Morfea superficial / Superficial morphea

La morfea superficial es una variante rara de morfea que se distingue de la clásica tanto en la clínica como en la histopatología. Se caracteriza por máculas hipopigmentadas o hiperpigmentadas, con mínima o ninguna induración, sin síntomas asociados, contractura ni atrofia. En la histopatología, se observa un compromiso limitado a las fibras colágenas en la dermis reticular superficial. Se comunica el caso de una paciente con diagnóstico de morfea superficial tratada con fototerapia ultravioleta B y metotrexato.

Superficial morphea is a rare variant of morphea that is distinguished from the classic variant both clinically and histopathologically. It is characterized by hypo or hyperpigmented patches with minimal to no induration, without associated symptoms, without contracture or atrophy. At the histopathological level, a limited involvement of collagen fibers is observed at the level of the uperficial reticular dermis. The case of a patient with superficial morphea treated with ultraviolet B phototherapy and methotrexate is presented.

Esclerodermia localizada en Coup de Sabre: a propósito de un caso / Localized Scleroderma en Coup de Sabre: a case report

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.

Morphea: a practical review of its diagnosis, classification and treatment

Morphea, or localized scleroderma, is a rare disease of the connective tissue that manifests itself with localized sclerosis of the skin and, in some cases, with extracutaneous manifestations. Its etiology is not fully understood, but it is believed that there is genetic predisposition, in addition to environmental triggering factors. Classification of the disease is not simple due to its multiple presentations; however, it is useful in order to define the treatment, which should be individualized and started early to avoid cosmetic and functional complications. In this review, we summarize the most important practical aspects of the classification, diagnostic methods and evaluation of morphea activity, as well as available therapeutic options, with an emphasis on existing clinical evidence regarding their efficacy and safety.

Case for diagnosis. Sclerodermiform manifestations of porphyria cutanea tarda secondary to hepatitis C

Abstract: A 63-year-old black female patient with blisters and exulcerations on the face, neck, upper limbs, and subsequent evolution with hypochromic sclerotic areas and alopecia, is reported. Chronic hepatitis C and presence of high levels of porphyrins in urine were demonstrated. There was complete remission with the use of hydroxychloroquine, photoprotection, and treatment of hepatitis. Significant sclerodermoid involvement of the skin as a manifestation of porphyria cutanea tarda secondary to hepatitis C emphasizes the importance of diagnostic suspicion regarding skin manifestation in order to indicate the appropriate therapy, and to minimize the hepatic morbidity.

Morfea o esclerodermia localizada juvenil: caso clínico / Morphea or juvenile localised scleroderma: Case report

Introducción: La morfea o esclerodermia localizada juvenil (ELJ) es una enfermedad autoinmune, inflamatoria, crónica, lenta y progresiva del tejido conectivo, de causa desconocida, que afecta preferentemente la piel y los tejidos subyacentes. Objetivos: Comunicar un caso de esclerodermia localizada juvenil en una escolar, y contribuir a un diagnóstico y tratamiento oportuno de esta patología. Caso clínico: Niña de 8 años con placas induradas hipopigmentadas, de distribución lineal en la extremidad superior derecha de 2 años de evolución y placas induradas hiperpigmentadas de textura acartonada, con áreas de piel adelgazada, blanquecina y edema en la pierna y el tobillo. Los elementos clínicos y los exámenes de apoyo diagnóstico, incluyendo la histología, fueron compatibles con ELJ lineal, panesclerótica. Se inició tratamiento inmunosupresor y simultáneamente realizó fisioterapia y terapia ocupacional intensivas. Conclusiones: Presentamos un caso de ELJ de tipo lineal y panesclerótico, en el que hubo retraso de 2 años en el diagnóstico, no obstante la respuesta al tratamiento inmunosupresor fue favorable según lo esperado.

Introduction: Morphea or juvenile localised scleroderma (JLS) is an autoimmune, inflammatory, chronic, slowly progressive connective tissue disease of unknown cause that preferably affects skin and underlying tissues. Objective: To report a case of Juvenil Localised scleroderma in an 8-year old girl, contributing to an early diagnosis and treatment. Clinical case: The case is presented of an 8 year-old girl who presented with indurated hypopigmented plaques, of linear distribution in the right upper extremity of two years onset, together with papery texture hyperpigmented indurated plaques with whitish areas of thinned skin in right lower extremity, and leg and ankle swelling. The clinical features and diagnostic tests, including histology were compatible with linear and pansclerotic JLS. She started with immunosuppressive therapy, physiotherapy, and occupational therapy. Conclusions: We report a case of linear and pansclerotic ELJ type, in which there was a 2 year delay in diagnosis, however the response to treatment was positive as expected.

Localized scleroderma: clinical spectrum and therapeutic update

Scleroderma is a rare connective tissue disease that is manifested by cutaneous sclerosis and variable systemic involvement. Two categories of scleroderma are known: systemic sclerosis, characterized by cutaneous sclerosis and visceral involvement, and localized scleroderma or morphea which classically presents benign and self-limited evolution and is confined to the skin and/or underlying tissues. Localized scleroderma is a rare disease of unknown etiology. Recent studies show that the localized form may affect internal organs and have variable morbidity. Treatment should be started very early, before complications occur due to the high morbidity of localized scleroderma. In this review, we report the most important aspects and particularities in the treatment of patients diagnosed with localized scleroderma.

MORFEAS: clasificación y tratamiento / MORFEAS: classification and treatment

Las morfeas (esclerodermias localizadas) constituyen un espectro de afecciones cutáneas caracterizadas por endurecimiento por fibrosis exagerada de la dermis y la hipodermis que adoptan típicas aunque diferentes morfologías clínicas, localizaciones, profundidad lesional, número de lesiones, curso evolutivo y pronóstico. Las morfeas comparten además un inicio predominante antes de los 15 años de edad, una gran variedad de autoanticuerpos séricos, potenciales y serias consecuencias funcionales y estéticas. Complicaciones extracutáneas aparecen sólo excepcionalmente, principalmente trastornos encefálicos en los pacientes con morfea cefálica. Un diagnóstico correcto y por tanto el inicio de un manejo terapéutico suelen ocurrir con un infortunado retardo de meses o años. Se han reportado diversas formas de tratamiento, algunas bastante controvertidas, basadas principalmente en experiencias anecdóticas o series limitadas de pacientes. Por otra parte, ha sido difícil validar parámetros confiables para monitorear la actividad, la severidad y la mejoría objetiva de los pacientes. El resultado terapéutico actual es sólo parcialmente exitoso y no hay guías terapéuticas aceptadas por todos. Esta revisión se limita a discutir las dificultades de clasificación de las morfeas, proponer una clasificación personal y revisar las modalidades terapéuticas usadas por los diferentes grupos de dermatólogos y reumatólogos a lo largo de los últimos 25 años

The morpheas (localized sclerodermas) represent a spectrum of cutaneous disorders characterized by skin sclerosis caused by exaggerated dermal and hypodermal fibrosis adopting different although typical clinical morphologies, localizations, lesional depth, number of lesions, course and prognosis. Other aspects shared by the morpheas are an onset mainly in patients before 15 years old, a wide variety of serum autoantibodies and potential serious functional and aesthetic consequences. Extracutaneous complications appear only by exception, mainly encephalic disorders in patients with cephalic morphea. The correct diagnosis and consequently the onset of therapy both usually happen with an unfortunate delay of months or years. Many ways of treatment have been reported, some quite controversial, mainly based in anecdotic experiences or limited series of patients. Furthermore it has been difficult to validate reliable parameters for monitoring the activity, severity and objective improvement of patients. Therapy results are at present only partially successful and there are no widely accepted therapeutic guidelines. This paper is limited to discuss the difficulties in classifying the morpheas, to propose a personal classification and to review the therapeutic modalities used by different groups of dermatologists and rheumatologists through the last 25 years

Presentación inusual de morfea en placas en un niño / Unusual presentation of morphea in plates in child

La morfea de presentación infantil se caracteriza por manifestarse como lesiones lineares, localizadas con mayor frecuencia en uno de los miembros o en la región facial. La morfea tiene mayor prevalencia en el sexo femenino. Se presenta el caso de un niño de once años sin morbilidad adicional, con lesiones planas, hiperpigmentadas y atróficas, localizadas en la parte medialy lateral del tercio inferior de ambas piernas. Las lesiones fueron confirmadas histopatologicamente como morfea superficial.

Child onset morphea is usually characterized by linear lesions, more frequently located in one limb or in the facial region, more prevalent in white female children. We report the case of a 11year-old male child without additional morbidity, with atrophic and hyperpigmented flat plaque lesions, located in the inner and outer side of the lower third part of both legs. The lesions were pathologically confirmed as superficial morphea.

Localized scleroderma: assessment of the therapeutic response to phototherapy / Esclerodermia cutânea: avaliação da resposta terapêutica à fototerapia

BACKGROUND: Scleroderma is a chronic autoimmune disease characterized by progressive connective tissue sclerosis and microcirculatory changes. Localized scleroderma is considered a limited disease. However, in some cases atrophic and deforming lesions may be observed that hinder the normal development. Literature reports indicate phototherapy as a therapeutic modality with favorable response in cutaneous forms of scleroderma. OBJECTIVES: This study had the purpose of assessing the phototherapy treatment for localized scleroderma. METHODS: Patients with localized scleroderma were selected for phototherapy treatment. They were classified according to the type of localized scleroderma and evolutive stage of the lesions. Clinical examination and skin ultrasound were used to demonstrate the results thus obtained. RESULTS: Some clinical improvement was observed after an average of 10 phototherapeutic sessions. All skin lesions were softer at clinical palpation with scores reduction upon pre and post treatment comparison. The ultrasound showed that most of the assessed lesions presented a decrease in dermal thickness, and only five maintained their previous measure. Treatment response was similar regardless of the type of phototherapeutic treatment employed. CONCLUSIONS: The proposed treatment was effective for all lesions, regardless of the phototherapeutic modality employed. The improvement was observed in all treated skin lesions and confirmed by clinical evaluation and skin ultrasound.

FUNDAMENTOS: A esclerodermia é uma doença autoimune caracterizada pela esclerose progressiva do tecido conjuntivo e alterações da microcirculação. A forma cutânea é considerada uma doença autolimitada. No entanto, em alguns casos, ocorrem lesões atróficas, deformantes, que dificultam o desenvolvimento normal. Relatos da literatura apontam a fototerapia como uma modalidade terapêutica com resposta favorável nas formas cutâneas da esclerodermia. OBJETIVOS: Este trabalho teve como objetivo avaliar o tratamento da esclerodermia cutânea com fototerapia. MÉTODOS: Foram selecionados pacientes com diagnóstico de esclerodermia cutânea para o tratamento com fototerapia, os quais foram classificados de acordo com o tipo clínico e o estágio evolutivo das lesões. Utilizou-se o exame clínico e a ultrassonografia da pele como metodologia para demonstrar os resultados obtidos com o tratamento proposto. RESULTADOS: Foi observado o início da melhora clínica das lesões com média de 10 sessões de fototerapia. A palpação clínica mostrou amolecimento em todas as lesões estudadas, com redução nos escores de avaliação estabelecidos. No exame de ultrassom, a maioria das lesões avaliadas mostrou diminuição da espessura da derme, e apenas cinco mantiveram sua medida. Não se observou diferença na resposta ao tratamento de acordo com o tipo de fototerapia instituída. CONCLUSÕES: O tratamento proposto foi efetivo em todas as lesões, independentemente do tipo de fototerapia realizada. A melhora foi observada em todas as lesões tratadas e comprovada pela avaliação clínica e pelo exame de ultrassom da pele.

Tratamiento de la esclerodermia localizada con corticoides y metrotexato / Treatment of localized scleroderma with corticosteroids and methotrexate

La morfea (o esclerodermia localizada) es una enfermedad inflamatoria, que se caracteriza por el engrosamiento de áreas circunscriptas de la piel. Por lo general, es un proceso benigno y autolimitado, pero en algunas oportunidades puede presentar posibles complicaciones. Estos casos potencialmente severos deben ser detectados y tratados tempranamente para evitar secuelas a largo plazo. Si bien existen múltiples tratamientos propuestos para este tipo de morfea pediátrica severa, la combinación de metotrexato y corticoides sistémicos parece ser la más efectiva y tener un buen perfil de seguridad. En el presente artículo se realiza una revisión de la literatura sobre el tema

Morphea (also known as localized scleroderma) is an inflammatory disease of the skin characterized by the thickening of certain areas of the skin. It is usually a benign and self-limited disorder, but in some cases it may be more severe. These patients should be detected early and be treated in time to avoid long-term complications. Although several treatments have been proposed for this type of severe morphea, the use of systemic steroids plus methotrexate seems to be the most effective, with few side effects. We here-by perform a literature review on this subject

Tratamento de úlceras crônicas secundárias à esclerodermia com laser de baixa potência: relato de caso / Treatment of chronic ulcers secondary to scleroderma with low-level laser: case report

Introdução - Os pacientes com esclerodermia podem apresentar complicações como ulcerações em terço distal de membros inferiores. A exata incidência dessa complicação recorrente é desconhecida, mas sabe-se que são lesões de mau prognóstico. O laser de baixa potência é amplamente explorado no tratamento de úlceras crônicas, mas até o momento não foram encontrados estudos publicados sobre a utilização do laser em úlceras secundárias à esclerodermia. Este estudo visa relatar os efeitos do laser de baixa potência no tratamento de úlceras cutâneas decorrentes de esclerodermia em paciente do sexo feminino. Materiais e Métodos - Realizou-se aplicação do laser de baixa potência nas feridas secundárias à esclerodermia, em paciente do sexo feminino com quinze anos de lesão. O laser utilizado apresentava comprimento de onda de 660 nm e foi utilizada a técnica pontual com contato na margem das lesões e pontual sem contato no leito das mesmas. A distância entre os pontos era de 1 cm. A energia aplicada por ponto foi de 200 mJ aplicada em 4 rajadas de 50 mJ, duas vezes por semana, durante 10 semanas. As áreas das lesões foram mensuradas através do programa Image J antes e após o tratamento. Resultados - A paciente apresentou diminuição significativa da área de todas as lesões de pele. Além disso, observou-se melhora no aspecto vascular e na sensibilidade dos membros inferiores, assim como, uma diminuição da área de hiperestesia em úlcera na região lateral do membro inferior esquerdo (MIE). Conclusão - O presente estudo documenta o sucesso de uma terapêutica menos invasiva do que cirurgias atualmente eleitas como tratamento adequado e propõe a continuidade de estudos sobre esse tipo de ferida.

Introduction - Ulcers in lower limbs extremity are a frequent problem in patients with systemic sclerosis; but the exact incidence is not defined, whereas these damages are difficult prognostic.The low-level laser is widely studied in treatment of venous ulcers and its utilization is well understood, but there are no studies about laser in ulcers secondary to scleroderma published yet. This study aims to report the low-level laser treatment efficacy over skin ulcers originated by scleroderma in a female patient. Materials and Methods - Low-level laser was applied on wounds secondary to scleroderma in a female patient who presented these injuries during fifteen years. The low-level laser applied had 660 nm wavelength, and it was used the punctual technique with contact around the ulcer, and inside the ulcer without contact. The distance between points was 1 cm. The energy applied per point was 200 mJ applied in 4 discharges of 50 mJ, twice a week, during ten weeks. The wound areas were measured through Image J software before and after treatment. Results - As a result of the treatment it was observed that wound areas significantly decreased. Additionally, there was an improvement of vascular aspect and sensibility in lower-limbs, as well a decrease of an area of hyperesthesia in ulcer at lateral region of left low-limb. Conclusion - This research presents the benefits of an alternative therapy less invasive than surgeries currently considered as adequate treatment, and it suggests continuity of studies about this type of wound.

Esclerodermia localizada na criança: aspectos clínicos, diagnósticos e terapêuticos: [revisão] / Localized scleroderma in children: clinical, diagnostic and therapeutic aspects: [revision]

A esclerodermia localizada, ou morféia, acomete crianças em idade escolar e, em geral, é autolimitada, apesar de localmente desfiguradora. A literatura descreve inúmeros fatores etiopatogênicos, bem como modalidades de investigação e tratamento. Este artigo reúne os estudos mais recentes e discute sua aplicação clínica.

Localized scleroderma or morphea affects school-aged children, is usually self-limited and a disfiguring condition. Several etiopathogenic factors, investigations and treatment options are described. This article reviews the recent literature and discusses its clinical applications.

Scleroderma esophagus.

A 40 year-old female presented with gradual onset generalised swelling, followed by thickening of skin for one year. She had symmetrical polyarthritis and firm, shiny skin with areas of depigmentation and absence of normal skin wrinkling. She had flexion contracture of fingers, masked facies, difficulty in opening mouth and Raynoud’s phenomenon. She had dysphagia to solid for last two months with recurrent heartburn. ESR was 120mm/1st hr. ANF was positive. X-ray hands showed periarticular osteopenia. Barium swallow X-ray showed dilated distal esophagus with loss of peristaltic contractions and a stricture at lower end of esophagus. Fundic gas was visible (Fig. 1). Upper GI endoscopy excluded any malignancy but showed features of reflux esophagitis with a stricture at lower esophagus causing difficulty in passing the endoscope through it. The esophagus is involved in 50-90% of patients of scleroderma.1 Smooth muscle atrophy and fibrosis lead to thinness and weakening in lower two-third of esophagus and incompetence of lower esophageal sphincter (LES). The proximal one third with its striated muscle is spared. The commonest manifestation is reflux esophagitis, which may be complicated by peptic stricturing near the junction with stomach. Dysphagia occurs due to esophageal dysmotility or stricture. Barium swallow X-ray shows dilatation and loss of peristaltic contractions in lower esophagus. LES is patulous. Esophageal mucosal ulceration and stricture may be visible. Manometry shows decreased amplitude or absence of peristaltic waves in lower esophagus. Resting pressure of LES is subnormal, but sphincter relaxation is normal. The differential diagnosis, achalasia shows esophageal dilatation with persistent beaklike narrowing of terminal esophagus due to non-relaxing LES. Chest X-ray shows absence of gastric air bubble and an air-fluid level in mediastinum on erect posture. Manometry shows normal or elevated resting pressure of LES and elevated resting pressure in esophageal body. Management of scleroderma esophagus includes proton pump inhibitors and dietary adjustments with soft foods. Bougie dilatation may be required for stricture. REFERENCE Kahan A, Menkes CJ. Gastrointestinal involvement in systemic sclerosis.Clin Dermatol 1994;12:259-65.

Correlação clínica e ultra-sonográfica na esclerodermia localizada cutânea / Clinical and ultrasonographic correlation in localized cutaneous scleroderma", "_i": "en

OBJETIVO: Apresentar os aspectos ultra-sonográficos da esclerodermia localizada e relacioná-los com os aspectos clínicos. MATERIAIS E MÉTODOS: Foram analisadas 23 lesões de esclerodermia localizada em 21 pacientes. Foi utilizado equipamento Logiq 700 com transdutor linear de 6-14 MHz. Foram avaliados, pelo dermatologista, o estágio da doença (inflamatório ou atrófico), e pelo radiologista, a espessura e a ecogenicidade da derme nas regiões afetadas e sãs adjacentes. Foi feito acompanhamento de sete casos após tratamento. RESULTADOS: Todas as lesões apresentaram perda do padrão ultra-sonográfico normal da derme. Os casos de lesão clinicamente atrófica (52,2 por cento; 12/23) corresponderam a redução da espessura e aumento da ecogenicidade da derme e os casos de lesão clinicamente inflamatória (47,8 por cento; 11/23) corresponderam a aumento da espessura e redução da ecogenicidade da derme. Controles pós-tratamento mostraram alterações na espessura da derme. CONCLUSÃO: Os achados ultra-sonográficos nos permitem associar o aumento da espessura e a redução da ecogenicidade da derme com a fase inflamatória da doença, e a redução da espessura e o aumento da ecogenicidade da derme com a fase atrófica da doença. Notamos também que é possível quantificar a espessura da derme e usar essa informação no controle pós-tratamento associada à avaliação clínica.

OBJECTIVE: To describe ultrasonographic findings of localized cutaneous scleroderma and correlating them with clinical findings. MATERIALS AND METHODS: Twenty-three lesions of localized cutaneous scleroderma in 21 patients were evaluated with a Logiq 700 equipment coupled with a 6-14 MHz linear transducer. The disease stage (athrophic or inflammatory) was evaluated by a dermatologist, and the ultrasonographic findings (skin thickness and echogenicity) for both the affected and adjacent healthy regions were evaluated by a radiologist. Seven of the cases underwent post-treatment follow-up. RESULTS: All the affected regions presented loss of the normal ultrasonographic pattern of the dermis. Cases with clinically atrophic lesions (52.2 percent; 12/23) corresponded to reduction in the thickness and increase in the echogenicity of the dermis, and clinically inflammatory lesions (47.8 percent; 11/23) corresponded to decrease in echogenicity and increase in the thickness of the dermis. Post-treatment follow-up demonstrated alterations in the dermis thickness. CONCLUSION: The ultrasonographic findings allow the correlation between increase in the thickness/decrease in echogenicity of the dermis with the inflammatory phase of the disease, and decrease of the thickness/increase in echogenicity of the dermis with the atrophic phase. Also, it could be observed that it is possible to quantify the thickness of the dermis, utilizing this information associated with the clinical evaluation in the post-treatment follow-up.

Morfea Segmentaria con Patrón de Blaschko / Segmentary Morphea with Blaschkco Pattern

La esclerodermia localizada, también denominada morfea, es una enfermedad del tejido conectivo que involucra principalmente la piel y el tejido celular subcutáneo y cuya principal característica es la esclerosis hialina inflamatoria de la piel, puede llegar a comprometer estructuras profundas como la afasia, el músculo y el tejido óseo. Presentamos el caso de un paciente varón de 34 años de edad, que presenta lesiones en placas, induradas, eritemato-brunas, distribuidas en forma segmentaria en hemicuerpo izquierdo y un estudio histopatológico compatible con morfea.

Localized sclerodermia, named also morphea, is a disease of connective tissue that compromised skin and subcutaneous cellular tissue and whose principal feature is the inflammatory hyaline sclerosis of skin that can also compromised deep structures like fascia, muscle and osseous tissue. We present the case of a 34 years old male with indurate plaques, erythematosus, dark brown, in segmentary distribution on left side of the body and with a histopathology study compatible with morphea.