[lung]] or [[Bronchogenic carcinoma|bronchogenic]] [[cancer]], [[esophageal]] [[cancer]], and [[breast cancer]]. Erythema gyratum repens can also be associated with [[Neoplastic|non-neoplastic]] diseases such as [[tuberculosis]], [[autoimmune]] disorders, or [[CREST syndrome]]. Erythema gyratum repens is characterized by its [[Pathognomonic|pathogonomic]] figurate, gyrate, or annular [[erythematous]] skin [[Eruption|eruptions]]. The intense [[pruritus]] can be debilitating and usually urges the patient to go to the [[emergency department]]. The [[microscopic]] [[histopathological]] features of erythema gyratum repens consist of acanthosis, focal parakeratotic, and spongiosis of the [[epidermis]] with perivascular [[mononuclear]], [[lymphocytic]], and [[histiocytic]] infiltrate in the [[superficial]] [[Plexuses|plexus]] of the [[dermis]]. Erythema gyratum repens is very rare and it mainly affects people in their seventieth decade, the male to female ratio is 2:1. Erythema gyratum repens is [[Diagnosis|diagnosed]] clinically by its characteristic skin [[eruption]] and an [[Urgent care|urgent]] thorough [[paraneoplastic]] workup should be initiated to look for internal [[malignancies]]. Patients with erythema gyratum repens presents with intensely [[Pruritic disorders|pruritic]], gradually progressive, skin lesions that crawl rather than migrate from one body region to the other. It can start in the [[upper trunk]] or upper back and extends to involve the [[extremities]] sparing the [[face]]. The mainstay of the treatment of erythema gyratum repens is finding and treating the underlying [[malignancy]]. [[Symptomatic treatment|Symptomatic]] treatment is not very effective in relieving the [[pruritus]] and its associated pain. The management can be [[surgical]] removal of the [[tumor]], [[chemotherapy]], or [[palliative]] conservative management. The skin [[Eruption|eruptions]] can improve completely after the removal of the underlying [[Tumor cell|tumor]], or can recur especially if the [[Tumor cell|tumor]] recurred or [[metastasized]]. Patients can live a few weeks, months or up to five years depending on when and at what stage the [[malignancy]] was detected.


==Historical Perspective==
==Historical Perspective==
Erythema gyratum repens was first described by Dr. John A Gammel, a dermatologist, in 1952 in a patient with metastatic breast cancer. The patient was a 55 year old woman with skin eruption for nine months, associated with poorly differentiated adenocarcinoma of the breast. The skin lesions started in the face and neck as erythema and scales lesions that clear while other lesions flared.


The association between [important risk factor/cause] and [disease name] was made in/during [year/event].
*The [[Association (statistics)|association]] between [[cutaneous]] manifestations and [[systemic]] [[malignancies]] was first studied in 1925 by Rothman, the Hungarian investigative [[dermatologist]], who wrote a comprehensive review on this subject and since then, cases were added to proof for the relationship between internal [[neoplasm]] and some [[skin lesions]].<ref name="Rothman1925">{{cite journal|last1=Rothman|first1=Stephan|title=Über Hauterscheinungen bei bösartigen Geschwülsten innerer Organe|journal=Archiv für Dermatologie und Syphilis|volume=149|issue=1|year=1925|pages=99–123|issn=0340-3696|doi=10.1007/BF02297811}}</ref><ref name="pmid25373439">{{cite journal| author=Burgdorf WHC, Bickers DR| title=The scientific legacy of Stephen Rothman. | journal=J Invest Dermatol | year= 2015 | volume= 135 | issue= 4 | pages= 954-959 | pmid=25373439 | doi=10.1038/jid.2014.447 | pmc=4366295 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25373439  }} </ref>
 
*Erythema gyratum repens was first described by Dr. John A Gammel, the [[dermatologist]], who was trained to link bizarre or recalcitrant [[dermatoses]] to internal [[malignancy]], In 1952, in a 55-year-old patient who had been complaining of [[Pruritic disorders|pruritic]] scaly skin [[eruption]] and [[Diagnosis|diagnosed]] nine months later with [[Adenocarcinoma|poorly differentiated adenocarcinoma]] of the [[breast]] with [[metastasis]] to [[axillary lymph nodes]].<ref name="Gammel1952">{{cite journal|last1=Gammel|first1=John A.|title=ERYTHEMA GYRATUM REPENS|journal=A.M.A. Archives of Dermatology and Syphilology|volume=66|issue=4|year=1952|pages=494|issn=0096-5979|doi=10.1001/archderm.1952.01530290070010}}</ref><ref name="Purdy1959">{{cite journal|last1=Purdy|first1=M. J.|title=Erythema Gyratum Repens|journal=A.M.A. Archives of Dermatology|volume=80|issue=5|year=1959|pages=590|issn=0096-5359|doi=10.1001/archderm.1959.01560230076020}}</ref>
In [year], [scientist] was the first to discover the association between [risk factor] and the development of [disease name].
*In 1950, Dr. Gammel presented his case of Erythema gyratum repens before the [[Cleveland Clinic|Cleveland]] [[Dermatological]] Society as Erythema gyratum migrans then he changed the term to erythema gyratum repens because the eruption does not "migrate" from one place to another but "crawls" constantly in the areas involved, like "ants on an anthill".<ref name="Gammel1952">{{cite journal|last1=Gammel|first1=John A.|title=ERYTHEMA GYRATUM REPENS|journal=A.M.A. Archives of Dermatology and Syphilology|volume=66|issue=4|year=1952|pages=494|issn=0096-5979|doi=10.1001/archderm.1952.01530290070010}}</ref>
 
*In 1973, 45 year old man was [[Diagnosis|diagnosed]] with erythema gyratum repens associated with [[metastatic]], [[Adenocarcinoma|undifferentiated adenocarcinoma]] which was removed following a right- sided [[craniotomy]]. The patient was misdiagnosed with  [[Erythema|erythema perstans]] and the [[malignancy]] was discovered after 8 months of the skin manifestations.<ref name="Skolnick1975">{{cite journal|last1=Skolnick|first1=Marvin|title=Erythema Gyratum Repens With Metastatic Adenocarcinoma|journal=Archives of Dermatology|volume=111|issue=2|year=1975|pages=227|issn=0003-987X|doi=10.1001/archderm.1975.01630140085011}}</ref>
In [year], [gene] mutations were first implicated in the pathogenesis of [disease name].
* Up to 1992, there were only 49 cases in the literature, 41 of which (84%) were associated with a [[neoplasm]] and that is why erythema gyratum repens has been considered as a [[paraneoplastic]] syndrome.<ref name="pmid1583177">{{cite journal| author=Boyd AS, Neldner KH, Menter A| title=Erythema gyratum repens: a paraneoplastic eruption. | journal=J Am Acad Dermatol | year= 1992 | volume= 26 | issue= 5 Pt 1 | pages= 757-62 | pmid=1583177 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1583177  }}</ref>
 
* Between 1990 and 2010, a literature review was done by collecting [[data]] from the [[Medical record|medical records]] of patients form [[dermatology]] department in University of Genoa and from [[databases]] as [[Pubmed|pubMed]] and [[medline]], to conclude that erythema gyratum repens is no longer considered as an [[obligate]] [[paraneoplastic]] [[syndrome]]. More than expected cases of EGR were found with no [[neoplasm]] association.<ref name="RongiolettiFausti2014">{{cite journal|last1=Rongioletti|first1=F.|last2=Fausti|first2=V.|last3=Parodi|first3=A.|title=Erythema gyratum repens is not an obligate paraneoplastic disease: a systematic review of the literature and personal experience|journal=Journal of the European Academy of Dermatology and Venereology|volume=28|issue=1|year=2014|pages=112–115|issn=09269959|doi=10.1111/j.1468-3083.2012.04663.x}}</ref>
There have been several outbreaks of [disease name], including -----.
 
In [year], [diagnostic test/therapy] was developed by [scientist] to treat/diagnose [disease name].


==Classification==
==Classification==
There is no established system for the classification of [disease name].
* Erythema gyratum repens has no established system for the [[classification]]. However, we can classify erythema gyratum repens based on its [[Association (statistics)|association]] with systemic [[malignancy]] into: <ref name="RongiolettiFausti2014" /><ref name="pmid28690517">{{cite journal| author=Fukunaga M, Harada K, Mae K, Wakamatsu K, Kiriyama N, Tsuboi R et al.| title=Erythema Gyratum Repens-Like Purpura in a Patient with Sjögren Syndrome. | journal=Case Rep Dermatol | year= 2017 | volume= 9 | issue= 2 | pages= 40-43 | pmid=28690517 | doi=10.1159/000477375 | pmc=5498950 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28690517  }}</ref><ref name="pmid12168480">{{cite journal| author=Günther R, Nasser S, Hinrichsen H, Fölsch UR| title=[Erythema gyratum repens: drug reaction following azathioprine administration in a patient with type I [[autoimmune]] [[hepatitis]]. | journal=Med Klin (Munich) | year= 2002 | volume= 97 | issue= 7 | pages= 414-7 | pmid=12168480 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12168480  }}</ref><ref name="RongiolettiFausti2012">{{cite journal|last1=Rongioletti|first1=Franco|last2=Fausti|first2=Valentina|last3=Parodi|first3=Aurora|title=Erythema Gyratum Repens Induced by Pegylated Interferon Alfa for Chronic Hepatitis C|journal=Archives of Dermatology|volume=148|issue=10|year=2012|pages=1213|issn=0003-987X|doi=10.1001/archdermatol.2012.1968}}</ref>
 
OR
 
[Disease name] may be classified according to [classification method] into [number] subtypes/groups: [group1], [group2], [group3], and [group4].


OR
{| style="border: 0px; font-size: 90%; margin: 3px;" align="center"
! style="background: #4479BA; padding: 5px 5px;" rowspan=1 | {{fontcolor|#FFFFFF| Types of Erythema gyratum repens}}
! style="background: #4479BA; padding: 5px 5px;" rowspan=1 | {{fontcolor|#FFFFFF| Characterestics}}
|-
| style="padding: 5px 5px; background: #DCDCDC;" | '''[[Paraneoplastic]] EGR'''
| style="padding: 5px 5px; background: #F5F5F5;" |
* Erythema gyratum repens is associated with internal [[malignancy]] in 82% of cases<ref name="RongiolettiFausti2014" />
*The most common [[neoplasms]] are [[Lung cancer|Lung]]/[[Bronchogenic carcinoma|bronchogenic]], [[breast cancer]], and GI tract ([[Stomach Cancer|stomach,]] [[Esophageal Cancer|esophageal]]) cancer.
*The other associated neoplasms are: [[Urinary bladder cancer|Urinary bladder,]] [[Prostate Cancer|prostate]], [[Uterine cancer|uterine]] and/or [[cervix]], and [[anal cancer]]
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold;" rowspan="8;" | Non-[[paraneoplastic]] EGR
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold;" rowspan="1;"|[[Idiopathic]] EGR
|-
| style="padding: 5px 5px; background: #F5F5F5;" |
*Erythema gyratum repens with no underlying [[malignancy]], associated conditions, or precipitating cause
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold;" rowspan="1;"| <nowiki>|</nowiki>EGR-like [[Eruption|eruptions]] <ref name="pmid28690517" />
|-
| style="padding: 5px 5px; background: #F5F5F5;" |
* Can appear in various [[autoimmune]] conditions


[Disease name] may be classified into [large number > 6] subtypes based on [classification method 1], [classification method 2], and [classification method 3].
* Characterized by [[Lesions|annular lesions]] with expanding concentric pattern and coalescing to form a zebra-like pattern or [[grain]] [[of wood pattern]]
[Disease name] may be classified into several subtypes based on [classification method 1], [classification method 2], and [classification method 3].
*EGR-like eruption in [[Sjögren syndrome]] (SS) is extremely rare
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold;" rowspan="1;"| EGR with concomitant [[skin disease]]
|-
| style="padding: 5px 5px; background: #F5F5F5;" |
**[[Pityriasis rubra pilaris]], [[psoriasis]], [[ichthyosis]], [[CREST|CREST (calcinosis, Raynaud phenomenon, esophageal motility disorder, sclerodactyly, and telangiectasia) syndrome]], virginal breast hypertrophy, [[rheumatoid arthritis]], [[tuberculosis]], [[bullous pemphigoid]], [[linear]] [[IgA]] [[disease]], and [[hypereosinophilic syndrome]], [[cryptogenic organizing pneumonia]]<ref name="pmid26765132">{{cite journal| author=Samotij D, Szczech J, Bencal-Kusinska M, Reich A| title=Erythema gyratum repens associated with cryptogenic organizing pneumonia. | journal=Indian J Dermatol Venereol Leprol | year= 2016 | volume= 82 | issue= 2 | pages= 212-3 | pmid=26765132 | doi=10.4103/0378-6323.173594 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26765132  }}</ref>
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold;" rowspan="1;"|[[Drug-induced]] EGR
|-
| style="padding: 5px 5px; background: #F5F5F5;" |
*''[[Azathioprine]]'' with [[type I]] [[autoimmune]] [[hepatitis]]<ref name="pmid12168480" />


OR
*''[[Interferon]]'' given for [[hepatitis C]] virus–related [[chronic hepatitis]]<ref name="RongiolettiFausti2012" />
 
|-
Based on the duration of symptoms, [disease name] may be classified as either acute or chronic.
|}
 
OR
 
If the staging system involves specific and characteristic findings and features:
According to the [staging system + reference], there are [number] stages of [malignancy name] based on the [finding1], [finding2], and [finding3]. Each stage is assigned a [letter/number1] and a [letter/number2] that designate the [feature1] and [feature2].
 
OR
 
The staging of [malignancy name] is based on the [staging system].
 
OR
 
There is no established system for the staging of [malignancy name].


==Pathophysiology==
==Pathophysiology==
The cause of EGR has not been identified. However, there are theories that suggest various immunologic mechanisms have been implicated in its pathogenesis. The first theory is that the tumor may induce antibodies that cross-react with the basement membrane of skin. The second theory is hat the tumor may produce polypeptides that bind skin antigens and render them immunogenic. Other researchers believe that deposition of tumor antigen-antibody complexes onto the basement membrane accounts for this reactive dermatitis.
* The [[pathogenesis]] of erythema gyratum repens is unclear<ref name="pmid3390794">{{cite journal| author=Appell ML, Ward WQ, Tyring SK| title=Erythema gyratum repens. A cutaneous marker of malignancy. | journal=Cancer | year= 1988 | volume= 62 | issue= 3 | pages= 548-50 | pmid=3390794 | doi=10.1002/1097-0142(19880801)62:3<548::aid-cncr2820620318>3.0.co;2-h | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3390794  }}</ref><ref name="pmid22224159" />
* Many [[Immunology|immunologic]] theories have been implicated in its [[pathogenesis]].
*The [[Immunology|immunologic]] mechanism theory is evidenced by the observed [[immunofluorescence]] patterns of [[IgG]], C3, and C4 at the [[basement membrane]]: <ref name="pmid22224159">{{cite journal| author=Gore M, Winters ME| title=Erythema gyratum repens: a rare paraneoplastic rash. | journal=West J Emerg Med | year= 2011 | volume= 12 | issue= 4 | pages= 556-8 | pmid=22224159 | doi=10.5811/westjem.2010.11.2090 | pmc=3236141 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22224159  }}</ref>
** Theory 1: the [[tumor]] induces [[antibodies]] that cross-react with the [[basement membrane]] of skin.
** Theory 2: the [[tumor]] produces [[polypeptides]] that bind skin [[antigens]] and render them [[Immunogenicity|immunogenic]]. 
** Theory 3: deposition of tumor antigen-antibody complexes onto the [[basement membrane]] causes [[Dermatitis|reactive dermatitis]] seen in erythema gyratum repens.
*The [[gross]] appearance of the unique [[Eruption|eruptions]] are:
** Wavy [[erythematous]] [[concentric]] [[bands]] that can be figurate, gyrate, or annular
** The [[bands]] are arranged in parallel rings and lined by a fine trailing edge of scales, a pattern often described as “wood grained”.
** The distinctive [[wood]][[grain|-grain]] appearance of the eruption is [[pathognomonic]].
** The [[rash]] typically involves large areas of the [[body]] but tends to spare the [[face]], [[hands]], and [[feet]] and it can expand as fast as 1 cm a day.
** Bullae can also form from within the areas of [[erythema]].
* The [[microscopic]] histologic features of erythema gyratum repens are not characteristics but the following are the [[biopsy]] specimen findings that are compatible with the [[diagnosis]]:<ref name="Gammel1952" /><ref name="Skolnick1975" /><ref name="pmid8339188">{{cite journal| author=Tyring SK| title=Reactive erythemas: erythema annulare centrifugum and erythema gyratum repens. | journal=Clin Dermatol | year= 1993 | volume= 11 | issue= 1 | pages= 135-9 | pmid=8339188 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8339188  }}</ref>
** The [[epidermis]] has thin [[atrophic]] patches with areas of acanthosis, focal parakeratotic horny layers, and spongiosis.
**The [[dermis]] contains a moderate perivascular [[mononuclear]], [[lymphocytic]], and [[histiocytic]] infiltrate in the [[superficial]] [[plexus]] as well as mild focal spongiosis and parakeratosis.
**[[Eosinophils]] and melanophages have also been reported in the [[dermal]] infiltrate.
**Diffuse to moderate [[edema]] of the [[Connective tissues|connective tissue]] can be seen.


The exact pathogenesis of [disease name] is not fully understood.


OR
It is thought that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3].
OR
[Pathogen name] is usually transmitted via the [transmission route] route to the human host.
OR
Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.
OR
[Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].
OR
The progression to [disease name] usually involves the [molecular pathway].
OR
The pathophysiology of [disease/malignancy] depends on the histological subtype.


==Causes==
==Causes==
may be caused by [cause1], [cause2], or [cause3].
* The exact cause of erythema gyratum repens is unknown.
 
* Various [[Immunology|immunologic]] mechanisms suggest that erythema gyratum repens [[etiology]] is stemmed from an [[Immunological|immunologic]] reaction.
OR
*The [[Association (statistics)|association]] between erythema gyratum repens and systemic [[malignancy]] is evidenced by the disappearance of the [[Pruritic disorders|pruritic]] [[Eruption|eruptions]] after the treatment of the underlying [[neoplasm]].
 
*The [[Association (statistics)|association]] doesn't necessarily mean causation.
Common causes of [disease] include [cause1], [cause2], and [cause3].
 
OR
 
The most common cause of [disease name] is [cause 1]. Less common causes of [disease name] include [cause 2], [cause 3], and [cause 4].


OR
==Differentiating Erythema Gyratum Repens from Other Diseases==
*EGR has a narrow [[differential diagnosis]] and it has to be differentiated from reactive (figurate or gyrate) erythematous skin eruptions.
*[[Differential diagnosis]] of reactive (figurate or gyrate) erythematous skin [[Eruption|eruptions]] based on their association with underlying systemic [[malignancy]]:


The cause of [disease name] has not been identified. To review risk factors for the development of [disease name], click [[Pericarditis causes#Overview|here]].
{| style="border: 0px; font-size: 90%; margin: 3px;" align=center
! style="background: #4479BA; padding: 5px 5px;" rowspan=1 | {{fontcolor|#FFFFFF|Types}}
! style="background: #4479BA; padding: 5px 5px;" rowspan=1 | {{fontcolor|#FFFFFF|examples}}
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold;" rowspan="3;"|With underlying [[malignancy]]''' '''<ref name="pmid8339188" /><ref name="pmid861171">{{cite journal| author=Holt PJ, Davies MG| title=Erythema gyratum repens--an immunologically mediated dermatosis? | journal=Br J Dermatol | year= 1977 | volume= 96 | issue= 4 | pages= 343-7 | pmid=861171 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=861171  }}</ref>'''
| style="padding: 5px 5px; background: #F5F5F5;" |
*Erythema gyratum repens (EGR)
|-
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Erythema annulare centrifugum]] (EAC)
|-
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Necrolytic migratory erythema]] (NME)
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold;" rowspan="6;" | Without underlying [[malignancy]]
| style="padding: 5px 5px; background: #F5F5F5;" |
* Erythema marginatum rheumaticum
|-
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[Erythema chronicum migrans]] 
|-
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[Familial|Familial annular erythema]]
|-
| style="padding: 5px 5px; background: #F5F5F5;" |
* The [[carrier]] state of [[chronic granulomatous disease]]
|-
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Subacute]] [[cutaneous]] [[lupus erythematosus]]
|-
| style="padding: 5px 5px; background: #F5F5F5;" |  
* [[Neonatal lupus erythematosus]]
|-
|}


==Differentiating ((Page name)) from Other Diseases==
EGR has a narrow differential diagnosis and given its distinctive appearance, it has to be differentiated from those with gyrate erythematous eruptions, such as, necrolytic migratory erythema (NME), erythema annulare centrifugum (EAC), and Erythema migrans. Necrolytic migratory erythema (NME) is usually seen in association with pancreatic glucagonomas and favors the perioral and perianal areas. Erythema annulare centrifugum (EAC) has pruritic concentric lesions with central clearing and a trailing edge of scale but usually involves smaller areas of the trunk and extremities and it migrates at a much slower rate than the lesions of EGR. Erythema migrans is the lesion of Lyme disease, it has no scaling and is a more localized skin reaction.


[Disease name] must be differentiated from other diseases that cause [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as [differential dx1], [differential dx2], and [differential dx3].
*[[Differential diagnosis]] of reactive (figurate or gyrate) erythematous skin [[Eruption|eruptions]] associated with underlying [[malignancy]]:'''<ref name="pmid8339188" /><ref name="pmid861171" />''' <br />


OR
{| style="border: 0px; font-size: 90%; margin: 3px;" align=center
! style="background: #4479BA; padding: 5px 5px;" rowspan=1 | {{fontcolor|#FFFFFF| Disease}}
! style="background: #4479BA; padding: 5px 5px;" rowspan=1 | {{fontcolor|#FFFFFF|Erythema Characteristics}}
! style="background: #4479BA; padding: 5px 5px;" colspan=1 | {{fontcolor|#FFFFFF|Signs and Symptoms}}
! style="background: #4479BA; padding: 5px 5px;" rowspan=1 | {{fontcolor|#FFFFFF|Associated Conditions}}
! style="background: #4479BA; padding: 5px 5px;" rowspan=1 | {{fontcolor|#FFFFFF|Histopathology}}
! style="background: #4479BA; padding: 5px 5px;" colspan=1 | {{fontcolor|#FFFFFF|Lab finding 
&
Other evaluation}}
! style="background: #4479BA; padding: 5px 5px;" rowspan=1 | {{fontcolor|#FFFFFF| Prognosis}}
|-
| style="padding: 5px 5px; background: #DCDCDC;" | '''[[Erythema gyratum repens|Erythema gyratum repens (EGR)]]'''
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Migratory]] annular and configurate erythematous bands that form concentric rings
* Wood grain scaly appearance
*[[scales]] follows the leading edge of the bands
*[[Eruption]] migrates more rapidly, 1cm/d<br />
| style="padding: 5px 5px; background: #F5F5F5;" |
* Skin [[Eruption|eruptions]]
* Severe Generalized  itching ([[pruritus]])
*[[Scaly]] erythematous patches over  trunk and proximal extremities, sparing the hands, feet, and face, can eventually involve the face.   
*[[Weight loss]]
*[[Malaise]] and [[fatigue]]
*[[Fever]]
*[[Anorexia]]
*[[Lymphadenopathy]]
*[[Headache]] and [[convulsion]] (intracranial metastasis)
*[[Shortness of breath]] ([[Bronchogenic carcinoma|bronchogenic carcinoma)]]
| style="padding: 5px 5px; background: #F5F5F5;" |
* Dermatologic conditions:
**[[Ichthyosis]] palmar/plantar [[hyperkeratosis]]
* Less frequently EGR co-present with:
**[[Pityriasis Rubra Pilaris]], [[Bullous pemphigoid]], [[Pemphigus vulgaris|Pemphigus vulgaris,]] [[Discoid lupus erythematosus|discoid lupus eythemutosus]], [[psoriusiform]] lesions, and nonspecific vesicles and bullae
*[[Tuberculosis]]


[Disease name] must be differentiated from [[differential dx1], [differential dx2], and [differential dx3].
*[[CREST syndrome]]


==Epidemiology and Demographics==
([[Calcinosis]], [[Raynaud's phenomenon|Raynaud’s phenomenon,]] [[Esophageal dysmotility]], [[Sclerodactyly]], and [[Telangiectasia]])
EGR commonly affects Caucasians, with an average age of onset in the seventh decade of life, and the male to female ratio is 2:1. EGR is usually precedes the diagnosis of the associated cancer by several months. EGR is mainly seen in patients with paraneoplastic syndrome, it can rarely be seen in patients with nonneoplastic conditions such as tuberculosis, hypereosinophilic syndrome, bullous pemphigoid vulgaris, systemic lupus erythematosis, and ulcerative colitis.
| style="padding: 5px 5px; background: #F5F5F5;" |
* The [[epidermis]]:
**[[Acanthosis]]
**Focal [[parakeratotosis]] and [[spongiosis]]
*The [[dermis]]:
**Mild focal spongiosis and parakeratosis
**Moderate [[perivascular]] [[Mononuclear cells|mononuclear]], [[lymphocytic]], and [[histiocytic]] infiltrate
**[[Eosinophils]] and [[melanophages]] have also been reported in the infiltrate
*Diffuse to moderate [[edema]] of the [[Connective tissue|connective tissue c]]<nowiki/>an be seen
| style="padding: 5px 5px; background: #F5F5F5;" |
*There are no diagnostic laboratory findings associated with erythema gyratum repens
*[[Eosinophilia]] is observed in 60% of cases<ref name="pmid22224159" />
*Decreased [[T lymphocytes]] and increased [[B lymphocytes]] observed in an erythema gyratum repens patient with increased [[luteinizing hormone]] and [[follicle-stimulating hormone]]<ref name="pmid8339188" />
*Decreased serum levels of [[Complement system|C3]]<ref name="pmid8339188" />
*Normal percentages of B and T [[lymphocytes]] and normal T-cell function were reported in an EGR patient without [[Cancer (disease)|cancer]]<ref name="pmid8339188" />


The incidence/prevalence of [disease name] is approximately [number range] per 100,000 individuals worldwide.
<br />
| style="padding: 5px 5px; background: #F5F5F5;" |
* Skin manifestations can be improved within 48 hours of the resection of the underlying [[tumor]] with on of the following:
** Complete cure of the skin [[eruption]] and [[pruritus]]
** Temporary improvement then recurrence of the [[eruption]] (specially in cases of [[metastasis]])
** No effect of the tumor treatment on the course of EGR
*** Death can occur few weeks after the detection of the [[malignancy]], few months, or four years as in Gammel's patient.
|-
| style="padding: 5px 5px; background: #DCDCDC;" | '''[[Erythema annulare centrifugum]] ([[Erythema annulare centrifugum|EAC]]) <ref name="pmid8339188" />'''
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Migratory]] annular and configurate erythematous


OR
or [[polycyclic]] lesions     


In [year], the incidence/prevalence of [disease name] was estimated to be [number range] cases per 100,000 individuals worldwide.
*[[Urticaria|Urticarial]] in appearance, ringed, [[arcuate]] figures


OR
*[[Eruption]] migrate at a slower rate (2 -3 mm/d) reaching up to 10 cm in diameter with central clearing


In [year], the incidence of [disease name] is approximately [number range] per 100,000 individuals with a case-fatality rate of [number range]%.
* Cover only a small percentage of the total body surface    
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Annular]] or [[polycyclic]] [[lesions]] which may begin as [[urticaria]]-like [[Papules|papule]]
* Eventually old lesions can spontaneously resolve in several days to a few weeks while new eruptions develop.
* The [[deep]] form of erythema annulare centrifugum has a firm, indurated border, is rarely [[Pruritic disorders|pruritic]], and has no scale
* The superficial type of erythema annulare centrifugum has an indistinct scaly border and is usually [[Pruritic disorders|pruritic]]  
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Infections]]


*[[Allergic Reaction|Allergic reactions]] to drugs
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Deep form:]]
**[[Mononuclear cells|Mononuclear]], [[Perivascular cell|perivascular]] [[infiltrate]] in the middle and lower portions of the [[dermis]] ([[coat sleeve-like configuration]])
**[[Infiltrate]] is primarily of [[lymphocytes]], but [[eosinophils]] are occasionally present
**Extravasation of [[erythrocytes]] is associated with [[Endothelial|endothelial swelling]]   
** No epidermal changes   
* Superficial:
** More non-specific
** Slight superficial perivascular [[Lymphocyte|lympho-]][[Histiocyte|histiocytic]] infiltrate   
** Focal parakeratosis and mild spongiosis with microvesiculation
| style="padding: 5px 5px; background: #F5F5F5;" |
* No specific laboratory changes


*[[Eosinophilia]] of the peripheral blood, as well as tissue, can be observed in erythema annulare centrifugum associated with a [[drug reaction]] or [[parasitic]] [[infection]]   


Patients of all age groups may develop [disease name].
* Evaluation for possible [[infection]] or [[drug reaction]] (prescribed and non-prescribed)


OR
*[[Complete blood counts|Complete blood count]]
*[[Urinalysis]]
*[[Liver function tests]]
*[[Renal function tests|Kidney function test]]
| style="padding: 5px 5px; background: #F5F5F5;" |
* Lesions disappear after the underlying etiology is managed ([[allergy]], [[infection]], [[malignancy]])
* if no underlying cause, lesions can recur after discontinuation of the supportive treatment
|-
| style="padding: 5px 5px; background: #DCDCDC;" | '''[[Necrolytic migratory erythema|Necrolytic migratory erythema (NME)]]'''
| style="padding: 5px 5px; background: #F5F5F5;" |
* Migratory circinate [[erythema]]/[[plaques]] with areas of [[necrosis]] and [[sloughing]]


The incidence of [disease name] increases with age; the median age at diagnosis is [#] years.
*[[Crusted]]  [[Erythematous]] scaly plaques with centrifugal growth


OR
*
| style="padding: 5px 5px; background: #F5F5F5;" |
* Red [[erythematous]] scaly [[plaques]] over [[Perineum]], distal [[extremities]], lower [[abdomen]], and [[face]]
* Spontaneous exacerbation and [[remission]] periods without knowing what the trigger is
*[[Weight loss]]
*[[Anemia]]
*[[Diabetes]]
*[[Diarrhea]]
*[[Stomatitis]].
| style="padding: 5px 5px; background: #F5F5F5;" |
* Obligatory [[paraneoplastic]] [[syndrome]]
*First manifestation of the rare [[pancreatic neuroendocrine tumor]] ([[Glucagonoma|glaucagonoma]])
*No other association
* Can be misdiagnosed as:
**[[Contact dermatitis]]
**[[Intertrigo]]
**[[Psoriasis|Inverse psoriasis]]
**[[Zinc deficiency]]
** Other [[nutritional deficiencies]]
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Paleness]] and spongiosis of the upper layer of the [[epidermis]]


[Disease name] commonly affects individuals younger than/older than [number of years] years of age.
* A [[Perivascular cell|perivascular]] [[lymphocytic]] and [[histiocytic]] infiltrate


OR
*[[Necrotic]] [[Keratinocyte|keratinocytes]] are common and can lead to erosions, crusting and [[Scaling skin|scaling]]
| style="padding: 5px 5px; background: #F5F5F5;" |
* Increased [[glucagon]] level


[Chronic disease name] is usually first diagnosed among [age group].
* Evaluation of the associated [[tumor]]:
**[[CT-scans|CT]] or [[MRI]] [[abdomen]]


OR
* * [[Visceral angiography|Selective visceral angiography]] to localize the tumor


[Acute disease name] commonly affects [age group].
* * [[Positron Emission Tomography]] (PET)


* * [[Octreotide]] [[scintigraphy]]
| style="padding: 5px 5px; background: #F5F5F5;" |
* Due to the difficulty of necrolytic migratory erythema recognition, and its association with [[glucagonoma]], diagnosis is usually delayed


* Necrolytic migratory erythema usually resolved after the resection and treatment of the [[Pancreatic tumor|pancreatic tumor,]] eg.10 days after tumor resection


There is no racial predilection to [disease name].
* Early recognition is crucial for better diagnosis and prognosis <br />
|}


OR
==Epidemiology and Demographics ==
* Erythema gyratum repens is a [[rare]], characteristic, and [[paraneoplastic syndrome]] with the following [[demographics]]:<ref name="pmid22224159" />


[Disease name] usually affects individuals of the [race 1] race. [Race 2] individuals are less likely to develop [disease name].
'''Age'''
* The [[average]] age of onset of erythema gyratum repens i is in the seventh decade of life (65 years old).


'''Gender'''
* The male to female ratio is 2:1.


 
'''Race'''
[Disease name] affects men and women equally.
* EGR commonly affects Caucasians.
 
OR
 
[Gender 1] are more commonly affected by [disease name] than [gender 2]. The [gender 1] to [gender 2] ratio is approximately [number > 1] to 1.
 
 
 
The majority of [disease name] cases are reported in [geographical region].
 
OR
 
[Disease name] is a common/rare disease that tends to affect [patient population 1] and [patient population 2].


==Risk Factors==
==Risk Factors==
There are no established risk factors for [disease name].
* There are no established [[risk factors]] for erythyma gyratum repens.
 
*Many patients with erythyma gyratum repens and [[malignancy]] had a history of [[tobacco smoking]].
OR
*Some patients with erythyma gyratum repens and [[malignancy]] have a family history of [[neoplasm]].
 
The most potent risk factor in the development of [disease name] is [risk factor 1]. Other risk factors include [risk factor 2], [risk factor 3], and [risk factor 4].
 
OR
 
Common risk factors in the development of [disease name] include [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].
 
OR
 
Common risk factors in the development of [disease name] may be occupational, environmental, genetic, and viral.


==Screening==
==Screening==
There is no screening tests for EGR but the skin rash shouldn't be missed in the the ED and patients should be referred for urgent evaluation and screening for internal malignancies.  
* There are no [[screening]] tests for erythema gyratum repens.
 
*[[Screening]] for internal [[malignancy]] should be done immediately after erythema gyratum repens is [[Diagnosis|diagnosed]].
There is insufficient evidence to recommend routine screening for [disease/malignancy].
 
OR
 
According to the [guideline name], screening for [disease name] is not recommended.
 
OR
 
According to the [guideline name], screening for [disease name] by [test 1] is recommended every [duration] among patients with [condition 1], [condition 2], and [condition 3].


==Natural History, Complications, and Prognosis==
==Natural History, Complications, and Prognosis==
Patients with EGR usually presents with the severely pruritic rash few months prior to the diagnosis of the internal malignancy. The pruritis can be debilitating and it may persist to the time of death.  
* The majority of patients with erythema gyratum repens presents with severely [[Pruritic disorders|pruritic]] [[erythematous]] skin lesions that appear several months prior to the [[malignancy]] diagnosis<ref name="pmid22224159" />
 
* If the underlying [[malignancy]] left untreated, the debilitating [[pruritus]] could persist until the patient dies<ref name="pmid22224159" />
If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
*[[Prognosis]] depends on the type of the underlying [[tumor]] and the probability of its treatment. It depends on the time of the erythema gyratum repens onset and the [[neoplasm]] discovery. The course and [[prognosis]] of erythema gyratum repens can be one of the following: 
 
** Complete [[cure]] of the skin [[eruption]] and [[pruritus]] after removal and treatment of the internal [[neoplasm]].
OR
** Temporary improvement then recurrence of the [[eruption]] (specially in cases of [[metastasis]]).
 
** No effect of the [[tumor]] treatment on the course of erythema gyratum repens.
Common complications of [disease name] include [complication 1], [complication 2], and [complication 3].
** Death can occur few weeks after the discovery of the [[malignancy]], few months, or four years as in Gammel's patient.
 
OR
 
Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.


==Diagnosis==
==Diagnosis==
===Diagnostic Study of Choice===
===Diagnostic Study of Choice===
EGR is mainly diagnosed clinically. Eosinophilia is observed in 60% of cases and immunofluorescence shows patterns of IgG, C3, and C4 at the basement membrane.
* Erythyma gyratum repens is mainly [[Diagnosis|diagnosed]] [[Clinical|clinically]] by its characteristic [[skin lesions]].
 
* It is considered as a [[cutaneous]] [[marker]] of [[malignancy]] with high [[specificity]] so physicians shouldn't miss its unique [[clinical]] skin presentation.
The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met: [criterion 1], [criterion 2], [criterion 3], and [criterion 4].
 
OR
 
The diagnosis of [disease name] is based on the [criteria name] criteria, which include [criterion 1], [criterion 2], and [criterion 3].
 
OR
 
The diagnosis of [disease name] is based on the [definition name] definition, which includes [criterion 1], [criterion 2], and [criterion 3].
 
OR
 
There are no established criteria for the diagnosis of [disease name].


===History and Symptoms===
===History and Symptoms===
The hallmark of EGR is skin rash  and pruritus is almost a universal symptom that can be extreme and debilitating. Patient may also complain of weight loss, anorexia, fatigue, and fever. 
* The universal symptoms of erythema gyratum repens are:
 
**[[Eruption|Skin eruptions]]
The majority of patients with [disease name] are asymptomatic.
**[[Pruritus|Intense pruritus]]
 
* Other symptoms related to the associated internal [[malignancy]] may include:
OR
** [[Weight loss]]
 
** [[Fatigue]]
The hallmark of [disease name] is [finding]. A positive history of [finding 1] and [finding 2] is suggestive of [disease name]. The most common symptoms of [disease name] include [symptom 1], [symptom 2], and [symptom 3]. Common symptoms of [disease] include [symptom 1], [symptom 2], and [symptom 3]. Less common symptoms of [disease name] include [symptom 1], [symptom 2], and [symptom 3].
**[[Anorexia]]
** [[Vomiting]]
** [[Fever]]
**[[Headache]]
**[[Convulsion]]
**[[Shortness of breath]]
**[[Abdominal distention]]


===Physical Examination===
===Physical Examination===
Patients with EGR presents with a rash consisting of wavy erythematous concentric bands that can be figurate, gyrate, or annular. The bands are arranged in parallel rings and lined by a fine trailing edge of scale, a pattern often described as “wood grained. The rash typically involves large areas of the body but tends to spare the face, hands, and feet and it can expands as fast as a cm a day. Bullae can also form from within the areas of erythema.
* Patients with erythyma gyratum repens usually are ill-appearing and [[lethargic]]
 
*[[Physical examination]] may be remarkable for:
by  usually appear [general appearance]. Physical examination of patients with [disease name] is usually remarkable for [finding 1], [finding 2], and [finding 3].
**[[Wood]]-[[grain]] erythematous scaly skin [[eruption]].
 
**Bullae can also form within the areas of erythema.
OR
**Typically involves large areas of the [[body]] but tends to spare the [[face]], [[hands]], and [[feet]] and it can expand as fast as 1 cm a day.<ref name="pmid22224159" />
 
**[[Signs]] of [[malignancy]] can be seen based on the [[neoplasm]] location such as:
Common physical examination findings of [disease name] include [finding 1], [finding 2], and [finding 3].
***[[Lymphadenopathy]]
 
***[[Palpable]] [[mass]]
OR
***[[Abdominal]] [[ascites]]
 
***[[Pleural effusion]]
The presence of [finding(s)] on physical examination is diagnostic of [disease name].
***[[Papilloedema]]
 
OR
 
The presence of [finding(s)] on physical examination is highly suggestive of [disease name].


===Laboratory Findings===
===Laboratory Findings===
An elevated/reduced concentration of serum/blood/urinary/CSF/other [lab test] is diagnostic of [disease name].
* There are no [[diagnostic]] [[laboratory]] findings associated with erythema gyratum repens.
 
* [[Eosinophilia]] is observed in 60% of cases.<ref name="pmid22224159" />
OR
*Decreased [[T lymphocytes]] and [[increased]] [[B lymphocytes]] observed in an erythema gyratum repens patient with increased [[luteinizing hormone]] and [[follicle-stimulating hormone]].<ref name="pmid8339188" />
 
*Decreased serum levels of [[C3 (complement)|C3]].<ref name="pmid8339188" />
Laboratory findings consistent with the diagnosis of [disease name] include [abnormal test 1], [abnormal test 2], and [abnormal test 3].
*Normal percentages of [[B lymphocyte|B]] and [[T lymphocytes]] and normal [[T-cell|T-cell function]] were reported in an erythema gyratum repens patient without [[cancer]].<ref name="pmid8339188" />
 
OR
 
[Test] is usually normal among patients with [disease name].
 
OR
 
Some patients with [disease name] may have elevated/reduced concentration of [test], which is usually suggestive of [progression/complication].
 
OR
 
There are no diagnostic laboratory findings associated with [disease name].
 
===Electrocardiogram===
There are no ECG findings associated with [disease name].


OR
=== Imaging Findings===
 
* There are no [[imaging]] findings associated with erythyma gyratum repens.
An ECG may be helpful in the diagnosis of [disease name]. Findings on an ECG suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
*[[Imaging]] to look for systemic [[neoplasms]] are:<ref name="pmid22224159" />
 
*<nowiki>* </nowiki>[[Computed tomography]] of the [[head]], [[neck]], [[chest]], [[abdomen]], and [[pelvis]].
===X-ray===
**[[Positron emission tomography]]/[[computed tomography]]
There are no x-ray findings associated with [disease name].
** Upper and lower [[gastrointestinal]] [[endoscopy]]
 
*The abnormal findings that heightened concern for systemic or widespread [[malignancy]] are:
OR
**[[Brain]], [[chest]], [[lung]], [[abdominal]], [[peritoneal]], or [[Pelvic masses|pelvic mass.]]
 
**[[Lymphadenopathy]].
An x-ray may be helpful in the diagnosis of [disease name]. Findings on an x-ray suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
** Enhanced bone lucencies suggestive of diffuse [[metastasis]].
 
OR
 
There are no x-ray findings associated with [disease name]. However, an x-ray may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].
 
===Echocardiography or Ultrasound===
There are no echocardiography/ultrasound  findings associated with [disease name].
 
OR
 
Echocardiography/ultrasound  may be helpful in the diagnosis of [disease name]. Findings on an echocardiography/ultrasound suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
 
OR
 
There are no echocardiography/ultrasound  findings associated with [disease name]. However, an echocardiography/ultrasound  may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].
 
===CT scan===
There are no CT scan findings associated with [disease name].
 
OR
 
[Location] CT scan may be helpful in the diagnosis of [disease name]. Findings on CT scan suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
 
OR
 
There are no CT scan findings associated with [disease name]. However, a CT scan may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].
 
===MRI===
There are no MRI findings associated with [disease name].
 
OR
 
[Location] MRI may be helpful in the diagnosis of [disease name]. Findings on MRI suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
 
OR
 
There are no MRI findings associated with [disease name]. However, a MRI may be helpful in the diagnosis of complications of [disease name], which include [complication 1], [complication 2], and [complication 3].
 
===Other Imaging Findings===
 
[[File:EGR1.jpg]]
 
There are no other imaging findings associated with [disease name].
 
OR
 
[Imaging modality] may be helpful in the diagnosis of [disease name]. Findings on an [imaging modality] suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].


===Other Diagnostic Studies===
===Other Diagnostic Studies===
Although skin appearance is characteristic, EGR has nonspecific histopathologic features. Biopsy specimens display acanthosis, mild hyperkeratosis, focal parakeratosis, and spongiosis confined to the epidermis and superficial dermis. Mononuclear, lymphocytic, and histiocytic perivascular infiltrate in the superficial plexus can also be seen. Basement membrane deposits of IgG, C3, or C4 have been noted under direct immunofluorescence in some cases.


There are no other diagnostic studies associated with [disease name].
* The [[histopathologic]] features of EGR is non-specific.
 
* [[Biopsy]] specimens show the following:<ref name="pmid22224159" />
OR
**Acanthosis, mild [[hyperkeratosis]], focal parakeratosis, and spongiosis confined to the [[epidermis]] and superficial [[dermis]]
 
** Mononuclear, [[Lymphocyte|lymphocytic]], and [[histiocytic]] perivascular infiltrate in the superficial [[plexus]] can also be seen
[Diagnostic study] may be helpful in the diagnosis of [disease name]. Findings suggestive of/diagnostic of [disease name] include [finding 1], [finding 2], and [finding 3].
**[[Eosinophils]] and melanophages have also been reported in the dermal infiltrate
 
**Diffuse to moderate [[edema]] of the [[connective]] [[tissue]] can be seen
OR
**Direct [[immunofluorescence]] can show patterns of [[IgG]], [[C3 (complement)|C3]], and C4 at the [[basement membrane]]
 
* Thorough paraneoplastic and systemic workup includes:<ref name="pmid22224159" /><ref name="pmid31111084">{{cite journal| author=Ridge A, Tummon O, Laing M| title=Response to "Transformation from pityriasis rubra pilaris to erythema gyratum repens-like eruption without associated malignancy: A report of 2 cases". | journal=JAAD Case Rep | year= 2019 | volume= 5 | issue= 5 | pages= 461-462 | pmid=31111084 | doi=10.1016/j.jdcr.2019.03.012 | pmc=6510971 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=31111084  }}</ref>
Other diagnostic studies for [disease name] include [diagnostic study 1], which demonstrates [finding 1], [finding 2], and [finding 3], and [diagnostic study 2], which demonstrates [finding 1], [finding 2], and [finding 3].
**[[Computed tomography]] of [[head]], [[neck]], [[chest]], [[abdomen]], and [[pelvis]].
**[[Positron emission tomography]]/[[computed tomography]].
** Upper and lower [[gastrointestinal]] [[endoscopy]].
**Complete [[blood]] [[chemistry]] ([[CBC]]).
**[[Comprehensive metabolic panel]] (CMP).
**[[Urinalysis|Urinanalysis.]]
**[[Rapid plasma reagin]] test [to exclude [[syphilis]]].
**[[Anti-nuclear antibody]] test [to exclude [[autoimmune]] disorders as [[systemic lupus erythematosus]] ([[SLE]])].
**Guaiac stool test.
**[[Serum protein electrophoresis]] [to exclude [[cancers]] as [[multiple myeloma]]].
**[[Lactate dehydrogenase]] [tissue damage, [[kidney disease]], [[liver disease]]].
**[[QuantiFERON]] [to exclude [[tuberculosis]]].
**[[Tumor markers]].


==Treatment==
==Treatment==
Various dermatologic and immunosuppressive therapies have been used to treat EGR. Systemic steroids are frequently ineffective. Topical steroids, vitamin A, and azathioprine have also failed to relieve skin manifestations. Improvement or resolution of EGR, and its associated intense pruritus, depends on recognition and treatment of the underlying malignancy. In patients with widely metastatic disease, the response of EGR to chemotherapy is variable. In such cases, patients may not experience resolution of the rash until just before the time of death, a time of significant immunosuppression.
=== Medical Therapy ===
*Treatment of erythema gyratum repens, and its associated intense [[pruritus]] depends on the recognition and treatment of the underlying [[malignancy]]<ref name="pmid22224159" />
*[[Symptomatic treatment|Symptomatic management]]:
**[[Hydroxyzine]] for itching, [[ibuprofen]] and [[oxycodone]] for pain, and t[[riamcinolone]] 0.1% [[cream]] for the rash.
*Management of the [[neoplasm]] depends on its type, location, stage, and time of its discovery and on patient preference:
**[[Surgical]] removal
** [[Chemotherapy]]
** [[Conservative]] [[palliative]] management
*Various [[Dermatological|dermatologic]] and [[immunosuppressive]] therapies have been used to treat erythema gyratum repens.
*Systemic [[steroids]] are frequently ineffective.
* Topical [[steroids]], [[vitamin A]], and [[azathioprine]] have also failed to relieve skin manifestations


===Medical Therapy===
===Surgery ===
There is no treatment for [disease name]; the mainstay of therapy is supportive care.
* [[Surgical]] [[resection]] of the discovered [[malignancy]] could be recommended as part of the management of Erythyma gyratum repens.


OR
=== Prevention ===


Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].
*[[Primary prevention]]:
 
** There are no [[Primary prevention|primary preventive]] measures available for erythema gyratum repens
OR
*[[Secondary prevention]]:
 
** If the thorough screening after Erythyma gyratum repens diagnosis detected the [[malignancy]] in its earliest stages.
The majority of cases of [disease name] are self-limited and require only supportive care.
* Tertiary [[prevention]]:
 
** If the thorough screening after Erythyma gyratum repens [[diagnosis]] detected the [[malignancy]] in its late stages or with widespread [[metastasis]].
OR
** Tertiary [[prevention]] aims to improve the [[quality of life]] and [[life expectancy]].
 
[Disease name] is a medical emergency and requires prompt treatment.
 
OR
 
The mainstay of treatment for [disease name] is [therapy].
 
OR
 
The optimal therapy for [malignancy name] depends on the stage at diagnosis.
 
OR
 
[Therapy] is recommended among all patients who develop [disease name].
 
OR
 
Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].
 
OR
 
Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
 
OR
 
Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
 
OR
 
Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].
 
===Surgery===
Surgical intervention is not recommended for the management of [disease name].
 
OR
 
Surgery is not the first-line treatment option for patients with [disease name]. Surgery is usually reserved for patients with either [indication 1], [indication 2], and [indication 3]
 
OR
 
The mainstay of treatment for [disease name] is medical therapy. Surgery is usually reserved for patients with either [indication 1], [indication 2], and/or [indication 3].
 
OR
 
The feasibility of surgery depends on the stage of [malignancy] at diagnosis.
 
OR
 
Surgery is the mainstay of treatment for [disease or malignancy].
 
===Primary Prevention===
There are no established measures for the primary prevention of [disease name].
 
OR
 
There are no available vaccines against [disease name].
 
OR
 
Effective measures for the primary prevention of [disease name] include [measure1], [measure2], and [measure3].
 
OR
 
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==References==
==References==
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Huda A. Karman, M.D.

Synonyms and keywords: Gammel's disease


Overview

Erythema gyratum repens is a rare highly specific and characteristic paraneoplastic syndrome that usually affect older people. It is characterized by wood-grain scaly skin eruption with intense pruritus. The cause of erythema gyratum repens is unknown but many theories suggest immunologic etiology or toxicologic products that are released by the associated tumor. The first case of erythema gyratum repens was described by a dermatologist named Gammel in the year 1952. For many years after erythema gyratum repens original description, there was little progress in defining the pathogenesis of erythema gyratum repens. Erythema gyratum repens has no specific classification but we can classify it based on its association with an internal malignancy into para-neoplastic and non-para-neoplastic erythema gyratum repens. The most common malignancies associated with erythema gyratum repens are lung or bronchogenic cancer, esophageal cancer, and breast cancer. Erythema gyratum repens can also be associated with non-neoplastic diseases such as tuberculosis, autoimmune disorders, or CREST syndrome. Erythema gyratum repens is characterized by its pathogonomic figurate, gyrate, or annular erythematous skin eruptions. The intense pruritus can be debilitating and usually urges the patient to go to the emergency department. The microscopic histopathological features of erythema gyratum repens consist of acanthosis, focal parakeratotic, and spongiosis of the epidermis with perivascular mononuclear, lymphocytic, and histiocytic infiltrate in the superficial plexus of the dermis. Erythema gyratum repens is very rare and it mainly affects people in their seventieth decade, the male to female ratio is 2:1. Erythema gyratum repens is diagnosed clinically by its characteristic skin eruption and an urgent thorough paraneoplastic workup should be initiated to look for internal malignancies. Patients with erythema gyratum repens presents with intensely pruritic, gradually progressive, skin lesions that crawl rather than migrate from one body region to the other. It can start in the upper trunk or upper back and extends to involve the extremities sparing the face. The mainstay of the treatment of erythema gyratum repens is finding and treating the underlying malignancy. Symptomatic treatment is not very effective in relieving the pruritus and its associated pain. The management can be surgical removal of the tumor, chemotherapy, or palliative conservative management. The skin eruptions can improve completely after the removal of the underlying tumor, or can recur especially if the tumor recurred or metastasized. Patients can live a few weeks, months or up to five years depending on when and at what stage the malignancy was detected.

Historical Perspective

Classification

Types of Erythema gyratum repens Characterestics
Paraneoplastic EGR
Non-paraneoplastic EGR Idiopathic EGR
  • Erythema gyratum repens with no underlying malignancy, associated conditions, or precipitating cause
|EGR-like eruptions [8]
EGR with concomitant skin disease
Drug-induced EGR

Pathophysiology


Causes

Differentiating Erythema Gyratum Repens from Other Diseases

Types examples
With underlying malignancy [14][15]
  • Erythema gyratum repens (EGR)
Without underlying malignancy
  • Erythema marginatum rheumaticum


Disease Erythema Characteristics Signs and Symptoms Associated Conditions Histopathology Lab finding

& Other evaluation

Prognosis
Erythema gyratum repens (EGR)
  • Migratory annular and configurate erythematous bands that form concentric rings
  • Wood grain scaly appearance
  • scales follows the leading edge of the bands
  • Eruption migrates more rapidly, 1cm/d

(Calcinosis, Raynaud’s phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia)


  • Skin manifestations can be improved within 48 hours of the resection of the underlying tumor with on of the following:
    • Complete cure of the skin eruption and pruritus
    • Temporary improvement then recurrence of the eruption (specially in cases of metastasis)
    • No effect of the tumor treatment on the course of EGR
      • Death can occur few weeks after the detection of the malignancy, few months, or four years as in Gammel's patient.
Erythema annulare centrifugum (EAC) [14]
  • Migratory annular and configurate erythematous

or polycyclic lesions

  • Eruption migrate at a slower rate (2 -3 mm/d) reaching up to 10 cm in diameter with central clearing
  • Cover only a small percentage of the total body surface   
  • Annular or polycyclic lesions which may begin as urticaria-like papule
  • Eventually old lesions can spontaneously resolve in several days to a few weeks while new eruptions develop.
  • The deep form of erythema annulare centrifugum has a firm, indurated border, is rarely pruritic, and has no scale
  • The superficial type of erythema annulare centrifugum has an indistinct scaly border and is usually pruritic  
  • No specific laboratory changes
  • Lesions disappear after the underlying etiology is managed (allergy, infection, malignancy)
  • if no underlying cause, lesions can recur after discontinuation of the supportive treatment
Necrolytic migratory erythema (NME)
  • Due to the difficulty of necrolytic migratory erythema recognition, and its association with glucagonoma, diagnosis is usually delayed
  • Necrolytic migratory erythema usually resolved after the resection and treatment of the pancreatic tumor, eg.10 days after tumor resection
  • Early recognition is crucial for better diagnosis and prognosis

Epidemiology and Demographics

Age

  • The average age of onset of erythema gyratum repens i is in the seventh decade of life (65 years old).

Gender

  • The male to female ratio is 2:1.

Race

  • EGR commonly affects Caucasians.

Risk Factors

Screening

Natural History, Complications, and Prognosis

  • The majority of patients with erythema gyratum repens presents with severely pruritic erythematous skin lesions that appear several months prior to the malignancy diagnosis[13]
  • If the underlying malignancy left untreated, the debilitating pruritus could persist until the patient dies[13]
  • Prognosis depends on the type of the underlying tumor and the probability of its treatment. It depends on the time of the erythema gyratum repens onset and the neoplasm discovery. The course and prognosis of erythema gyratum repens can be one of the following:
    • Complete cure of the skin eruption and pruritus after removal and treatment of the internal neoplasm.
    • Temporary improvement then recurrence of the eruption (specially in cases of metastasis).
    • No effect of the tumor treatment on the course of erythema gyratum repens.
    • Death can occur few weeks after the discovery of the malignancy, few months, or four years as in Gammel's patient.

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Prevention

References

  1. Rothman, Stephan (1925). "Über Hauterscheinungen bei bösartigen Geschwülsten innerer Organe". Archiv für Dermatologie und Syphilis. 149 (1): 99–123. doi:10.1007/BF02297811. ISSN 0340-3696.
  2. Burgdorf WHC, Bickers DR (2015). "The scientific legacy of Stephen Rothman". J Invest Dermatol. 135 (4): 954–959. doi:10.1038/jid.2014.447. PMC 4366295. PMID 25373439.
  3. 3.0 3.1 3.2 Gammel, John A. (1952). "ERYTHEMA GYRATUM REPENS". A.M.A. Archives of Dermatology and Syphilology. 66 (4): 494. doi:10.1001/archderm.1952.01530290070010. ISSN 0096-5979.
  4. Purdy, M. J. (1959). "Erythema Gyratum Repens". A.M.A. Archives of Dermatology. 80 (5): 590. doi:10.1001/archderm.1959.01560230076020. ISSN 0096-5359.
  5. 5.0 5.1 Skolnick, Marvin (1975). "Erythema Gyratum Repens With Metastatic Adenocarcinoma". Archives of Dermatology. 111 (2): 227. doi:10.1001/archderm.1975.01630140085011. ISSN 0003-987X.
  6. Boyd AS, Neldner KH, Menter A (1992). "Erythema gyratum repens: a paraneoplastic eruption". J Am Acad Dermatol. 26 (5 Pt 1): 757–62. PMID 1583177.
  7. 7.0 7.1 7.2 Rongioletti, F.; Fausti, V.; Parodi, A. (2014). "Erythema gyratum repens is not an obligate paraneoplastic disease: a systematic review of the literature and personal experience". Journal of the European Academy of Dermatology and Venereology. 28 (1): 112–115. doi:10.1111/j.1468-3083.2012.04663.x. ISSN 0926-9959.
  8. 8.0 8.1 Fukunaga M, Harada K, Mae K, Wakamatsu K, Kiriyama N, Tsuboi R; et al. (2017). "Erythema Gyratum Repens-Like Purpura in a Patient with Sjögren Syndrome". Case Rep Dermatol. 9 (2): 40–43. doi:10.1159/000477375. PMC 5498950. PMID 28690517.
  9. 9.0 9.1 Günther R, Nasser S, Hinrichsen H, Fölsch UR (2002). "[Erythema gyratum repens: drug reaction following azathioprine administration in a patient with type I [[autoimmune]] [[hepatitis]]". Med Klin (Munich). 97 (7): 414–7. PMID 12168480. URL–wikilink conflict (help)
  10. 10.0 10.1 Rongioletti, Franco; Fausti, Valentina; Parodi, Aurora (2012). "Erythema Gyratum Repens Induced by Pegylated Interferon Alfa for Chronic Hepatitis C". Archives of Dermatology. 148 (10): 1213. doi:10.1001/archdermatol.2012.1968. ISSN 0003-987X.
  11. Samotij D, Szczech J, Bencal-Kusinska M, Reich A (2016). "Erythema gyratum repens associated with cryptogenic organizing pneumonia". Indian J Dermatol Venereol Leprol. 82 (2): 212–3. doi:10.4103/0378-6323.173594. PMID 26765132.
  12. Appell ML, Ward WQ, Tyring SK (1988). "Erythema gyratum repens. A cutaneous marker of malignancy". Cancer. 62 (3): 548–50. doi:10.1002/1097-0142(19880801)62:3<548::aid-cncr2820620318>3.0.co;2-h. PMID 3390794.
  13. 13.00 13.01 13.02 13.03 13.04 13.05 13.06 13.07 13.08 13.09 13.10 13.11 Gore M, Winters ME (2011). "Erythema gyratum repens: a rare paraneoplastic rash". West J Emerg Med. 12 (4): 556–8. doi:10.5811/westjem.2010.11.2090. PMC 3236141. PMID 22224159.
  14. 14.0 14.1 14.2 14.3 14.4 14.5 14.6 14.7 14.8 14.9 Tyring SK (1993). "Reactive erythemas: erythema annulare centrifugum and erythema gyratum repens". Clin Dermatol. 11 (1): 135–9. PMID 8339188.
  15. 15.0 15.1 Holt PJ, Davies MG (1977). "Erythema gyratum repens--an immunologically mediated dermatosis?". Br J Dermatol. 96 (4): 343–7. PMID 861171.
  16. Ridge A, Tummon O, Laing M (2019). "Response to "Transformation from pityriasis rubra pilaris to erythema gyratum repens-like eruption without associated malignancy: A report of 2 cases"". JAAD Case Rep. 5 (5): 461–462. doi:10.1016/j.jdcr.2019.03.012. PMC 6510971 Check |pmc= value (help). PMID 31111084.