Cutis verticis gyrata L91.8

Author: Prof. Dr. med. Peter Altmeyer

Co-Autor: Jeton Luzha

All authors of this article

Last updated on: 29.10.2020

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Synonym(s)

Audry Syndrome; bull-dog-scalp-syndrome; Bull-dog-scalp syndrome; cutis capitis gyrata; Cutis gyrata Unna; cutis verticis plicata; nevus cerebriformis; pachydermia occipitale verticellée; Pachydermia Perin wrinkle; pachydermia plicaturée; pachydermia verticis gyrata; Pachydermia wrinkly; wrinkled pachydermia; wrinkled pachydermia perin

History
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Roberts, 1843; Jadassohn, 1906; Unna, 1907

Definition
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Rare deformity of the scalp with wrinkles and deep furrows, which remind of brain convolutions, standing in rows next to each other, running straight from front to back, sometimes merging with each other.

Classification
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One can distinguish a primary (idiopathic) cutis verticis gyrata with and without associated symptoms and a secondary cutis verticis gyrata.

Occurrence/Epidemiology
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Worldwide the occurrence of cutis verticis gyrata has been described in 0.5-3.4% of all mentally retarded people. The etiology of cutis verticis gyratais is still unknown, although genetic and endocrinological factors of pathogenetic relevance are suspected. Furthermore, a connection between steroid abuse and the development of cutis verticis gyratais has been demonstrated.

Manifestation
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  • Primary form: During or after puberty, in 90% of patients before the age of 30. Men are affected 5 times more frequently than women.
  • Secondary form: Depending on the underlying disease, often at birth or during the first years of life.

Localization
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Mainly hairy head, also forehead and neck area. Rarely Palmae.

Clinical features
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Bead-shaped, up to finger-thick folds. Possibly reduced hair growth on the skin folds. Possible maceration and superinfection with very dense wrinkles. The primary form is often associated with mental retardation, neurological deficits or ophthalmological changes such as cataract, strabismus or retinitis pigmentosa. Cases of cutis verticis gyrata with hyper-IgE syndrome have been described.

Differential diagnosis
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Therapy
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A causal therapy of the disease is not known. If necessary excision, clarification and treatment of the accompanying symptoms.

Progression/forecast
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Slow increase in intensity. Quoad vitam favourable.

Literature
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  1. Audry C (1909) Pachydermia occipitale vorticalée (cutis verticis gyrata). Ann Dermatol Syphiligr (Paris) 10: 257-258
  2. Cribier B et al (1993) Cutis verticis gyrata: reduction surgical treatment. Ann Dermatol Venerol 120: 542-545
  3. Fesel R et al (1990) Cutis verticis gyrata. dermatologist 41: 501-505
  4. Jadassohn J (1906) A peculiar furrow, widening and thickening of the skin at the back of the head. Verh Dtsch Dermatol Ges, IX Congress, p. 451
  5. Nguyen NQ (2003) Cutis verticis gyrata. Dermatol Online J 9: 32
  6. Schöttler L et al (2007) Cerebriforms asymptomatic furrows of the scalp. dermatologist 58: 1058-1062
  7. Snyder MC et al (2002) Congenital primary cutis verticis gyrata. Plast Reconstr Surgery 110: 818-821
  8. Unna PG (1907) Cutis verticis gyrata. Practical Dermatol 45: 227
  9. by Geest AJ et al (2002) Cerebriform intradermal naevus (a rare form or secondary cutis verticis gyrata). J Eur Acad Dermatol Venereol 16: 529-531
  10. Woollons A et al (2000) Cutis verticis gyrata of the scalp in a patient with autosomal dominant insulin resistance syndrome. Clin Exp Dermatol 25: 125-128

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020