Clinical effects of topical pimecrolimus in a patient with Fox-Fordyce disease

Fox-Fordyce disease (FFD) is characterized by a pruritic eruption of skin-coloured or yellowish papules in areas rich in apocrine glands. The histology comprises dilatation of follicular infundibula with hyperkeratosis, acanthosis, and spongiosis of the infundibular epithelium with perifollicular infiltration of lymphocytes and foamy histiocytes. We treated a 12-year-old girl with FFD with topical pimecrolimus for 12 weeks, this resulted in a complete clearance of lesions. After the therapy, the patient was followed for an additional 19 months without signs of relapse. The effects of pimecrolimus in FFD might imply that an inflammatory process inducing secondary reactive hyperkeratosis could be involved in the pathogenesis of FFD.