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

Skin biopsy.


  • 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.

Differential diagnosis

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.

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