A Case of Pseudoxanthomatous Mastocytosis

Oh, Hee Soo; Lee, Moo Hyung; Kim, Nack In; Haw, Choong Rim

Abstract

We report a case of pseudoxanthomatous mastocytosis that is an uncommon variant of urticaria pigmentosa. A 3 month-old boy visited our hospital with yellowish cutaneous nodules At the age of 2 months, small yellowish macule developed on the posterior neek, and during the following months, lesions spread to trunk, upper extremities, face, and scalp, most of them became gradually to the nodules. Darier's sign could be elicited easily. A biopsy of one. of the nodules or the back showed a very dense oval or cuboidal cells infiltrate in the dermis. Special stain with toluiddine blue showed this to he composed of mast cells. Electron microscopic examination revealed crowded mast. cells which was showing numerous cytoplasmic granules and long, thin villi. The patient: was managed symptomaticvlly and recommended to avoid mechanical or chemical irritation to the lesions.