Adult-onset Bilateral en coup de sabre – Parry–Romberg syndrome overlap

A 22-year-old immunocompetent male presented with a 4-year history of progressive skin hardening and hair loss over the left side of the forehead and scalp. Over time, it extended to the nose and chin, leading to facial asymmetry. There was no history of associated headache, visual changes, or trauma to scalp, and no significant medical, family, or drug history were found. Cutaneous examination showed two asymmetric, shiny, ivory-colored, atrophic, hairless plaques, and the right plaque extending longitudinally from frontal scalp to middle forehead, while the left plaque extending until ala of nose and mentum, associated with alopecia of involved left eyebrow, and minimal atrophy of ipsilateral tongue [Figure 1]. Ocular, neurological, and dental examination was normal. Routine laboratory investigations were normal. Brain magnetic resonance imaging could not be done due to financial restraints. Histopathology reported mild hyperkeratosis, dermal sclerosis, sparse perifollicular infiltrate of lymphocytes and neutrophils, consistent with localized scleroderma [Figures 2 and 3]. Clinicohistopathological corelation was compatible with linear morphea en coup de sabre – Parry–Romberg syndrome overlap, such case was also cited by Abdelnour et al.^[1] and Pekiner et al.^[2] The patient was started on oral methotrexate 25 mg once weekly and topical application of clobetasol propionate 0.05%–calcitriol 0.0003% ointment once daily. No further progression was noted at 2 months follow-up.

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Figure 1:

Photographs of the patient from different views show obvious atrophy of skin and subcutaneous tissue (black arrows) affecting scalp, forehead, left side of the nose, and left upper lip and mentum.

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Figure 2:

Photomicrograph from biopsy scalp shows dermis with thickened collagen and mild inflammation. No hair follicles seen (H&E; × 100).

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Figure 3:

Photomicrograph from biopsy forehead shows thinned epidermis. Dermis shows thickened collagen, sparse perifollicular infiltrate, and eccrine glands present high up in the dermis (H&E; × 100).

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