Early Onset Childhood Cicatricial Pemphigoid: A Case Report and Review of the Literature

Abstract

Cicatricial pemphigoid is an extremely rare condition in children, since only 17 cases have been reported in the English literature, so far. We describe a new case in a 20-month-old boy, who is to our knowledge the youngest patient reported yet. The disorder had begun 10 months before he was referred to our department by mucosal crusted erosions of the oral and nasal cavities and conjunctivae. Cutaneous examination showed buccal erosions with limited mouth opening, entropion of the lower eyelids, trichiasis, cicatricial cornea, synechia of the nasal cavities and hypopigmented lesions of the abdomen. There were no anal or genital lesions. Cicatricial pemphigoid was confirmed by positive direct and indirect immunofluorescence on mucous biopsy. Systemic corticosteroids (2 mg/kg/day), maintained for 12 months, had led to complete healing of lesions. But due to cicatrization, synechia of the nasal cavities and corneal opacities, leading to a dramatic visual loss, have occurred. Dapsone 25 mg/day and topical ocular cyclosporine are now maintained to avoid relapse. Our review of the literature of all cases of CP showed that ocular and to a less degree, vulvar lesions are the most severe ones, due to the serious complications with scar formation.

Mucous Membrane Pemphigoid

“…Approximately 20 cases of childhood onset MMP have been reported, among whom the youngest one was 10 months old. 21–23 There is no known racial or geographic predilection.…”

A case of antiepiligrin cicatricial pemphigoid with extensive cutaneous erosions mimicking pemphigus vulgaris

“…It is the disease of elderly (fifth–sixth decade) but can present in younger age group 2. Primarily affecting mucous membranes, the disease can involve skin in 25%–30% cases.…”

Vulvar Sikatrisyel Pemfigoid: Bir Çocuk Olgu

“…Erişkin büllöz pemfigoid olgularının %9'unda vulvar tutulum saptanırken bu oranın çocukluk çağı büllöz pemfigoidinde %50 gibi oldukça yüksek bir oranda olduğu belirtilmektedir. Literatürde bildirilen yaklaşık yirmi çocukluk çağı sikatrisyel pemfigoid olgusundan sadece üçünde olgumuzda saptandığı gibi izole vulvar tutulum olduğu belirtilmektedir 6,7 . Vulvar sikatrisyel pemfigoidde oküler tutulum, görme kaybı veya körlüğe yol açabilmesi nedeni ile mutlaka araştırılmalıdır.…”