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June 2, 2008

Picture of the Month—Diagnosis

Author Affiliations


Arch Pediatr Adolesc Med. 2008;162(6):582. doi:10.1001/archpedi.162.6.582

Denouement and Discussion: Chronic Bullous Dermatosis of Childhood

The physical finding of blisters arranged in the cluster of jewels pattern is highly suggestive of chronic bullous dermatosis of childhood (CBDC). With this presumed diagnosis, the patient's home medications were withheld and an empirical regimen of dapsone, 50 mg/d, and oral prednisone, 40 mg/d, was initiated. The patient was admitted for 48 hours for observation and wound care to denuded areas. Histopathologic examination of an intact vesicle demonstrated subepidermal cleft formation with a superficial perivascular, predominantly neutrophilic, infiltrate. Direct immunofluorescence performed on perilesional skin demonstrated linear staining along the basement membrane zone with IgA and C3, and minimally with IgG. These histopathologic findings confirmed the diagnosis of linear IgA bullous dermatosis/CBDC.

Chronic bullous dermatosis of childhood is an acquired autoimmune bullous disease of young children. It is the most common autoimmune bullous disease encountered within the pediatric population.1,2The age at onset is reported to be between 6 months and 10 years, with a mean age at onset of 4½ years.3No racial or sexual predilection has been reported.4The lesions of CBDC are typically most prominent on the abdomen and perineum but may also involve the trunk, extremities, face, and mucous membranes.5These lesions are frequently pruritic and consist of multiple large, tense bullae filled with clear or hemorrhagic fluid. The cluster of jewels or string of pearls sign is considered characteristic and refers to the annular arrangement of new vesicles or bullae around a central crust or erythematous plaque.

The differential diagnosis of childhood bullous diseases is extensive. Autoimmune blistering diseases include pemphigus vulgaris, childhood bullous pemphigoid, cicatricial pemphigoid, dermatitis herpetiformis (DH), and CBDC or linear IgA bullous disease. The hypersensitivity phenomenon of bullous papular urticaria can occasionally present with frank blisters and should be included in the differential diagnosis. Pemphigus vulgaris usually starts in the oral mucosa and is followed by blistering of the skin, which is often painful. The blisters are flaccid with a positive Nikolsky sign. Childhood bullous pemphigoid is characterized by large, tense bullae similar to those seen in the adult form of the disease. Cicatricial pemphigoid presents with severe erosive lesions of the mucous membranes that result in severe scarring. The lesions of DH are intensely pruritic and chronic. Papulovesicular lesions and urticarial wheals on the extensor surfaces in a herpetiform or grouped distribution are characteristic of DH. It is uncommon to see intact vesicles or bullae in DH because the primary lesions are often ruptured by excoriation owing to intense pruritus.6Despite the differences we have listed, skin biopsy of an early, intact bulla for pathologic examination and of perilesional skin for immunofluorescence is the most important tool for accurate diagnosis.7

Although several anecdotal therapeutic options have been reported, no controlled or comparative studies have been performed to our knowledge. Most reports support the use of dapsone or sulfapyridine, with or without corticosteroids, as first-line therapy.8However, dicloxacillin sodium may be an option for patients with CBDC that is refractory to dapsone or sulfapyridine, as well as for those patients who are not candidates for standard therapy.8If the onset of the bullous disease coincides with the use of a new prescription drug, consideration should be given to stopping the use of that medication. The list of agents implicated in CBDC continues to grow. Of all reported causative drugs, vancomycin hydrochloride is the best documented in the literature.9Other potential documented triggers include ampicillin, amiodarone hydrochloride, captopril, cyclosporine, lithium, and multiple antiepileptic agents.10

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Correspondence:Michael Sheehan, MD, Department of Dermatology, Indiana University School of Medicine, 550 N University Blvd, Ste 3240, Indianapolis, IN 46202.

Accepted for Publication:June 19, 2007.

Author Contributions:Study concept and design: Sheehan, Huddleston, and Mousdicas. Acquisition of data: Sheehan and Huddleston. Analysis and interpretation of data: Huddleston. Drafting of the manuscript: Sheehan. Critical revision of the manuscript for important intellectual content: Sheehan, Huddleston, and Mousdicas. Study supervision: Huddleston and Mousdicas.

Financial Disclosure:None reported.

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