History

In primary cutis verticis gyrata, skin plaques develop after puberty, usually in the vertex and occipital region. In secondary cutis verticis gyrata, skin plaques can be present at birth. The skin gradually becomes thicker, and folds and furrows are formed.

No other symptoms are usually present; however, pain was observed in cases with cutis verticis gyrata due to intradermal nevus and in traction alopecia. The progression of changes is visible.^[59]

Physical Examination

In the primary form, only the scalp is involved. Cutis verticis gyrata typically affects the vertex and occipital region, but some forms can involve the entire scalp. Folds are soft and spongy and cannot be corrected by pressure or traction. In primary cutis verticis gyrata, folds are usually symmetric; in secondary cutis verticis gyrata, folds are asymmetric.

In most cases, the direction of the folds is anterior to posterior, but it may be transverse in the occipital region. The number of folds varies from 2-12, although some atypical cases with 1 fold have been described. The skin color is unchanged. The hair over the folds may be sparse but normal in the furrows. Maceration and an unpleasant smell may be present in patients with secondary infections in the furrows.

See the image below.

Cutis verticis gyrata. Courtesy of DermNet New Zealand (http://www.dermnetnz.org/assets/Uploads/dermal-infiltrative/cvg2.jpg).

Differential Diagnoses

Unna PG. Cutis verticis gyrata. Monatschr Prakt Derm. 1907. 45:227-33.
Polan S, Butterworth T. Cutis verticis gyrata; a review with report of seven new cases. Am J Ment Defic. 1953 Apr. 57(4):613-31. [QxMD MEDLINE Link].
Garden JM, Robinson JK. Essential primary cutis verticis gyrata. Treatment with the scalp reduction procedure. Arch Dermatol. 1984 Nov. 120(11):1480-3. [QxMD MEDLINE Link].
Tucci A, Pezzani L, Scuvera G, Ronzoni L, Scola E, Esposito S, et al. Is cutis verticis Gyrata-Intellectual Disability syndrome an underdiagnosed condition? A case report and review of 62 cases. Am J Med Genet A. 2017 Mar. 173 (3):638-646. [QxMD MEDLINE Link].
Kolawole TM, Al Orainy IA, Patel PJ, Fathuddin S. Cutis verticis gyrata: its computed tomographic demonstration in acromegaly. Eur J Radiol. 1998 May. 27(2):145-8. [QxMD MEDLINE Link].
Zangeneh F, Carpenter PC. Visual vignette. Cutis verticis gyrata (GVG) in acromegaly. Endocr Pract. 2002 Nov-Dec. 8(6):475. [QxMD MEDLINE Link].
Landenberger GMC, Ongaratti BR, Pereira-Lima JFS, Oliveira MDC. Cutis verticis gyrata: a cutaneous finding in acromegaly. An Bras Dermatol. 2022 May-Jun. 97 (3):369-371. [QxMD MEDLINE Link]. [Full Text].
Saoji V, Chaudhari S, Gohokar D. Primary systemic amyloidosis: three different presentations. Indian J Dermatol Venereol Leprol. 2009 Jul-Aug. 75(4):394-7. [QxMD MEDLINE Link].
Woollons A, Darley CR, Lee PJ, Brenton DP, Sonksen PH, Black MM. Cutis verticis gyrata of the scalp in a patient with autosomal dominant insulin resistance syndrome. Clin Exp Dermatol. 2000 Mar. 25(2):125-8. [QxMD MEDLINE Link].
Marque M, Gardie B, Bressac de Paillerets B, Rustin P, Guillot B, Richard S, 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 Dec. 163(6):1337-9. [QxMD MEDLINE Link].
Bilen H, Atasoy M, Akcay G, Akcay M, Capoglu I, Gursan N, et al. Elephantiasic pretibial myxedema and cutis verticis gyrata caused by graves' disease. Thyroid. 2006 Aug. 16(8):815-6. [QxMD MEDLINE Link].
Jeannet PY, Watts GD, Bird TD, Chance PF. Craniofacial and cutaneous findings expand the phenotype of hereditary neuralgic amyotrophy. Neurology. 2001 Dec 11. 57(11):1963-8. [QxMD MEDLINE Link].
Khanijow K, Unemori P, Leslie KS, Mulligan K, Schambelan M, Maurer T. Cutis Verticis Gyrata in Men Affected by HIV-Related Lipodystrophy. Dermatol Res Pract. 2013. 2013:941740. [QxMD MEDLINE Link].
Kim HS, Teo RY, Tan AW. Cutis verticis gyrata in a patient with hyper-IgE syndrome. Acta Derm Venereol. 2009. 89(4):413-4. [QxMD MEDLINE Link].
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 Oct. 66(10):1421-3. [QxMD MEDLINE Link].
Ulrich J, Franke I, Gollnick H. Cutis verticis gyrata secondary to acne scleroticans capitis. J Eur Acad Dermatol Venereol. 2004 Jul. 18(4):499-502. [QxMD MEDLINE Link].
Lehmann P. [Cutis verticis gyrata-like acne inversa. Combined medical and surgical management]. Hautarzt. 2009 Apr. 60(4):328-30. [QxMD MEDLINE Link].
Larsen F, Birchall N. Cutis verticis gyrata: three cases with different aetiologies that demonstrate the classification system. Australas J Dermatol. 2007 May. 48(2):91-4. [QxMD MEDLINE Link].
Racz E, Kornsee Z, Csikos M, Dobos M, Salacz P, Karpati S. Darier's disease associated with cutis verticis gyrata, hyperprolactinaemia and depressive disorder. Acta Derm Venereol. 2006. 86(1):59-60. [QxMD MEDLINE Link].
Nachinolcar SN, Pai V, Shukla P, Muthuprabhakaran K. Primary nonessential cutis verticis gyrata with acne keloidalis nuchae. Indian J Dermatol Venereol Leprol. 2021 Sep-Oct. 87 (5):748. [QxMD MEDLINE Link]. [Full Text].
Kolawole TM, al Orainy IA. Cutis verticis gyrata (case reports of rare causes). West Afr J Med. 1999 Apr-Jun. 18(2):130-2. [QxMD MEDLINE Link].
Passarini B, Neri I, Patrizi A, Masina M. Cutis verticis gyrata secondary to acute monoblastic leukemia. Acta Derm Venereol. 1993 Apr. 73(2):148-9. [QxMD MEDLINE Link].
Jeanfils S, Tennstedt D, Lachapelle JM. Cerebriform intradermal nevus. A clinical pattern resembling cutis verticis gyrata. Dermatology. 1993. 186(4):294-7. [QxMD MEDLINE Link].
Hayashi Y, Tanioka M, Taki R, Sawabe K, Kore-eda S, Utani A, et al. Malignant melanoma derived from cerebriform intradermal naevus. Clin Exp Dermatol. 2009 Dec. 34(8):e840-2. [QxMD MEDLINE Link].
Ramos-e-Silva M, Martins G, Dadalti P, Maceira J. Cutis verticis gyrata secondary to a cerebriform intradermal nevus. Cutis. 2004. 73:254-6. [QxMD MEDLINE Link].
Saha D, Kini UA, Kini H. Cutaneous Neurocristic Hamartoma Presenting as Cutis Verticis Gyrata. Am J Dermatopathol. 2013 Jun 27. [QxMD MEDLINE Link].
Rao AG, Koppada D, Haritha M. Giant Cerebriform Congenital Cellular Blue Nevus Presenting as Cutis Verticis Gyrata. Indian J Dermatol. 2016 Jan-Feb. 61 (1):126. [QxMD MEDLINE Link].
Corbalan-Velez R, Perez-Ferriols A, Aliaga-Bouiche A. Cutis verticis gyrata secondary to hypothyroid myxedema. Int J Dermatol. 1999 Oct. 38(10):781-3. [QxMD MEDLINE Link].
Commens CA, Greaves MW. Cutis verticis gyrata due to an intradermal naevus with an underlying neurofibroma. Clin Exp Dermatol. 1978 Sep. 3(3):319-22. [QxMD MEDLINE Link].
Fox LP, Geyer AS, Anyane-Yeboa K, Garzon MC. Cutis verticis gyrata in a patient with Noonan syndrome. Pediatr Dermatol. 2005 Mar-Apr. 22(2):142-6. [QxMD MEDLINE Link].
Thappa DM, Sethuraman G, Kumar GR, Elangovan S. Primary pachydermoperiostosis: a case report. J Dermatol. 2000 Feb. 27(2):106-9. [QxMD MEDLINE Link].
Arikan S, Sen I, Bahceci M, Tuzcu A, Ayli M. An interesting case of pachydermoperiostosis with idiopathic myelofibrosis associated with monosomy 22. Int J Dermatol. 2009 Aug. 48(8):882-5. [QxMD MEDLINE Link].
Beauregard S. [Cutis verticis gyrata and pachydermoperiostosis. Several cases in a same family. Initial results of the treatment of pachyderma with isotretinoin]. Ann Dermatol Venereol. 1994. 121(2):134-7. [QxMD MEDLINE Link].
Kanwar AJ, Ghosh S, Thami GP, Kaur S. Alopecia and cutis verticis gyrata due to traction presenting as headache. Int J Dermatol. 1992 Sep. 31(9):671-2. [QxMD MEDLINE Link].
Nguyen KH, Marks JG Jr. Pseudoacromegaly induced by the long-term use of minoxidil. J Am Acad Dermatol. 2003 Jun. 48(6):962-5. [QxMD MEDLINE Link].
Koregol S, Yatagiri RV, Warad SR, Itagi NR. A rare association of scleromyxedema with cutis verticis gyrata. Indian Dermatol Online J. 2016 May-Jun. 7 (3):186-9. [QxMD MEDLINE Link].
Keller K, Williams C, Seagle B. Klinefelter syndrome and cutis verticis gyrata. Am J Med Genet. 2001 Oct 15. 103(3):249-51. [QxMD MEDLINE Link].
George AA, George L, Mahabal G, Bindra M, Pulimood S. Systemic T cell lymphoma presenting as cutis verticis gyrata. Indian J Dermatol Venereol Leprol. 2015 Nov-Dec. 81 (6):631-3. [QxMD MEDLINE Link].
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 Oct. 190 (11):1080-1. [QxMD MEDLINE Link].
Harding JJ, Barker CA, Carvajal RD, Wolchok JD, Chapman PB, Lacouture ME. Cutis verticis gyrata in association with vemurafenib and whole-brain radiotherapy. J Clin Oncol. 2014 May 10. 32 (14):e54-6. [QxMD MEDLINE Link].
Palazzo R, Schepis C, Ruggeri M, Baldini L, Pizzimenti A, Arcoraci V, et al. An endocrinological study of patients with primary cutis verticis gyrata. Acta Derm Venereol. 1993 Oct. 73(5):348-9. [QxMD MEDLINE Link].
Seppalainen AM, Iivanainen M. The cutis verticis gyrata and mental retardation syndrome: electroencephalographic analysis of nineteen cases. J Ment Defic Res. 1974 Dec. 18(4):277-83. [QxMD MEDLINE Link].
Kara IG. Forehead lifting for cutis verticis gyrata. Plast Reconstr Surg. 2003 Apr 15. 111(5):1777-8. [QxMD MEDLINE Link].
Radwanski HN, Rocha Almeida MW, Pitanguy I. Primary essential cutis verticis gyrata - a case report. J Plast Reconstr Aesthet Surg. 2008 Oct 22. [QxMD MEDLINE Link].
Dahir GA, Miller LK, Butler MG. Survey of mentally retarded males for cutis verticis gyrata and chromosomal fragile sites. Am J Med Genet. 1992 Sep 1. 44(1):118-20. [QxMD MEDLINE Link].
Marini R, Cappa M, Neri G, Romana M. Cutis verticis gyrata and chromosomal abnormalities. Am J Dis Child. 1989 Mar. 143(3):269-70. [QxMD MEDLINE Link].
Musumeci SA, Ferri R, Viglianesi M, Elia M, Ragusa RM, Bergonzi P. Cutis verticis gyrata and chromosomal fragile sites. Am J Med Genet. 1991 Feb-Mar. 38(2-3):249-50. [QxMD MEDLINE Link].
Schepis C, Palazzo R, Cannavo SP, Ragusa RM, Barletta C, Spina E. Prevalence of primary cutis verticis gyrata in a psychiatric population: association with chromosomal fragile sites. Acta Derm Venereol. 1990. 70(6):483-6. [QxMD MEDLINE Link].
Striano S, Ruosi P, Guzzetta V, Perone L, Manto A, Cirillo S. Cutis verticis gyrata--mental deficiency syndrome: a patient with drug-resistant epilepsy and polymicrogyria. Epilepsia. 1996 Mar. 37(3):284-6. [QxMD MEDLINE Link].
Auada MP, Cintra ML, Puzzi MB, Viana D, Cavalcanti DP. Scalp lesions in Turner syndrome: a result of lymphoedema?. Clin Dysmorphol. 2004 Jul. 13(3):165-8. [QxMD MEDLINE Link].
Larralde M, Gardner SS, Torrado MV, Fernhoff PM, Santos Munoz AE, Spraker MK, et al. Lymphedema as a postulated cause of cutis verticis gyrata in Turner syndrome. Pediatr Dermatol. 1998 Jan-Feb. 15(1):18-22. [QxMD MEDLINE Link].
Kennedy A, Perry D, Battin M. Antenatal imaging of cutis verticis gyrata. Pediatr Radiol. 2008 May. 38(5):583-7. [QxMD MEDLINE Link].
Schunter JA, Metze D, Böhm M. Is IGF-1 a key player in the pathogenesis of acromegaly-associated cutis verticis gyrata?. Exp Dermatol. 2017 Jan. 26 (1):85-86. [QxMD MEDLINE Link].
Akesson Ho. Cutis verticis gyrata and mental deficiency in Sweden. I. Epidemiologic and clinical aspects. Acta Med Scand. 1964 Jan. 175:115-27. [QxMD MEDLINE Link].
Akesson Ho. Cutis verticis gyrata and mental deficiency in Sweden. II. Genetic aspects. Acta Med Scand. 1965 Apr. 177:459-64. [QxMD MEDLINE Link].
MacGillivray RC. Cutis verticis gyrata and mental retardation. Scott Med J. 1967. 12:450-4.
Lamelas AM, Taub PJ, Silver L, Kressel A. Congenital Primary Essential Cutis Verticis Gyrata. Eplasty. 2016. 16:ic13. [QxMD MEDLINE Link].
Gross PR, Carter DM. Malignant melanoma arising in a giant cerebriform nevus. Arch Dermatol. 1967 Nov. 96(5):536-9. [QxMD MEDLINE Link].
Roque J, Marques P. Clinico-radiological features of acromegaly-related cutis verticis gyrata. Hormones (Athens). 2022 Apr 18. [QxMD MEDLINE Link].
van Geest AJ, Berretty PJ, Klinkhamer PJ, Neumann HA. Cerebriform intradermal naevus (a rare form of secondary cutis verticis gyrata). J Eur Acad Dermatol Venereol. 2002 Sep. 16(5):529-31. [QxMD MEDLINE Link].
Parolin Marinoni L, Taniguchi K, Giraldi S, Carvalho VO, Furucho M, Bertogna J. Cutis verticis gyrata in a child with Turner syndrome. Pediatr Dermatol. 1999 May-Jun. 16(3):242-3. [QxMD MEDLINE Link].
Yazici AC, Ikizoglu G, Baz K, Polat A, Ustunsoy D. Cerebriform intradermal nevus. Pediatr Dermatol. 2007 Mar-Apr. 24(2):141-3. [QxMD MEDLINE Link].
Oh DJ, Park JH, Kang SH, Hwang SW, Park SW. Primary non-essential cutis verticis gyrata revealed with 3-D magnetic resonance imaging. Acta Derm Venereol. 2006. 86(5):458-9. [QxMD MEDLINE Link].
Okamoto K, Ito J, Tokiguchi S, Ishikawa K, Furusawa T, Sakai K. MRI in essential primary cutis verticis gyrata. Neuroradiology. 2001 Oct. 43(10):841-4. [QxMD MEDLINE Link].
Demirpolat G, Sener RN, Stun EE. MR imaging of pachydermoperiostosis. J Neuroradiol. 1999 Mar. 26(1):61-3. [QxMD MEDLINE Link].
Mutter D, Lipsker D, Cribier B, Evrard S, Roy C. Primary cutis verticis gyrata: CT findings. J Comput Assist Tomogr. 1993 Jul-Aug. 17(4):663-4. [QxMD MEDLINE Link].
Jain K, Jain VK, Aggarwal K, Bansal A. Late onset isotretinoin resistant acne conglobata in a patient with acromegaly. Indian J Dermatol Venereol Leprol. 2008 Mar-Apr. 74(2):139-41. [QxMD MEDLINE Link].
Farah S, Farag T, Sabry MA, Simeonov ST, al-Khattam S, Abulhassan SJ, et al. Cutis verticis gyrata-mental deficiency syndrome: report of a case with unusual neuroradiological findings. Clin Dysmorphol. 1998 Apr. 7(2):131-4. [QxMD MEDLINE Link].
Filosto M, Tonin P, Vattemi G, Bongiovanni LG, Rizzuto N, Tomelleri G. Cutis verticis gyrata, mental retardation and Lennox-Gastaut syndrome: a case report. Neurol Sci. 2001 Jun. 22(3):253-6. [QxMD MEDLINE Link].
Sen F, Cagatay A, Ozsut H, Calangu S, Eraksoy H, Ozbey N. An unusual association of cutis verticis gyrata with empty sella. Eur J Intern Med. 2008 Oct. 19(6):e23-5. [QxMD MEDLINE Link].
Kosumi H, Izumi K, Natsuga K, Yamaguchi Y, Itami A, Shimizu H. Cutis Verticis Gyrata Fluctuation with Atopic Dermatitis Disease Activity. Acta Derm Venereol. 2017 Nov 15. 97 (10):1245-1246. [QxMD MEDLINE Link].
Beier JP, Kneser U, Horch RE. Surgical treatment of facial cutis verticis gyrata with direct excision. J Cutan Med Surg. 2007 Jan-Feb. 11(1):4-8. [QxMD MEDLINE Link].
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 Feb 1. [QxMD MEDLINE Link].
Dumas P, Medard de Chardon V, Balaguer T, Cardot-Leccia N, Lacour JP, Lebreton E. [Primary essential cutis verticis gyrata: Case report and literature review.]. Ann Chir Plast Esthet. 2009 Nov 23. [QxMD MEDLINE Link].
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 Mar. 48(3):307-9. [QxMD MEDLINE Link].
Zhao D, Li J, Wang K, Guo X, Lang Y, Peng L, et al. Treating cutis verticis gyrata using skin expansion method. Cell Biochem Biophys. 2012 Mar. 62(2):373-6. [QxMD MEDLINE Link].

Media Gallery

Cutis verticis gyrata. Courtesy of DermNet New Zealand (http://www.dermnetnz.org/assets/Uploads/dermal-infiltrative/cvg2.jpg).

of 1

Author

Malgorzata D Skibinska, MD, PhD Senior Lecturer, Department of Dermatology, Pediatric Dermatology and Oncology, Medical University of Łódź; Consultant Dermatologist and Allergist, Private Practice, Łódź, Poland

Malgorzata D Skibinska, MD, PhD is a member of the following medical societies: British Society of Allergy and Clinical Immunology, Polish Dermatological Society, Royal College of Physicians

Disclosure: Nothing to disclose.

Coauthor(s)

Camila K Janniger, MD Clinical Professor of Dermatology, Clinical Associate Professor of Pediatrics, Chief of Pediatric Dermatology, Rutgers New Jersey Medical School

Camila K Janniger, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Former Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Military Dermatologists, Phi Beta Kappa, Texas Dermatological Society

Disclosure: Nothing to disclose.

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Professor of Pediatrics, Professor of Medicine, Rutgers New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, New York Academy of Medicine, Royal College of Physicians of Edinburgh, Sigma Xi, The Scientific Research Honor Society

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Daniel J Hogan, MD Clinical Professor of Internal Medicine (Dermatology), Nova Southeastern University College of Osteopathic Medicine; Investigator, Hill Top Research, Florida Research Center

Daniel J Hogan, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Contact Dermatitis Society, Canadian Dermatology Association

Disclosure: Nothing to disclose.

Cutis Verticis Gyrata Clinical Presentation

History

Physical Examination

References

Tables

Contributor Information and Disclosures

What would you like to print?