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.

Darier's disease and acrokeratosis verruciformis are inherited with the same pattern and they share similar clinical properties. We present a patient with both Darier's disease and acrokeratosis verruciformis. The patient had had brownish and skin-colored papules, initially on her face and neck, since the age of 25. Similar lesions had gradually spread to various parts of her body and lastly the dorsal aspect of her feet. On dermatological examination, brownish and skin-colored, 2 to 5 mm, keratotic papules were observed on her face, neck, both axillae, inframammary regions, and dorsa of the hands and feet. The nail examination revealed "V" shaped notches and longitudinal ridges on the fingers of both hands. The biopsy taken from the neck showed typical findings for Darier's disease. The biopsy from the dorsa of the left foot revealed the distinctive structures of acrokeratosis verruciformis. In this article the clinical and histopathological findings of these diseases are reviewed based on our patient.
Acrodermatitis/*pathology ; Adult ; Biopsy ; Female ; Humans ; Keratosis/*pathology ; Keratosis Follicularis/*pathology ; Skin/pathology

No abstract available.
Darier Disease*

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Darier Disease*

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Darier Disease*

Darier's disease is a well-known genodermatosis characterized by recurrent waxy, hyperkeratotic papules usually occurring over the seborrheic area. The major histopathological changes are characteristic acantholysis and dyskeratosis which are diagnostic of the disease with typical clinical features. There are less common variants including the hypertrophic, vesicobullous and linear type. However, comedonal lesions are very rare. We report an unusual case of Darier's disease, which showed prominent comedonal papules and plaques over the face, scalp and upper trunk with the typical findings of Darier's disease.
Acantholysis ; Darier Disease* ; Scalp

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Darier Disease* ; Lichens* ; Neurodermatitis*

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Darier Disease* ; Minocycline* ; Tetracycline

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Darier Disease* ; Pityriasis*

Darier's disease is a genetic disorder of keratinization with autosomal dominant inheritance. Its appearance is usually in the form of greasy, crusted, keratotic yellow-brown papules and plaques found particularly on seborrheic areas of the body. However, there are some clinical variants showing atypical skin lesions. Here we report an unusual case of Darier's disease, which mainly showed prominent comedonal papules over the face.
Darier Disease ; Keratins ; Skin ; Wills