(Last Updated On: October 30, 2018)

Authors: Maria Rosa Noliza F. Encarnacion, MD, Milali T. Torres, MD FPDS

Abstract

Introduction: CBDC is a rare autoimmune bullous disorder presenting with tense bullae with some lesions showing ‘cluster of jewel” or “string of pearls” appearance. Histopathology shows subepidermal bulla with predominant neutrophilic infiltrate. The most important feature is the presence of continuous linear IgA deposits at the basement membrane zone (BMZ) against heterogenous BMZ antigens. CBDC responds to Dapsone.

Case Summary: AV, a 2 year old female, sought consult due to multiple vesicles which started 6 months PTC, gradually increasing in size and number forming bullae and erosive plaques, which would spontaneously resolve, associated with pruritus. On physical examination, there are few well defined round tense vesicles and bullae, on top of erythematous to hyperpigmented plaques, on the trunk, upper and lower extremities forming annular and polycyclic patter giving a “cluster of jewels” morphology. There are no oral lesions, no genital lesions, no lesions on palms and soles. Nikolsky sign and Asboe Hansen sign were negative. CBC, urinalysis, chest x ray and G6PD revealed normal results. Skin punch biopsy on the upper back revealed findings consistent with CBDC. The patient was given topical medication such as Clobetasol ointment + Petroleum jelly to be applied 2x/day and Mupirocin ointment BID. Systemic medications include Cetirizine 5mg/5ml 3mL once a day, Prednisone 10mg/ml BID for 5 days then 1mL OD evnetually shifted to Dapsone 50mg/tab ½ tablet once a day which afforded gradual resolution of symptoms.

Conclusion: CBDC carries a good prognosis. Spontaneous remissions, often within 2 years are frequent. Occasionally, the disease persists well into puberty but often is less severe than the initial eruption.

 

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Keywords

cluster of jewels, chronic bullous disease of childhood, dapsone

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