Nkechi Anne Enechukwu1, Gabriel Olabiyi Ogun2, Ogochukwu Ifeanyi Ezejiofor1, Adebola Olufunmilayo Ogunbiyi3, Lidia Rudnicka4. 1. Department of Internal Medicine, Dermatology Unit, Nnamdi Azikiwe University, Nnewi Campus, Nnewi, Anambra, Nigeria. 2. Department of Pathology, University of Ibadan, Oyo State, Nigeria. 3. Department of Medicine, University of Ibadan, Oyo State, Nigeria. 4. Department of Dermatology, Warsaw University of Medicine, Warsaw, Poland.
Dear Editor,Central centrifugal cicatricial alopecia (CCCA) is the most common cause of scarring central scalp alopecia among black females.[1] It is reportedly rare in Males of African descent.[2] Earlier reports had suggested that the use of hot combs and hair relaxers by African women were important pathogenetic factors in its development and also explained its prevalence in this group when compared with men.[3] However, genetic mutations in PADI3, a gene encoding an enzyme that modifies other proteins that are necessary for normal hair-shaft formation has been linked to CCCA.[4]A 31-year-old male presented with 8 years' history of progressive hair loss, which started at the crown and later progressively expanded to other areas of the scalp associated with pain, occasional itching, pustules on the scalp, increased hair shedding, and breakage. The symptoms were worse following haircuts. He did not use chemical hair relaxers, dyes, or any other form of hair processing methods. He had regular haircuts every 3–4 weeks with an electric clipper. There is a family history of asymptomatic central scalp alopecia (maternal grandfather and paternal uncle). Examination revealed multiple shiny areas of patchy alopecia devoid of follicular openings with few scattered papules on the vertex sparing the occipital area [Figure 1].
Figure 1
(a) Vertex of the index patient showing multiple shiny areas devoid of hair follicle openings. (b) Left Temporal scalp region of the index patient. (c) Occipital scalp region of the index patient
(a) Vertex of the index patient showing multiple shiny areas devoid of hair follicle openings. (b) Left Temporal scalp region of the index patient. (c) Occipital scalp region of the index patientTrichoscopy [Figure 2a and b] showed peripilar gray halos, honeycomb pigmentation interrupted by areas of hyperpigmentation; fibrotic and pinpoint white dots, white areas of fibrosis, multiple single hair follicle units with wide interfollicular distance, minimal perifollicular scale, and barely visible erythema.
Figure 2
(a and b) showing peripilar gray halos, honeycomb pigmentation occasionally interrupted by scattered areas of hyperpigmentation; fibrotic white dots, some pinpoint white dots, white areas of fibrosis, multiple single hair follicle units with a wide interfollicular distance, minimal perifollicular scale, and barely visible erythema
(a and b) showing peripilar gray halos, honeycomb pigmentation occasionally interrupted by scattered areas of hyperpigmentation; fibrotic white dots, some pinpoint white dots, white areas of fibrosis, multiple single hair follicle units with a wide interfollicular distance, minimal perifollicular scale, and barely visible erythemaHistology showed severely reduced follicular density with perifollicular fibrosis and absent inner root sheath [Figure 3].
Figure 3
(a) severe reduced follicular density with few follicles present with perifollicular fibrosis (blue arrows), hematoxylin and eosin (×40). These surviving follicles show no inner root sheath. (b) The marked perifollicular fibrosis (blue arrow), hematoxylin and eosin (×100). (c) A single focus of preservation of the multilobular sebaceous glands “hugging” a vellus follicle in the middle and adjacent naked hair shaft (red arrow), hematoxylin and eosin (×100). (d) Note the presence of naked hair shafts in the dermis (red arrows), hematoxylin and eosin (×40)
(a) severe reduced follicular density with few follicles present with perifollicular fibrosis (blue arrows), hematoxylin and eosin (×40). These surviving follicles show no inner root sheath. (b) The marked perifollicular fibrosis (blue arrow), hematoxylin and eosin (×100). (c) A single focus of preservation of the multilobular sebaceous glands “hugging” a vellus follicle in the middle and adjacent naked hair shaft (red arrow), hematoxylin and eosin (×100). (d) Note the presence of naked hair shafts in the dermis (red arrows), hematoxylin and eosin (×40)A diagnosis of CCCA was made. He was given monthly intralesional triamcinolone; doxycycline and topical clobetasol counseled to increase intervals between haircuts and avoid close shaves. He had a significant reduction in symptoms with some hair regrowth during the subsequent visit.Diagnosis of CCCA in males is often missed or delayed (as in the index patient) as central scalp alopecia is often attributed to androgenetic alopecia. CCCA classically presents as central scarring alopecia, which starts on the vertex and spreads centrifugally to the rest of the scalp; hence, its close clinical resemblance to male pattern hair loss (MPHL) which often presents as central scalp hair loss.[5] This creates a diagnostic difficulty which can easily lead to a misdiagnosis of CCCA as MPHL.[5] Trichoscopy is useful in differentiating between CCCA and MPHL. Distinguishing trichoscopic features include the absence of peripilar halo, miniaturization of the hair shaft, hair shaft variability, and intact pigmentary network.Where a history of hair breakage (an early clue to CCCA) cannot be ascertained, especially in African males who shave their hair regularly, a history of worsening symptoms following haircuts as in the index patient rules out androgenetic alopecia as the cause of alopecia.The role of hair shaving practices in the progression of CCCA in males should also be investigated.Central scalp alopecia in males is commonly attributed to androgenetic alopecia. A family history of balding in males does not exclude other causes of alopecia in men. Symptomatic alopecia in men should be investigated. Modified hair shaving practices are a useful adjunct in the treatment of CCCA in males. Clinical practitioners should always have a high index of suspicion of CCCA in males with symptomatic central scalp alopecia.
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Authors: Liron Malki; Ofer Sarig; Maria-Teresa Romano; Marie-Claire Méchin; Alon Peled; Mor Pavlovsky; Emily Warshauer; Liat Samuelov; Laura Uwakwe; Valeria Briskin; Janan Mohamad; Andrea Gat; Ofer Isakov; Tom Rabinowitz; Noam Shomron; Noam Adir; Michel Simon; Amy McMichael; Ncoza C Dlova; Regina C Betz; Eli Sprecher Journal: N Engl J Med Date: 2019-02-13 Impact factor: 91.245