Darier's disease

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keratosis

 [ker″ah-to´sis]
any horny growth, such as a wart or callosity.
actinic keratosis a sharply outlined wartlike or keratotic growth, which may develop into a cutaneous horn, and may become malignant; it usually occurs in the middle aged or elderly and is due to excessive exposure to the sun. Called also senile or solar keratosis. (See Atlas 3, Part F).
keratosis follicula´ris a slowly progressive autosomal dominant disorder of keratinization characterized by pinkish to tan or skin-colored papules on the seborrheic areas of the body that coalesce to form plaques, which may become crusted and secondarily infected; over time, the lesions may become darker and may fuse to form papillomatous and warty malodorous growths. Called also Darier's disease and Darier-White disease.
keratosis palma´ris et planta´ris palmoplantar keratoderma.
keratosis pharyn´gea horny projections from the tonsils and pharyngeal walls. Called also pharyngokeratosis.
keratosis pila´ris hyperkeratosis limited to the hair follicles.
keratosis puncta´ta a hereditary hyperkeratosis in which the lesions are localized in multiple points on the palms and soles.
seborrheic keratosis (keratosis seborrhe´ica) a benign, noninvasive tumor of epidermal origin, marked by numerous yellow or brown, sharply marginated, oval, raised lesions.
senile keratosis (solar keratosis) actinic keratosis.
Miller-Keane Encyclopedia and Dictionary of Medicine, Nursing, and Allied Health, Seventh Edition. © 2003 by Saunders, an imprint of Elsevier, Inc. All rights reserved.

Darier's disease

Keratosis follicularis, a rare autosomal dominant skin disease caused by mutations on Chromosome 12. It features warty, foul-smelling plaques formed of coalesced greasy plaques of seborrhoeic dermatitis with severe itching and sometimes pain. The disease is chronic and may worsen with age. Treatment is with oral retinoids. Prenatal diagnosis by DNA analysis of cells obtained by chorionic villus sampling is possible.
Collins Dictionary of Medicine © Robert M. Youngson 2004, 2005
References in periodicals archive ?
(3) By adulthood, the lesions either resolve or some lightly colored non-urticating macules remain; however, some patients will continue to have a positive Darier's sign.
Pemphigus Desmosomes Psoriasis Increased T-cell activity Darier's disease Gene encoding Autosomal intracellular dominant calcium pump (ATP2A2 gene) gene) Dyskeratosis Dyskerin (X-linked), X-linked, congenita TINF2 and sometimes TERT (autosomal autosomal dominant) dominant Disorders Clinical features Histopathological features Defect in [alpha]-helical rod pattern Epidermolysis Blistering of hands and Intraepithelial bullosa simplex feet which heal clefting without scarring.
Darier's disease occurs worldwide, with an estimated incidence of 4 per million per decade (3) and an estimated prevalence ranging from 1 in 30,000 (4) to 1 in 100,000.
Grover's disease, despite histological similarity to Darier's disease, does not share an abnormality in the ATP2A2 gene.
"There's nothing really known about Darier's," said Sonia, 31, of Penarth Road, Cardiff, who has suffered with the condition since she was 12.
Calluses visually identified as resulting from physical labor and hereditary keratoderma or Darier's disease, identified by visual inspection and family history, were excluded.
Darier's disease may look similar to cases of CARP without pigmentary change, but it often will have accompanying nail changes.
Clinical features appear within the first two years of life, in the form of multiple oval or round hyperpigmented macules, papules, or patches that are brown-red-yellow and 2-4 mm in diameter.11 They urticate within a few minutes of gentle rubbing (Darier's sign) causing localized itch, redness, and wealing, which subsides within an hour.
A positive Darier's sign on physical exam and lab testing for elevated histamine can aid in the diagnosis.
Under a microscope, Grover's disease has a characteristic appearance of acantholytic dyskeratosis (FIGURE 2); it can be similar in appearance to Darier's disease, Hailey-Hailey disease, or pemphigus.