Concomitant en coup de sabre and plaque type morphea in the same patient: a rare occurrence

Linear scleroderma is a rare form of morphea which mainly affects children, with 67% of patients diagnosed before 18 years of age. Linear scleroderma may involve the forehead and fronto-parietal area of the scalp, where it is called linear scleroderma en coup de sabre because the appearance of skin lesions simulates the stroke of a saber [1]. Co-existence of en coup de sabre and plaque morphea has been described in very few case reports in the literature [2]. Here we describe a 17-year-old female with concomitant en coup de sabre and plaque type morphea. Additionally, this is probably the first case where en coup de sabre was followed by the development of plaque type morphea in the same patient.
A 17-year-old female presented to our outpatient department of dermatology with a history wherein she suffered a blunt trauma to the frontal part of the scalp 2 years previously. After 3 months of trauma she developed an indurated scar over the trauma site with associated hair loss. Subsequently this indurated scar with hair loss progressed vertically in a linear fashion. For the last 2 months, she had also experienced thickening and tightening of the skin over the left thigh. There was no history of headache, seizures or weakness of any body parts. The patient denied any systemic complaints. Physical examination revealed a well-defined indurated plaque 6 × 3 cm over the frontal region of the scalp and forehead in the left paramedian location associated with scarring alopecia. There was thickened, indurated and light brownish colored skin over the forehead in a vertical fashion (Fig. 1 A). The medial aspect of the left thigh revealed a single shiny ivory white to light brownish colored indurated plaque of the size of 8 × 5 cm as measured by a calibrated measuring tape (Fig. 1 B). The rest of the cutaneous examination, neurological examination, brain magnetic resonance imaging (MRI) and ANA were unremarkable. Histopathological examination of the skin biopsy from the left thigh and frontal scalp lesion showed atrophy of the epidermis, perivascular infiltrate of lymphocytes and plasma cells in the dermis and subcutaneous tissue. Densely packed collagen in thick bundles with sparse adnexal structures were seen in the dermis and subcutaneous tissue (Figs. 2, 3). Based on the history and clinical findings and further supported by histopathology, a diagnosis of concomitant en coup de sabre and plaque morphea was made. The...


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