Abstract
Hailey-Hailey disease (HHD) is a rare autosomal dominant skin condition discovered in 1939 by Hailey brothers. Prevalence is found to be around 1 in 50,000 and is relatively uncommon in India. First onset of disease occurs between 20 to 40 years of age, usually presented in the 3rd and 4th decades of life. Here, we report a case of a 50-year-old female who presented with clinical features of the HHD and showed greater response to the treatment.
Highlights
Hailey-Hailey disease (HHD) called benign familial pemphigus is a rare autosomal dominant disorder that affects the adhesion of epidermal keratinocytes which recurs with remitting and relapsing episodes
It is caused by mutations in the ATP2C1 gene which results in altered cellular connections within desmosomes and adherens junctions of epidermis [1]
HHD is primarily localized in intertriginous areas usually characterized by painful blistering, erosions, maceration, and frequent secondary infection in flexural areas such as neck, axillae, groins, and perineum [1,2]
Summary
Hailey-Hailey disease (HHD) called benign familial pemphigus is a rare autosomal dominant disorder that affects the adhesion of epidermal keratinocytes which recurs with remitting and relapsing episodes. It is caused by mutations in the ATP2C1 gene which results in altered cellular connections within desmosomes and adherens junctions of epidermis [1]. A 50-year-old married female with h/o T2DM and microadenoma was presented to the medical OP department with itchy, erythematous papules mainly over the flexural areas on and off since 20 years (Fig. 1). The patient developed increased blood sugar levels associated with the use of steroids She developed wide spread staphylococcal skin infection with rapid generalization of the lesions. New lesion developed on foot and managed with prednisolone 40 mg OD and T clindamycin 300 mg BD daily
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