Primary cutaneous follicle centre lymphoma differential diagnosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Soroush Seifirad, M.D.[2], Sogand Goudarzi, MD [3], Sowminya Arikapudi, M.B,B.S. [4]

Overview

Primary cutaneous follicle centre lymphoma a must be differentiated from other diseases such as eczema and psoriasis.

Differentiating Primary Cutaneous Follicle Centre lymphoma from other Diseases

• Primary cutaneous follicle centre lymphoma must be differentiated from mycosis fungoides or any other diseases with cutaneous patch or plaque that not respond to first- and second-line treatment such as:
  • Sezaruy syndrome
    • Sezaruy syndrome(SS) is more symptoI contrast to patch or plaque mycosis fungoides, SS is much more symptomatic. Sezary syndrome patients tend to present with diffuse skin involvement,not like mycosis fungoides usually evolve through patches and plaques to erythroderma
    • In Sezary syndrome infiltration of skin is generally much less dense than plaque in mycosis fungoides (MF)
  • Eczema
  • Adult T cell leukemia/lymphoma
  • Psoriasis
  • Pityriasis rubra pilaris
  • dermatitis
  • Hypereosinophilic syndrome
  • Adult T-cell leukemia
  • Atopic dermatitis
  • Contact dermatitis ( Allergic, irritant)
  • Chronic actinic dermatitis
  • Scabies
  • Subcutaneous panniculitis like T cell lymphoma (SPTCL)
  • Drug eruption
  • Graft versus host disease
  • Lichen planus
  • Pediatric atopic dermatitis
  • Tinea corporis
  • Primary cutaneous anaplastic large cell lymphoma (ALCL)
  • Cutaneous gamma/delta T cell lymphoma (G/D TCL)

Disease	Rash Characteristics	Signs and Symptoms	Associated Conditions
Primary Cutaneous Follicle Centre lymphoma	Firm erythematous or violaceous plaques, nodules, or tumors of varying sizes May be ulcerated	Fever Rash, ulcer, firm erythematous or violaceous plaques, nodules, or tumors of varying sizes Fatigue Weight loss Night sweats Chest pain Abdominal pain Bone pain Painless swelling in the neck, axilla, groin, thorax, and abdomen (lymphadenopathy)	Lyme disease (Borrelia burgdorferi) Hepatitis C Human herpes virus 8 (HHV8)
Cutaneous T cell lymphoma/Mycosis fungoides	Premycotic phase: A scaly, red rash in areas of the body that usually are not exposed to the sun. This rash does not cause symptoms and may last for months or years. Patch phase: Thin, reddened, eczema -like rash. Plaque phase: Small raised bumps (papules) or hardened lesions on the skin, which may be reddened. Tumor phase: Tumors form on the skin. These tumors may develop ulcers and the skin may get infected.	Epidermal atrophy or poikiloderma Generalized itching (pruritus) Pain in the affected area of the skin. Insomnia Red (erythematous) patches scattered over the skin of the trunk and the extremities Tumor-like lobulated outgrowths form on the skin in the latter part of the disease Weight loss Lymphadenopathy Malaise and fatigue Anemia May progress to Sezary syndrome (Skin involvement plus hematogenous dissemination)	Sezary syndrome
Pityriasis rosea	Pink or salmon in colour, which may be scaly, termed as "herald patch" Oval in shape Long axis oriented along the clevage lines Distributed on the trunk and proximal extremities Squamous marginal collarette and a “fir-tree” or “Christmas tree” distribution on the posterior trunk Develops after viral infection Resolves spontaneously after 6-8 weeks	Preceded by a prodrome of: Sore throat Gastrointestinal disturbance Fever Arthralgia	Infection by any of the following: HHV-6 HHV-7 HHV-8
Pityriasis lichenoides chronica	Recurrent lesions are usually less evenly scattered than psoriasis Brownish red or orange-brown color Lesions are capped by a single detachable opaque mica-like scale Often leave hypopigmented macules	High fever Malaise Myalgias Skin burning Pruritis	Infection by any of the following: Epstein-Barr virus (EBV) Toxoplasma gondii Human immunodeficiency virus (HIV)
Nummular dermatitis	Multiple coinshaped eczematous lesions Commonly affecting the extremities (lower>upper) and trunk May ooze fluid and become dry and crusty	Often appears after a skin injury, such as a burn, abrasion (from friction), or insect bite Lesions commonly relapse after occasional remission or may persist for long periods Pruritis	Associated with: Dry skin Emotional stress Allergens(rubber chemicals, formaldehyde, neomycin, chrome, mercury and nickel) Staphylococcus infection Seasonal variation Alcohol Drugs Atopy
Secondary syphilis	Round coppery red color lesions on palms and soles Papules with collarette of scales	Fever Generalized lymphadenopathy Sore throat Patchy hair loss Headaches Weight loss Myalgia Fatigue	Associated with: Condylomata lata Corona verinata Positive VDRL test
Bowen’s disease	Erythematous little scaly plaque, which enlarges over time in an erratic manner Scale is usually yellow or white and it is easily detachable without producing any bleeding Well defined margins	Pruritis Pain Bleeding lesions	Associated with: Erythroplasia of Queyrat (Bowen's disease of the penis) Squamous cell carcinoma Solar radiation and ultraviolet (UV) exposure Radiotherapy Immunosuppression Arsenic exposure Human papilloma virus (HPV) type 16 Merkel cell polyomavirus Sjögren’s syndrome
Exanthematous pustulosis	Numerous small, primarily non-follicular, sterile pustules, arising within large areas of edematous erythema	Fever Leukocytosis Intracorneal, subcorneal, and/or intraepidermal pustules with papillary dermal edema containing neutrophils and eosinophils	Associated with: Antibiotics (penicillins, sulfonamides, tetracyclines) Carbamazepine Calcium channel blockers(Diltiazem) Hydroxychloroquine
Hypertrophic lichen planus	Classically involves shin and ankles and is characterized by hyperkeratotic plaques and nodules covered by a scale Lesions may transform into hyperkeratotic thickened elevated purplish or reddish plaques and nodules	Chronic pruritis Scaling May be asymptomatic	Associated with Hepatitis C virus infection
Sneddon–Wilkinson disease	Flaccid pustules that are often generalized and have a tendency to involve the flexural areas Have an annular configuration	Pruritis May be asymptomatic	Associated with: Monoclonal gammopathy, usually an IgA paraproteinemia Crohn's disease Osteomyelitis Adalimumab
Small plaque parapsoriasis	Erythematous plaques which are covered with fine scale. May present with elongated, finger-like patches symmetrically distributed on the flanks, also known as digitate dermatosis	Lesions may be asymptomatic May be mildly pruritic May fade or disappear after sun exposure during the summer season, but typically recur during the winter	May progress to mycosis fungoides
Intertrigo	Red and fleshy looking lesion in skin folds Itching Oozing May be sore	Pruritis Musty odor	Associated with: Infections (Fungal, bacterial, viral) Allergies Diabetes Obesity
Langerhans cell histiocytosis	Scaling and crusting of the scalp	Pathological fractures Visceromegaly (hepatomegaly, spleenomegaly) Chronic cough Dyspnea Lymphadenopathy	Associated with: Diabetes insipidus Pancytopenia
Tinea manuum/pedum/capitis	Scaling, flaking, and sometimes blistering of the affected areas Hair loss with a black dot on scalp in case of tinea capitis	Pruritis KOH preparation of the lesions confirms fungal infection	Associated with: Diabetes Immunosupression Intimate contact with infected person May lead to asthma exacerbation
Seborrheic dermatitis	Papulosquamous, scaly, flaky, itchy, and red rash found particularly at sebaceous gland-rich areas of the body		Associated with: AIDS Stress Fungal infection Fatigue Sleep deprivation Change of season Parkinson's disease Biotin deficiency

References

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