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Condition report · Dermatological

Darier and Hailey-Hailey Disease

Inherited skin conditions in which skin cells do not stick together properly, causing crusted papules or painful, blistering patches, often in skin folds. Flares are often triggered by heat, sweat, friction or sunlight.

A condition report looks for rare variants known to cause the conditions it covers.

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The 3 conditions we analyse

Darier disease

Autosomal dominant

An ATP2A2 disorder causing greasy, crusted papules on the chest, back and scalp, with nail changes.

What a diagnosis changes

Avoiding heat, sunlight and friction, and treating infections early, reduces flares; it has also been associated with mood disorders, which is useful for clinicians to know.

Genes analysed

Also known as

DAR; Darier White disease; Darier's disease; Keratosis Follicularis

MONDO:0007417OMIM 124200Orphanet 218MedGen 5956

Acrokeratosis verruciformis

Autosomal dominant

Flat, wart-like papules on the backs of the hands and feet, related to Darier disease.

Genes analysed

Also known as

acrokeratosis verruciformis of Hopf; AKV; AKV of Hopf; Hopf disease

MONDO:0007048OMIM 101900Orphanet 79151MedGen 75589

Hailey-Hailey disease

Autosomal dominant

An ATP2C1 disorder causing recurrent painful blisters and erosions in skin folds such as the armpits and groin.

What a diagnosis changes

Keeping skin folds cool and dry and treating infection early help control flares.

Genes analysed

Also known as

BCPM; benign chronic familial pemphigus of Hailey-Hailey; benign chronic pemphigus; benign familial pemphigus

MONDO:0008218OMIM 169600Orphanet 2841MedGen 43100

Other dermatological conditions

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