Fototerapia UVB banda estrecha en el tratamiento de la enfermedad injerto contra huésped crónica cutánea: reporte de caso / Narrowband UVB phototherapy for the treatment of cutaneous chronic graft versus host disease: case report

Resumen La enfermedad injerto contra huésped crónica (EICHc) es una complicación frecuente en los pacientes que reciben trasplante de progenitores hematopoyéticos (TPH) alogénico, siendo la piel el órgano más frecuentemente afectado. La EICHc cutánea se presenta con lesiones esclerodermiformes y no esclerodermiformes y frecuentemente requiere tratamiento con inmunosupresores sistémicos, fotoféresis extracorpórea o fototerapia. Los inmunosupresores tienen el potencial de producir importantes efectos adversos, por lo que terapias con mejor perfil de seguridad son claramente necesarias. Presentamos el caso de una paciente de 11 años a quien se le realizó un TPH haploidéntico como tratamiento de una leucemia linfocítica aguda. En su evolución desarrolló EICHc cutánea esclerodermiforme. La paciente recibió tratamiento con luz ultravioleta B de banda estrecha (UVBbe), respondiendo satisfactoriamente en los 2 primeros meses. Existen múltiples reportes y series de casos exitosos sobre el tratamiento con fototerapia en distintas modalidades. En relación a la fototerapia con UVBbe, la literatura es escasa, sin embargo, muestran importantes resultados tanto en las formas esclerodermiformes y no esclerodermiformes de la EICHc cutánea y un buen perfil de seguridad. De todas formas, se requieren estudios prospectivos controlados a gran escala para determinar su efectividad como terapia adjuvante o incluso de primera línea y para definir los esquemas terapéuticos y dosis más efectivas.

Summary Chronic graft-vs-host disease (GVHD) is a frequent complication in patients who receive allogeneic hematopoietic cell transplants (HCTs), and the skin is the most common site of involvement. Chronic cutaneous GVHD can present with sclerotic or nonsclerotic changes and often requires treatment with systemic immunosuppressants, extracorporeal photopheresis, or phototherapy. Immunosuppressants carry the potential of causing important side effects, so additional modes of therapy with better security profiles are clearly needed. We report a case of an eleven year old girl, who received allogeneic HCTs to treat acute lymphocytic leukemia. She developed sclerotic chronic GVHD. The patient underwent treatment with narrowband UV-B phototherapy, and a significant improvement was seen over the first 2 months. There are a number of successful series and case reports on different forms of phototherapy. In relation to narrowband UV-B phototherapy, literature is scarce, although shows important results in sclerotic and nonsclerotic forms of chronic cutaneous GVHD and a good safety profile have been seen. Anyway, large-scale controlled prospective trials are needed to evaluate the effectiveness of phototherapy as adjuvant o even first-line therapy, and to establish the most effective therapy schemes and doses.

Calcinosis pseudotumoral en esclerodermia / Pseudotumoral calcinosis in systemic sclerosis

La calcinosis es una manifestación frecuente en la esclerodermia sistémica. Sin embargo, la calcinosis pseudotumoral es de presentación excepcional. Las radiografías o tomografía axial computada son de utilidad para el diagnóstico y el seguimiento. Aunque no existe un tratamiento estandarizado efectivo, la remoción quirúrgica de las lesiones sintomáticas y algunos fármacos son útiles en algunos enfermos. Se presenta el caso de una paciente de 23 años con esclerodermia sistémica variedad limitada que desarrolló múltiples lesiones pseudotumorales.

Soft tissue calcinosis is a common cutaneous manifestation in systemic sclerosis. However, pseudotumoral calcinosis is rare. Radiographs or computed tomography are useful tools to diagnose and assess this condition. Although no treatment was uniformly effective, surgical excision of symptomatic lesions and medical treatment provided benefit for some patients. We report here a case of a 23 year old woman, suffering from limited cutaneous systemic sclerosis, who developed many pseudo tumoural calcinosis lesions.

Validación del índice Duruõz en una población argentina con esclerodermia / Reliability and validity of the Duruoz Hand Index in an argentinian population with scleroderma

Objetivo: el objetivo de nuestro estudio fue adaptar y validar el Indice Duruõz (ID) en una población argentina con esclerodermia. Métodos: Para la validación, reumatólogos tradujeron al español la versión original en francés. Para evaluar la validez constructiva se utilizó la escala global visual análoga del paciente (EVA), EVA para cada una de las preguntas de la misma actividad, el cuestionario de evaluación de salud (HAQ) y el Score modificado de Rodnan para evaluar la piel. Resultados: Se incluyeron 45 pacientes con diagnóstico de esclerodermia. La correlación entre la puntuación total de ID y el EVA global del paciente fue de 0,58, con el HAQ fue 0,63 y con el Rodnan fue 0,08. El coeficiente de correlación entre el EVA y cada grupo de preguntas para la misma actividad en el ID, indicó buena correlación para las preguntas que se refieren a las actividades de cocina, así como para vestirse, de higiene, y para las preguntas de oficina. Se encontró excelente nivel de correlación con los ítems relacionados con las actividades de motricidad fina. La reproducibilidad fue de 0,88 y la confiabilidad de 0,98. Conclusión: Los resultados mostraron que el ID es un cuestionario confiable y válido para esta población argentina con esclerodermia.

Objective: the aim of our study was to adapt and to validate theDHI questionnaire in an argentinian population with scleroderma. Methods:  For validation, rheumatologists translated to Spanish the original version in French. To evaluate the construct validity, we used thepatient global visual analogue scale (VAS), VAS for questions for the sameactivity, the health assessment questionnaire (HAQ) and the Rodnan. Results: a total of 45 patients with scleroderma were includedin the study. The correlation between the total score of DHI and thepatient global VAS was 0.58, with the HAQ was 0.63 and with Rodnan0.08. The correlation coefficient between the VAS and each group of questions for the same activity in the DHI questionnaire, indicatedgood correlation for the questions that refer to activities of kitchen, aswell as for dressing, for hygiene, and for the office questions. Therewas excellent level of correlation with those related to  fine motoractivities. The reproducibility was 0.88 and the reliability was 0.98. Conclusion: The results from this study show the DHI to be a reliableand valid test for this argentinian population with scleroderma.

Síndrome de Evans Fisher asociado con esclerodermia / Fisher Evans syndrome associated with scleroderma

El síndrome de Evans Fisher, descrito por primera vez en 1951, es un desorden autoinmune caracterizado por la presencia simultánea o secuencial de anemia hemolítica, trombocitopenia inmune y, en ocasiones, neutropenia inmune; con una prueba de antiglobulina directa positiva. Puede ser de causa primaria o secundaria a otras condiciones, como el lupus eritematoso sistémico, los síndromes linfoproliferativos o inmunodeficiencias primarias. Es muy rara su asociación con la esclerodermia. Con el término esclerodermia, que en sentido literal significa piel dura, se designa un grupo de enfermedades y síndromes que tienen como característica común la induración y el engrosamiento cutáneos. Se caracteriza por la presencia de un depósito excesivo de los componentes del tejido conjuntivo, expresado en forma de fibrosis hística, y por alteraciones estructurales del lecho vascular. Con un cuadro clínico muy amplio, afecta fundamentalmente la piel y ciertos órganos internos, como tubo digestivo, pulmón, corazón y riñón. Se presenta una paciente femenina de 75 años de edad, piel negra, con antecedentes de hipertensión arterial, diabetes mellitus tipo 2, cardiopatía isquémica y esclerodermia, esta última diagnosticada seis meses antes de su ingreso. Acudió por decaimiento marcado, palidez cutáneo-mucosa intensa y petequias generalizadas. En los estudios realizados se detectó anemia y trombocitopenia severas, reticulocitosis, prueba de antiglobulina directa positiva e hipercelularidad medular con hiperplasia severa de los sistemas megacariopoyético y eritropoyético. Se diagnosticó un síndrome de Evans Fisher y se trató con esteroides e inmunomoduladores; se logró la mejoría clínica y la remisión hematológica

The Evans syndrome, first described in 1951, is an autoimmune disorder characterized by the simultaneous or sequential development of hemolytic anemia and immune thrombocytopenia or immune neutropenia. It may be of primary origin or secondary to other diseases or conditions such as systemic lupus erythematosus, lymphoproliferative disorders or primary immunodeficiencies. Its association with scleroderma is considered very rare. The word scleroderma, which literally means hard skin, designates a group of diseases and syndromes of common feature in induration and thickening the skin. It is characterized by the presence of excessive deposition of connective tissue components, expressed as histic fibrosis, and structural alterations of the vascular bed. With a broad clinical view, it primarily affects the skin and certain internal organs such as gastrointestinal tract, lung, heart and kidney. We present a 75 year-old female, black skin, with a history of hypertension, type 2 diabetes, ischemic heart disease and scleroderma, the latter diagnosed six months before admission. The patient referred marked weakness, pale skin and generalized petechiae. The complete blood count detected severe anemia, thrombocytopenia and reticulocytosis. Other studies showed positive direct Coombs test and severe hypercellularity. Evans Fisher syndrome was diagnosed and treated with steroids and immunomodulators; clinical improvement and hematologic remission was achieved

Hallazgos dermatoscópicos en enfermedades del tejido conectivo en pacientes pediátricos / Dermoscopic findings in connective tissue diseases in pediatric patients

Introducción: La dermatoscopía de superficie ha mostrado utilidad en el examen de pacientes con enfermedades de tejido conectivo. Anormalidades de los vasos capilares del repliegue ungueal proximal se pueden presentar en etapas iniciales de dichas enfermedades y su hallazgo puede ayudar al diagnóstico temprano de éstas. Este estudio tuvo como objetivo describir los hallazgos capilaroscópicos del repliegue ungueal proximal en pacientes pediátricos con lupus eritematoso sistémico, dermatomiositis y esclerodermia. Materiales y métodos: Estudio observacional, prospectivo, transversal y descriptivo. Se incluyeron 53 pacientes de entre 2 y 18 años de edad. El repliegue ungueal proximal de todos ellos fue observado con dermatoscopio manual de luz polarizada. Resultados: Todos los pacientes con dermatomiositis y esclerodermia y 14 de 30 pacientes con lupus eritematoso sistémico mostraron hallazgos anormales en el repliegue ungueal proximal. Éstos consistieron en una desorganización de la distribución capilar normal, aumento de los lazos capilares, capilares ramificados, pérdida progresiva de capilares, megacapilares y microhemorragias. Discusión: Los hallazgos capilaroscópicos del repliegue ungueal proximal en pacientes pediátricos con lupus eritematoso sistémico, dermatomiositis y esclerodermia tienen utilidad para el diagnóstico.

Introduction: Superficial dermatoscopy has proved useful in the examination of connective tissue diseases. Anomalies in capillary vessels of the proximal nailfold may be present in early stages of these diseases, and this finding may aid in their early diagnosis. The objective of this study was to determine capillaroscopic findings of the proximal nailfold in pediatric patients with systemic lupus erythematosus, dermatomyositis and sclerodermia. Materials and methods: Observational, prospective, transversal and descriptive study. Fifty-three patients with an age range between 2 and 18 years were included. The proximal nailfold of each patient was observed with a manual polarized light dermatoscopeResults: All of the patients diagnosed with dermatomyositis and sclerodermia, and 14 of 30 patients with systemic lupus erythematosus showed significant signs in the proximal nailfold, which consisted of capillary distribution disorganization, branched capillaries, progressive loss of capillaries, megacapillaries and microhemorrages. Discussion: Capillaroscopic findings of the proximal nailfold in pediatric patients with systemic lupus erythematosus, sclerodermia and dermatomyositis are useful for diagnosis.

Combined therapy of interferon alpha. 2b with cyclosporine and prednisolone in scleroderma versus methotrexate and prednisolone

Scleroderma is a rare connective tissue disease characterized by widespread small vessels obliteration and fibrosis of the skin and multiple internal organs. No therapy is of proven value in scleroderma. To evaluate the value of combination of interferon alpha. 2b, cyclosporine and small dose of Prednisolone in the treatment of scleroderma. 46 patients with different histopathological phases of scleroderma were enrolled in this study. The patients was divided randomly in two groups. Group one was composed of 32 patients treated with interferon alpha. 2b 3MIU/day, cyclosporine 100mg/day and Prednisolone 10mg/day. Group two received methotrexate 10 mg/week with 15 mg Prednisolone / day. After one year follow up group one [who received interferon alpha. 2b, cyclosporine and Prednisolone] showed remarkable improvement in the shape of skin, tightness of skin and raynauds phenomena who used prednisolone plus methotrexate who did not show any improvement, in comparison with group two. Combined therapy of interferon alpha. 2b, cyclosporine and small dose of prednisolone could be useful therapy for patients with scleroderma

[Prevalence of hypothyroidism in Shariati Hospital 2003-2004]

Scleroderma is an important chronic disease with unknown ethiology and two subtypes: limited type: Skin involvement limited to distal of extremity and face. Diffuse type: Skin involvement is both distal and proximal of extremity, face and thrunk. Thyroid dysfunction is a main problem in these patients but there is no published data of Iranian scleroderma patients This is a cross-sectional study to determine the prevalence of hypothyroidism [clinical, subclinical and autoimmune] in patients with scleroderma 125 patients with scleroderma selected and T3, T4, TSH Anti Tpo Ab and Anti TG Ab measured in them. 33 patients with scleroderma had hypothyroidism. [%26/4]. Two patients with scleroderma had hyperthyroidism. [%1/6].%12/8 had clinical hypothyroidism. And%13/6 had subclinical hypothyroidism,%33/3 of patients with subclinical hypothyroidism and positive autoantibody had limited type. Where as%66/6 of patients with subclinical hypothyroidism and autoantibody had diffuse type,%28/5 of patients with clinical hypothyroidism and positive auto anti body had limited type. Where as%71/4 of patients with clinical hypothyroidism and auto anti body had diffuse type. All of patients with hyper thyroidism had diffuse type and autoantibody positive. It seems hypothyroidism has an increased prevalence in patients with scleroderma and we suggest that thyroid function test must be done in primary evaluation of these patients

Desfecho da gravidez em portadoras de esclerodermia difusa e limitada / Pregnancy outcome in patients with diffuse and limited scleroderma

OBJETIVO: verificar se existe diferença no número de abortamentos e recém-nascidos (RN) de baixo peso entre as pacientes com esclerodermia (SSc) se comparadas com as mulheres sem a doença entre as formas da doença e quando a manifestação da SSc ocorre antes ou após a gestação. MÉTODOS: foram incluídas 26 pacientes com SSc. Os casos foram analisados quanto ao número de gestações, abortamentos, peso ao nascer e sexo dos filhos, correlacionando-se com a forma da doença e a relação temporal do diagnóstico com a gestação. As pacientes não haviam feito uso de medicações que interferissem na gestação, nem apresentavam outra doença. Para controle foram incluídas 26 mulheres saudáveis, sem doenças que alterassem o curso da gestação, pareadas para nível socioeconômico e idade. Para estudo estatístico foram feitas tabelas de freqüência, de contingência e empregados os testes de Fisher, chi2 e Mann-Whitney. RESULTADOS: entre as pacientes com SSc, houve 96 gestações com 13,5 por cento (n=13) de abortamentos. No grupo controle, 94 gestações com 9,6 por cento (n=9) de abortamentos. Não houve diferença quanto ao número de RN de baixo peso nestes dois grupos (n=8 e controles, n=6, com p=0,54) e tampouco entre o número de abortos (p=0,46). Entretanto, o número de RN de baixo peso foi significativamente maior entre os casos com a forma difusa da doença (forma difusa, n=4 e limitada, n=4, com p=0,04) e em pacientes que engravidaram após o diagnóstico estabelecido de SSc (37,5 por cento em mulheres sabidamente doentes e 6,7 por cento em mulheres que engravidaram antes de adoecer, com p=0,03). O número de RN masculinos foi maior no grupo das SSc (p=0,002). CONCLUSÕES: as mulheres portadoras de SSc apresentam um número maior de RN de baixo peso na forma difusa da doença e quando a gravidez se estabelece após o diagnóstico clínico da doença.

Study on the clinical characteristics of systemic sclerosis / 대한내과학회지

BACKGROUND: Systemic sclerosis (scleroderma) is a connective tissue disorder of unknown etiology characterized by fibrosis of the skin and internal organs. It is remarkably heterogeneous in initial presentations and internal organ involvement. Limited and diffuse cutaneous subsets of systemic sclerosis (SSc) are known to be different in clinical and laboratory features. The aim of the present study was to determine the clinical characteristics of systemic sclerosis in Koreans. METHODS: Fifty-six patients with systemic sclerosis at the Rheumatology Clinic of Seoul National University Hospital were studied for age, sex, symptoms, signs, and laboratory results. The differences in clinical and laboratory features between limited and diffuse cutaneous subsets were investigated. RESULTS: The mean age at diagnosis of 56 patients (male:female=1:4.6) was 42.4 years (range 11-72 years). The patients consisted of 30 limited and 26 diffuse cutaneous SSc. Cutaneous involvement was as follows: sclerodactyly (100%), Raynaud's phenomenon (94.6%), digital pitting scar (66.1%), subcutaneous calcinosis (1.8%). In musculoskeletal system, 25 cases (46.3%) developed arthralgia/arthritis, 14 cases (25.9%) myalgia. In gastrointestinal system, esophagus was affected in 11 cases (20.7%). Respiratory involvement consisted of interstitial lung disease (24 cases, 43.7%) and pulmonary hypertension (2 cases, 3.6%). Total skin score and functional vital capacity showed significant negative correlation (p<0.05). Cardiovascular involvement consisted of congestive heart failure (3 cases, 5.5%) and pericardial effusion (1 case, 1.8%). Azotemia was found in one patient (1.8%). Antinuclear antibody was positive in 53 cases (94.6%) and anticentromere antibody 2 cases (3.6%). Anti-Scl 70 antibody was positive in 46.4% of all patients, 40.0% of limited scleroderma and 53.8% of diffuse scleroderma. When comparing clinical features between limited and diffuse cutaneous subsets, musculoskeletal involvement was more common in limited scleroderma. CONCLUSION: Systemic sclerosis in Koreans showed various systemic and organ involvement, musculoskeletal system, lung and esophagus being commonly affected. There was no significant difference between limited and diffuse scleroderma in clinical features except musculoskeletal involvement. Investigation of major internal organs, especially lung and esophagus, is needed, regardless of cutaneous subsets in systemic sclerosis.

A case of Limited Scleroderma Associated with Antiphospholipid Syndrome / 대한류마티스학회지

The antiphospholipid syndrome is characterized by arterial thrombosis, venous thrombosis, pregnancy wastage, and thrombocytopenia associated with a persis tently positive lupus anticoagulant and/or moderate to high positive anticardiolipin antibodies(IgG or IgM). The antiphospholipid antibodies have been detected in many medical conditions, but the antiphospholipid syndrome (APS) has mainly been restricted to the primary antiphospholipid syndrome and APS associated with systemic lupus erythematosus. Rarely, the APS has been reported in other autoimmune disorders in the literature. We describe a woman with a limited form of scleroderma and the APS manifested by complete occlusion of left axillary artery with probable thrombotic occlusive nature, thrombocytope nia, prolonged aPTT, and persistently positive lupus anticoagulant.

Two Cases of Scleroderrna with Peripheral Vascular diseases / 대한류마티스학회지

Scleroderma is a systemic disorder with multiorgan involvement. About 90% of patients with scleroderma has Raynaud's phenomenon and microvascular involvement is well recognized in scleroderma, but macrovascular involvement is not recognized. We experienced 2 cases of scleroderma with peripheral vascular occlusions that had been diagnosed by fernoral angiography. The one patient with limited scleroderma had anticentromere antibody and angiography of both fernoral arteries showed nonvisualization of posterior tibial artery. The other patient with diffuse scleroderma had anti-Scl-70 antibody and angiography of right fernoral artery showed occlusion of both anterior and posterior tibial arteries and stenosis of distal portion of peronial artery. Both patients didn't have the risk factors of atherosclerosis such as hypertension, hypercholesterolemia, obesity, and smoking. In recent years, there have been reports that scleroderma is associated with macrovascular disease. In the future, the study on the freguency, prevention and treatment of macrovasular disease in scleroderma is necessary. We report 2 cases of scleroderma with peripheral vascular occlusions that had been diagnosed by fernoral angiography with review of literatures.