ABSTRACT

Chronic Bullous Disease of Childhood (CBDC) is an acquired autoimmune blistering disorder of childhood, and it occurs without preferance to race and gender. CBDC may even temporarily improve with the brief course of antibiotics. Disease onset usually occurs at the age of 6 months to 10 years with a mean age of 4.5 years. However, disease onset has been reported as early as within 24 to 48 hours after birth and up to the age of 12 years. The ‘clusters of jewels’ pattern is typical of CBDC. In this pattern of distribution tense vesicles arise at the periphery of old lesions. Tense pruritic blisters of variable sizes, small clear-filled vesicles normal appearing skin or at periphery of an annular erythema, along with crusts, excoriations and erosions may be present in the patient. It is characterized by the deposition of linear band of IgA along the dermal-epidermal junction. CBDC is thought to be the result of a humoural response to a normal constituent of the epidermal basement membrane zone (BMZ). There may be genetic susceptibility to CBDC with an increased incidence of HLA-B8, HLA-DR3 and HLA-DQW2. However, the molecular basis of CBDC has not yet been clearly determined. This condition is seen in all ethnic groups seems to be more common in developing countries. Despite its good prognosis, most children are offered treatment to decrease the disease severity and shorten its duration. The most frequent medication is dapsone, starting at 0.5 to 1 mg/kg of body weight per day increasing up to2 mg/kg depending on the response. Keywords: Chronic Bullous Disease of Childhood, Linear IgA dermatosis
Publication date: 01/09/2023

https://ijbpas.com/pdf/2023/September/MS_IJBPAS_2023_7415.pdf

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https://doi.org/10.31032/IJBPAS/2023/12.9.7415