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Visual Treats in Dermatology
2023
:3;
29
doi:
10.25259/CSDM_156_2022

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

Department of Dermatology, Venereology and Leprosy, Vardhman Mahavir Medical College, Opposite AIIMS Hospital, New Delhi, India
Department of Pathology, ICMR National Institute of Pathology, Safdardung Hospital Campus, New Delhi, India
Corresponding author: Kanupriya Kapil, Department of Dermatology, Venereology and Leprosy, Vardhman Mahavir Medical College, Opposite AIIMS Hospital, New Delhi, India. priya1kanu.kk@gmail.com
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This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, transform, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

How to cite this article: Kapil K, Bansal S, Sharma S. Adult-onset bilateral en coup de sabre – Parry–Romberg syndrome overlap. CosmoDerma 2023;3:29.

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.

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.
Figure 2:
Photomicrograph from biopsy scalp shows dermis with thickened collagen and mild inflammation. No hair follicles seen (H&E; × 100).
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).

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent.

Conflicts of interest

There are no conflicts of interest.

Financial support and sponsorship

Nil.

References

  1. , , , . Parry-Romberg syndrome associated with en coup de sabre in a patient from South Sudan-a rare entity from East Africa: A case report. J Med Case Rep. 2019;13:138.
    [CrossRef] [PubMed] [Google Scholar]
  2. , , , . Frontal linear scleroderma (en coup de sabre): A case report. J Dent Child. 2006;73:175-8.
    [Google Scholar]

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