A Candidate Syndromic Triad of Acne Keloidalis Nuchae, Dissecting Cellulitis, and Secondary Cutis Verticis Gyrata

Acne keloidalis nuchae (AKN) affects the posterior hairline/nape; dissecting cellulitis of the scalp (DCS) is a suppurative scarring alopecia; and cutis verticis gyrata (CVG) describes scalp folds that may arise from chronic inflammation. This small retrospective, hypothesis-generating case series describes a clinicopathologically corroborated constellation of AKN, scalp-wide DCS, and extensive secondary CVG.

At one Los Angeles hair clinic, 12 consecutive men evaluated for suspected or confirmed DCS from December 2022 through November 2024 underwent full-scalp and posterior hairline/nape examination with trichoscopy. Triad eligibility required clinicopathologically confirmed AKN, clinicopathologically confirmed DCS involving at least 3 scalp zones, and clinically extensive CVG involving at least 3 zones. Biopsy compartments were standardized, while exact sites were selected by clinician judgment using morphology and trichoscopy.

Three patients met prespecified criteria (age range, 30-40 years; 2 Hispanic and 1 African American). This referral-enriched case-ascertainment observation is not a prevalence estimate. All had class II or III tumorous AKN, draining boggy nodules with scarring alopecia, and extensive cerebriform folds. Posterior hairline symptoms preceded diffuse scalp inflammation and furrowing by approximately 1-2 years. Nuchal and scalp biopsies supported AKN and DCS, respectively; clinically normal-appearing scalp biopsies showed perifollicular infundibulo-isthmic lymphocytic inflammation with fibrosis.

The AKN-DCS-CVG constellation may represent a candidate syndromic triad characterized by high inflammatory burden and extensive fibrotic scalp remodeling. Recognition should prompt reciprocal examination of the full scalp and posterior hairline/nape. Multicenter studies are needed to validate the constellation and define its epidemiology, chronology, and clinical outcomes.