HISTOPATHOLOGICAL STUDY AND CLINICAL PRESENTATION OF DARIER'S DISEASE

Darier's disease is infrequent, inherited autosomal dominant disease of the skin categorised by intense brownish keratotic papules in the palmar fossa, nail dystrophy and seborrheic parts of the body. The disease is usually aggravated by the exposure to sunlight, heat and sweating. Aim: The purpose of this analysis was to determine the clinical profile, epidemiology and Darier’s disease histopathological characteristics. Study Design: An Observational Study. Place and Duration: In the dermatology outpatient department of Pakistan Institute of Medical Sciences (PIMS), Islamabad for one year duration from March 2018 to March 2019. Methods: Thirty Darier's disease patients from the OPD of dermatology selected for the study. In all subjects; analysis of histopathology was done. Results: The usual clinical feature seen in almost every patients were Yellow brown crusted greasy papules and in 86.6% of patient’s cobble-stoning of palate was seen, in 73.3% of the cases have keratotic papules and palmar pits in 83.3% of the subjects. In all subjects, Alternative white and red bands were seen and V-shaped notches were found on the free edges of the nails in 95.3%. The observed histopathological characteristics were suprabasal acantholysis (90%), hyperkeratosis (100%), grains and corps ronds (83.3% and acantholytic cells (86.6%). Conclusion: Cutaneous, histopathological and clinical changes in Darier's disease are same to those defined in the prose. Keywords: Dyskeratosis, keratosis follicularis, Darier's disease