Successful treatment of lipedematous alopecia using mycophenolate mofetil.

Lipedematous alopecia (LA) is a rare form of non-scarring alopecia described by Coskey in 1961. To date, 78 cases have been reported in the literature (1). LA generally starts from the vertex, with a centrifuge extension that may affect the whole scalp. Patients may present pain or pruritus along the affected area. Palpation reveals a smooth, diffuse and cotton-wool-like induration (1, 2). Currently there is no effective treatment to halt or delay progression. The use of topical and intralesional steroids has, in general, had a negative response (3). High & Hoang reported no response to hydroxychloroquine in a female patient with LA and discoid lupus erythematous (DLE) (4). Yip et al. (5) reported surgical treatment with a successful debulking of the scalp after achieving inactivity of the disease, with no recurrence after a 1-year follow-up. We describe here a patient with LA who responded successfully to oral mycophenolate mofetil (MMF), an immunosuppressive drug not previously used to treat this condition. A 51-year-old man with no history of comorbidities or medication intake presented with diffuse and progressive hair loss, increased scalp volume, and pruritus in the last 5 months. Physical examination revealed non-cicatricial alopecia with erythema on the vertex and bilaterally on the parietal zone (Fig. 1A). Palpation revealed a cotton wool-like induration on the affected area. Dermoscopy showed multiple linear telangiec-tasias with conservation of the follicular openings. Ultrasound revealed thickening of the hypodermis in the affected areas, with marked effacement of the dermal-hypodermic interphase. Laboratory tests showed a normal complete blood count (CBC), antinuclear antibody (ANA) and extractable nuclear antigen (ENA). Thyroid, kidney, and hepatic function tests were also normal. Biopsy highlighted a superficial perivascular lymphocytic infiltrate with marked thickening of the subcuta-neous cellular tissue. There was also loss of normal lobular architecture at the hypodermis due to interstitial oedema. Ectatic lymphatic vessels were seen in the deep dermis and hypodermis (Fig. 2). Direct immunofluorescence was negative. Treatment began with intralesional steroid infiltration every 14 days, totalling 8 sessions in 4 months. Follow-up showed refractoriness and even worsening of the condition, characterized by centrifugal progression of alopecia and increased signs of inflammation. After new clinical and laboratory tests that demonstrated normal function of other organs, treatment with 1 g/day of MMF was initiated, obtaining the first satisfactory results after the third month (Fig. 1B). MMF was well tolerated by the patient, with no side-effects. The same dose was therefore maintained …