6.1.6 Dyskeratosis follicularis (Darier)
Grading & Level of Importance: C
Darier’s disease, Morbus Darier, Morbus Darier-White.
Incidence 1:30,000–100,000. Men are more severely affected than women. Start in early life or adolescence. Life expectancy normal.
Autosomal dominant inherited, acantholytic cornification disorder of epidermis, hair follicles and nails.
Aetiology & Pathogenesis
A mutation on chromosome 12 leads to disruption calcium ATPase. The formation of desmosomal tonofilaments and the differentiation of keratinocytes is disturbed, resulting in acantholysis. Trigger factors: UV light, sweating, virus infections (herpes simplex), caffeine, alcohol, stress.
Signs & Symptoms
Skin: small grayish-brown, itchy keratotic papules; occasionally confluent, weeping, foul-smelling. Punctiform interruptions of the papillary strip structure on the fingers and toes. Verrucous papules on the back of the hand are also known as Acrokeratosis verruciformis Hopf.
Oral mucosa and hard palate: leukoplakia-like changes with small papules.
Nails: dystrophy with formation of grooves and subungual hyperkeratosis.
Seborrheic regions: centrofacial, scalp, axillary region, central breast and back regions, inguinal region, anogenital.
Special form: Verrucous papules on the back of the hand are also referred to as Acrokeratosis verruciformis Hopf.
Laboratory & other workups
Dyskeratosis: defective differentiation of keratinocytes with corps ronds (eosinophilic cells in Str. spinosum) and corps grains (granular nuclear residues in St. Granulosum) Verrucous hyperkeratosis, especially in acrokeratosis verruciformis Hopf.
Acantholysis: suprabasal cleft formation with numerous eosinophilic granulocytes.
Chronic with worsening in the first years of illness.
Clinical and histological picture.
Seborrheic dermatitis, pemphigus benignus familiaris (Hailey-Hailey), transient acantholytic dermatosis (Grover's disease), pemphigus vegetans (intertriginous), acanthosis nigricans, verrucous epidermal nevi.
Prevention & Therapy
Prevention: Avoidance of direct exposure to the sun (UV; Sweating).
Topical: Retinoids, antiseptic baths, short-term weak glucocorticosteroids.
Systemic: Acitretin, in need of (superinfection) Antibiotica.
Surgical: exceptionally dermabrasion, laser.
Grover's disease (transitory acantholytic dermatosis) is an important differential diagnosis predominantly in older men with a tendency to relapse after transient phases of regression.
Be the first one to leave a comment!