Cutaneous Mastocytosis with Persistent Blistering: Successful Treatment with Methylprednisolone and 3-Year Follow-Up Management

Abstract

Background: Mastocytosis is a rare disorder with diverse clinical manifestations. In cutaneous mastocytosis the mast cell infiltration is limited to the skin, but is often associated with systemic symptoms due to the release of mast cell mediators. Case Report: We report a 6-month-old male infant who had skin lesions of various morphologies (macules, papules, plaques, and nodules) and sizes, persistent blistering and frequent flushing episodes for half a year. Vital signs and physical examinations were unremarkable. No abnormalities in the laboratory tests were found except for a serum tryptase level (STL) of 11.8 ng/ml. The histological and immunohistochemical examinations confirmed the diagnosis of cutaneous mastocytosis. The patient was first treated with methylprednisolone, oral levocetirizine, and topical fusidic acid/betamethasone cream. Subsequently the treatment was tapered and stopped within 9 weeks. The child’s symptoms improved and were successfully controlled with intermittent courses of ketotifen and topical hydrocortisone over 3 years. Conclusion: Childhood cutaneous mastocytosis usually has a favorable prognosis, but in some cases the disease can progress with skin manifestations necessitating a more active systemic and topical treatment.