Erythema gyratum repens
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Synonyms and keywords: Gammel's disease.
Overview
Historical Perspective
- In 1953, the dermatologist, Dr. John A Gammel who was trained to link skin lesions to internal malignancy was the first one who described and labeled Erythema Granulatum Repens in a 55-year-old patient with poorly differentiated breast adenocarcinoma
- Up to 1992, there were only 49 cases in the literature, 41 of which (84%) were associated with a neoplasm
- EGR is associated with internal malignancy in 82% of cases. However, between 1990 and 2010, data was collected from the medical records of patients form dermatology department in University of Genoa and from databases as pubmed and medline, the conclusion of this literature review was that EGR is no longer considered as an obligate paraneoplastic syndrome. More than expected cases of EGR were found with no neoplasm association. Non-paraneoplastic EGR could be:
- Idiopathic EGR
- EGR-like eruptions (different dermatologic lesions that mimic EGR)
- Drug-induced EGR
Classification
- There is no established system for the classification of EGR.
Pathophysiology
- The cause of EGR has not been identified.
- Many theories suggest that EGR is due to immunologic mechanisms
- The immunologic mechanism theory is evidenced by the observed immunofluorescence patterns of IgG, C3, and C4 at the basement membrane
- 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 immunogenic
- Theory 3 deposition of tumor antigen-antibody complexes onto the basement membrane causes the reactive dermatitis seen in EGR
Causes
The cause of erythema gyratum repens has not been identified.
Differentiating ((Page name)) from Other Diseases
- EGR has a narrow differential diagnosis and it has to be differentiated from skin lesions with gyrate erythematous eruptions, such as:
- Necrolytic migratory erythema (NME)
- Erythema annulare centrifugum (EAC)
- Erythema migrans
Epidemiology and Demographics
EGR commonly affects Caucasians, with an average age of onset in the seventh decade of life (an average age of 63 years), 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.
Risk Factors
There are no established risk factors for EGR.
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.
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 pruritus can be debilitating and it may persist to the time of death.
Diagnosis
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.
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.
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.
Laboratory Findings
There are no diagnostic laboratory findings associated with EGR.
Electrocardiogram
There are no ECG findings associated with EGR.
X-ray
There are no x-ray findings associated with EGR.
Echocardiography or Ultrasound
There are no echocardiography/ultrasound findings associated with EGR.
CT scan
CT findings could be seen in the associated visceral malignancy in EGR.
MRI
There are no MRI findings associated with EGR.
Other Imaging Findings
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.
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
There is no treatment for EGR]; the mainstay of therapy is underlying malignancy.
Surgery
Surgical intervention is not recommended for the management of EGR.
Primary Prevention
There are no established measures for the primary prevention of EGR.
Secondary Prevention
There are no established measures for the secondary prevention of EGR.