Bullous Pemphigoid in Infancy: Case Report and Literature Review

Abstract

We report a 2-month-old girl who developed bullous pemphigoid on her hands and feet shortly after receiving her routine immunizations. Infantile bullous pemphigoid has a clinical presentation distinct from bullous pemphigoid seen in older children and should be included in the differential diagnosis of blisters involving the hands and feet. Our patient responded well to topical corticosteroid therapy. CASE REPORTA 3-month-old healthy white female infant presented to the dermatology clinic with a 1 month history of blisters which began on her feet. The blisters developed 3 days after she received her routine 2 month immunizations (oral polio, diphtheria-tetanus-pertussis (DPT), hemophilus type B, and hepatitis B). The blisters had been increasing in both number and size and had spread to the patient's hands. She had no prior history of skin disorders and no family history of atopy. Physical examination revealed a well-appearing infant girl who was noted to have multiple 0.25 to 1 cm tense bullae on erythematous bases (Fig. 1). The lesions were clustered most intensely on the ventral surfaces of the palms and soles, but also were present on the lateral digits and the dorsal hands and feet. Solitary lesions were also noted on her arms and legs. No lesions were noted on the patient's trunk, face, or perineum.Punch biopsies were performed of the lesional and perilesional skin from the dorsal right foot for both standard histologic analysis and for immunofluorescence studies. Laboratory evaluation including a complete blood count with differential was within normal limits, with the exception of a mild peripheral eosinophilia (8%). Hematoxylin and eosin staining clearly demonstrated a subepidermal bullous dermatosis with an intense mixed inflammatory infiltrate containing numerous eosinophils within the dermis.Periodic acid-Schiff stain for fungi was negative. Gram's stain was negative. Direct immunofluorescence of perilesional skin revealed linear staining of IgG along the basement membrane zone (Fig. 2). Staining of complement component C3 was also seen in the same distribution. Staining for IgA, IgM, and fibrinogen was negative, and no circulating antibasement membrane zone antibodies were detected in the patient's serum. The mother's serum was tested and found to be devoid of circulating antibasement membrane zone antibodies. Neither salt-split skin testing or immunoblot assay was performed. After a potent topical steroid (Cyclocort ointment) was applied, her blisters resolved completely at 6 weeks with only mild postinflammatory hyperpigmentation and no textural changes. One year after resolution of her lesions the patient has had no further outbreaks and has continued the recommended schedule for routine childhood immunizations.

Infantile bullous pemphigoid successfully treated with combined pulse corticosteroids and high-dose IVIG

“…Differential diagnosis should include both infectious (bullous impetigo, scabies, herpes simplex, Hand, Foot, and Mouth disease) and noninfectious entities (linear IgA dermatosis, acropustulosis of infancy, and erythema multiforme) (Brad, Deng, Flynn, & Suarez, ).…”