We present a case of a 22-year-old man who presented with pain and swelling of both knees and hands. Enlarged wrists, ankles and feet were also noted, along with facial seborrhoea, thickening of the skin and deformity of the fingers. [ncbi.nlm.nih.gov]

Similar lesions were present in periorbital region and nipple-areola complex [Figure 1]. Macroglossia and periorbital purpura were also present. Nails and mucosae were normal. [ijtrichology.com]

Primary non-essential is often present at birth or early childhood and is associated with obvious mental, physical, or functional abnormalities/deficiencies. Secondary CVG varies in time of presentation but may also be present at birth. [dermatologyadvisor.com]

We present a case of secondary CVG in a patient with longstanding underlying fibromatosis who presented with CVG two years into his disease. [scholars.direct]

• Strabismus

  Keywords: Cutis verticis gyrata, pachydermoperiostosis, strabismus How to cite this article: Date P, Jain S. One-sided brain over the head: Cutis verticis gyrata. [mjdrdypv.org]

  These may involve cerebral palsy, epilepsy, mental deficiency, deafness, cataracts, strabismus, retinitis pigmentosa, microcephaly, schizophrenia, etc. [dermatologyadvisor.com]

  The primary form has no known cause and may occur in isolation (primary essential CVG) or in association with other neurologic (intellectual disability, deafness, microcephaly, or epilepsy) and/or ophthalmologic abnormalities (cataracts, strabismus, blindness [actasdermo.org]

  Three years later Ganter reported two new cases, both mentally retarded with microcephaly; one with epilepsy had strabismus and the other had rotatory nystagmus (5). [scielo.org.co]

  […] only formation of skin folds on the scalp is found.1 The nonessential form presents association with several neurological manifestations (microcephaly and seizures, mental retardation, cerebral palsy, epilepsy) or ophthalmological changes (cataract, strabismus [scielo.br]

• Skin Lesion

  Abstract The primary form of cutis verticis gyrata, a rare skin lesion, is described for the first time in a neonate in whom it was associated with other congenital anomalies. [ncbi.nlm.nih.gov]

  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 and more recently scalp subcision[5] See also Skin lesion List of cutaneous conditions [dictionnaire.sensagent.leparisien.fr]

  (poly-L-lactic acid)).[11] See also[edit] Skin lesion List of cutaneous conditions References[edit] Notes ^ James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology (10th ed.). Saunders. p. 572. [en.wikipedia.org]

  Furthermore, in one of the images of the patient provided by the authors, several skin lesions are observed on the forehead as well as multiple lesions on the nose and nasolabial folds, suggestive of fibrous plaque and angiofibromas, respectively. [elsevier.es]

• Normal Hair

  –Typically normal hair in furrows but possibly thinner hair on folds. Infection may arise in deep furrows and cause subsequent malodor, erythema, and maceration. [dermatologyadvisor.com]

• Hand Enlargement

  Bone enlargement of the hands, knees, ankles and feet was also found. Secondary causes of pachydermoperiostosis were negative. [ncbi.nlm.nih.gov]

• Spastic Quadriplegia

  Language disorder was the next category with 18 cases (27.7%), chronic gastritis in 10 cases (15.38%), spastic quadriplegia in 8 cases (12.3%) and dyslipidemia in 8 cases (12.3%) In the medical record family abandonment was stated in 19 cases (29.23%) [scielo.org.co]

• Quadriplegia

  In 1962 Berg and Windrath-Scott described a man with mental retardation, CVG, and quadriplegia with onset at puberty (1). [scielo.org.co]

• Dysesthesia

  Neurocutaneous disease: Neurocutaneous dysesthesias. J Am Acad Dermatol, 74 (2016), pp. 215-228 [10] T.A. Kouwenhoven, P.C.M. van de Kerkhof, M. Kamsteeg. Use of oral antidepressants in patients with chronic pruritus: A systematic review. [actasdermo.org]

Based on the clinical feature and examination findings, acromegaly was suspected. 1–3 Endocrine workup revealed elevated serum growth hormone (48.1 ng.ml; reference range 0–30.0 ng/ml), unsuppressed post-glucose growth hormone (PGGH) (60 min PGGH, 35.2 [academic.oup.com]

The patient's disease improved after treatment and is currently stable. A year post treatment initiation scalp lesions developed. [scholars.direct]

Treatment is surgical, involving the excision of the most densely folded areas of skin and the tightening of the scalp in longitudinal and transverse directions. Surgical treatment is indicated when the patient requests it. [docteurmedarddechardon.co.uk]

Treatment[edit] The only current medical treatment for this condition is limited to plastic surgery with excision of the folds by means of scalp reduction/surgical resection. [en.wikipedia.org]

Treatment with capsaicin gel 0.075% was instated but the patient's condition worsened; oral gabapentin was then instated with no improvement, and both treatments were therefore suspended. [actasdermo.org]

Ya‐Jing Hsu, Ying‐Jui Chang, Lin‐Hui Su and Yu‐Ling Hsu, Using novel subcision technique for the treatment of primary essential cutis verticis gyrata, International Journal of Dermatology, 48, 3, (307-309), (2009). Ibrahim A. [doi.org]

The prognosis is benign. Other organ systems are spared and there is no malignant transformation of scalp tissue or brain parenchyma. [ncbi.nlm.nih.gov]

Cutis verticis gyrata may be disfiguring, but the process is essentially benign, and the prognosis is good[1]. [statpearls.com]

The prognosis is benign. Other organ systems are spared and there is no malignant transformation of scalp tissue or brain parenchyma. NEUROLOGY 1996;47: 573-575 Copyright 1996 by Advanstar Communications Inc. [n.neurology.org]

Treatment and prognosis Management depends on severity of the disease 2. In mild disease, good scalp hygiene is recommended to avoid accumulations of malodourous secretions in furrows 2. [radiopaedia.org]

Etiology The etiologies of cutis verticis gyrata can be classified as either primary or secondary 1-6: primary primary essential: no other associated anomalies primary non-essential: associated with neuropsychiatric or ophthalmologic anomalies secondary [radiopaedia.org]

Differentiation of primary and secondary forms of cutis verticis gyrata and currently recognized etiologies are also briefly reviewed. [ncbi.nlm.nih.gov]

Epidemiologic and clinical aspects”. Acta Med Scand. vol. 175. 1964. pp. 115-27. (Large analysis in Swedish population that provides epidemiologic, clinical, and genetic data concerning CVG.) Akesson, HO. [dermatologyadvisor.com]

Epidemiologic and clinical aspects. Acta med. scand., 175, 115. Google Scholar | Crossref | Medline Äkesson, H. O. (1965). Cutis verticis gyrata and mental deficiency in Sweden. Genetic aspects. Acta med. scand., 177, 459. [journals.sagepub.com]

However, a study of plasma levels of thyroid hormone, cortisol, sex hormone, and prolactin levels in 15 patients with cutis verticis gyrata did not show any abnormal hormone levels.[6] The possible role of genetic transmission of CVG is uncertain.[3][6] Epidemiology [statpearls.com]

Epidemiologic and clinical aspects. Acta med. scand., 1964, 175: 115. 2. Åkesson, H. O.: Cutis verticis gyrata and mental deficiency in Sweden II. Genetic aspects. Acta med. scand., 1965, in press. 3. McDowall, T. [cambridge.org]

Epidemiologic and clinical aspects. Acta Med Scand. 1964 Jan. 175:115-27. [Medline]. Akesson Ho. Cutis verticis gyrata and mental deficiency in Sweden. II. Genetic aspects. Acta Med Scand. 1965 Apr. 177:459-64. [Medline]. MacGillivray RC. [emedicine.medscape.com]

Author information 1 Pediatric Highly Intensive Care Unit, Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy. 2 Division of Neuroradiology, Fondazione [ncbi.nlm.nih.gov]

A hormonal influence has been postulated as this disorder usually manifests in postpubertal men.[5][3] Secondary cutis verticis gyrata is considered to be a manifestation of a variety of underlying causes, and the pathophysiology, in this case, can be [statpearls.com]

^ "Cutis Verticis Gyrata: Background, Pathophysiology, Etiology". February 2019. ^ Unna PG. Cutis verticis gyrata. Monatschr Prakt Derm. 1907. 45:227-33. ^ Levine, Norman (2004). Dermatology Therapy A-Z Essentials. [en.wikipedia.org]

Etiology Pathophysiology In primary CVG, the etiology and pathophysiology are unknown; however, primary lesions may be influenced by genetic and/or endocrinologic factors such as X-linked inheritance and increased utilization of testosterone in peripheral [dermatologyadvisor.com]

[…] without monoclonal gammopathy [34] Supernumerary X chromosome syndromes (including Klinefelter syndrome) [35] Systemic T-cell lymphoma [36] Turner syndrome Vemurafenib and whole-brain radiotherapy combination treatment in melanoma patients [37, 38] Pathophysiology [emedicine.medscape.com]

Early diagnosis is extremely important to prevent the development of malignant melanoma. [ncbi.nlm.nih.gov]

Surgery is the morphological treatment of choice for cutis verticis gyrata, but does not prevent the continued progression of the disease. [docteurmedarddechardon.co.uk]

The surgical technique must spare the scalp vascularization to prevent healing complications and to allow for a subsequent surgery if needed. [cureus.com]

Local scalp hygiene is important to avoid secretion accumulation, maceration, unpleasant odor, and also to prevent secondary infections[3][4][5] Primary cutis verticis gyrata is a benign cutaneous disorder. [statpearls.com]

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs [emedicine.medscape.com]