Abstract

A 48-year-old woman initially presented with a 2-year history of progressive scalp alopecia associated with arthralgia and tenosynovitis, mainly in the joints of the hands, which had begun 3 years before that presentation. The patient had hypercholesterolaemia, but was otherwise healthy, and there was no family history of alopecia. The results of laboratory investigations carried out at the time were normal, apart from a mild elevation in her lactate dehydrogenase levels and liver-function tests. The persistent tenosynovitis was successfully treated with low-dose prednisolone and hydroxychloroquine. A few months later, the patient returned to our department, presenting with exerciseinduced and paroxysmal nocturnal dyspnoea, recurrent diarrhoea, and progressive worsening of the alopecia. On physical examination, diffuse alopecia was seen, without evidence of scarring. The scalp was diffusely thickened (Fig. 1), while the eyebrows, eyelashes, axillary, genital and body hair appeared normal. Skin biopsies were taken from the scalp.

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