Mucin-poor follicular mycosis fungoides

Abstract

A 49-year-old woman was admitted with generalized pruritus which she had for the last 4 years. Three months ago she developed erythema on her face, alopecia totalis, an erythematous macular eruption with follicular hyperkeratoses on the trunk and limbs (Fig. 1). She had bilateral palpable axillary lymphadenopathy. Histologic examination of the biopsies taken from the erythematous areas on the trunk and scalp revealed a folliculocentric infiltration composed of atypical, small and medium-sized mononuclear cells, intermingled with reactive lymphocytes, histiocytes, plasmocytes, rare eosinophils, and giant cells without involvement of the epidermis (Fig. 2). The infiltrate surrounded and invaded the hair follicle epithelium without destroying it. With alcian blue staining, only small amounts of mucin were detected within the epithelium of the hair follicles, By the immunohistochemistry performed, folliculocentric infiltration was mainly composed of CD3(+), CD4(+), CD8(-) lymphocytes. A full blood count, peripheral smear, erythrocyte sedimentation rate (ESR), the concentration of nitrogen in the form of urea in the blood (BUN), creatinin, transaminases, serum electrolytes, C-reactive protein (CRIP), IgE, serum lipids, serum lactate dehydrogenase, urinalysis, roentgenogram and CT scan of chest were normal. Beta 2 microglobulin was 3.15 mg/L (normal range 1.2-2.5 mg/L). In the biopsy of the axillary lymph node, there was a focal infiltration of atypical T-cells in the interfollicular area. She was given psoralen-UVA (PUVA) treatment for 6 months (85 sessions, in escalating doses with a total 71.8 j/cm(2)) which resulted in a partial healing of the pruritus, erythematous plaques, follicular hyperkeratoses, and patchy hair regrowth. She then received oral methyl prednisolone, 40 mg daily for 3 weeks and the dose was gradually decreased and eventually reduced to 16 mg daily in 2 months. This resulted in healing of the pruritus, improvement in the erythematous plaques and follicular hyperkeratoses and axillary lymphadenopathy. Beta 2 microglobulin levels decreased to normal range (2.27 mg/ L). After taking the 16 mg daily dose of the prednisolone for 6 months, her complaint of itching recurred. Erythematous plaques and follicular hyperkeratoses were noted again. The dosage of the steroids was increased to 40 mg daily and PUVA treatment was restarted. She is currently receiving oral methyl prednisolone and PUVA.