CLINICAL HISTORY:

Signs and symptoms:  Non pruritic erythematous patch on the patients right buttock that has been present for at least four years and was previously diagnosed and treated as Eczema. Reports minimal change over the last four years with no period of clearance.

Previous Treatment:  Topical antifungal creams, Class I-VI topical corticosteroids and an intramuscular Triamcinolone injection. Pt reports slight benefit from the topical combination of Nystatin / Triamcinolone but denies any benefit from the other medications.

Other information:  Pt works in a day care center. She is not taking oral prescription medications or herbal supplements.

PHYSICAL EXAM:

A 62 year old Caucasian female with a 35 x 60mm patch of erythema on her right superior buttock. The lesion is non-tender and surrounding erythema blanches with diascopy. No palpable lymph nodes are present.

LABORATORY TESTS:

CBC with peripheral smear: Within Normal Limits
Complete Metabolic Panel: Within Normal Limits

DERMATOHISTOPATHOLOGY:

See Photos Below

DIFFERENTIAL DIAGNOSIS:

1.   Eczema
2.   Psoriasis
3.   Candidiasis
4.   Mycosis Fungoides
5.   Majocci Granuloma

SCROLL DOWN FOR ANSWER AND DISCUSSION.

CORRECT DIAGNOSIS:

Mycosis Fungoides

DISCUSSION:

Mycosis Fungoides, despite its name, is not a fungal disease. It is the most common type of cutaneous T-cell lymphoma (CTCL) and is typically diagnosed in the 5th or 6th decade of life. Mycosis Fungoides is more prevalent in men then in woman and in African Americans versus Caucasians. The etiology remains a mystery although genetic, immunologic and environmental factors have been implicated.

The disease course is often lingering and evolves over several clinical phases: pre-MF, patch, plaque, and tumor. Lesions are most common in the bathing suit area and can be pruritic. The pre-MF phase is commonly misdiagnosed as eczema or when biopsied as a non-specific spongiotic dermatitis. It can take up to 35 years for the lesion to progress into a patch or plaque phase with the median length to diagnosis around 5 years.

Distinguishing histological features depend on the clinical stage. Early lesions demonstrate patchy bandlike lymphocytic infiltrate in the papillary dermis with coarse fibrosis. Epidermotropism is a common feature and Pautrier microabscesses filled with atypical lymphocytes containing cerebriform nuclei are characteristic of MF. Immunophenotyping often reveals CD2+, CD3+, CD4+, CD5+ and CD8- with loss of CD7. T cell receptor gene rearrangement is typically clonal.

Staging of MF, like most cancerous conditions, is done using the TNM system: Tumor (clinical phase and percent of involved skin), Nodal (lymph node involvement), Metastasis (visceral organ involvement) and in addition, also includes Blood evaluation (presence of Sezary cells). Prognosis depends on staging, with infection or systemic involvement accounting for cause of death.

Our patient is T1, N0, M0, B0 placing her in the clinical stage IA which carries a 95% five year survival rate.

TREATMENT:

Our patient was referred to an oncologist for full systemic workup. She declined treatment of the lesion at this time which could include topical corticosteroids, topical chemotherapy (nitrogen mustard or carmustine), topical bexarotene and PUVA or UVB. Additional treatment options for more progressive cases include: radiotherapy, systemic multiagent chemotherapy (cyclophosphamide, doxorubicin, vincristine and prednisone), interferons, systemic retinoids, and other biologic response modifiers. However, it is important to note that treatment has not been shown to prolong life.

REFERENCES:

1)Arnold, Odom, James. (2000) Andrews Diseases of the Skin. Philadelphia, PA:
Elsevier
2)Bolognia JL, Jorizzo JL, Rapini RP, et. al. (2008) Dermatology. Spain: Mosby
3)Habif. (2004) Clinical Dermatology. China: Mosby
4)Lever. (2009) Histopathology of the Skin. China: Lippincott Williams & Wilkins

Additional Comment:

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