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]

• Hepatosplenomegaly

  The present report describes a man with acute myelogenous leukemia (AML) whose course was complicated by cutis verticis gyrata (CVG), a mediastinal mass, neuropathies, and hepatosplenomegaly as a result of visceral involvement. [ncbi.nlm.nih.gov]

• Ulcer

  The CVG developed under systemic corticosteroid treatment of an ulcerative colitis. Corticosteroids were discontinued and a therapeutic attempt was made with isotretinoin. [ncbi.nlm.nih.gov]

• Lacrimation

  This report describes an unusual case of a neonate with giant congenital melanocytic nevi covering more than 80% of the body surface area, with cutis verticis gyrata and abnormal lacrimal glandular tissue rests in the epibulbar area. [ncbi.nlm.nih.gov]

• Hand Enlargement

  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]

• Long Arm

  The single locus on the long arm of chromosome 8 that showed homozygosity by descent comprised the gene responsible for Cohen syndrome (CS), VPS13B. We then sequenced VPS13B in the patients and found a homozygous splice site mutation. [ncbi.nlm.nih.gov]

• Vertigo

  The patient did not have a history of neuropsychiatric disorders and was generally in good health except for diabetes mellitus, hypertension, and benign positional vertigo. [dermatology.cdlib.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]

• Mediastinal Mass

  The present report describes a man with acute myelogenous leukemia (AML) whose course was complicated by cutis verticis gyrata (CVG), a mediastinal mass, neuropathies, and hepatosplenomegaly as a result of visceral involvement. [ncbi.nlm.nih.gov]

• Colitis

  The CVG developed under systemic corticosteroid treatment of an ulcerative colitis. Corticosteroids were discontinued and a therapeutic attempt was made with isotretinoin. [ncbi.nlm.nih.gov]

The CVG developed under systemic corticosteroid treatment of an ulcerative colitis. Corticosteroids were discontinued and a therapeutic attempt was made with isotretinoin. [ncbi.nlm.nih.gov]

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]

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]

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

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]

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

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]

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]

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]

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]

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]

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]