Porfiria cutánea tarda que simula esclerosis sistémica progresiva / Porphyria cutanea tarda mimicking progressive systemic sclerosis

Resumen Los síndromes esclerodermiformes suelen imitar muy bien una esclerosis sistémica progresiva, y es la presencia de ampollas cutáneas en áreas fotoexpuestas con hiperpigmentación los datos diferenciales para diagnosticar una porfiria. Presentamos el caso de un varón de 48 años con fotosensibilidad, fragilidad capilar, ampollas cutáneas e hiperpigmentación asociado a esclerodactilia, con pérdida cicatrizal distal de tejido en los dedos de las manos, que simuló a la perfección una esclerosis sistémica progresiva. La analítica mostró negatividad para anticuerpos antinucleares, antitopoisomerasa y anticentrómero, con valores altos de uroporfirinas en orina. El tratamiento con flebotomías e hidroxicloquina mejoró la fotosensibilidad y la fragilidad cutánea.

Abstract Sclerodermiform syndromes usually mimic progressive systemic sclerosis very well, with the presence of skin blisters in photo-exposed areas with hyperpigmentation being the differential data for diagnosing porphyria. We present the case of a 48-year old man with photosensitivity, capillary fragility, skin blisters, and hyperpigmentation associated with sclerodactyly with distal scar tissue loss on the fingers, which perfectly simulated progressive systemic sclerosis. The analysis showed negativity for antinuclear, antitopoisomerase and anticentromere antibodies, with high levels of uroporphyrins in urine. Phlebotomy and hydroxycloquine treatment improved photosensitivity and skin fragility.

Enfermedad indiferenciada del tejido conectivo con progresión a esclerosis sistémica / Undifferentiated disease of the connective tissue with progression to systemic sclerosis

La enfermedad indiferenciada del tejido conectivo es una condición de etiología desconocida que comparte características clínicas, patológicas y de laboratorio de varias colagenosis, sin cumplir los criterios del Colegio Americano de Reumatología para el diagnóstico de una enfermedad reumática específica y muchos pacientes evolucionan a condiciones definidas a lo largo del tiempo tales como Lupus, Esclerosis sistémica progresiva, Enfermedad de Sjögren entre otros. Antecedentes: Linfoma Hodgkin diagnosticado desde 2012 para lo cual recibió múltiples esquemas de quimioterapia. Las muestras de ganglio y médula ósea se habían enviado al laboratorio de Inmunopatologia de la Universidad de Stanford y allí no se apreciaron hallazgos compatibles con enfermedad linfoproliferativa. Enfermedad actual: Mujer de 27 años de edad con cuadro clínico de 1 mes de evolución, caracterizado por edema blando en miembros inferiores acompañado de edema palpebral matutino; concomitantemente presenta aumento de temperatura intermitente sin patrón especifico y dolor osteomuscular generalizado con limitación para la deambulación. Se ingresa. Al examen físico, regulares condiciones clínicas. En la piel se aprecia engrosamiento cutáneo importante. Se realizó biopsia cutánea y los hallazgos fueron compatibles con Esclerosis Sistémica(AU)

Undifferentiated connective tissue disease is a condition of unknown etiology that shares clinical, pathological and laboratory characteristics of several collagenopathies that do not meet the criteria of the American College of Rheumatology for the diagnosis of a specific disease; a large number of patients evolve to conditions defined over time such as Lupus, Systemic Sclerosis, Sjogren's Disease, among others. Past history: Hodgkin lymphoma was diagnosed since 2012 for which she received multiple chemotherapy schemes. A gland biopsy was sent to the Stanford University, as well as a bone marrow sample, and lymphoma was discarded. Present history: this 27-year-old female consulted for edema in lower limbs present during one month, accompanied by eyelid edema in the mornings; also fever without a specific pattern, myalgias and arthralgias. On physical examination, the skin was thickened and limb edema was present. A skin biopsy was performed, and the findings were consistent with Systemic Sclerosis. The patient is receiving cyclophosphamide and Azathioprine and leading her normal life(AU)

Review of common conditions associated with periodontal ligament widening

PURPOSE: The aim of this article is to review a group of lesions associated with periodontal ligament (PDL) widening. MATERIALS AND METHODS: An electronic search was performed using specialized databases such as Google Scholar, PubMed, PubMed Central, Science Direct, and Scopus to find relevant studies by using keywords such as “periodontium”, “periodontal ligament”, “periodontal ligament space”, “widened periodontal ligament”, and “periodontal ligament widening”. RESULTS: Out of nearly 200 articles, about 60 were broadly relevant to the topic. Ultimately, 47 articles closely related to the topic of interest were reviewed. When the relevant data were compiled, the following 10 entities were identified: occlusal/orthodontic trauma, periodontal disease/periodontitis, pulpo-periapical lesions, osteosarcoma, chondrosarcoma, non-Hodgkin lymphoma, progressive systemic sclerosis, radiation-induced bone defect, bisphosphonate-related osteonecrosis, and osteomyelitis. CONCLUSION: Although PDL widening may be encountered by many dentists during their routine daily procedures, the clinician should consider some serious related conditions as well.

Esclerosis sistémica progresiva en una mujer joven / Progressive systemic sclerosis in a young woman

Se presenta el caso clínico de una fémina de 23 años de edad, con diagnóstico clínico y radiográfico de enfermedad de Cronh desde hacía 2 años aproximadamente, quien fue ingresada en el Servicio de Medicina Interna del Hospital General Docente "Dr. Juan Beuno Zayas Alfonso'' de Santiago de Cuba, por presentar síndrome febril prolongado, cefalea intensa, mialgias, artralgia y poliserositis, con una evolución desfavorable, además de complicaciones que fueron apareciendo posteriormente (insuficiencias cardíaca, renal y respiratoria). Los resultados de los exámenes complementarios efectuados y de los estudios histopatológicos, reafirmaron la presencia de esclerosis sistémica progresiva, lo cual se confirmó luego mediante biopsia de piel. Se indicó el tratamiento, según las diferentes eventualidades clínicas y finalmente la paciente logró reincorporarse a la vida social, pero con ciertas limitaciones.

The case report of a 23 years female patient with clinical and radiographic diagnosis of Cronh disease for approximately 2 years who was admitted in the Internal Medicine Service of "Dr. Juan Bruno Zayas Alfonso" Teaching General Hospital in Santiago de Cuba is presented for presenting prolonged feverish syndrome, intense migraine, myalgias, artralgia and polyserositis, with an unfavorable clinical course, besides complications that were emerging later on (heart, renal and respiratory failures). The results of the complementary exams and of pathological and histological studies carried out, reaffirmed the presence of progressive systemic sclerosis, which was confirmed later by means of skin biopsy. The treatment was indicated, according to the different clinical events and finally the patient was could be reinserted to the social life, but with certain limitations.

Fascitis eosinofílica: a propósito de un caso / Eosinophilic fasciitis: an account of a case

Eosinophilic fasciitis is a rare entity characterized by induration of skin and peripheral eosinophilia. The pathogenesis is still not well known, but is known association with hematological, autoimmune or neoplastic diseases. The complex and not well standardized treatment. For a patient of 66 years presented with a history of morphea...

La Fascitis Eosinofílica (FE) es una entidad muy infrecuente, caracterizada por induración de piel y eosinofilia periférica. La patogenia aún no es bien conocida, pero es sabida la asociación con enfermedades hematológicas, autoinmunes o neoplásicas. El tratamiento es complejo y no bien estandarizado. Se presenta el caso de una paciente de 66 años, con antecedentes de morfea...

Progressive systemic sclerosis: Using high-resolution CT to detect and characterize interstitial lung disease in Siriraj Hospital.

Objective: To detect and characterize interstitial lung disease in patients with progressive systemic sclerosis in siriraj hospital using high-resolution computed tomography (HRCT) and to detect other associated findings in the chest. Methods: Retrospective reviewed chest radiography and HRCT of twenty patients with progressive systemic sclerosis during January 2000- January 2005. The HRCT scans were reviewed by two radiologists for reticular opacities (interlobular septal thickening, traction bronchiectasis, honeycombing and bronchovascular interstitial thickening), nodular pattern (miliary, centrilobular and perilymphatic nodule), consolidation and ground-glass opacities. The associated findings in the chest such as esophageal dilatation, mediastinal lymphadenopathy and main pulmonary artery enlargement were also evaluated. Results: Chest radiography detect interstitial lung disease in eighteen patients (90%). The findings are reticular opacities and fibrosis at both lower lobes. The HRCT shows interstitial lung disease in twenty patients (100%) with the greatest proportion of interlobular septal thickening (85%), followed by traction bronchiectasis (75%), honeycombing (40%) and ground-glass opacities (25%). The distribution of disease is peripheral and lower lung predominant. The associated findings: esophageal dilatation and mediastinal lymphadenopathy were presented in 55% of patients and main pulmonary artery enlargement was presented in 90% of patients. Conclusion: HRCT is more sensitive than chest radiography for detecting and characterize interstitial lung disease in patients with progressive systemic sclerosis. Our study has evidences of interstitial lung disease in all patients with greatest proportion of interlobular septal thickening, followed by traction bronchiectasis, honeycombing and ground-glass opacities. Moreover HRCT can recognize cases with predominance of inflammatory process and direct clinician into more aggressive treatment, may be benificial in preventing irreversible disease. HRCT was useful for detecting other associated findings in the chest such as esophageal dilatation, mediastinal lymphadenopathy and main pulmonary artery enlargement.

Preliminary results on study of subtypes of T lymphocytes in peripheral blood of progressive systemic sclerosis patients

In order to have the primary results of subtypes of T-lymphocytes in peripheral blood of progressive systemic sclerosis patients, 20 patients were studied. The results showed that the counts of TCD3 was 1505 + 505 (cells/ml), TCD4 was 738 + 332 (cells/ml), TCDs was 668 + 291 (cells/ml). There was no significant difference of T-lymphocytes between progressive systemic sclerosis patients and the healthy people. Especially, one patient with severe clinical and paraclinical manifestations had remarkable decrease of TCD3, TCD4, and TCD8.

Manometric Investigation of Anorectal Dysfunction in Patients with Progressive Systemic Sclerosis

PURPOSE: The aim of this study was to investigate the anorectal function in patients with progressive systemic sclerosis (PSS), thus to define the clinical role of anorectal manometry in the earlier diagnosis of anorectal involvement of PSS. METHODS: Seventeen consecutive patients (all females) with PSS were evaluated with anorectal manometry by the stationary pullthrough technique using the 8-channel hydraulic capillary infusion system for anorectal function. Functional parameters of the manometry were compared between patients with PSS and 20 normal control subjects, matched for age and sex. RESULTS: The mean resting pressure over the high pressure zone (HPZ) in patients with PSS was significantly lower than that in the control group (70.8 3.4 mmHg vs. 81.5 3.2 mmHg: P=0.046). The HPZ in patients with PSS was also significantly reduced compared with that in the control (1.5 0.1 cm vs. 2.5 0.1 cm: P=0002). The rectoanal inhibitory reflex (RAIR) was detected in only 10 patients (59%) in the PSS group, but was present in all except one (95%) in the control (P=0.022). More interestingly, RAIR in patients with PSS responded at a higher volume of the air insufflated than that in the control (74% vs. 30% at 20 cc, 21% vs. 30% at 30 cc, and 0% vs. 40% at 50 cc, respectively: P=0.031). Other functional parameters, including maximal squeeze pressure, minimal sensory and maximal tolerable volume of the rectum, and rectal compliance were not significantly different between two groups. CONCLUSIONS: Anorectal involvement reflected by the anorectal manometric dysfunction may be rather an earlier event in patients with PSS. An awareness to perform an anorectal manometric study in every case of PSS may be necessary for earlier subclinical detection of anorectal involvement by the disease.

Polyneuropathy in Progressive Systemic Sclerosis

Progressive systemic sclerosis (PSS) is a chronic inflammatory disease of the connective tissue which affects the skin and other internal organs. Involvement of the peripheral nervous system is rare, although muscle abnormalities as a component of PSS are well recognized. 29-year-old (patient 1) and 64-year-old (patient 2) women had PSS for 3 and 5 years, respectively. They complained of having a tingling sensation in the legs. Examination showed reduced strength in the distal muscles, bilateral hypesthesia for pain and vibration below the knees and reduced or absent tendon reflexes in the lower limbs. Sensorimotor neuropathies were demonstrated by decreased motor conduction velocities and pro-longed distal sensory latencies. Sural nerves were of normal thickness. The density of myelinated fibers was not decreased and axonal degeneration was scanty or absent. Characteristic findings were intimal hypertrophy and perivas-cular inflammatory infiltrates in both patients. Inflammatory cell invasions in the peripheral nerve is extremely are in patients with PSS. It may be suggested that an inflammatory or immune mechanism is another contributing factor to polyneuropathy in PSS as well as microangiopathy.

A Case of progressive Systemic Sclerosis with Silicosis / 대한피부과학회지

Scleroderma is sclerosis of the skin characterized by the appearance of circumscribed or diffuse hard, smooth, ivory-colored anea that are immobile upon the underlying tissues and give the appearance of hide-bound skin. Several environmental factors and chemicals have been described as being able to induce systemic scleroderma and scleroderma-like diseases. A possible association between scleroderma and workers exposed to silica has been suggested by reports in the literature since the turn of the century. We report a 55-year-old man, with a history of Raynaud's phenomenon and dysphagia, who had been exposed to silica dust over fifteen years. He had a mask-like face, flexian deformity of fingers, and hyperpigmented induration on the skin of all the extremities and the trunk. Antinuclear antibodies were positive in nucleolar type. On radiologic study, streaky, mottled, and nodular increased densities were noted in the both lung fields and these findings were consistent with silicosis. Endoscopic and manometric examination of the esophagus represented scleroderrna esophagus. Histopathological findings were compatible with scleroderma. He was treated with azathioprine and nifedipine with good improvement.

A Case of progressive Systemic Sclerosis with Silicosis / 대한피부과학회지

Scleroderma is sclerosis of the skin characterized by the appearance of circumscribed or diffuse hard, smooth, ivory-colored anea that are immobile upon the underlying tissues and give the appearance of hide-bound skin. Several environmental factors and chemicals have been described as being able to induce systemic scleroderma and scleroderma-like diseases. A possible association between scleroderma and workers exposed to silica has been suggested by reports in the literature since the turn of the century. We report a 55-year-old man, with a history of Raynaud's phenomenon and dysphagia, who had been exposed to silica dust over fifteen years. He had a mask-like face, flexian deformity of fingers, and hyperpigmented induration on the skin of all the extremities and the trunk. Antinuclear antibodies were positive in nucleolar type. On radiologic study, streaky, mottled, and nodular increased densities were noted in the both lung fields and these findings were consistent with silicosis. Endoscopic and manometric examination of the esophagus represented scleroderrna esophagus. Histopathological findings were compatible with scleroderma. He was treated with azathioprine and nifedipine with good improvement.