En coup de sabre.

A man born in 1961 was apparently well until his marriage in May 1986. In August of that year, his attention was drawn to the presence of a reddish patch over the right forehead. It was initially asymptomatic. It continued to progress to form an apparent linear furrow extending from the receding hairline to the vertex and temporal part of the scalp. At this juncture, the patient experienced moderate pain when laughing. Five years later the patient experienced a constant boring pain affecting the right eyeball. Consultation with an ophthalmologist led to sacrificial enucleation of the right eye despite the lack of a precise diagnosis. Nevertheless, the initial condition continued to progress causing disfigurement of the right side of the face. Examination of the face was marked by perceptible asymmetry. A linear atrophic plaque in the form of a furrow was identified on the right side of the forehead extending from the eyebrow to the vertex and temporal part of the scalp. The skin over the furrow was taut and bound down (Fig. 1). A hematoxylin and eosin-stained section prepared from the lesion revealed marked thickening of the dermis. The collagen bundles were hypertrophied and closely packed together. The staining was homogeneously eosinophilic. It was largely devoid of inflammatory infiltrate. The changes in the blood vessels were conspicuous by narrowing of the lumina, thickening of the walls, and a sparse perivascular lymphocytic infiltrate. Pilosebaceous units were completely absent, whereas a few atrophic pulled-up sweat glands were located in the mid-dermis. Similar changes were observed in the subcutaneous tissue. The epidermis was largely atrophied, with flattening of the rete ridges. Figure 1. En coup de sabre: linear scleroderma occupying the frontoparietal part of the scalp and associated with facial hemiatrophy Download figure to PowerPoint