Darier disease is an autosomal dominant manner, with complete penetrance and variable expressivity. The condition is caused by mutations in the ATP2A2 gene, which encodes SERCA2. Family history is often overlooked due to subtle clinical presentations and presentation is common at around 20 years of age.

Case Report:This is the case of a 79-year-old elderly female who presented with a three year history of intensely pruritic erythematous hyperkeratotic papules on the face, trunk, and all extremities aggravated by heat and sweating. She reported of similar skin lesions seen on her father and brother, however they were undiagnosed and not treated. Multiple medications, including Prednisone, topical steroids, and antibiotics, provided temporary relief. Skin punch biopsy was done which revealed Acantholytic acanthoma consistent with Darier’s disease. CBC, SGPT, SGOT, Alkaline Phosphatase, BUN, Creatinine, Lipid Profile, FBS were requested and she was managed with topical corticosteroids, isotretinoin, Urea 10% lotion, and oral anti-histamines.

Darier disease is an autosomal dominant genodermatosis caused by mutations in the ATP2A2 gene, often presenting during puberty with chronic symptoms like hyperkeratotic lesions and nail abnormalities. Diagnosis relies on clinical and histopathologic correlation, aided by family history, though variable expressivity can complicate it. Coexisting infections must be evaluated due to potential morbidity. Treatment options include systemic retinoids, immunomodulators, and topical therapies like retinoids and calcineurin inhibitors. Personalized therapies have shown promise. Patient education on trigger avoidance and genetic counseling is crucial for managing recurrence risk, while life expectancy remains comparable to the general population.