Bullous Rash in an Adolescent Girl
13-year-old girl presented with a history of 6 weeks of itching, partially crusted, perioral (Figure, A), and palmoplantar lesions. The perioral lesions had previously been treated as presumed impetigo contagiosa. Over the course of a few weeks, tense bullae developed on the inflamed skin (Figure, B and C) and the patient was referred to our hospital. Routine laboratory investigations, including full blood count, C-reactive protein, kidney and liver test, were normal except for mild eosinophilia (0.64 109/L). Although serum antibodies against hemidesmosomes (anti-BP180 and anti-BP230) were negative, a skin biopsy was performed and histopathology showed sub-epidermal blisters with eosinophilia. Direct immunofluorescence staining showed linear deposits of C3 (not shown) and IgG (Figure, D) at the epidermal basal membrane zone, and IgA deposits were absent. The linear C3 and IgG deposit and the eosinophil-rich infiltrate in the dermis and the subepidermal blister are typical for bullous pemphigoid. Local and systemic corticosteroids (2 mg/kg/d prednisone) were started and resulted in significant improvement within 2 weeks. Gradual tapered doses of
prednisone were subsequently given over 3 months and the patient remained well. Almost 2 years after the initial presentation, the patient returned with a small blister on the forehead, which was considered to be the first relapse and local and systemic corticosteroids (0.5 mg/kg/d) were restarted. Bullous pemphigoid usually occurs in adults older than 60 years and is extremely rare in children and adolescents. At onset, the disease may mimic more common skin conditions, including bullous impetigo contagiosa, urticarial eczema, or erythema multiforme. The differential diagnosis of subepidermal blisters in childhood also includes linear IgA bullous dermatosis, epidermolysis bullosa, and bullous systemic lupus erythematodes. As demonstrated in our patient, a skin biopsy is important for the suspected diagnosis of bullous pemphigoid. The histopathology findings and the facial and palmo-plantar localization of the lesions, together with absence of other systemic symptoms and a prompt response to treatment with corticosteroids made the diagnosis of a bullous pemphigoid most likely. Treatment of bullous pemphigoid consists of topical and systemic corticosteroids. Relapses are commonly reported in adults; however, information on incidence and risk of relapses in children and adolescents is limited. n Nadine M€ uller, MD General Pediatrics
Ulrich Heininger, MD General Pediatrics Infectious Diseases Unit University Children’s Hospital Basel
Andreas W. Arnold, MD Department of Dermatology University Hospital Basel
Nicole Ritz, MD, PhD Figure. A-C, Cutaneous findings at presentation, and D, direct immunofluorescence staining showing linear deposits of IgG.
J Pediatr 2015;166:1088. 0022-3476/$ - see front matter. Copyright ª 2015 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpeds.2014.12.065
General Pediatrics Infectious Diseases Unit University Children’s Hospital Basel Basel, Switzerland