Background: A change in a gene called Ca2+-ATPase type 2, which controls the movement of calcium in the endoplasmic reticulum, causes Darier disease. Darier disease is a rare skin condition that affects how cells stick together. Also, this illness gets worse with heat and exposure to the sun, sweating, and rubbing, and it is characterized by many bumpy, greasy spots with an oily skin distribution. Sometimes, it can also harm the mucosa (the lining inside our body) and cause our nails to grow strangely. There is a special type of Darier disease called unilateral segmental Darier disease. It causes red, scaly bumps to appear on one side of the body and they are not related to any other illnesses. Objective: To show an example of a person with Darier's disease. Also, to talks about the main characteristics and the treatments that are available. Subject and methods: A case report. Results: 51 years old male patient presented with multiple red to brown hyperkeratotic papules coalesce into plaque and led to thickened skin affecting the face, trunk, axilla and dorsa of both upper and lower extremities. Histopathology, there was hyperkeratosis, acanthosis, and papillomatosis. Supra basal acantholytic blister containing acantholytic cells, some of them showed Evidence of dyskeratosis. Corps ronds are present in the uppermost layer, especially the granular layer. Grains are present within the horney layer. No signs of malignancy or granuloma. Conclusion: Because there are no confirmed ways to cure DD, it is hard to treat. There are many different ways to treat this problem, like using special creams or medications, having surgery, or using lasers. But these treatments don't work very well and only help a little bit. It is important to ensure a multidisciplinary approach in the management of patients with Darier's disease because systemic retinoids have shown to be more effective in achieving some reduction of symptoms in 90% of patients. Isotretinoin was used in our patient with a good outcome because it reduces hyperkeratosis, smoothens papules, and reduces odour.
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