Progressive facial hemiatrophy with linear morphea en coup de sabre: a coexistence

Progressive facial hemiatrophy (PFH), or Parry-Romberg syndrome, is a rare disease characterised by atrophy of the craniofacial tissues seen in the distribution of one or more divisions of the trigeminal nerve with minimal epidermal involvement, while en coup de sabre (ECDS) manifests as a paramedian depression of the scalp and forehead, with prominent epidermal changes [1, 2]. We present a rare case of PFH and ECDS in a male patient and discuss the differences between the two conditions.
A 24-year-old man presented to us with disfigurement of the left side of the face for the past 2 years, 3 months after sustaining blunt injury to the forehead. On examination, the patient was found to have atrophy of the left side of the face. There was a linear hyperpigmented indurated plaque present over the left side of the forehead extending to the frontal scalp and downwards to the middle of the bridge of the nose. There was loss of hair from the area on the scalp and the eyebrow (fig. 1). On palpation, the left supraorbital ridge and the frontal bone were found to be indented at the location of the plaque. A similar plaque extending from the left mandibular angle to the left angle of the mouth and from the zygomatic prominence to the inferior mandibular margin was present causing loss of beard hair (fig. 2). The oral mucosa showed atrophy of the left side of the tongue (fig. 3). Teeth and the palate were normal. Ophthalmologic examination revealed no abnormality. The rest of the cutaneous, systemic and general examination was normal.
Routine investigations including blood counts and liver and renal function tests were within the normal range. An orthopantomogram showed no abnormality. Electroencephalography (EEG) and computed tomography (CT) of the head showed no abnormality. Histopathology from the involved skin of the cheek showed slightly atrophic epidermis and a thickened dermis containing thick homogenised bundles of collagen and minimal mononuclear inflammatory cell infiltrate. Based on these findings, a diagnosis of progressive facial hemiatrophy with ECDS was made.
Progressive facial hemiatrophy is a rare disease of unknown cause characterized by degeneration of the face on one side. Apart from causing cosmetic disfigurement, it may also lead to loss of function on the involved side of the face [3]. The disease afflicts people in the first two decades of life, has a predilection for females and more frequently has been reported on...


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