Pemphigoid in Children

Abstract

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.

Misdiagnosis, Treatments, and Outcomes in the Immunobullous Diseases in Children

“…Some children respond to dapsone or sulfapyridine, but we did not use these agents in our patients (15,19). Oral er>'thromycin and niacinamide have also been reported to be helpful, but there is very limited experience with this treatment, and we did not attempt it.…”

Bullous Pemphigoid and Epidermolysis Bullosa Acquisita: Presentation, Prognosis, and Immunopathology in 11 Children

“…Our findings agree with these observations. It has been suggested that almost all juvenile BP patients show disseminated disease (27), and all our patients except the child with localized vulval BP developed generalized blistering. Localized vulval BP has been suggested to be a distinct variant of childhood BP which responds well to topical corticosteroids, as in this patient, and does not lead to scarring (28).…”

Childhood bullous pemphigoid: a case report and 10‐year follow up

“…However, they differ in some aspects: mucous membrane involvement has been reported more frequently in children. Oranje 4 reported mucosal findings in almost 50% of the patients studied, while Nemeth et al 2 . found mucous membrane involvement in 13 out of 18 (72%) children.…”

“…It usually clears the lesions rapidly, preventing the formation of new ones, as observed in our patient. Addition of dapsone or sulfapyridine as adjunctive therapy to steroids may be useful 4 . Oral erythromycin alone has also been used with good responses 16 .…”