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. 2023 Aug 25:16:2315-2327.
doi: 10.2147/CCID.S422310. eCollection 2023.

Unveiling a Shared Precursor Condition for Acne Keloidalis Nuchae and Primary Cicatricial Alopecias

Sanusi Umar  1   2   3 , Donna Ton  3 , Marissa J Carter  4 , Paul Shitabata  1   2   5
Affiliations

Unveiling a Shared Precursor Condition for Acne Keloidalis Nuchae and Primary Cicatricial Alopecias

Sanusi Umar et al. Clin Cosmet Investig Dermatol. .

Abstract

Purpose: Small observational studies suggest subclinical disease occurrence in the normal-appearing scalp zones of several primary cicatricial alopecias. To aid patient management, we began routinely evaluating the entire scalp of patients with acne keloidalis nuchae (AKN), including trichoscopy-guided biopsies.

Patients and methods: This retrospective study evaluated 41 patients sequentially presenting with AKN at a single clinic between June and December 2022. Primary lesions and normal-appearing scalp in the superior parietal scalp at least 5 cm away from AKN-affected zones were clinically evaluated, and areas showing perifollicular erythema or scales/casts on trichoscopy were biopsied and histologically analyzed.

Results: Forty-one men with AKN, including 20 men of African descent, 17 Hispanic, and 4 European-descended Whites, were evaluated. All patients, including 22% with associated folliculitis decalvans, showed scalp-wide trichoscopy signs of perifollicular erythema or scaling in normal-appearing scalp areas. All patients showed histologic evidence of perifollicular infundibulo-isthmic lymphocytoplasmic infiltrates and fibrosis (PIILIF), with 96% showing Vellus or miniaturized hair absence. PIILIF was often clinically mistaken for seborrheic dermatitis (44-51%). All White patients had mild papular acne keloidalis nuchae lesions mistaken for seborrheic dermatitis.

Conclusion: PIILIF may be a precursor to a wide spectrum of primary cicatricial alopecias, including AKN and folliculitis decalvans. This finding carries implications for the early diagnosis and management of AKN and other primary cicatricial alopecias.

Keywords: central centrifugal cicatricial alopecia; folliculitis decalvans; frontal fibrosing alopecia; lichen planopilaris; scarring alopecia; subclinical.

Plain language summary

Acne keloidalis nuchae (AKN) is a type of hair loss and scalp condition marked by scarring and inflammation. This condition falls under a group of chronic hair and scalp issues known as primary cicatricial alopecia (PCA). Current treatments for AKN and similar PCAs often do not work well, and the condition tends to return. We have found a hidden scalp condition that could be causing AKN and other PCAs. It’s a subtle disease that affects the entire scalp, even though it might not show noticeable symptoms. We have observed this condition in all 41 AKN patients in our study, and it’s characterized by certain changes in the hair and scalp’s structure and immune system response. Other studies have linked this condition to various other PCAs. We believe this hidden condition could be causing AKN and making it come back after treatment. This study suggests that treating AKN might require a broader approach beyond just treating the visible symptoms. Since this hidden condition exists in other PCAs, it might be a common cause.

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Conflict of interest statement

Sanusi Umar owned shares and issued patents and patent applications in FineTouch Laboratories Inc. and Dr. U Devices Inc. at the time of this work. The other authors declare that they have no conflicts of interest in this work.

Figures

Figure 1
Figure 1
(A) Posterior view of a patient with histopathologically proven diagnosis of AKN in the nape area (red arrow) and FD in the right vertex area (Blue arrow). There is a vast zone of NAS outside of the AKN and FD zones. (B) Same patient showing AKN (nape – red arrow) and FD (right vertex – blue arrow) and a marked parietal NAS area targeted for biopsy. There is a vast zone of NAS outside of the AKN and FD zones.
Figure 2
Figure 2
(A) Posterior view of a patient with histopathologically proven diagnosis of AKN in the nape area (red arrow) and FD in the entire vertex area (Blue arrow). There is a vast zone of NAS outside of the AKN and FD zones. (B) Posterior view of a patient with histopathologically proven diagnosis of AKN in the nape area. There is a vast zone of NAS outside of the AKN zone.
Figure 3
Figure 3
(A) Typical trichoscopy finding in the NAS of patients with AKN showing perifollicular scales (Blue arrow) and erythema (Red Arrow), manifesting as hyperpigmentation in a Black African patient. The blue ink mark indicates the biopsy site. (B) Typical trichoscopy finding in the NAS of patients with AKN showing perifollicular scales (Blue arrow) and erythema Red arrow) in a nonblack Hispanic patient. The blue ink mark indicates the biopsy site.
Figure 4
Figure 4
(A) Hematoxylin and eosin stain of the vertical section of an NAS biopsy specimen showing PIILIF. Shows a follicle with lichenoid inflammatory cell infiltrate composed of lymphocytes and plasma cells effacing the dermal-epidermal junction at the level of the infundibulum (Red arrow). There is basal squamatization associated with a Max-Josef space (Green arrow). The interfollicular epidermis is spared of inflammation. Perifollicular and interfollicular fibrosis is present. No sebaceous glands or Vellus or miniaturized hairs are present. Magnification 4x. (B) Hematoxylin and eosin stain of the horizontal section of the same NAS biopsy specimen showing PIILIF. Shows perifollicular inflammatory cell infiltrate composed of lymphocytes and plasma cells effacing the dermal-epidermal junction (Red arrow), basal desquamatization associated with a Max-Josef space (Green arrow), and perifollicular fibrosis (Blue arrows). Magnification 4x.
Figure 5
Figure 5
(A) Trichoscopy of normal-appearing beard area of a patient with AKN showing perifollicular and interfollicular erythema Red arrows) and scales (Blue arrows). (B) Hematoxylin and eosin staining of trichoscopy guided-normal appearing in the beard area of a patient with AKN showing PIILIF. A horizontally sectioned follicle with a scant lichenoid inflammatory cell infiltrate composed of lymphocytes and plasma cells, effacing the dermal-epidermal junction associated with perifollicular fibrosis (Blue arrows). Premature desquamation associated with a Max-Josef space (Green arrow) is visible. No Vellus or miniaturized hairs or sebaceous glands are present. Magnification 4x.
Figure 6
Figure 6
A European-descended White male showing scattered erythematous papules (Red arrows) in the nape area found to be AKN upon histologic analysis.
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