Darier disease in Adult
Darier disease presents in early adolescence to mid-adult life, with peak onset in the second decade of life. The disease manifests with greasy, hyperkeratotic papules in a seborrheic distribution, along with palmoplantar pits, acrokeratosis verruciformis-like papules, and characteristic nail findings (candy-cane nails). Leukodermic macules are a rarely reported finding. These small macules occur most frequently on the ventral aspect of limbs and trunk. Their onset is prior to puberty. They have been recognized and reported most frequently in individuals with darker skin phototypes.
In additional to cutaneous findings, 15%-50% of patients present with oral involvement, including cobblestoning of the oral mucosa, gingival hypertrophy, and sialadenitis. Esophageal involvement with erosions has been described. The severity of oral disease may parallel that of the cutaneous disease.
After onset, the disease is lifelong. It may be accentuated or only prominent in the spring and summer, when exposures to heat, perspiration, and ultraviolet (UV) light are increased. Other exacerbating conditions / factors may include trauma, menstruation, and certain drugs (eg, lithium, oral corticosteroids).
The lesions of Darier disease may be pruritic, painful, or malodorous. Along with the appearance, these symptoms may lead to significant psychosocial distress. Patients are at an increased risk of bacterial or viral skin infections.
Linear, segmental, or unilateral presentations are uncommon variants of the disease caused by mutations in the same gene.
For more information, see OMIM.
Q82.8 – Other specified congenital malformations of skin
48611009 – Darier's disease
- Confluent and reticulated papillomatosis
- Seborrheic dermatitis
- Grover disease (transient acantholytic disease) has a similar biopsy but has a different age of onset (eg, fourth or fifth decade).
- Follicular atopic dermatitis (eczema) has a similar distribution, as do follicular occlusion syndromes, perforating disorders, granuloma annulare, and some tumors of the appendages.
- Hailey-Hailey disease is more erosive and is more common in intertriginous areas.
- Pemphigus foliaceus
- Acanthosis nigricans
- Drug-induced gingival hypertrophy
- Denture-induced hyperplasia
- Oral squamous cell carcinoma
- Oral leukemic infiltration
- Granulomatosis with polyangiitis
- Kaposi sarcoma
- Acrokeratosis verruciformis of Hopf – An isolated finding in the absence of Darier disease; conditions are allelic.
- Flat warts
- Epidermodysplasia verruciformis
Last Updated: 04/09/2018