A case of cutis verticis gyrata developing in a patient with primary scarring alopecia: A unique presentation of a rare disorder

A 39-year-old female with a medical history including hydronephrosis, kidney stones, hyperlipidemia, anxiety, and gastroesophageal reflux disease, presented to the dermatology clinic with complaints of scalp tenderness and pruritus with associated vertex hair loss. The patient's hair loss began 13 years prior, and she subsequently developed scalp undulations a few years later, which progressed in prominence over time. Scalp pruritus and tenderness within these undulations developed 3 years prior to presentation to our clinic. She was of normal intelligence; denied any history of ocular disease, scalp injuries, endocrine disorders, or tumors. The patient’s current medications include fluticasone propionate, cetirizine HCl, and semaglutide. She denied a family history of autoimmune diseases and hair loss was experienced by her father only. On examination she was found to have 2 adjacent linear deep furrows running anterior to posterior, forming a single undulation from the mid-scalp to the right crown, traversing the area of hair sparsity. These undulations were palpable to touch and visible on magnetic resonance imaging of the scalp (Fig 1). On trichoscopic examination, this area also exhibited evidence of follicular dropout, perifollicular scaling, and erythema (Fig 2), with additional scalp bogginess in the surrounding area. Differential diagnoses were cutis verticis gyrata (CVG), linear morphea, and cutis laxa, as well as a scarring alopecia.Fig 2Trichoscopy/Dermoscopy findings of follicular dropout, perifollicular scaling, and erythema.View Large Image Figure ViewerDownload Hi-res image Download (PPT) A 4-mm punch biopsy from the right crown revealed a gently papillated and hyperplastic epidermis with multiple dilated and compounded follicular infundibula opening up into the surface, which is suggestive of CVG (Fig 3). Additionally, polytrichia with perifollicular fibrosis was present most consistent with a cicatricial alopecia (Fig 4). The histologic differential included lichen planopilaris, central centrifugal cicatricial alopecia, as well as folliculitis decalvans.Fig 410× Histology: Follicular units show fusion of hair follicles (follicular bigeminy and trigeminy) with prominent perifollicular fibrosis and a surrounding infiltrate characterized by lymphohistiocytic infiltrate with scattered neutrophils.View Large Image Figure ViewerDownload Hi-res image Download (PPT) The patient was treated with topical clobetasol 0.05% 5 times a week as well as intralesional triamcinolone 5 mg/mL, 1 mL, repeated every 1-3 months. The patient was also started on oral minoxidil 0.625 mg daily for 1 month and increased to 1.25 mg daily thereafter. In addition to the scalp biopsy, other interventions included a chest x-ray to rule out lung cancer, a complete blood count, thyroid stimulation hormone levels, and thyroid peroxidase antibody. All of the results were normal. CVG is primarily observed in men, with a prevalence of approximately 1 in 100,000 males and 0.026 in 100,000 females.1Shareef S. Horowitz D. Kaliyadan F. Cutis verticis gyrata.in: StatPearls. StatPearls Publishing, 2022Google Scholar The pathogenesis of CVG remains uncertain, though a hormonal etiology has been postulated due to the male predominance and postpubertal onset of the disorder.1Shareef S. Horowitz D. Kaliyadan F. Cutis verticis gyrata.in: StatPearls. StatPearls Publishing, 2022Google Scholar The various presentations of the 3 categories of CVG are summarized in Table I. CVG-Intellectual Disability and secondary CVG have been linked to ophthalmological abnormalities, neurologic disease, and mental deficiency.1Shareef S. Horowitz D. Kaliyadan F. Cutis verticis gyrata.in: StatPearls. StatPearls Publishing, 2022Google Scholar Secondary CVG is caused by underlying systemic inflammatory processes such as eczema and psoriasis. Primary scarring alopecias are rarely reported in association with CVG. There is 1 report of a patient with CVG, folliculitis decalvans, and acne keloidalis nuchae (AKN).3Alonso Pereira L. Teixeira M. Andrade J. et al.An overlap of secondary cutis verticis gyrata, folliculitis decalvans, folliculitis keloidalis nuchae and the use of dreadlocks: the role of inflammation due to traction.Skin Appendage Disord. 2016; 2: 130-134Crossref PubMed Scopus (4) Google Scholar The authors note all 3 conditions are associated with dermal collagen thickening which may result from fibroblast growth factor activation. A database study conducted over 11 years from July 2009 to November 2020 involving a total of 108 patients with AKN at a dermatology clinic in Los Angeles revealed a higher incidence of CVG in patients with AKN. This study reported a CVG incidence rate of 3.7% amongst patients diagnosed with AKN, which is significantly higher than the 0.001% incidence rate observed in the general population.4Umar S. Lullo J.J. Carter M.J. et al.Acne keloidalis nuchae is associated with cutis verticis gyrata.Clin Cosmet Investig Dermatol. 2022; 15: 1421-1427Crossref PubMed Scopus (3) Google Scholar Another report is of a patient with scarring alopecia and pachydermoperiostosis, which is associated with CVG although the patient did not exhibit CVG.5Anansiripun P. Suchonwanit P. A case of progressive thickening and furrowing of facial skin and scalp with scarring alopecia.Skin Appendage Disord. 2021; 7: 237-242Crossref PubMed Scopus (1) Google Scholar There is a case of CVG concurrent with alopecia areata.6Yoo K.H. Lee J.W. Jang W.S. et al.Cutis verticis gyrata and alopecia areata: a synchronous coincidence?.Yonsei Med J. 2010; 51: 612-614Crossref PubMed Scopus (11) Google Scholar The authors postulate a possible genetic association as there is evidence of a mutation in the FGFR2 gene which may be involved in the development of CVG and this gene is located on a chromosome that has been linked with alopecia areata.Table IDiagnostic findings of CVG and cicatricial alopeciaEpidemiology/clinical historyPhysical examinationTrichoscopy/dermoscopyPathologic featuresCVG- Primary essential CVG1Shareef S. Horowitz D. Kaliyadan F. Cutis verticis gyrata.in: StatPearls. StatPearls Publishing, 2022Google ScholarMen > WomenPostpubertal men before the age of 30Idiopathic and typically no associated abnormalitiesSymmetric, redundant scalp folds that exhibit deep furrows and convolutions, and the appearance mimicking that of cerebral gyri, furrows are typically anterior to posteriorVariableRange from normal skin architecture to thickened connective tissue with hypertrophy or hyperplasia of adnexal structures with or without an associated increase in collagen fibers. The folds in secondary CVG are that of the underlying disease process.Epidermal hypertrophy and hyperplasiaCVG- Intellectual disability (CVG-ID)1Shareef S. Horowitz D. Kaliyadan F. Cutis verticis gyrata.in: StatPearls. StatPearls Publishing, 2022Google ScholarUsually associated with neurological and ophthalmological disordersFolds may be more asymmetricalSecondary CVG1Shareef S. Horowitz D. Kaliyadan F. Cutis verticis gyrata.in: StatPearls. StatPearls Publishing, 2022Google ScholarMay be associated with hormonal and inflammatory disease processesCharacteristics of underlying disease processPrimary Cicatricial alopecia2Bagherani N. Hasić-Mujanović M. Smoller B. et al.Disorders of hair.in: Smoller B. Bagherani N. Atlas of dermatology, dermatopathology and venereology: inflammatory dermatoses. Springer International Publishing, 2022: 669-742Crossref Google ScholarPrevalence of approximately 7%CCCA and FD have higher prevalence in those of African descentCCCA more common in womenFD more common in menWell-circumscribed, areas of hair loss on the scalp. The hair loss may be accompanied by itching, burning, and pain. The affected area may also have a shiny, smooth appearance.Broken hairs, perifollicular erythema, and white scalesPeritubular hyperkeratosisPerifollicular fibrosis, destruction of the hair follicle, dilated eccrine glands, and a lymphocytic or neutrophilic infiltrate.CCCA, Central centrifugal cicatricial alopecia; CVG, cutis verticis gyrata; FD, folliculitis decalvans. Open table in a new tab CCCA, Central centrifugal cicatricial alopecia; CVG, cutis verticis gyrata; FD, folliculitis decalvans. An increased understanding of the pathogenesis of CVG is necessary to determine possible associations with alopecia, including primary scarring alopecia. Dr Shapiro is a consultant for Aclaris Therapeutics, Incyte, and Replicel Life Sciences. Drs Shapiro and Lo Sicco have been investigators for Regen Lab and are investigators for Pfizer. Dr Lo Sicco is a consultant for Pfizer and Aquis. MGB, LA, EK, CO, RHK, and EAR have no conflicts to disclose. We would like to extend our appreciation to Sammy Leung, the Dermatopathology Manager at NYU Langone, for obtaining the histology slides for our publication.

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