Introduction: Premature desquamation of the inner root sheath is described as a defining histologic feature of follicular degeneration syndrome/central centrifugal cicatricial alopecia; moreover, the immunological features of this disease are not well established.Case report: A 46-year-old African American female was evaluated for an asymptomatic scarring alopecia after using several chemicals on her hair. The clinical examination revealed visible, well defined patches of hair loss.Methods: Biopsies for hematoxylin and eosin examination, as well as for direct immunofluorescence and immunohistochemistry analysis were performed. We evaluated molecules involved in signaling of growth factor pathways (e.g. the Akts), specifically VEGF and Oct-4 to investigate involvement of these molecules in this disease. Hematoxylin and eosin staining demonstrated histopathologic findings of premature desquamation of the inner root sheath and eccentric thinning of the follicular epithelium, supporting the diagnosis of central centrifugal cicatricial alopecia. Direct immunofluorescence revealed strong depositions of IgG, Complement/C3 and fibrinogen around the multiple hair follicles and their supply vessels. Immunohistochemistry staining of the base of the hair follicle was seen with fibrinogen and Oct-4 antibodies. Immunohistochemistry also demonstrated increased expressions of VEGF around supply vessels of the hair follicle, as well as some overexpression of anti-human Akt-pS473 phosphorylation site specific antibody.Conclusions: Our immunologic findings suggest that the etiology of centrifugal cicatricial alopecia includes not only hair traction, but also a possible reactive immune response
