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Autosomal dominant ichthyosis vulgaris

MedGen UID:
609440
Concept ID:
C0432300
Congenital Abnormality
Synonyms: ADI - Autosomal dominant ichthyosis vulgaris; autosomal dominant ichthyosis vulgaris; dominant congenital ichthyosiform erythroderma; dominant ichthyosis vulgaris; ichthyosis simplex; ichthyosis vulgaris; ichthyosis vulgaris, autosomal dominant; NON RARE IN EUROPE: Autosomal dominant ichthyosis vulgaris
SNOMED CT: Autosomal dominant ichthyosis vulgaris (254158000); ADI - Autosomal dominant ichthyosis vulgaris (254158000)
 
Monarch Initiative: MONDO:0007810
Orphanet: ORPHA462

Definition

Ichthyosis vulgaris is a common skin disorder passed down through families that leads to dry, scaly skin. It often begins in early childhood. Treatment may include heavy duty moisturizers which contain chemicals that help the skin to shed normally, including lactic acid, salicylic acid, and urea. Ichthyosis vulgaris can be a nuisance, but it rarely affects overall health. The condition usually disappears during adulthood, but may return in later years. This condition is inherited in an autosomal dominant pattern. [from MONDO]

Professional guidelines

PubMed

Hofmann B, Stege H, Ruzicka T, Lehmann P
Br J Dermatol 1999 Oct;141(4):642-6. doi: 10.1046/j.1365-2133.1999.03101.x. PMID: 10583110
Lucker GP, Steijen PM, Suykerbuyk EJ, Kragballe K, Brandrup F, van de Kerkhof PC
Acta Derm Venereol 1996 Mar;76(2):97-101. doi: 10.2340/000155557697101. PMID: 8740259
Anton-Lamprecht I
J Invest Dermatol 1983 Jul;81(1 Suppl):149s-56s. doi: 10.1111/1523-1747.ep12540961. PMID: 6345689

Recent clinical studies

Etiology

Hofmann B, Stege H, Ruzicka T, Lehmann P
Br J Dermatol 1999 Oct;141(4):642-6. doi: 10.1046/j.1365-2133.1999.03101.x. PMID: 10583110
Lavrijsen AP, Oestmann E, Hermans J, Boddé HE, Vermeer BJ, Ponec M
Br J Dermatol 1993 Nov;129(5):547-53. doi: 10.1111/j.1365-2133.1993.tb00482.x. PMID: 8251350
Piccirillo A, Auricchio L, Fabbrocini G, Parenti G, Ballabio A, Delfino M
Br J Dermatol 1988 Aug;119(2):185-8. doi: 10.1111/j.1365-2133.1988.tb03200.x. PMID: 3166940
Happle R, van de Kerkhof PC, Traupe H
Dermatologica 1987;175 Suppl 1:107-24. doi: 10.1159/000248867. PMID: 2961628
Anton-Lamprecht I
J Invest Dermatol 1983 Jul;81(1 Suppl):149s-56s. doi: 10.1111/1523-1747.ep12540961. PMID: 6345689

Diagnosis

Hofmann B, Stege H, Ruzicka T, Lehmann P
Br J Dermatol 1999 Oct;141(4):642-6. doi: 10.1046/j.1365-2133.1999.03101.x. PMID: 10583110
Fartasch M, Haneke E, Anton-Lamprecht I
Arch Dermatol Res 1987;279(4):270-2. doi: 10.1007/BF00417327. PMID: 2445305
Traupe H, Kolde G, Happle R
Clin Genet 1984 Nov;26(5):457-61. doi: 10.1111/j.1399-0004.1984.tb01089.x. PMID: 6499258
Anton-Lamprecht I
J Invest Dermatol 1983 Jul;81(1 Suppl):149s-56s. doi: 10.1111/1523-1747.ep12540961. PMID: 6345689
Anton-Lamprecht I, Hofbauer M
Humangenetik 1972;15(3):261-4. doi: 10.1007/BF00702363. PMID: 4117150

Therapy

Kumar V, Kalra S, Mutreja D, Arya A
Paediatr Int Child Health 2012 May;32(2):119-20. doi: 10.1179/2046905511Y.0000000013. PMID: 22595223
Hofmann B, Stege H, Ruzicka T, Lehmann P
Br J Dermatol 1999 Oct;141(4):642-6. doi: 10.1046/j.1365-2133.1999.03101.x. PMID: 10583110
Lucker GP, Steijen PM, Suykerbuyk EJ, Kragballe K, Brandrup F, van de Kerkhof PC
Acta Derm Venereol 1996 Mar;76(2):97-101. doi: 10.2340/000155557697101. PMID: 8740259
Steijlen PM, Reifenschweiler DO, Ramaekers FC, van Muijen GN, Happle R, Link M, Ruiter DJ, van de Kerkhof PC
Arch Dermatol Res 1993;285(4):221-6. doi: 10.1007/BF00372013. PMID: 7688204
Happle R, van de Kerkhof PC, Traupe H
Dermatologica 1987;175 Suppl 1:107-24. doi: 10.1159/000248867. PMID: 2961628

Prognosis

Hofmann B, Stege H, Ruzicka T, Lehmann P
Br J Dermatol 1999 Oct;141(4):642-6. doi: 10.1046/j.1365-2133.1999.03101.x. PMID: 10583110
Lucker GP, Steijen PM, Suykerbuyk EJ, Kragballe K, Brandrup F, van de Kerkhof PC
Acta Derm Venereol 1996 Mar;76(2):97-101. doi: 10.2340/000155557697101. PMID: 8740259
Happle R, van de Kerkhof PC, Traupe H
Dermatologica 1987;175 Suppl 1:107-24. doi: 10.1159/000248867. PMID: 2961628

Clinical prediction guides

Hofmann B, Stege H, Ruzicka T, Lehmann P
Br J Dermatol 1999 Oct;141(4):642-6. doi: 10.1046/j.1365-2133.1999.03101.x. PMID: 10583110
Steijlen PM, Reifenschweiler DO, Ramaekers FC, van Muijen GN, Happle R, Link M, Ruiter DJ, van de Kerkhof PC
Arch Dermatol Res 1993;285(4):221-6. doi: 10.1007/BF00372013. PMID: 7688204
Ford GP, Friedmann PS, Ross J
Br J Dermatol 1983 Sep;109(3):309-11. doi: 10.1111/j.1365-2133.1983.tb03546.x. PMID: 6615717
Passarge E, Post B, Schöpf E
Birth Defects Orig Artic Ser 1971 Jun;7(8):46-9. PMID: 5173313

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