[Localization of sarcoidosis in a patient suffering from hemorrhoids].
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Biomedical subjects
Publications and source records attributed to M Grossin.
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INTRODUCTION: Paraneoplastic pemphigus is an autoimmune bullous disease described by precise clinical, histological and immunological features presented by Anhalt in 1990. Prognosis is very severe and depends on the associated neoplasia and the gravity of the mucosal damage. CASE REPORT: Paraneoplastic pemphigus was diagnosed in a 62-year-old man with chronic lymphoid leukemia. The course was favorable up to one-year follow-up after general corticosteroid therapy. DISCUSSION: This case illustrates that paraneoplastic pemphigus can be controlled by general corticosteroids and would suggest the severe prognosis may be improved.
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Extrapulmonary sarcoidosis, and particularly the presence of musculoskeletal complications of the disease, may require chronic corticosteroid therapy. In five patients with biopsy-proven sarcoidosis and presenting recalcitrant forms of the disease, we introduced low-dose oral methotrexate (MTX) [10 mg/week (7.5-15)] for 30 months (16-34) to control the clinical and biological symptoms as well as to try to reduce the intolerated steroid posology. Beneficial effects were observed within 8-12 weeks in all patients, which allowed a reduction of 59% (35-75) of the corticosteroid posology, and maintained with a follow-up of 3 yr in 4/5 patients. No significant toxicity was observed. MTX appears to be an efficient, safe and corticosteroid-sparing therapeutic agent for the treatment of recalcitrant musculoskeletal manifestations of sarcoidosis.
Angiotropic large cell lymphoma (ALCL), the so-called malignant angioendotheliomatosis, is characterised by proliferation of tumorous cells within small vessels. Manifestations in the CNS and cutaneous lesions prevail in the clinical presentation, although any organ can be involved. The recent classification of this lymphoma as part of the large cell lymphomas has modified the therapeutic approaches employed. This should improve the prognosis of this usually fatal disease. An unusual case presenting with fever, mononeuritis multiplex, and cutaneous lesions is reported. Peripheral neuropathy without other neurological symptoms is uncommon, and, to our knowledge, such isolated mononeuritis multiplex with nerve lesions has not been previously reported in ALCL. The clinical diagnosis was a systemic necrotising vasculitis and it is considered that its differential diagnosis must include angiotropic large cell lymphoma.
PURPOSE: To determine the magnetic resonance (MR) imaging features of subungual glomus tumors. MATERIALS AND METHODS: Thirty-one patients with a clinical suspicion of glomus tumor and 10 control subjects underwent MR imaging at 1.5 T. MR images of normal glomus bodies of a cadaver finger were correlated with histologic slices. With a local surface gradient coil, the pixel size reached 117 microns in one direction. Relaxation times were measured. Gadoterate meglumine was injected in 19 patients. RESULTS: Normal glomus bodies were visualized in the reticular dermis of the nail bed. Twenty-seven of 28 pathologically confirmed glomus tumors were detected with MR imaging. A peripheral capsule was present in most tumors. The nail matrix was compressed in 13 cases. The authors were able to differentiate three subtypes of glomus tumors (vascular, solid, and myxoid) on the basis of relaxation times and enhancement characteristics. Four patients had mucoid cysts or angioma in the nail bed. CONCLUSION: MR imaging can help accurately define the location and limits of glomus tumors before excision.
Disseminated infection by Fusarium is being increasingly reported in immunocompromised patients. We report the first case of Fusarium oxysporum disseminated infection with well-documented fungal myocarditis. Despite antifungal therapy and hematologic recovery, the patient died in cardiogenic shock, myocardial involvement clearly contributing to the fatal outcome.
The aim of this study was to assess the prevalence of malignant lymphomas in patients with long-standing primary Sjögren's syndrome (pSS). We retrospectively studied a cohort of 55 patients with pSS over a mean follow-up period of 12 years. Five patients (9%) developed malignant lymphoma. The interval between the diagnoses of SS and lymphoma ranged from four to 12 years (mean = 6.5 years). The lymphoma arose in the lymph nodes in two cases, the parotid gland in one case, the lacrimal gland in one case, and the lung in one case. All five cases were B-cell low-grade lymphomas. Among our SS patients, those with extraglandular manifestations and/or a mixed cryoglobulin were at increased risk for lymphoma development. Secondary lymphoma carried a poor prognosis in our study. Three of the six SS patients who died during the follow-up period had lymphoma.
INTRODUCTION: A case of a cutaneous metastasis as a first sign of a linitis plastica is reported. CASE REPORT: A 57 year-old man presented for a cervical infiltrated skin plaque. Histological examination and immunohistochemical staining gave the diagnosis of metastasis probably of gastrointestinal origin. Gastric endoscopy and biopsy confirmed the diagnosis of a linitis plastica. DISCUSSION: Cutaneous metastases from gastric carcinoma are uncommon and exceptionally the first sign of the disease. Their clinical and histological aspects are reviewed.
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OBJECTIVES: The aim of this retrospective study of 136 patients was to specify the natural history and the systemic prognosis of chronic cutaneous lupus erythematosus. It has been stated that in most of the cases, the disease only affects the skin. METHODS: From 10 October 1980 to 31 December 1990, 136 patients with the following criteria were included in this retrospective study: clinical signs suggestive of chronic cutaneous lupus erythematosus, characteristic histology, insufficient evidence for the diagnosis of systemic lupus erythematosus. RESULTS: The prevalence of systemic clinical involvement in the population under study was nearly the same as in the general population. The following biologic or immunologic abnormalities were quite common: leukopenia, lymphopenia, thrombopenia (significantly more frequent among patients having widespread chronic cutaneous lupus erythematosus), low titers of complement levels, positive antinuclear antibodies (usually at a low titer). Eleven out of the 136 patients developed systemic lupus erythematosus, most often over 5 years after the onset of the cutaneous lesions. Four cases out of these 11 had poor prognosis: renal and/or neurologic involvement. CONCLUSION: This data suggests that patients with chronic cutaneous lupus erythematosus could benefit from long-term follow up, since the course to systemic disease occurs in only a few. Usually, antimalarials used singly or in combination with topical steroids may lead to the clearing of the lesions. Thalidomide will occasionally be useful whenever the disease is unresponsive to the preceding measures.
Diffuse sclerosing osteomyelitis of the mandible has been described as a localized disease of unknown origin. We offer evidence that mandibular involvement in the recently described synovitis acne pustulosis hyperostosis osteitis syndrome exactly fits the accepted description for diffuse sclerosing osteomyelitis of the mandible. The clinical, radiologic, and pathologic findings in seven such cases are presented and discussed.
Two new cases of mid-dermal elastolysis are reported. In the first case, a 32-year-old woman, the clinical picture included the typical perifollicular protrusions of the trunk and wrinkled skin of the members. There was no prodrome and no other signs were associated. In the second case, a 39-year-old woman, after a 14-year clinical course, the skin manifestations involved the entire body with the exception of the face. Over the years, the initial follicular papules progressively formed plaques of wrinkled skin.
Sixteen cases of acquired mid-dermal elastolysis have been reported in the literature. In 1977, Shelley identified this type of elastolysis as a separate entity and described three variants: post-inflammatory elastolysis with cutis laxa predominates in facial areas in African children. The initial inflammatory lesions are replaced by rough wrinkling of the skin; post-inflammatory elastolysis without cutis laxa; non-inflammatory elastolysis without cutis laxa as mid-dermal elastolysis. It is difficult nevertheless to classify certain cases reported in the literature. This would suggest that there is in reality a continuum of acquired elastolysis which could be classed according to a scale of decreasing initial inflammation. The non-inflammatory pole would represent mid-dermal elastolysis. The common features of the different types of elastolysis are dominated by the absence of a causative agent in most cases, unknown pathogenesis and the fact that the empirical treatments which have been used have been ineffective.
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Sarcoidosis is rare in children, who usually have only asymptomatic pulmonary involvement. We report granulomatous cheilitis in two children with sarcoidosis. Both children had systemic sarcoidosis with lymph node and pulmonary involvement.
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