Premium
Cicatricial Pemphigoid in a 6‐Year‐Old Child: Report of a Case and Review of the Literature
Author(s) -
Rosenbaum Marjorie Mintz,
Esterly Nancy Burton,
Greenwald Mark J.,
Gerson Carol Roberts
Publication year - 1984
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/j.1525-1470.1984.tb00435.x
Subject(s) - medicine , cicatricial pemphigoid , prednisone , epiglottis , bulla (seal) , dysphagia , surgery , vomiting , pemphigoid , dapsone , dermatology , lesion , airway obstruction , biopsy , airway , bullous pemphigoid , larynx , pathology , lung , antibody , immunology
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 subepidennal 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.