Localized Scleroderma en Coup de Sabre: A case report

Abstract

Introduction: Localized scleroderma or morphea is an idiopathic autoimmune dis- order that causes subcutaneous sclerotic changes and is different from systemic sclerosis or scleroderma. The morphea in “coup de Sabre” is a subtype of linear morphea that usually involves the forehead and scalp causing contraction and stiffness of the skin that culminates in a depression and that may be associated with ocular and neurological symptoms. We present a case of a young male pa- tient with morphea in coup de sabre.

Case presentation: A 23 years old male patient presents with a skin lesion of tri­angular morphology in the left-frontal region. He was initially treated with topical corticosteroids, but had persistent growing of the skin lesion associated with new onset occipital headache. Ultrasound of the lesion as well as skin biopsy were per- formed confirming morphea in coup de sabre. Brain magnetic resonance imaging with gadolinium was normal. Inmunosuppresive tratment was started.

Discussion: Morphea in Coup de sabre is an rare disease. It is more frequent in women and children. Because it involves the deep tissues of the face and fore- head, it is relevant to rule out any ocular or neurological involvement. The sero- logical study is usually not necessary and results are of uncertain interpretation. When the diagnosis is unclear, a biopsy of the compromised tissue may help to identify inflammation and/or atrophy and to evaluate the degree of activity of the lesion. Ultrasound is also an useful tool for evaluation of the activity of the skin lesion, comparable to biopsy. Regarding treatment, topical corticosteroids are the first line therapy for acute lesions. Methotrexate has proven to be useful in deeper active lesions, with or without corticosteroids. Finally, there is an important aso- ciation between this type of lineal morphea and progressive hemifacial atrophy (Parry Romberg syndome), which may involve the brain and needs to be referred to the specialist as soon as possible.