(41) Darier's disease

Bingham, E.A.; Burrows, D.

doi:10.1111/j.1365-2133.1984.tb05311.x

Cited by 12 publications

(3 citation statements)

References 1 publication

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“…Nail changes are rare before the age of 5 years, but have been reported in younger children (47). Nail changes are rare before the age of 5 years, but have been reported in younger children (47).…”

Section: Darier Diseasementioning

confidence: 99%

“…This is an autosomal dominant genodermatosis characterized by a widespread keratotic rash, acral flat keratoses, palmar pits, and distinctive nail changes. Nail changes are rare before the age of 5 years, but have been reported in younger children (47). The nail changes may be the only sign of the disease in young patients, as they usually precede the appearance of the rash.…”

Section: Darier Diseasementioning

confidence: 99%

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Nail manifestations of some important genetic disorders in children

Rogers¹

2002

Dermatol Ther

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There are some genetic disorders in which nail changes are the major feature, such as the nail patella syndrome and pachyonychia congenita. Nail abnormalities are a constant feature of other disorders such as hidrotic ectodermal dysplasia, dyskeratosis congenita, some forms of epidermolysis bullosa, and ichthyosis follicularis with alopecia and photophobia. Nail changes often occur in mal de Meleda, Papillon–Lefèvre syndrome, and the keratitis‐ichthyosis‐deafness (KID) syndrome and are an occasional feature in some other ichthyoses and in incontinentia pigmenti. In Goltz syndrome, nail changes can be supportive of the diagnosis in a case in which the other features are minimal or subtle. In Darier disease, nail changes may be the first sign of the disorder and lead to the diagnosis being made. Nail changes may occur in some conditions such as Apert syndrome and Adams–Oliver syndrome as a result of abnormalities of the distal phalanges.

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Section: Darier Diseasementioning

confidence: 99%

Section: Darier Diseasementioning

confidence: 99%

Nail manifestations of some important genetic disorders in children

Rogers¹

2002

Dermatol Ther

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“…In a study by Burge and Wilkinson 1 they were found in 92% of 163 patients, and in 95% of a smaller number of patients in a similar study 2 . Several authors 2–5 have reported the occurrence of nail changes in the absence of other evidence of disease. The number of abnormal nails range from two or three to all nails in a minority of patients.…”

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confidence: 93%

An effective surgical treatment for nail thickening in Darier's disease

Baran

2005

Acad Dermatol Venereol

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Isolated Congenital Nail Dysplasia

Hamm

Karl²,

Bröcker³

2000

Arch Dermatol

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Developmental nail abnormalities are extremely heterogeneous, with hereditary isolated conditions being a small and rare subgroup. An unusual congenital nail dysplasia observed in a large South German kindred was characterized clinically to review the question of uniqueness.Design: Case series of affected family members.Setting: University department of dermatology and houses of patients. Patients:The history and clinical features in 22 affected family members (13 females and 9 males, aged 5 to 74 years) were recorded and documented by photographs. Nail biopsy samples were taken from 2 patients. Interventions: None.Results: The pedigree spanning 5 consecutive generations was best compatible with autosomal dominant in-heritance with complete penetrance. Nail alterations were mostly present since birth and soon reached an individually variable degree of severity. Affected persons showed longitudinal streaks and thinning of nail plates, mostly of all fingernails and toenails, with some accentuation of the thumbnail and big toenails, poorly developed lunulae, longitudinal angular ridges of individual nail plates occasionally starting proximally from a reddish prominence, platonychia and koilonychia of individual nails often overgrowing the lateral folds, and notches and fissures of the free margins. Histological abnormalities included a prominent granular layer of the nail matrix and epithelial strands and buds extending from the nail bed. There were no associated anomalies. Other nail dystrophies were excluded by differences in clinical and histological features. Conclusion:The nail abnormality observed in our family represents a new autosomal dominant disorder for which we propose the term isolated congenital nail dysplasia.

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(41) Darier's disease

Cited by 12 publications

References 1 publication

Nail manifestations of some important genetic disorders in children

Nail manifestations of some important genetic disorders in children

An effective surgical treatment for nail thickening in Darier's disease

Isolated Congenital Nail Dysplasia

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