Fibrosing Alopecia in a Pattern Distribution (FAPD) in 16 African-Descent and Hispanic Female Patients: A Challenging Diagnosis.

BACKGROUND: Fibrosing alopecia in a pattern distribution (FAPD) has only been described in Caucasian patients, and it is not clear whether it can develop in dark-skin ethnicities. MATERIALS AND METHODS: Sixteen Brazilian female patients, 12 of African descent and 4 Hispanic, with progressive scarring alopecia in a pattern distribution were analyzed. RESULTS: Dermatoscopic features showed perifollicular erythema and scaling (14/16), hair fiber diameter diversity (16/16), loss of follicular ostia (16/16), and follicular keratosis (3/16). Late stages showed a honeycomb pigmented network (12/16), a hyperpigmented perifollicular halo (12/16), and small white patches (12/16). Histopathological features showed lichenoid perifollicular infiltrate (14/16), follicular miniaturization (16/16), concentric fibrosis (16/16), perifollicular lymphocytic infiltrate (16/16), and vellus hair involvement (10/16). Premature desquamation of the inner root sheath was found in 11 patients. CONCLUSIONS: The concomitant findings of cicatricial pattern hair loss (with or without the recess of the front hair line), hair fiber diversity, perifollicular erythema and scaling, a whitish perifollicular halo, and histological findings of androgenetic alopecia, with vacuolar interface alteration of the upper portion of the follicular epithelium, are the main key features to suggest the diagnosis of FAPD. FAPD is a possible diagnosis in patients of color with cicatricial pattern hair loss. Clinical, dermatoscopic, and histopathological examination allow a proper final differential diagnosis.

Graft versus Host Disease Presenting as Fibrosing Alopecia in a Pattern Distribution: A Model for Pathophysiological Understanding of Cicatricial Pattern Hair Loss.

A case of cutaneous graft versus host disease (GvHD) presenting as fibrosing alopecia in a pattern distribution (FAPD) is discussed, possibly providing a mechanistic model for a better understanding of the pathogenic events underlying cicatricial pattern hair loss. The implication of a follicular inflammation and fibrosis associated with patterned hair loss has emerged from several independent studies. Eventually, Zinkernagel and Trüeb reported a peculiar type of cicatricial pattern hair loss with histopathological features consistent with lichen planopilaris (LPP) associated with androgenetic alopecia (AGA). With regard to its pathogenesis, LPP is regarded to constitute a T-cell-mediated autoimmune reaction. An as yet unknown antigenic stimulus from the malfunctioning hair follicle may initiate a lichenoid tissue reaction that triggers apoptosis of the follicular epithelial cells in the susceptible individual. GvHD is a complication following allogeneic tissue transplantation and is induced and maintained by immunocompetent cells from the donor tissue that particularly attack epithelia of fast-proliferating tissues in the recipient. Due to its analogies with lichen planus, GvHD constitutes a valid immunologic model for lichen planus, LPP and ultimately FAPD. Specifically, the presentation of GvHD of the scalp combines features of AGA and of LPP, as originally proposed in earlier observations on permanent alopecia after bone marrow transplantation.

Familial Cicatricial Alopecia: Report of Familial Frontal Fibrosing Alopecia and Fibrosing Alopecia in a Pattern Distribution.

Frontal fibrosing alopecia (FFA) and fibrosing alopecia in a pattern distribution (FAPD) as originally reported by Kossard in 1994 and by Zinkernagel and Trüeb in 2000, respectively, represent two distinct patterns of cicatricial pattern hair loss. Both share a patterned distribution and histological evidence of a lichenoid follicular inflammation with fibrosis. FFA is characterized by a marginal alopecia along the frontotemporal hairline, and FAPD by a progressive alopecia of the centroparietal scalp. Since the original reports, evidence has accumulated that there exists considerable clinical overlap among FFA, FAPD, and lichen planopilaris, with coexistence of features of the three conditions within the same individual. Moreover, familial cases of FFA have been reported, pointing to a possible genetic background to the condition. Our observation of familial occurrence of FFA and FAPD in daughter and mother, respectively, further underscore a nosologic relationship between the two conditions with respect to both an androgenetic background and the (lichenoid) inflammatory reaction pattern.