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

• Published in: Pediatric dermatology Vol. 27; no. 2; pp. 119 - 124
• Main Authors: Kharfi, Monia, Khaled, Aida, Anane, Raja, Fazaa, Becima, Kamoun, Mohamed Ridha
• Format: Journal Article
• Language: English
• Published: Oxford, UK Blackwell Publishing Ltd 01-03-2010; Wiley
• Subjects: Adolescent; Adrenal Cortex Hormones - therapeutic use; Biological and medical sciences; Blindness - prevention & control; Bullous diseases of the skin; Child; Child, Preschool; Chronic Disease; Corneal Opacity - diagnosis; Corneal Opacity - drug therapy; Corneal Opacity - prevention & control; Cyclosporine - therapeutic use; Dapsone - therapeutic use; Dermatology; Entropion - complications; Entropion - pathology; Female; General aspects; Humans; Hypopigmentation - diagnosis; Hypopigmentation - drug therapy; Immunosuppressive Agents - therapeutic use; Infant; Male; Medical sciences; Pemphigoid, Benign Mucous Membrane - complications; Pemphigoid, Benign Mucous Membrane - diagnosis; Pemphigoid, Benign Mucous Membrane - drug therapy; Bullous dermatosis; Human; Immunopathology; Pediatrics; Skin disease; Scarring pemphigoid; Stomatology; Dermatology; Autoimmune disease; Conjunctiva disease; Case study; Eye disease; Oral cavity disease; Mucosa disease; Child; Bibliographic review
• ISSN: 0736-8046; 1525-1470
• DOI: 10.1111/j.1525-1470.2009.01079.x

:  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.