Departments of 1Dermatology and 2Radiology & Diagnostic Imaging, Hospital General Universitario de Ciudad Real, Ciudad Real, Spain *Corresponding Author’s e-mail: mgarciaa73@yahoo.es املعلومات األولية عن تثخن الفروة حالة مرضية تصيب فروة الرأس حتدث يف عدد من األمراض مونيكا قار�شيا-اأربا، مقيول فلوري�س-تريي، لو�شيا قونزل�س-روي�س، من�رصات فرانكو-منوز، مانولو-كالفو-قار�شيال Primary Essential Cutis Verticis Gyrata A scalp condition that may appear in various disorders *Mónica García-Arpa,1 Miguel A. Flores-Terry,1 Lucía González-Ruiz,1 Monserrat Franco-Muñoz,1 Manolo Calvo-García2 A 35-year-old male patient of bolivian origin was referred to the Dermatology Depart- ment of the Hospital General de Ciudad Real, Ciudad Real, Spain, in 2017 with intense scalp pruritus and nodules of one year’s duration. A physical exam- ination revealed skin redundancy with deep cerebri- form folds and wrinkles in the scalp consistent with a diagnosis of cutis verticis gyrata (CVG) [Figure 1]. There was no reported family history of similar scalp conditions or any evidence of intellectual disabilities or neurological, psychiatric or ophthalmological manifestations. The rest of the physical examination revealed no other abnormalities, including digital clubbing. In order to exclude secondary disorders, a cutaneous biopsy and various complementary examinations were performed. This included a complete blood count, bio- chemistry testing, electrophoresis and urine analysis and testing for immunoglobulins, serum thyroid-stimulating hormone, free thyroxine, growth hormone, insulin-like growth factor 1, insulin and insulin resistance index. In addition, the patient underwent serological screening for the hepatitis virus, HIV and syphilis, as well as karyo- typing, hand X-raying and an ophthalmological exam- ination. There was no evidence of any abnormalities. Magnetic resonance imaging (MRI) with volume-rend- ering revealed subcutaneous thickening of the scalp [Figure 2]. Intracranial abnormalities were ruled out by axial MRI [Figure 3]. Given the benign nature of the condition, the patient was discharged and localised hygiene practices were recommended. Comment Overall, CVG cases are classified into primary and sec- ondary forms; primary CVG cases are sometimes further subclassified as either non-essential (i.e. those with assoc- iated neurological, psychiatric and ophthalmological manifestations) or essential (i.e. those without abnorm- alities or secondary disorders).1–3 Primary essential interesting medical image Sultan Qaboos University Med J, November 2018, Vol. 18, Iss. 4, pp. e560–562, Epub. 28 Mar 19 Submitted 6 Jun 18 Revision Req. 10 Jul 18; Revision Recd. 31 Jul 18 Accepted 30 Aug 18 Figure 1: Photographs of the scalp of a 35-year-old male patient with intense scalp pruritus and nodules showing skin redundancy and deep symmetrical cerebriform folds in the frontoparietal, vertex and occipital areas, typical of cutis verticis gyrata. doi: 10.18295/squmj.2018.18.04.025 Mónica García-Arpa, Miguel A. Flores-Terry, Lucía González-Ruiz, Monserrat Franco-Muñoz and Manolo Calvo-García Interesting Medical Image | e561 CVG is a rare disorder of unknown aetiology which is five times more frequent in men.4 Most cases are sporadic, although hereditary cases have been des- cribed.2 The prevalence of CVG is unknown in most South American countries, including Bolivia; however, a Colombian study indicated the prevalence of CVG to be 5.9% among institutionalised patients.5 Primary essential CVG usually presents in puberty as symmetric scalp folds extending from the vertex to the occiput which cannot be flattened by traction or pressure.1–3 Typically, the folds resemble the brain cortex and extend anteroposteriorly, although they may also occur in a horizontal direction in the occipital region. Biopsies of the folds show normal skin histopathology.1,3 On MRI, CVG has a typical appearance due to the thickening of the skin and subcutaneous tissues, esp- ecially with three-dimensional volume rendering.2 Primary essential CVG must be differentiated from secondary CVG. The latter type can manifest in areas other than the scalp and has been associated with different conditions, such as acromegaly and pachydermo- periostosis.6,7 Furthermore, CVG has been described among patients receiving external-beam whole-brain radiotherapy, vemurafenib and anabolic steroids.8,9 Prim- ary essential CVG may also be associated with certain genetic disorders, like fragile X syndrome.10 Other cond- itions that may present with CVG include endocrine diseases (i.e. thyroid disease and diabetes mellitus), intracranial and benign scalp tumours, intracerebral aneurysms, internal malignancy, HIV-related lipodys- trophy, inflammatory dermatoses and direct cutaneous infiltration in the setting of malignant haematological disease.1,2 In the present case, the patient had a normal karyotype and did not present with any features of other disorders. Apart from the cutaneous scalp lesions, the physical examination was normal. Furthermore, the patient had no medical or family history of genetic diseases; as such, he was diagnosed with primary essential CVG. As the condition is benign, only symptomatic treatment is necessary.2 Careful local hygiene is some- times recommended in order to avoid secretions, unplea- sant odours and pruritus. Surgical treatment is only necessary for aesthetic reasons and ranges from simple excision to tissue expansion and skin grafts, depending on the size and location of the lesions.2 References 1. Yang JJ, Sano DT, Martins SR, Tebcherani AJ, Sanchez AP. Primary essential cutis verticis gyrata: Case report. An Bras Dermatol 2014; 89:326−8. https://doi.org/10.1590/abd1806-48 41.20142949. 2. 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; 173:638−46. https://doi.org/10. 1002/ajmg.a.38054. 3. Zeljko-Penavić J, Šimić D, Jurišić K, Gunarić A. Cutis verticis gyrata: A case report. Acta Dermatovenerol Croat 2016; 24:140−3. 4. Akesson HO. Cutis verticis gyrata and mental deficiency in Sweden: II - Genetic aspects. Acta Med Scand 1965; 177:459−64. Figure 2: Volume-rendering magnetic resonance imaging of the scalp of a 35-year-old male patient showing typical features of cutis verticis gyrata. Figure 3: Axial magnetic resonance imaging scan of the brain of a 35-year-old male patient showing no evidence of intracranial abnormalities. Primary Essential Cutis Verticis Gyrata A scalp condition that may appear in various disorders e562 | SQU Medical Journal, November 2018, Volume 18, Issue 4 5. Fernando Hernández J, Espejo Jiménez LM, Correa Díaz RL, de la Espriella R. Cutis verticis gyrata in adult male institut- ionalized population in Colombia: Case series. Acta Neurol Colomb 2015; 31:404−11. https://doi.org/10.22379/2422402259. 6. Yerawar C, Bandgar T, Lila A, Shah NS. Acromegaly presenting as cutis verticis gyrata. QJM 2016; 109:423. https://doi.org/10. 1093/qjmed/hcw020. 7. Sandoval AR, Flores-Robles BJ, Llanos JC, Porres S, Dardón JD, Harrison RM. Cutis verticis gyrata as a clinical manifestation of Touraine-Solente-Golé syndrome (pachydermoperiostosis). BMJ Case Rep 2013; 2013:bcr2013010047. https://doi.org/10. 1136/bcr-2013-010047. 8. Harding JJ, Barker CA, Carvajal RD, Wolchok JD, Chapman PB, Lacouture ME. Cutis verticis gyrata in association with vem- urafenib and whole-brain radiotherapy. J Clin Oncol 2014; 32:e54−6. https://doi.org/10.1200/JCO.2013.49.3528. 9. Sommer A, Gambichler T, Altmeyer P, Kreuter A. A case of cutis verticis gyrata, induced by misuse of anabolic substances? Clin Exp Dermatol 2005; 31:129–156. https://doi.org/10.1111/ j.1365-2230.2005.01911.x. 10. 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; 70:483−6.