Scalp disorders may form distinct syndrome, small study suggests

Image courtesy Dr. U Hair and Skin Clinic

Three severe scalp conditions usually diagnosed separately may represent a single clinical syndrome, according to a small case series that could help physicians identify extensive disease earlier.

The study, published in Clinical, Cosmetic and Investigational Dermatology, linked acne keloidalis nuchae (AKN), dissecting cellulitis of the scalp (DCS), and secondary cutis verticis gyrata, or CVG. AKN produces inflammatory papules and thick scars at the nape, while DCS is marked by painful or draining nodules and cicatricial alopecia. CVG causes deep scalp folds and furrows.

Researchers at a Los Angeles clinic reviewed 12 consecutive males with suspected or confirmed DCS from Dec. 2022 through Nov. 2024. They underwent scalp and nape examinations, trichoscopy, and multisite biopsies.

Three men, aged 30 to 40 years, met predefined criteria for the proposed triad: class II or III tumorous AKN, DCS affecting at least three scalp zones and CVG in at least three zones. Each reported nape disease first, followed one to two years later by widespread inflammation and furrowing.

“These conditions can be diagnosed and treated separately, so their relationship may be missed,” said the lead author, Dr. Sanusi Umar, in a press release. “All three showed the same severe pattern across the same scalp regions, reported the same sequence of onset, and had related biopsy findings. Recognizing it may help patients reach specialist care sooner and help clinicians assess the whole scalp.” Dr. Umar is head of Scalp and Hair Disorders at Harbor-UCLA Medical Center and founder and medical director of Dr. U Hair and Skin Clinic in Manhattan Beach, Calif.

Biopsies supported AKN at the nape and DCS elsewhere. Normal-appearing scalp in all three patients showed perifollicular infundibulo-isthmic lymphocytic inflammation and fibrosis, or PIILIF, involving inflammation and early fibrosis around the upper hair follicle. AKN, DCS, and unaffected-appearing tissue showed similar immune-cell profiles, including more CD4-positive than CD8-positive T cells and prominent CD117-positive mast cells.

A similar, though unconfirmed, pattern in one patient’s father raised the possibility of shared susceptibility.

The findings do not establish a syndrome, given the study’s size and single-clinic design. But new scalp folds or widespread inflammatory lesions in patients with AKN should prompt a full-scalp examination for DCS. Clinicians treating DCS should likewise examine the nape for AKN, potentially using trichoscopy-guided multisite biopsies to clarify the diagnosis and reduce the risk of permanent scarring and disfigurement.