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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.
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