Track: Case Reports

Topic: Lymphoproliferative Disorders

Abstract:

A 48-year-old woman, tobacco user, presented with a 2-year history of asymptomatic atrophic plaques. On the antecubital fold, she had a 10cm circular well-circumscribed plaque with elevated erythematous-purple edge and a frankly atrophic and pendulous-like center. Near the right intergluteal fold, she presented a 6cm erythematous plaque covered by atrophic, pendulous-like and bulging skin. No lymphadenopathies were identified.

A previous skin biopsy of the forearm lesion informed a chronic non-necrotizing granulomatous dermatitis. She had undergone topical treatment with betamethasone for 18 months with progressive enlargement of the lesions.

Routine laboratory, LDH, ꞵ2microglobulin, calcemia, and angiotensin converting enzyme levels were normal. The Mantoux test was 0mm and the chest x-ray was normal.

Histopathological examination showed mild epidermal atrophy with epidermotropism. Small, atypical lymphocytes with hyperchromatic nuclei infiltrated the epidermis. The superficial and deep dermis showed dense diffuse, perivascular, and periadnexal infiltrates with abundant atypical lymphocytes, histiocytes and multinucleated giant cells arranged in granulomas. Verhoeff-Van Gieson stain showed loss of elastic fibers in the mid and superficial dermis. A few multinucleated cells engulfed elastic fibers (elastophagocytosis). Immunohistochemistry stains revealed lymphocytes were positive for CD3, CD5, and CD7, with a predominance of CD4 over CD8. CD30 was negative.

The patient was diagnosed with granulomatous slack skin (GSS). She received treatment with PUVA and tacrolimus, resulting in clinical improvement of the forearm lesion. The gluteal lesion was excised. This case highlights a rare type of cutaneous T-cell lymphoma and the importance of considering GSS in the differential diagnosis of granulomatous dermatosis.

Menu

Mauro Coringrato, MD