Disease Information for Keratosis follicularis (Darier Disease)

Ads
Clinical Manifestations
Signs & Symptoms
Brown nails/discolored
Cutaneous manifestations
Dandruff
Face skin lesions
Follicular hyperkeratosis
Hypertrophy nails/Onychogryposis
Koebner phenomenon/post-mechanical stimulation/rash lesion
Non-pruritic rash
Papular Rash
Papule
Papulosquamous Lesion
Papulosquamous rash
Plaques/cutaneous
Rash with well demarcated lesions
Rash/Characteristic pattern/distribution
Rash/Keratotic
Scalp rash
Symmetrical rash
Thickened nails/Onychauxis
Thorax skin lesions
Truncal distribution/rash
Oral mucosa white lesions
Behavior problems
Destructive Behavior
Clinical Presentation & Variations
Presentation/ Papular Rash Face Upper Arms
Demographics & Risk Factors
Family History
Family history/Skin rashes chronic
Laboratory Tests
Abnormal Lab Findings (Non Measured)
DNATest specific/genetics laboratory/abnormality (Lab)
Diagnostic Test Results
Pathology
BX/Skin biopsy/Hyperkeratosis
Associated Diseases & Rule outs
Rule Outs
Acne vulgaris (acne)
Basal cell carcinoma
Folliculitis
Warts/verruca vulgaris
Associated Disease & Complications
Neurobehavior Problems
Disease Mechanism & Classification
Class
CLASS/Dermatologic/Subcutaneous (category)
CLASS/Skin disorder (ex)
Process
PROCESS/Autosomal dominant hereditary disease (ex).
PROCESS/Eponymic (category)
PROCESS/Hereditofamilial (category)
PROCESS/INCIDENCE/Rare disease (ex)
PROCESS/Reference organ/system (category)
Synonyms
Synonym
DAR, DARIER DIS, DARIER DISEASE, Darier White disease, DARIERS DIS, Dariers Disease, Darier's Disease, DD, Disease Darier, Disease Darier's, Dyskeratosis follicularis, Follicular keratosis, follicularis keratosis, Heredit follicular keratosis, Hereditary follicular keratosis, Keratosis follicular, KERATOSIS FOLLICULARIS, Keratosis follicularis (disorder), Psorosperm follicularis veget, Psorospermosis, PSOROSPERMOSIS FOLLICULARIS, Psorospermosis follicularis vegetans, WHITE DARIER DISEASE, Synonym/Darier Disease (Keratosis Follicularis), Synonym/Darier-White Disease, Synonym/Dyskeratosis Follicularis Vegetans, Synonym/Psorospermosis Follicularis (Darier), Synonym/White-Darier disease
Definition

A slowly progressive autosomal dominant disorder of keratinization characterized by pinkish-to-tan papules that coalesce to form plaques; These lesions become darker over time and commonly fuse, forming papillomatous and warty malodorous growths.

Keratosis Follicularis; Darier Disease; Darier-White Disease; Dyskeratosis Follicularis Vegetans; Psorospermosis Follicularis; White-Darier Disease;

Keratosis follicularis (KF) is a rare, gradually progressive, hereditary skin disorder; It is characterized by wart-like spots and/or broader, rough areas (papules, plaques) on the scalp, forehead, face, neck, area behind the ears, and the central torso, back and front; In addition, thickening and discoloration of the nails in association with the skin changes is a reliable sign; Some mild neurological and psychiatric symptoms are sometimes associated with KF:

(Edit)

External Links Related to Keratosis follicularis (Darier Disease)
Google
Wikipedia
Merck
Images
PubMed (National Library of Medicine)
NGC (National Guideline Clearinghouse)
Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
Ads