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INTRODUCCIÓN: La esclerodermia localizada o morfea corresponde a una patología idiopática autoinmune que produce cambios escleróticos subcutáneos, que presenta diferencias con respecto a la esclerosis sistémica o esclerodermia. Un tipo de morfea lineal es la morfea "En Coup de Sabre" que consiste en la contracción y rigidez de la piel que culmina con una depresión de parte de la mitad del rostro, que puede asociarse a síntomas oftalmológicos y neurológicos. Aquí se describe un caso en un hombre joven con este tipo de morfea lineal. PRESENTACIÓN DEL CASO: Hombre de 23 años presenta lesión cutánea de morfología triangular en región frontal izquierda, por lo que decide consultar a dermatología, dónde se maneja con corticoides tópicos. Dos años después, la lesión sigue creciendo y se asocia a cefalea occipital, sin otros síntomas sistémicos. Se decide estudiar con biopsia, ecografía de cuero cabelludo y resonancia nuclear magnética (RNM) cerebral con gadolinio. Se diagnostica morfea en coup de sabre e indica tratamiento inmunosupresor. DISCUSIÓN: Dado que la Morfea en Coup de Sabre es una patología que compromete el rostro, es relevante realizar una derivación al oftalmólogo para evaluación de compromiso ocular y realizar una RNM para evaluación neurológica, en este caso ambos estudios resultaron negativos. El estudio serológico no es siempre necesario y debemos ser cautelosos en el uso de esta herramienta. Cuando existen dudas diagnósticas, se puede recurrir a una biopsia del tejido comprometido, la que debe incluir grasa subcutánea. La biopsia también ayuda para ver el grado de compromiso cutáneo que presenta el paciente. Con respecto al manejo, los corticoides tópicos son la elección para el manejo de lesiones agudas. El Metotrexato ha demostrado ser útil en lesiones agudas y profundas, asociado o no a corticoides.
INTRODUCTION: Localized scleroderma or morphea is an idiopathic autoimmune disorder 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 patient 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 performed 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 forehead, it is relevant to rule out any ocular or neurological involvement. The serological 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 asociation 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.
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Humans , Male , Adult , Scleroderma, Localized/diagnosis , Scleroderma, Localized/therapy , Physical Examination , Biopsy , Blood Cell Count , UltrasonographyABSTRACT
Objective:To analyze clinical, laboratory and imaging characteristics of different subtypes of linear morphea (LM) , and to propose an appropriate approach to the diagnosis and severity assessment of LM.Methods:Clinical data were collected from patients with clinically and/or pathologically confirmed LM in Department of Dermatology, West China Hospital, Sichuan University from January 2018 to December 2019, and retrospectively analyzed.Results:A total of 107 patients with LM were enrolled into this study, including 63 with LM of the limbs/trunk, 22 with morphea en coup de sabre, 11 with progressive hemifacial atrophy and 11 with eosinophilic fasciitis. Disease severity was evaluated by using the modified localized scleroderma skin severity index (mLoSSI) and localized scleroderma skin damage index (LoSDI) scores in 88 patients, with the mLoSSI scores ranging from 0 to 51 points, and the LoSDI scores ranging from 0 to 40 points. Routine blood examination of 10 patients with eosinophilic fasciitis showed increased eosinophil counts in 4 patients. Thirteen (14.8%) of 88 patients with LM were positive for antinuclear antibody, with titers of ≥ 1∶320. Magnetic resonance imaging (MRI) examination showed ipsilateral cerebral hemisphere atrophy and contralateral white matter hyperintensity on T2-weighted images in 2 out of 4 patients with progressive hemifacial atrophy, myofascial thickening in 26 out of 28 patients with LM of the limbs/trunk (92.9%) , subcutaneous septal and myofascial thickening in all 11 patients with eosinophilic fasciitis.Conclusions:The preliminary assessment of disease activity, severity and prognosis of LM can be made by mLoSSI and LoSDI. MRI examination is recommended for patients with clinical signs of involvement of subcutaneous structures.
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Parry朢omberg syndrome (PRS) may overlap localized scleroderma (morphea) lesions with linear depression (en coup de sabre [ECDS]). Overlap case with PRS and ECDS was presented. Enophthalmos, uveitis, ocular torticollis, keratic linear precipitates, and anti-double-stranded DNA positivity were identified. Subendothelial keratic precipitates detected by an in vivo laser scanning confocal microscopy were the first profiled in the literature. Patients must be evaluated and followed up carefully by their clinics to prevent misdiagnosis and unnecessary procedures such as surgery of ocular torticollis as muscular torticollis.
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Background: Scleroderma is a chronic connective tissue disease characterized by hardened or scaly skin and widespread abnormalities of the viscera, which is rare in the pediatric age group. Objective: In this study, we retrospectively reviewed 23 pediatric patients suffering systemic (SSc) and localized (LS) scleroderma. Methods: Twenty-three patients were enrolled and were diagnosed with SSc or LS from March 1993 to September 2009 in the Department of Pediatrics at Mackay Memorial Hospital in Taipei, Taiwan. These diagnoses were based on the criteria of the American College of Rheumatology and the clinicalmanifestations of hard skin. Data recorded included sex, age-at-onset, age-at-diagnosis, laboratory data, family history, trauma history, treatment, and outcomes. Results: Three patients suffered SSc and 20 patients had LS, including 16 girls and 7 boys. Mean age-at-onset was 6.55±3.28 years old. Antinuclear antibodies were positive in 15 patients. Tests for anti-Scl-70 antibodies were positive in 1 patient with SSc. One boy had en coup de sabre combined with a posterior fossa tumor. Twenty-two patients were treated with D-penicillamine. Oral prednisolone and methotrexate were added, if indicated. One girl with LS developed proteinuria after Dpenicillamine treatment. All patients with localized disease ultimately documented a softening of their skin lesions. Conclusions: While scleroderma is rare in children, the prognosis of SSc is poor but better than for adults. The prognosis for LS is usually benign, however, the skin may become progressively indurated and it may not only be a skin disease. No progression from LS to SSc was observed in our study.
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Progressive facial hemiatrophy (PFH) was first described by Parry and Romberg and this is a rare disorder of an unknown origin. PFH is characterized by slowly progressive, mostly unilateral facial atrophy of the skin, soft tissues, muscles and underlying bony structures and is usually develops in the first or second decade of life. Although the clinical presentation of PFH may appear similar to localized linear scleroderma en coup the sabre, some patients presenting with PFH have neither en coup the sabre nor generalized sclerosis. Herein, we report on an unusual case of a 7-year-old girl who presented with progressive hemiatrophy of the face.
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Child , Humans , Atrophy , Facial Hemiatrophy , Muscles , Scleroderma, Localized , Sclerosis , SkinABSTRACT
Localized scleroderma is a disease of unknown origin and is characterized by circumscribed sclerotic skin changes. When occurring in a linear pattern, it is called linear scleroderma. 'En coup de sabre' is a subtype of linear scleroderma which is characterized by band-like, ivory-colored depressions on the frontoparietal scalp region. Several treatment modalities have been used. However, they have not been effective or safe. We report our experience of two patients with 'en coup de sabre' on the forehead, who were treated successfully with polymethylmethacrylate microspheres (PMMA-NewPlastic(R)).