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30.    Scleroderma en coup de Sabre with secondary pseudo-cicatricial alopecia and partial loss of eyebrow and eyelashes. 
Andreas Finner, Jerry Shapiro,  Department of Dermatology and Skin Science, University of British Columbia, Vancouver, Canada.

History and Findings: We report a 44 yo caucasian male who developed linear progressive hairloss and a skin-coloured depression of his right scalp and forehead over the last 8 years with partial loss of the right eyebrow and upper eyelashes. The eyelids appear normal. There is no history of previous trauma or associated symptoms. Histology of a scalp biopsy is consistent with morphea and shows a normal pattern in elastic stain. ANA was negative. A neurologic examination was normal. Discussion: Scleroderma en coup de sabre (LSCS) is regarded to be a variant of morphea and can cause secondary cicatricial alopecia. Because end-stage lesions show a widened hyalinized dermis and preserved elastic fibres, the condition has been categorized as pseudo-cicatricial. Autoimmunity is thought to be involved in the etiology of morphea. Displacement, local inflammation or cytokines may cause the permanent alopecia. While loss of eyebrows is not uncommon in LCSC, involvement of eyelashes has only been reported once before. A close association between LSCS and Progressive Facial Hemiatrophy (PFH, Parry-Romberg Syndrome) has been suggested, with PFH being a deeper variant of LSCS with neurologic and ophthalmologic manifestations. In PFH, mosaicism or a dysregulation of the sympathetic nerves have been hypothesized (trophoneurosis). The latter is supported by PFH development after sympathectomy and the distribution along the branches of the trigeminal nerve, which is accompanied by sympathetic fibres. Hair follicles are surrounded by a network of nerves. We therefore hypothesize that the loss of eyelashes could also be related to alterations in piloneural interaction. Summary: LCSC can cause secondary pseudo-cicatricial alopecia. The pathogenesis of this condition and which factors lead to loss of the relatively distant eyelashes in this case are unclear.