Hailey Hailey disease (HHD) is a Beningn familial rare autosomal dominant disorder. The main cause of HHD is due to mutations in ATPC21 gene which encodes the human secretary pathway ca2+/mn2+ ATPase protein. Faulty calcium pump action leads to disorganized function of desmogleins which are calcium dependent adherence proteins. A 61 year old male patient admitted in our hospital in dermatology ward with chief complaints of itchy painful lesions skin lesions over groin and right axilla since 15 days. Report shows well defined vesicular, pustular, plagues over inner aspect of thighs, right axilla. Patient was treated with T. Prednisolone 20 mg, T. Cetirizine, T. Hydrochlorthiazide, T. BC, Vit C. Patient was discharged and advised to continue T. Prednisolone 20 mg OD, mupirocin cream. No reported cure is present but corticosteroids, topical antibiotics, antifungals useful in management of HHD in many cases.
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