Darier-White Disease: a Review of the Clinical Features in 163 Patients

Overview

Journal J Am Acad Dermatol

Specialty Dermatology

Date 1992 Jul 1

PMID 1619075

Citations 66

Authors

S M Burge

J D Wilkinson

Affiliations

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Abstract

Background: Darier's disease is a rare, dominantly inherited genodermatosis; there have not been any large clinical studies of patients with this disease.

Objective: Our purpose was to document the clinical features in a large group of patients with Darier's disease.

Methods: Data were collected from 163 affected persons.

Results: The onset usually occurred between the ages of 6 and 20 years. The disease has a predilection for the skin in seborrheic areas; 96% had acral signs; 6% had hypertrophic flexural involvement; and 13% had oral mucosal lesions. There was no remission. Topical therapy sometimes provided relief of symptoms but had no effect on the progress of the disease. Oral retinoids were effective, but long-term therapy was tolerated poorly. Most patients did not have other medical problems.

Conclusion: Although Darier's disease is a chronic and unremitting burden, most patients manage to lead a relatively normal life.

Citing Articles

Severe Darier's Disease by Mitochondrial DNA Insertion Causing Nonsense Mutations: In Silico Prediction of a Pathophysiological Mechanism to a Novel Mutation.

Shintani H, Ikuno Y, Kokubu H, Fujimoto S, Yamaguchi A, Takahashi T Exp Dermatol. 2025; 34(3):e70070.

PMID: 40062416 PMC: 11891979. DOI: 10.1111/exd.70070.

Dermoscopy of Onychopapilloma: A Benign Mimic of Subungual Malignancy.

Woodie B, Subhadarshani S Cureus. 2025; 17(1):e78103.

PMID: 40018485 PMC: 11865681. DOI: 10.7759/cureus.78103.

Patients with Darier disease have an increased risk of keratinocyte carcinoma: a Swedish registry-based nationwide cohort study.

Inci R, Gillstedt M, Kallionpaa R, Peltonen S, Polesie S Orphanet J Rare Dis. 2024; 19(1):463.

PMID: 39681873 PMC: 11648299. DOI: 10.1186/s13023-024-03497-z.

The use of photodynamic therapy in the management of darier disease and Hailey-Hailey disease: a systematic review.

Ueltschi O, Casola M, Rose L, Dulmage B, Kaffenberger J Arch Dermatol Res. 2024; 316(8):610.

PMID: 39259372 DOI: 10.1007/s00403-024-03356-8.

Dantrolene corrects cellular disease features of Darier disease and may be a novel treatment.

Hunt M, Wang N, Pupinyo N, Curman P, Torres M, Jebril W EMBO Mol Med. 2024; 16(9):1986-2001.

PMID: 39060641 PMC: 11392931. DOI: 10.1038/s44321-024-00104-3.