Cicatricial pemphigoid in a 6-year-old child: report of a case and review of the literature.
Cicatricial pemphigoid has been documented previously in only four patients under the age of 20 years. We report a 6-year-old male who had erosions of the oral and genital mucosa, conjunctivitis, hoarseness, dysphagia, recurrent vomiting, and weight loss. Upper airway obstruction due to a chronically inflamed and scarred epiglottis necessitated tracheostomy. Biopsy of tissue from a solitary cutaneous lesion demonstrated a subepidermal bulla. Direct and indirect immunofluorescence confirmed a diagnosis of cicatricial pemphigoid. Therapy was begun consisting of prednisone, 2 mg per kg per day, in combination with dapsone, 2 mg per kg per day. He did not improve until the prednisone dosage was increased to 4 mg per kg per day.
D000293:Adolescent; D000328:Adult; D000368:Aged; D001485:Basement Membrane / Q000276:immunology; D002648:Child; D002675:Child, Preschool; D003176:Complement C3 / Q000032:analysis; D003622:Dapsone / Q000627:therapeutic use*; D003937:Diagnosis, Differential; D004359:Drug Therapy, Combination; D004825:Epiglottis / Q000000981:diagnostic imaging; D005260:Female; D005455:Fluorescent Antibody Technique; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D008297:Male; D008875:Middle Aged; D009092:Mucous Membrane / Q000473:pathology; D010390:Pemphigoid, Benign Mucous Membrane / Q000175:diagnosis / Q000276:immunology / Q000628:therapy*; D011241:Prednisone / Q000627:therapeutic use*; D011859:Radiography; D012872:Skin Diseases, Vesiculobullous / Q000628:therapy*
D003176:Complement C3; D007074:Immunoglobulin G; D003622:Dapsone; D011241:Prednisone