CLINICAL HISTORY:

Signs and symptoms:  Lesions were very pruritic and scaly in nature

Previous Treatment:  Claritin, Atarax, Protopic, salicylic acid and various topical steroids

Other information:  No family history of a similar rash. No recent travels.

PHYSICAL EXAM:

On physical examination, thickening and furrowing of the scalp and facial skin was apparent, as was alopecia of the frontal and vertex scalp. The facial changes were consistent with leonine faces. Diffuse hyperkeratotic papules, plaques, and nodules with multiple areas of depigmentation and secondary impetiginization was also present. The palms and soles revealed extensive hyperkeratosis. All nails displayed onycholysis and subungual hyperkeratosis.

LABORATORY TESTS:

The CBC and CMP were WNL. ANA was found to be negative and RPR was non-reactive.

DERMATOHISTOPATHOLOGY:

Biopsy from the right preauricular area demonstrates an atypical lymphoid infiltrate consisting of numerous large cells with diffuse CD30+ staining.

DIFFERENTIAL DIAGNOSIS:

1.   Leishmaniasis
2.   Leprosy
3.   Chronic actinic dermatitis
4.   Mycosis fungoides
5.   Pityriasis rubra pilaris

SCROLL DOWN FOR ANSWER AND DISCUSSION.

CORRECT DIAGNOSIS:

Mycosis fungoides

DISCUSSION:

Mycosis fungoides is the most common type of cutaneous T-cell lymphoma (CTCL) as it accounts for approximately 50% of the cases. There is an incidence of 0.3 per 100,000 individuals. Median age of diagnosis is 55-60, and males are more commonly affected.

Patients generally progress from patches (premycotic stage) to plaques and finally nodules. The disease is usually located in the “bathing trunk” distribution. Prior to proper diagnosis, patients initially present with a nonspecific eczematous or psoriasiform dermatitis with inconclusive skin biopsies.

The distinguishing histologic features include nests of atypical cells with highly convoluted and hyperchromatic nuclei (Pautrier’s microabscesses) and epidermotropism of lymphocytes. Immunophenotyping typically stains positive for CD3, CD4, and CD45 RO and mostly negative for CD8. Unique to our case was the CD30+ large cell anaplastic transformation, which is associated with a poor prognosis.

Our patient presented with classic leonine faces. The differential that should be considered is as follows: carcinoid, chronic actinic dermatitis, cutis verticis gyrata, leishmaniasis, leprosy, lipoid proteinosis, lymphma/leukemia, MF, multicentric reticulohistiocytosis, multiple keratoacanthoma syndrome, progressive nodular histiocytoma, sarcoidosis, and scleromyxedema.

Prognosis is dependent on the stage, extent of skin lesions, and presence of extracutaneous manifestations. Patients with limited patch/plaque stage have normal life expectancy. Lymph node and visceral involvement, as well as transformation into large T-cell lymphoma portends an aggressive course.

TREATMENT:

Actual treatment for this patient: Pt was placed on topical corticosteroids and referred to an oncologist for systemic workup.

Other Treatment options: Treatment options include topical corticosteriods, topical chemotherapy (including nitrogen mustard and carmustine), radiotherapy, phototherapy, systemic chemotherapy (CHOP), biologic response modifiers, interferons, and retinoids. Novel immunomodulatory therapies such as receptor-targeted cytotoxic fusion proteins and various vaccines have been used.

REFERENCES:

1. Arnold, Odom, James. (2000) Andrews Diseases of the Skin. Philadelphia, PA.
Elsevier.

2. Bolognia JL, Jorizzo JL, Rapini RP, et. al. (2003) Dermatology. Spain: Mosby

3. Paulli M, Berti E. Cutaneous T-cell lymphomas (including rare subtypes). Current concepts. Haematologica. 2004 Nov; 89 (11):1372-1.

Additional Comment:

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