Congenital alopecia areata: a systematic review

Alopecia areata describes nonscarring, generally patchy, hair loss which can affect the entire scalp (alopecia totalis) and body (alopecia universalis).1, 2 Although the exact pathogenesis is not yet understood, alopecia areata is considered an acquired autoimmune condition.1, 2 Prevalence of alopecia areata peaks between ages 10 and 30, and younger age at presentation is generally considered a poor prognostic factor.1 However, cases of congenital alopecia areata have been reported.1, 3 Diagnosing congenital alopecia areata is difficult, as many other conditions should be ruled out including, but not limited to, tinea capitis, neonatal telogen effluvium, triangular alopecia, atrichia congenita, Marie-Unna hypotrichosis, and traction alopecia.1, 3 However, proper diagnosis of congenital alopecia areata is important, as treatment is possible.1-3 Therefore, this study aimed to analyze data from existing congenital alopecia areata reports. Following PRISMA guidelines,4 Web of Science and PubMed were searched, and cases of alopecia areata present at birth were included (Figure 1). Twelve congenital alopecia areata patients were identified, with a female predominance (7/12; 58.3%). Three were premature (25.0%). Alopecic pattern at birth was alopecia areata in eight cases (66.7%), alopecia totalis in two (16.7%), and alopecia universalis in two (16.7%). Positive family history of alopecia areata was reported in five cases (42.7%). Nail changes were noted in three patients (25.0%). Congenital alopecia areata diagnosis was clinical in eight cases (66.7%) and biopsy-proven in four (33.3%). Nine patients received/reported treatment (75.0%). Of those treated with topical corticosteroid alone (3/9; 33.3%), two demonstrated total hair regrowth (66.7%), while one progressed to alopecia totalis (33.3%). Two patients were treated with topical corticosteroid and a topical vasodilator, minoxidil (22.2%). One experienced total hair regrowth, while the other demonstrated partial hair regrowth, until the topical corticosteroid was discontinued (i.e. minoxidil was used alone), and alopecia totalis resulted. One patient was treated with topical corticosteroid and oral antihistamine (11.1%), and one was treated with placebo lotion (11.1%). Both experienced partial hair regrowth. Two patients were treated with oral pulse steroid, topical immunomodulator, and minoxidil, but no follow-up was provided (22.2%). Results are summarized in Table 1. Limitations of this review include small sample sizes and the possibility of missed cases due to lack of well-established terminology and underreporting. Additionally, biopsy is not necessary for alopecia areata diagnosis1 but is helpful in confirming unique (e.g. congenital) presentations of the condition. Given that alopecia areata is typically considered an acquired autoimmune condition, congenital cases call our understanding of alopecia areata into question and may provide additional insight into the underlying pathophysiology. Acquired alopecia areata and congenital alopecia areata seem to have many clinical/epidemiological similarities. Positive family history of alopecia areata in five cases suggests genetic factors are involved in the development of congenital alopecia areata. The female predominance of congenital alopecia areata is also in keeping with acquired alopecia areata.1, 3 Additionally, nail changes in 25.0% of congenital alopecia areata cases is similar to rates of nail changes typically seen in acquired alopecia areata.5 Finally, given that topical corticosteroids yielded partial or total hair regrowth in five of six patients who received them (83.3%), topical corticosteroids are a reasonable first-line treatment for congenital alopecia areata, as with acquired alopecia areata.1 In conclusion, congenital cases of alopecia areata may provide insight into the pathophysiology of the condition and should be considered on the differential diagnosis of alopecia in the newborn, thereby ensuring proper treatment of those affected.

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