Folliculotropic Mycosis Fungoides: Response to Bexarotene

摘要

Folliculotropic mycosis fungoides (FMF) is a variant of mycosis fungoides characterized by the presence of folliculotropic infiltrates, often with sparing of the epidermis, and preferential involvement of the head and neck. We report a man who was initially diagnosed with eosinophilic folliculitis but had classical FMF with ulceration and improved on bexarotene. Introduction Folliculotropic mycosis fungoides has been designated as a distinct entity in the World Health Organization-European Organization for Research and Treatment of Cancer classification system for cutaneous lymphomas. 1 It usually involves the scalp and face but may also compromise the torso, as in our patient. Its clinical spectrum includes scarring and non-scarring alopecia, erythematous plaques with follicular accentuation, acneiform lesions such as nodulocystic lesions, pustules, milia and comedones, tumors, and sometimes mucinous discharge. Severe pruritus is also a prominent feature. 2 Initial Presentation A 62-year-old man presented with a 20 year history of a pruritic erythematous rash on his scalp and trunk that had been diagnosed as seborrheic dermatitis and eosinophilic folliculitis. He had eosinophilia (13%) and IgE significantly elevated (2317 K/UL). He was initially treated with systemic antifungals, topical steroids, antihistaminics and oral antibiotics with partial improvement. This past last year, he developed a slowly growing ulceration in the middle of his back.Physical examination (Figure 1A) revealed an erythematous plaque with scarring alopecia on his scalp and (Figure 1B) erythematous telangiectatic patches and plaques on the trunk. Micropustules were seen within some of these lesions. (Figure 1C). On the back, he had a large 4 x 3 cm. ulcer down to the muscle containing purulent drainage (Figure 1D) and had palpable axillary and inguinal nodes. The cultures grew Staphylococcus aureus. Biopsies were performed of the edge of the ulcer, of the anterior chest surrounding a pustule, and of a lymph node.