Isolated cutis verticis gyrata of the glabella and nasal bridge: A case report and review of the literature

doi:10.1016/j.bjps.2013.01.040

Journal of Plastic, Reconstructive & Aesthetic Surgery

Volume 66, Issue 10, October 2013, Pages 1421-1423

https://doi.org/10.1016/j.bjps.2013.01.040 Get rights and content

Summary

Cutis verticis gyrata (CVG) is an uncommon morphological condition of the scalp characterised by ridges and furrows resembling the surface of the brain. We present a case of secondary CVG affecting both the glabella and nasal aesthetic unit. To our knowledge, isolated CVG of the glabella has never been reported nor has extension of CVG onto the nasal aesthetic unit of the face. We also describe an association between hyper-IgE syndrome and CVG, and review this rare disorder including options for its surgical management.

Section snippets

Case report

A 21-year-old male with a background of hyper-IgE syndrome was referred to our Unit with a chronic frontal headache associated with glabella skin changes (Figure 1). His additional past medical history included bronchiectasis secondary to hyper-IgE syndrome, including a right upper lobectomy for pulmonary abscess. He also gave a history of recurrent cutaneous and ear infections, and was on long term antibiotic and antifungal prophylaxis. The patient had no history of neuropsychiatric or

Discussion

CVG is a rare scalp condition characterised by convoluted folds and deep furrows of that resemble the surface of the cerebral cortex.¹ Usually, 2–30 folds can exist within 0.5–2.0 cm width, and the depth of furrow is about 1 cm.² The estimated prevalence of CVG is 1 in 100,000 in the male population and 0.026 in 100,000 in the female population.³ CVG is classified in two forms: ‘primary’ (subdivided into essential and non-essential cases) and ‘secondary’.⁴ The primary form of CVG is

Conclusions

CVG is traditionally described as an uncommon disorder of the scalp. However, we present a case of CVG involving both the forehead and nasal aesthetic units of the face indicating that there is a potential for facial involvement in CVG. We also describe an apparent association between hyper-IgE syndrome and CVG.

Conflict of interest statement

The authors declare that there is no source of financial or other support, or any financial or professional relationships which may pose a competing interest.

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There are more references available in the full text version of this article.

Cited by (16)

Acromegaly-related cutis verticis gyrata
2022, Growth Hormone and IGF Research
Citation Excerpt :
Here, we review acromegaly-related CVG, aiming to raise awareness for the possible occurrence of CVG in acromegaly, and we analyze its pathophysiology, presentation, management and differential diagnosis. CVG, also known by the terms paquidermia verticis gyrata, cutis verticis plicata, and “bulldog” scalp syndrome, is a rare benign skin lesion characterized by thickened and folded skin of the scalp and/or forehead and face, with furrows of up to 1 cm depth and folds ranging between 0.5 and 2 cm width, resembling the brain gyri and sulci [8,14–19]. This pattern seems to be related with the mechanical properties of the galea aponeurotica, a tough layer of dense fibrous tissue covering the cranium, thus limiting soft tissue expansion and leading to an uneven growth [20].
Acromegaly is a disorder caused by hypersecretion of growth hormone (GH), resulting in excessive levels of insulin-like growth factor 1 (IGF-1), and almost always due to a pituitary tumor. It is classically associated with acral enlargement, prominent facial features and soft tissue overgrowth. Skin manifestations include hirsutism, acne, skin tags, oily skin and acanthosis nigricans. However, other uncommon dermatological features, such as cutis verticis gyrata (CVG), may also occur. Here, we review acromegaly-related CVG aiming to raise awareness for its possible occurrence in this setting, and we discuss its pathophysiology, presentation, management and differential diagnosis.
A comprehensive literature search regarding CVG, particularly CVG related to acromegaly, has been carried out. Case reports, original studies and review papers, were considered.
CVG is a rare benign skin lesion characterized by thickened and folded scalp, resembling the brain gyri and sulci. The diagnosis of CVG mainly relies on clinical examination, although tissue biopsy may be necessary in case of uncertain etiology. In acromegaly, CVG appears to be driven by the trophic effects of GH and IGF-1 on skin and soft tissues. While CVG is uncommon in acromegaly, it seems to occur more frequently in male patients. The management of acromegaly-related CVG essentially relies on controlling the serum levels of GH and IGF-1. Surgical skin procedures should be reserved for patients with severe aesthetic distress, after achieving the best possible control of acromegaly.
CVG is a rare manifestation of acromegaly that may allow an earlier diagnosis and a swifter treatment of these patients, which in turn may improve or entirely reverse such remarkable skin lesions.
Cutis verticis gyrata: a cutaneous finding in acromegaly
2022, Anais Brasileiros de Dermatologia
Acromegaly is a rare disease characterized by changes in the bone and soft tissue systems, induced by excess growth hormone and insulin-like growth factor type 1. Among the skin lesions associated with acromegaly is cutis verticis gyrata, an hypertrophic, and coarse folding of the skin of the scalp, an association of uncommon incidence and unknown prevalence. This case report describes the case of a patient diagnosed with acromegaly at age 60 with previously unidentified cutis verticis gyrata. This report aims to review the literature on cutis verticis gyrata and its unusual association with acromegaly.
Cutis verticis gyrata and acromegaly
2015, Presse Medicale
Butterfly-shape scalp excision: A single stage surgical technique for cutis verticis gyrata
2014, Journal of Plastic, Reconstructive and Aesthetic Surgery
Congenital Cutis Verticis Gyrata in a Newborn with Turner Syndrome: A Rare Clinical Manifestation of This Chromosomal Disease with Trichoscopic Evaluation
2023, Diagnostics
Cutis verticis gyrata: Two cases associated with drug-resistant epilepsy
2020, Clinical Case Reports

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Case reportIsolated cutis verticis gyrata of the glabella and nasal bridge: A case report and review of the literature

Summary

Section snippets

Case report

Discussion

Conclusions

Conflict of interest statement

J Plast Reconstr Aesthet Surg

J Plast Reconstr Aesthet Surg

Curr Opin Immunol

Cutis verticis gyrata: three cases with different aetiologies that demonstrate the classification system

Australas J Dermatol

Treating cutis verticis gyrata using skin expansion method

Cell Biochem Biophys

Congenital primary cutis verticis gyrata

Plast Reconstr Surg

Case report
Isolated cutis verticis gyrata of the glabella and nasal bridge: A case report and review of the literature