A male patient comes with recurrent vesicles and erosions on the groin and axilla ,what is the diagnosis ?
Correct Answer: Hailey-Hailey Disease
Description: Hailey-hailey disease/chronic benign familial pemphigus - -recurrent flaccid vesicles develop over inter-triginous area (flexors) - groin, axilla - Occurs due to defect in ATP2C I gene - ATP2C I gene codes for SERCA (Sarco Endoplasmic reticulum Ca2+ ATPase). Therefore, in this disease Ca2+ ATPase is defective. - Ca2+ required for attachment of two desmosomes which doesn't happen in this disease resulting in separation of keratinocytes i.e. acantholysis. - Autosomal Dominant C/F: - recurrent flaccid vesicles develop over inter-triginous area (flexors) - groin, axilla - D/t friction at flexor region - rupture of these lesions occurs leading to erosion.- Nail: longitudinal white bands. Histology: Dilapidated Brick wall appearance Pemphigus - presents with blisters in mouth & genitals Tinea cruris/dhobi's itch - fungal infection causing infection in groin region caused by Trichophyton. Inverse psoriasis - presents with red & inflamed lesion in the skin folds of the body, therefore aka interiginous psoriasis.
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