Cutis verticis gyrata
- Fiona Larsen, MBChB, FRACP
Fiona Larsen, MBChB, FRACP
Cutis verticis gyrata (CVG) is a rare congenital or acquired scalp condition characterized by convoluted folds and deep furrows that resemble the surface of the cerebral cortex [1-3]. It may occur as a primary condition, often in association with neuropsychiatric or ophthalmologic abnormalities, or may be secondary to a number of localized or systemic inflammatory or neoplastic diseases. Cutis verticis gyrata has also been reported in association with several genetic syndromes, including Turner syndrome, Noonan syndrome, and craniosynostosis syndromes.
This topic will discuss the clinical presentation, diagnosis, and treatment of primary and secondary CVG. Other scalp disorders are discussed separately. (See "Approach to the patient with a scalp disorder".)
Cutis verticis gyrata is classified as primary, essential or nonessential, or secondary [3,4].
●Primary essential cutis verticis gyrata occurs in isolation in otherwise normal individuals.
●The primary nonessential form is associated with neuropsychiatric or ophthalmologic abnormalities.
- MacGillivray RC. Cutis verticis gyrata and mental retardation. Scott Med J 1967; 12:450.
- Unna PG. Cutis verticis gyrata. Monatsh Prakt Derm 1907; 45:227.
- POLAN S, BUTTERWORTH T. Cutis verticis gyrata; a review with report of seven new cases. Am J Ment Defic 1953; 57:613.
- Garden JM, Robinson JK. Essential primary cutis verticis gyrata. Treatment with the scalp reduction procedure. Arch Dermatol 1984; 120:1480.
- AKESSON HO. CUTIS VERTICIS GYRATA AND MENTAL DEFICIENCY IN SWEDEN. I. EPIDEMIOLOGIC AND CLINICAL ASPECTS. Acta Med Scand 1964; 175:115.
- Diven DG, Tanus T, Raimer SS. Cutis verticis gyrata. Int J Dermatol 1991; 30:710.
- Schepis C, Siragusa M. Primary cutis verticis gyrata or pachydermia verticis gyrata: a peculiar scalp disorder of mentally retarded adult males. Dermatology 1995; 191:292.
- Schepis C, Palazzo R, Cannavo SP, et al. Prevalence of primary cutis verticis gyrata in a psychiatric population: association with chromosomal fragile sites. Acta Derm Venereol 1990; 70:483.
- Palazzo R, Schepis C, Ruggeri M, et al. An endocrinological study of patients with primary cutis verticis gyrata. Acta Derm Venereol 1993; 73:348.
- Dahir GA, Miller LK, Butler MG. Survey of mentally retarded males for cutis verticis gyrata and chromosomal fragile sites. Am J Med Genet 1992; 44:118.
- Schepis C, Palazzo R, Ragusa RM, et al. Association of cutis verticis gyrata with fragile X syndrome and fragility of chromosome 12. Lancet 1989; 2:279.
- Musumeci SA, Ferri R, Viglianesi M, et al. Cutis verticis gyrata and chromosomal fragile sites. Am J Med Genet 1991; 38:249.
- Snyder MC, Johnson PJ, Hollins RR. Congenital primary cutis verticis gyrata. Plast Reconstr Surg 2002; 110:818.
- Hambrick GW Jr, Carter DM. Pachydermoperiostosis. Touraine-Solente-Golé syndrome. Arch Dermatol 1966; 94:594.
- Tanese K, Niizeki H, Seki A, et al. Pathological characterization of pachydermia in pachydermoperiostosis. J Dermatol 2015; 42:710.
- Harish V, Clarke F. Isolated cutis verticis gyrata of the glabella and nasal bridge: a case report and review of the literature. J Plast Reconstr Aesthet Surg 2013; 66:1421.
- Kara IG. Forehead lifting for cutis verticis gyrata. Plast Reconstr Surg 2003; 111:1777.
- Beier JP, Kneser U, Horch RE. Surgical treatment of facial cutis verticis gyrata with direct excision. J Cutan Med Surg 2007; 11:4.
- Zhao L, Fang F, Xu J, et al. Giant primary cutis verticis gyrata successfully treated with surgical excision and tissue expansion. Dermatol Surg 2006; 32:753.
- Castori M, Sinibaldi L, Mingarelli R, et al. Pachydermoperiostosis: an update. Clin Genet 2005; 68:477.
- Ramos-e-Silva M, Martins G, Dadalti P, Maceira J. Cutis verticis gyrata secondary to a cerebriform intradermal nevus. Cutis 2004; 73:254.
- Orkin M, Frichot BC 3rd, Zelickson AS. Cerebriform intradermal nevus. A cause of cutis verticis gyrata. Arch Dermatol 1974; 110:575.
- Larsen F, Birchall N. Cutis verticis gyrata: three cases with different aetiologies that demonstrate the classification system. Australas J Dermatol 2007; 48:91.
- Saha D, Kini UA, Kini H. Cutaneous neurocristic hamartoma presenting as cutis verticis gyrata. Am J Dermatopathol 2014; 36:e66.
- Laxmisha C, Thappa DM, Jayanthi S. Isolated scalp collagenoma mimicking cutis verticis gyrata. Indian J Dermatol Venereol Leprol 2006; 72:309.
- Alcántara González J, Truchuelo Díez MT, Carrillo Gijón R, et al. Letter: Cerebriform intradermal nevus presenting as secondary cutis verticis gyrata. Dermatol Online J 2010; 16:14.
- Plewig G. Cutis verticis gyrata secondary to acne scleroticans capitis. J Eur Acad Dermatol Venereol 2005; 19:772.
- Ross JB, Tompkins MG. Cutis verticis gyrata as a marker of internal malignancy. Arch Dermatol 1989; 125:434.
- Kim JE, Choi KH, Kang SJ, et al. Angiosarcoma mimicking cutis verticis gyrata. Clin Exp Dermatol 2011; 36:806.
- Rahman A, Mahmood A. Cutis verticis gyrata secondary to infiltrating ductal carcinoma breast. J Coll Physicians Surg Pak 2012; 22:120.
- Passarini B, Neri I, Patrizi A, Masina M. Cutis verticis gyrata secondary to acute monoblastic leukemia. Acta Derm Venereol 1993; 73:148.
- Al-Bedaia M, Al-Khenaizan AS. Acromegaly presenting as cutis verticis gyrata. Int J Dermatol 2008; 47:164.
- Kolawole TM, Al Orainy IA, Patel PJ, Fathuddin S. Cutis verticis gyrata: its computed tomographic demonstration in acromegaly. Eur J Radiol 1998; 27:145.
- Walia R, Bhansali A. Cutis verticis gyrata. BMJ Case Rep 2011; 2011.
- Yerawar C, Bandgar T, Lila A, Shah NS. Acromegaly presenting as cutis verticis gyrata. QJM 2016; 109:423.
- Araujo KM, da Silva FT, Bestane GC, et al. Delayed Diagnosis of Acromegaly in an Elderly Adult that Initially Presented as Cutis Verticis Gyrata. J Am Geriatr Soc 2016; 64:685.
- Bilen H, Atasoy M, Akcay G, et al. Elephantiasic pretibial myxedema and cutis verticis gyrata caused by Graves' disease. Thyroid 2006; 16:815.
- Woollons A, Darley CR, Lee PJ, et al. Cutis verticis gyrata of the scalp in a patient with autosomal dominant insulin resistance syndrome. Clin Exp Dermatol 2000; 25:125.
- Corbalán-Vélez R, Pérez-Ferriols A, Aliaga-Bouiche A. Cutis verticis gyrata secondary to hypothyroid myxedema. Int J Dermatol 1999; 38:781.
- Saoji V, Chaudhari S, Gohokar D. Primary systemic amyloidosis: three different presentations. Indian J Dermatol Venereol Leprol 2009; 75:394.
- Kim HS, Teo RY, Tan AW. Cutis verticis gyrata in a patient with hyper-IgE syndrome. Acta Derm Venereol 2009; 89:413.
- Harding JJ, Barker CA, Carvajal RD, et al. Cutis verticis gyrata in association with vemurafenib and whole-brain radiotherapy. J Clin Oncol 2014; 32:e54.
- Lang N, Sterzing F, Enk AH, Hassel JC. Cutis verticis gyrata-like skin toxicity during treatment of melanoma patients with the BRAF inhibitor vemurafenib after whole-brain radiotherapy is a consequence of the development of multiple follicular cysts and milia. Strahlenther Onkol 2014; 190:1080.
- Khanijow K, Unemori P, Leslie KS, et al. Cutis Verticis Gyrata in Men Affected by HIV-Related Lipodystrophy. Dermatol Res Pract 2013; 2013:941740.
- Larralde M, Gardner SS, Torrado MV, et al. Lymphedema as a postulated cause of cutis verticis gyrata in Turner syndrome. Pediatr Dermatol 1998; 15:18.
- Parolin Marinoni L, Taniguchi K, Giraldi S, et al. Cutis verticis gyrata in a child with Turner syndrome. Pediatr Dermatol 1999; 16:242.
- Keller K, Williams C, Seagle B. Klinefelter syndrome and cutis verticis gyrata. Am J Med Genet 2001; 103:249.
- Lobato-Berezo A, Imbernón-Moya A, Churruca-Grijelmo M, Gallego-Valdés MÁ. [Cutis verticis gyrata associated with Klinefelter syndrome]. Med Clin (Barc) 2015; 145:e21.
- Rácz E, Kornseé Z, Csikós M, et al. Darier's disease associated with cutis verticis gyrata, hyperprolactinaemia and depressive disorder. Acta Derm Venereol 2006; 86:59.
- Parlak M, Erdem T, Karakuzu A, et al. Darier's disease seen with cutis verticis gyrata. Acta Derm Venereol 2001; 81:75.
- Marque M, Gardie B, Bressac de Paillerets B, et al. Novel FH mutation in a patient with cutaneous leiomyomatosis associated with cutis verticis gyrata, eruptive collagenoma and Charcot-Marie-Tooth disease. Br J Dermatol 2010; 163:1337.
- Wenger TL, Bhoj EJ, Wetmore RF, et al. Beare-Stevenson syndrome: two new patients, including a novel finding of tracheal cartilaginous sleeve. Am J Med Genet A 2015; 167A:852.
- Auada MP, Cintra ML, Puzzi MB, et al. Scalp lesions in Turner syndrome: a result of lymphoedema? Clin Dysmorphol 2004; 13:165.
- Masson P, Fayon M, Lamireau T, et al. [Unusual form of Noonan syndrome: neonatal multi-organ involvement with chylothorax and nevoid Cutis verticis gyrata]. Pediatrie 1993; 48:59.
- Fox LP, Geyer AS, Anyane-Yeboa K, Garzon MC. Cutis verticis gyrata in a patient with Noonan syndrome. Pediatr Dermatol 2005; 22:142.
- Witt DR, Hoyme HE, Zonana J, et al. Lymphedema in Noonan syndrome: clues to pathogenesis and prenatal diagnosis and review of the literature. Am J Med Genet 1987; 27:841.
- Marini R, Cappa M, Neri G. Cutis verticis gyrata and chromosomal abnormalities. Am J Dis Child 1989; 143:269.
- Chandler KE, Kidd A, Al-Gazali L, et al. Diagnostic criteria, clinical characteristics, and natural history of Cohen syndrome. J Med Genet 2003; 40:233.
- Mégarbané A, Waked N, Chouery E, et al. Microcephaly, cutis verticis gyrata of the scalp, retinitis pigmentosa, cataracts, sensorineural deafness, and mental retardation in two brothers. Am J Med Genet 2001; 98:244.
- Mégarbané A, Slim R, Nürnberg G, et al. A novel VPS13B mutation in two brothers with Cohen syndrome, cutis verticis gyrata and sensorineural deafness. Eur J Hum Genet 2009; 17:1076.
- Mishra A, Tehrani H, Hancock K, Duncan C. Management of primary cutis verticis gyrata with tissue expansion and hairline lowering foreheadplasty. J Plast Reconstr Aesthet Surg 2010; 63:1060.
- Zhao D, Li J, Wang K, et al. Treating cutis verticis gyrata using skin expansion method. Cell Biochem Biophys 2012; 62:373.
- Hsu YJ, Chang YJ, Su LH, Hsu YL. Using novel subcision technique for the treatment of primary essential cutis verticis gyrata. Int J Dermatol 2009; 48:307.
- Hayashi Y, Tanioka M, Taki R, et al. Malignant melanoma derived from cerebriform intradermal naevus. Clin Exp Dermatol 2009; 34:e840.