A 6-week-old Girl with Recurrent Bullous Lesions and Erosions

Alexander K.C. Leung; Benjamin Barankin; Kin Fon Leong

doi:10.1542/pir.2017-0265

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Alexander K.C. Leung, MD*
Benjamin Barankin, MD^†
Kin Fon Leong, MD^‡

^*Department of Pediatrics, University of Calgary, Calgary, Alberta, Canada
^†Toronto Dermatology Centre, Toronto, Ontario, Canada
^‡Pediatric Institute, Kuala Lumpur General Hospital, Kuala Lumpur, Malaysia

AUTHOR DISCLOSURE
Drs Leung and Leong have disclosed no financial relationships relevant to this case. Dr Barankin has disclosed that he participates in continuing medical education speaking events for AbbVie, Actelion, Amgen, Celgene, Galderma, Janssen, La Roche Posay, Leo Pharma, Novartis, Pierre Fabre, and Valeant and that he receives grant funding from Boehringer Ingelheim, Eli Lilly, Galderma, Leo Pharma, Pfizer, Celgene, and Janssen for clinical research trials. This commentary does not contain a discussion of an unapproved/investigative use of a commercial product/device.

Presentation

A 6-week-old girl presents with recurrent generalized bullae and erosions since 1 week of age. The parents relate that the infant has tense bullae form after trivial physical trauma. The infant was born to a 24-year-old gravida 2, para 1 mother at 38 weeks’ gestation after an uncomplicated pregnancy and a normal vaginal delivery. Apgar scores were 7 and 9 at 1 and 5 minutes, respectively. Birthweight was 2,800 g, and length was 50.5 cm. Her parents are nonconsanguineous and healthy. There is no family history of atopy or similar skin diseases. The infant is exclusively breastfed. She has a history of intermittent flushing episodes.

Physical examination reveals multiple tense vesicles, bullae, erosions, and crusted lesions at different stages of their evolution over the trunk, extremities, face, and scalp (Figs 1–3). The skin is diffusely thickened, with a leather grain feel and varying degrees of erythroderma. Gentle rubbing or stroking of the skin results in a wheal with a surrounding erythematous flare (Darier sign). The rest of the physical examination findings are normal. In particular, there is no hepatosplenomegaly or palpable lymphadenopathy.

Figure 1.

Multiple bullae and erosions with diffuse thickening and erythema of the skin on the trunk and face.

Figure 2.

Bullous lesions and erosions with diffuse thickening and erythema of the skin on the right groin and …