Upsurging cases of en coup de sabre:distinctive clinical manifestations in a series of 41 patients

Authors

  • Khalifa E. Sharquie Department of Dermatology, College of Medicine, University of Baghdad. Medical City Teaching Hospital, Iraq
  • Raed I. Jabbar Department of Dermatology, Fallujah Teaching Hospital, Al-Anbar Health Directorate, Anbar, Iraq
  • Inas K. Sharquie Department of Microbiology & Immunology, College of Medicine, University of Baghdad, Baghdad, Iraq

Keywords:

En coupe de sabre, Morphea, Localized scleroderma

Abstract

Background En coupe de sabre is not uncommon connective tissue disorder of children and adult and often end with marked deep scarring that starts very early and ending with scarring of the skin and scarring alopecia of scalp. Objective The aim of current study is to screen for patients with en coup de sabre among cases with morphea and to evaluate the different clinical and histopathological features of the disease. Patients and methods This is cross sectional descriptive clinical study where all patients with morphea were screened for en coup de sabre and their clinical manifestations including scarring alopecia. Full clinical history and examination were carried for all patients that were seen during the period during from 2014-2023 years. Biopsies for histopathological evaluation were carried out Results A total number of 181 patients with different types of morphea were seen, their ages ranged between 4– 65 years with mean ± SD = 30 ± 15.345. There were141(77.9%) females and 40(22.09%) males with the ratio of 3.52:1. En coup de sabre was detected in 41(22.65%) cases, their ages ranged from 8-32 years with a mean of 20 years,31(75.6%)] cases under 18 years of age, with 30 (73.17%) females,11 (26.82%) males with female to male ratio was 2.72:1. The age onset of the disease was ranged from 4-28 years with mean of 14 years and in 30 (73.17%) of patients the age at onset of the disease was during childhood. Five (12.19 %) cases with face only but no scalp involvement while scarring alopecia was detected in 36(87.8%) of patients. One of the distinctive early features of the en coup de sabre of the face was the presence of rectangular mostly hyperpigmented and sometimes hypopigmented bands. Histopathological assessment showed mild acanthosis with normal dermal-epidermal junction but with pigmented basal layer while the dermis revealed mild inflammatory reaction with collagen deposition in early lesions while complete skin sclerosis was noticed in late lesions.   Conclusion En coup de sabre is a disease of children and young ages and mostly seen among females. There are early and late distinctive clinical and histopathological pictures. It is common cause of scarring of the face and scalp. Early detection of these patients is mandatory to prevent permanent complications.

References

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Published

2023-01-15

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Sharquie KE, Jabbar RI, Sharquie IK. Upsurging cases of en coup de sabre:distinctive clinical manifestations in a series of 41 patients . J Pak Assoc Dermatol [Internet]. 2023Jan.15 [cited 2024Jul.13];34(1):85-91. Available from: http://www.jpad.com.pk/index.php/jpad/article/view/2602

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