Lesiones ampollares en una paciente con morfea panesclerótica discapacitante de la infancia / Bullous lesions in a patient with panesclerotic morphea of the childhood

La morfea ampollar es un tipo enfrecente de esclerodermia localizada que se caracteriza por presentar ampollas sobre placas escleróticas. La presencia de este tipo de lesiones obliga a descartar la variante extraenital de liquen esclerodemias localizadas, es posible hallar ambas afecciones. Se describe el caso de una paciente de 19 años con diagnóstico de morfea panesclerótica y liquen escleroso ampollar.

Bollous morphea is an infreqent type of morphea characterized for developing bullae on sclerodermiformic plaques. The presence of bullae forces to discard lichen sclerosus, a disease that usually develops in the genital zone, the extragenital variant could belong to the same spectrum that localized sclerodermiformic diseases. We present a 19 year old female patient with the diagnosis of panesclerotic morphea and bullous lichen sclerosus.

Morfea panesclerótica incapacitante / Disabling panesclerotic morphea

La morfea panesclerótica incapacitante es una forma severa y rara de esclerodermia localizada, es una enfermedad inflamatoria e inmunomediada de etiología desconocida. Evoluciona con endurecimiento generalizado de la piel por la esclerosis progresiva de la dermis y tejido celular subcutáneo y el deterioro de las articulaciones, huesos, fascia y músculos. La respuesta terapéutica es pobre y la progresión de la enfermedad genera discapacidad física, disminución de la calidad de vida y complicaciones fatales. Presentamos un caso de un paciente que inició los primeros síntomas a los 7 años de edad, siendo diagnosticado con morfea panesclerótica incapacitante conforme evolución clínica e histología, evolucionando con empeoramiento clínico progresivo independiente de las terapias instituidas (AU)

Disabling panesclerotic morphea is a rare and severe form of localized scleroderma, an inflammatory and immune-mediated disease of unknown etiology. It evolves with generalized hardening of the skin due to progressive sclerosis of the dermis and subcutaneous tissue and involvement of joints, bones, fascias and muscles. The therapeutic response is poor and the progression of the disease leads to physical disability, decreased quality of life and fatal complications. We present a case of a patient whose first symptoms started at 7 years of age and was further diagnosed with disabling panesclerotic morphea according to clinical evolution and histology, evolving with progressive clinical worsening regardless of the therapies instituted (AU)

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.

Coexistence of morphea and granuloma annulare: a rare case report

ABSTRACT CONTEXT: Localized scleroderma (morphea) is characterized by fibrosis of skin and subcutaneous tissue. Granuloma annulare is a relatively common disease that is characterized by dermal papules and arciform plaques. CASE REPORT: Here, we present the case of a 42-year-old woman who developed granuloma annulare on the dorsum of her feet and abdominal region, and morphea on the anterior side of her lower limbs. We also discuss the etiological and pathogenetic processes that may cause the rare coexistence of these two diseases. CONCLUSION: Only a few cases in the literature have described coexistence of morphea and granuloma annulare.

Lichen sclerosus associated with localized scleroderma: dermoscopy contribution

Abstract: Lichen sclerosus is an uncommon inflammatory dermatosis with preferential involvement of the urogenital region. The extragenital involvement is uncommon and is characterized by small rounded macules or papules, pearly white in color. The coexistence of lichen sclerosus and scleroderma plaques in most cases with extragenital location has been reported in the literature. We report a case of lichen sclerosus associated with scleroderma in children, highlighting the importance of dermoscopy in diagnosis.

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.

Scleroderma en coup de sabre treated with polymethylmethacrylate - Case report

Abstract The scleroderma en coup de sabre is a variant of localized scleroderma that occurs preferentially in children. The disease progresses with a proliferative and inflammatory phase and later atrophy and residual deformity, which are treated with surgical techniques such as injectable fillers, transplanted or autologous fat grafting and resection of the lesion. Among the most widely used fillers is hyaluronic acid. However, there are limitations that motivate the search for alternatives, such as polymethylmethacrylate, a permanent filler that is biocompatible, non-toxic, non-mutagenic and immunologically inert. In order to illustrate its application, a case of scleroderma en coup de sabre in a 17-year-old patient, who was treated with polymethylmethacrylate with excellent aesthetic results, is reported.

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.

Presentación atípica de morfea tipo malformación vascular símil con síndrome Parry-Romberg secundario / Atypical presentation of vascular malformation morphea simile Parry-Romberg syndrome secondary

La Morfea es una forma de presentación clínica de la esclerodermia localizada, que generalmente se manifiesta como una placa eritematosa con un anillo lila que evoluciona a placa atrófica. Existen presentaciones atípicas de morfea en que al inicio la piel afectada simula ser una malformación vascular tipo nevus flameus o mancha en vino de oporto. El Síndrome Parry-Romberg corresponde a una morfea localizada que se caracteriza por hemiatrofia facial que afecta la funcionalidad de la cara y que puede comprometer piel, tejido muscular y hueso. El objetivo de este artículo es presentar, por medio de un caso clínico, una entidad rara de morfea que al inicio simula ser un nevusflameus. El nevus flameus adquirido es raro y requiere tiempo para que se inicien los cambios escleróticos. Se presenta el caso de un niño de 8 años que consultó por un cuadro atípico sospechoso de nevus flameus y que al cabo de 3 años evolucionó como una hemiatrofia facial progresiva. Los pacientes con diagnóstico de nevus flameus adquirido deberían monitorizarse en busca de signos de evolución a morfea y si la ubicación es en la cara siempre debe sospecharse un Síndrome Parry-Romberg para el inicio temprano de terapia y disminuir las consecuencias asociadas.

Morphea is a clinical presentation of the localized scleroderma, which usually manifests as an erythematous plaque with a purple halo that evolves to an atrophic plaque. There are atypical presentations of the morphea that, at the beginning, the affected skin simulates being a vascular malformation port wine stain simil. The Parry-Romberg Syndrome corresponds to a localized morphea that characterizes for showing a facial hemiatrophy that affects the facial functionality and may compromise the skin, muscular tissue and bones. The objective of this paper is to present, through a clinical case, a rare entity of the morphea that at the beginning simulates being a port wine stain. The acquired port wine stain is rare and requires some time to show sclerotic changes. A clinical case of an 8 years old patient that consulted for a suspicion of an atypical clinical picture of port wine stain and that after 3 years evolved in a progressive facial hemiatrophy is presented. The patients with an acquired port wine stain diagnosis should be monitored to find signs of an evolution to morphea and if it is located on the face there should always be a suspicion of a Parry Romberg syndrome in order to start earlier the therapy and reduce the associated consequences.

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.

Caso para diagnóstico / Case for diagnosis

A esclerodermia localizada (EL) ou morfeia é uma doença crônica do tecido conjuntivo, de provável etiologia autoimune, que tem como base alterações na síntese e deposição do colágeno, representadas clinicamente por lesões cutâneas escleróticas. Algumas placas podem apresentar coloração amarelada ou xantocrômica, causando confusão diagnóstica. Este artigo relata o caso de uma adolescente, com concomitância de lesões eritematosas e xantocrômicas, ambas manifestações clínicas da doença.

Localized scleroderma or morphea is a chronic disease of the connective tissue. Its etiology may be autoimmune and the condition results from a disturbance in collagen synthesis and deposition, clinically represented by sclerotic skin lesions. Some plaques may be yellowish, which can be misleading at diagnosis. This article reports the case of an adolescent girl who concomitantly presented erythematous lesions and yellowish lesions, both of which constitute clinical manifestations of the disease.

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.

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.

Intertriginous bullous morphea: A clue for the pathogenesis?

Bullae occurring in lesions of morphea are uncommon. The cause of bullae formation in morphea is multifactorial, although lymphatic obstruction from the sclerodermatous process is considered the likeliest cause. Bullous morphea may be confused clinically with lichen sclerosus et atrophicus since both diseases may cause bullae in sclerodermatous plaques. A 69-year-old woman presented with a history of generalized morphea diagnosed 9 years earlier; and a 1-month history of pruritic bullae on her inframammary folds, axillary regions, lower abdomen, upper extremities and inguinal folds. Physical examination revealed multiple erythematous erosions, hemorrhagic vesicles and eroded bullae with slight scale or crusts overlying hypopigmented, indurated, shiny plaques. Skin biopsy revealed prominent edema in the papillary dermis, resulting in bulla formation and thickening of collagen fibers within the dermis. Direct immunofluorescence was negative. According to histologic and clinical features, the diagnosis of bullous morphea was established.

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.