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Chronic Bullous Disease of Childhood

Chronic Bullous Disease of Childhood

Presentation
A 4-year-old, previously healthy girl presented to a tertiary care children’s hospital emergency department (ED) with large, tense bullae involving up to 40% of her body surface area. The patient’s parents described a 12-day history of itchy, papulovesicular lesions that had progressed into large blisters.

The rash began as pruritic pink bumps around the child’s umbilicus that spread over several days to her abdomen and extremities. She was evaluated by her pediatrician 4 days after the initial eruption; an empiric diagnosis of varicella was made although the patient’s temperature was normal and she had no constitutional symptoms. Calamine lotion was recommended to treat the pruritis.

Over the next 3 days, the lesions spread and enlarged, progressing from vesicles to bullae. The child was taken to the ED for re-evaluation. There, therapy with amoxicillin, mupirocin, and oral diphenhydramine was initiated for presumed bullous impetigo.

At home, over the next 5 days, the lesions continued to enlarge and spread, causing significant discomfort, and limiting ambulation. The parents brought the child back to the ED. She was evaluated and transferred for admission to the children’s hospital. The patient had no history of illness, medication use, or known allergy to medications and her immunizations were up-to-date. There was no personal or family history of similar skin lesions, abscesses, or cellulitis. The family had not traveled recently; they were not aware of exposure to ticks or other disease-carrying insects.

Figure 1
Figure 1

The patient’s vital signs were stable and temperature normal on admission. Her height and weight were at the 97th and 89th percentiles, respectively. The physical examination found an otherwise healthy child with a uniform bullous eruption covering most of her body. The presentation was worst on her legs (Figure 1), and her arms and trunk were also affected. There were a few smaller lesions on palms, soles, neck, and ears, but her face, scalp, and anogenital mucosa were spared.

Figure 1
Figure 2

The lesions were pink-to-red, discoid, sharply-circumscribed discreet and coalesced, 1- to 3-cm bullae and erosions. Many had central hemorrhagic crusting (Figure 2). Intact bullae were filled with straw-colored fluid. A few lesions had a raised border, suggestive of a “rosette” configuration (Figure 3).

Two 3-millimeter punch biopsy skin specimens were submitted on hospital day 1 for light and immunofluorescent microscopy. Histopathologic findings included a linear band of IgA at the basement membrane zone and a subepidermal split with a dense neutrophilic infiltrate. Additional investigation revealed a normal titer for glucose-6-phosphate dehydrogenase (G6PD).

Figure 1
Figure 3

The skin-care regimen during the patient’s 4-day hospitalization included a daily whirlpool with ½ cup added bleach followed by application of petroleum jelly and this was to be continued at home until her skin cleared. Oral dicloxacillin, 250 mg (12 mg/kg), was administered twice daily along with prednisolone, 30 mg (1.5 mg/kg) daily. After 1 month, there was significant improvement (Figure 4).  Topical dapsone 5% gel was recommended for application to affected areas but was not authorized by the patient’s insurance plan.

Figure 1
Figure 4

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