BH03: Systemic sclerosis and frontal fibrosing alopecia: a novel combination of scarring alopecia

B. Gaglani,¹ N. Rouhani,¹ C.M. Stefanato² and A. Martinez³

¹Department of Dermatology, Central Middlesex Hospital, London North West University Hospitals NHS Trust, London, UK; ²Department of Dermatopathology, St John’s Institute of Dermatology, Guy’s and St Thomas’ NHS Trust, London, UK; and ³Department of Dermatopathology, Central Middlesex Hospital, London North West University Hospitals NHS Trust, London, UK

A 43-year-old woman presented to dermatology with frontotemporal scarring alopecia extending to the occipital area. She had a 10-year history of systemic sclerosis diagnosed after the onset of hair loss, with associated with lung fibrosis, reflux pharyngitis, digital vasculitis and Raynaud syndrome with premature menopause. Histopathology from the frontal and right parietal scalp biopsies showed thickening of dermal collagen bundles with some degree of hyalinization, as well as peri-infundibular and isthmic lymphoid cell infiltrate with mild perifollicular fibrosis and miniaturization of the hair follicles. No vacuolar–interface change or lichenoid infiltrate of the interfollicular epidermis, and no histopathological signs of chronic traction alopecia were present. Blood tests were supportive of a diagnosis of systemic sclerosis, as confirmed by an erythrocyte sedimentation rate of 37 mm per hour, and positive antinuclear antibody 1 : 5120, Ro, ribonucleoprotein, extractable nuclear antigen and Scl-70 antibodies. Anticentromere antibodies were negative. Clinicopathological correlation suggested a combined scarring alopecia with features of frontal fibrosing alopecia (FFA) occurring on a background of systemic sclerosis. While these two types of alopecia may be coincidental, we hypothesized that FFA might be the result of epitope spreading (Koebnerization) with systemic sclerosis. Diffuse systemic sclerosis has been reported to occur with increased levels of tissue inhibitor of metal metalloproteinases in the serum causing increased deposition of matrix metalloproteins (MMPs) alongside increased levels of MMP-9. MMP 9 is also suggested to be responsible for the apoptosis of basal keratinocytes in FFA, which may lead to exocytosis of cytotoxic T cells into the epidermal layer leading genetically predisposed patients to develop FFA. Multifactorial alopecia is known to occur on the scalp. We describe this novel combination of scarring alopecia secondary to systemic sclerosis and FFA, which, to our knowledge, has not been previously reported.