A clinical and histopathological study of Darier’s disease

Neerja Puri

Abstract


Background Darier’s disease is an uncommon inherited skin disease transmitted in autosomal dominant pattern characterized by brownish keratotic papules particularly dense in the seborrheic areas of the body, palmar pits and nail dystrophy .The disease is often exacerbated by sun exposure, perspiration and heat.

 

Patients and methods

We selected 30 patients of Darier’s disease from the dermatology OPD for the study. The histopathological examination was performed in all the patients.

 

Results Yellow brown crusted greasy papules were the commonest clinical feature seen in 100% patients, cobblestoning of palate was seen in 86.6% patients, palmar pits in 83.3% patients and keratotic papules were seen in 73.3% patients. Regarding the nail changes alternating red and white bands were seen in all the patients (100%) and V shaped nicking at the free margins of nails was seen in 93.3% patients. The histopathological features noted were hyperkeratosis (100%), suprabasal acantholysis (90%), acantholytic cells (86.6%) and corps ronds and grains (83.3%).

 

Conclusion Cutaneous changes, clinical and histopathological, in Darier’s disease are similar to that described in the literature.

 


Keywords


Darier’s disease, keratosis follicularis, dyskeratosis

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References


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