Hyalinosis Cutis et Mucosae: Report of 4 Cases / 대한피부과학회지

We report four cases of hyalinosis cutis et mucosae. They developed hosrseness in their infancy and all had past history of frequent skin infections with accompanying varioliform scars. On physical examination, all the patients had beaded papules along their eyelid margins, hoarseness, varioliform scars and various skin and mucous membrane infiltrations. Pathologic examinations performed on skin infiltrations and eyelid papules revealed diastase resistant periodic acid Schiff positive materials deposited mainly in upper dermis. We have given three of four patients 1g/day of dimethyl sulphoxide from 6 to 12 months with no significant side effects nor remarkable clinical improvement.