Pemphigoid in children

• Published in: Pediatric dermatology Vol. 6; no. 4; p. 267
• Main Authors: Oranje, A P, van Joost, T
• Format: Journal Article
• Language: English
• Published: United States 01.12.1989
• Subjects: Adolescent; Adrenal Cortex Hormones - therapeutic use; Child; Child, Preschool; Drug Therapy, Combination; Female; Fluorescent Antibody Technique; Humans; Immunoglobulins - analysis; Infant; Male; Pemphigoid, Benign Mucous Membrane - drug therapy; Pemphigoid, Benign Mucous Membrane - epidemiology; Pemphigoid, Benign Mucous Membrane - physiopathology; Pemphigoid, Bullous - drug therapy; Pemphigoid, Bullous - epidemiology; Pemphigoid, Bullous - physiopathology; Prognosis; Skin Diseases, Vesiculobullous - physiopathology
• ISSN: 0736-8046
• DOI: 10.1111/j.1525-1470.1989.tb00910.x

Pemphigoid is an autoimmune blistering disease that is rare in childhood. A review of the English, German, and French literature published prior to 1989 revealed 31 cases of juvenile bullous pemphigoid and 12 of other forms (10 cases of juvenile cicatricial and 2 of juvenile localized bullous pemphigoid). Childhood pemphigoid, although less frequent, seems no different than its adult counterpart. While oral lesions are more common in the juvenile bullous form, there is no association with malignancy, which is a controversial and presumably incorrectly assumed association in the adult disease. The prognosis for children is good in most cases, and the disease is self-limiting. The mainstay of therapy has been oral corticosteroids, but dapsone also often produces a good response. A relatively new approach is to give a combination of erythromycin and niacinamide with dapsone for a steroid-sparing effect. In some patients the response to erythromycin plus niacinamide alone has been satisfactory; however, controlled therapeutic trials are lacking. It has been stated that high doses of steroids and immunosuppressive drugs are indicated for the cicatricial, as opposed to juvenile, bullous pemphigoid. From this review of the few cases of childhood cicatricial disease, it seems that the therapeutic approach might be the same for both forms.