Cutis verticis gyrata

Cutis verticis gyrata
Classification and external resources
OMIM 219300 605685 304200

Cutis verticis gyrata (CVG) is a superficial medical condition usually associated with thickening of the scalp.[1] The condition was first reported by Alibert in 1837,[2] who called it cutis sulcata.[3] A clinical description of the condition was provided by Robert in 1843[4] and was named by Unna in 1907.[5] It has also been called Robert-Unna syndrome, bulldog scalp, corrugated skin, cutis verticis plicata, and pachydermia verticis gyrata.[6]

Sufferers show visible folds, ridges or creases on the surface of the top of the scalp.[7] The number of folds can vary from 2 to roughly 10 and are typically soft and spongy. These folds cannot be corrected with pressure. The condition typically affects the central and rear regions of the scalp, but sometimes can involve the entire scalp.

Hair loss can occur over time where the scalp thickens, though hair within any furrows remains normal. Thus far, due to the (apparent) rarity of the condition, limited research exists and causes are as yet undetermined. What is known, is that the condition is not exclusively congenital.

Classifications

CVG is classified according to the presence, or lack of underlying cause. Studies suggest that CVG often occurs in individuals in a secondary form to other ailments. However, the condition can also be present on its own. CVG can be classified into two forms: ‘primary’ (essential and non-essential) and ‘secondary’.[8]

The classifications are:

  • Primary essential
  • Primary non-essential
  • Secondary

Primary essential CVG is where the cause of the condition in unknown. It has no other associated abnormalities. This occurs mainly in men, with a male:female ratio of 5 or 6:1, and develops during or soon after puberty. Because of the slow progression of the condition, which usually occurs without symptom, it often passes unnoticed in the early stage [9]

Primary non essential CVG can be associated with neuropsychiatric disorders including cerebral palsy, epilepsy, seizures and ophthalmologic abnormalities, most commonly cataracts.

Secondary CVG occurs as a consequence of a number of diseases or drugs that produce changes in scalp structure. These include: acromegaly (excessive growth hormone levels due to pituitary gland tumours), excessive drug use that mimics acromegaly (including the injection of growth hormone itself and drugs that stimulate growth hormone output, such as GHRP-6 and CJC-1295), melanocytic naevi (moles), birthmarks (including connective tissue naevi, fibromas and naevus lipomatosus), and inflammatory processes (e.g., eczema, psoriasis, Darier disease, folliculitis, impetigo, atopic dermatitis, acne).

Treatment

There is no 'cure' for this condition and currently, medical treatment is limited to plastic surgery with excision of the folds by means of scalp reduction/surgical resection. Scalp subcision has also been suggested as a treatment.[10] Additional suggestions also include injections of a dermal filler i.e. Sculptra (poly-L-lactic acid)[11]

See also

References

  1. ↑ James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology (10th ed.). Saunders. p. 572. ISBN 0-7216-2921-0.
  2. ↑ Tan O, Ergen D (July 2006). "Primary essential cutis verticis gyrata in an adult female patient: a case report". J. Dermatol. 33 (7): 492–5. doi:10.1111/j.1346-8138.2006.00116.x. PMID 16848824.
  3. ↑ http://scholar.valpo.edu/cgi/viewcontent.cgi?article=1040&context=jmms -- See page 82 of J Mind Med Sci. 2016; 3(1): 80-87.
  4. ↑ http://emedicine.medscape.com/article/1113735-overview
  5. ↑ Unna PG. Cutis verticis gyrata. Monatschr Prakt Derm. 1907. 45:227-33.
  6. ↑ Levine, Norman (2004). Dermatology Therapy A-Z Essentials. Berlin New York: Springer. p. 166. ISBN 978-3-540-00864-4.
  7. ↑ Rapini, Ronald P.; Bolognia, Jean L.; Jorizzo, Joseph L. (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. p. 1502. ISBN 1-4160-2999-0.
  8. ↑ Radwanski, Henrique N.; Rocha Almeida, Marcelo Wilson; Pitanguy, Ivo (2009). "Primary essential cutis verticis gyrata – a case report". Journal of Plastic, Reconstructive & Aesthetic Surgery. 62 (11): e430–e433. doi:10.1016/j.bjps.2008.06.062. ISSN 1748-6815.
  9. ↑ Okamoto K, Ito J, Tokiguchi S, Ishikawa K, Furusawa T, Sakai K (October 2001). "MRI in essential primary cutis verticis gyrata". Neuroradiology. 43 (10): 841–4. doi:10.1007/s002340100591. PMID 11688700.
  10. ↑ Hsu YJ, Chang YJ, Su LH, Hsu YL (March 2009). "Using novel subcision technique for the treatment of primary essential cutis verticis gyrata". Int. J. Dermatol. 48 (3): 307–9. doi:10.1111/j.1365-4632.2009.03927.x. PMID 19261024.
  11. ↑ Unusual and rare complication described in San Francisco | CATIE - Canada's source for HIV and hepatitis C information

Bibliography

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