Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 38-year-old man with systemic lupus erythematosus had nodular cutaneous mucinosis, leukocytoclastic vasculitis, and a vesiculobullous eruption. Immunopathologic study of a bullous lesion revealed granular deposits of IgA and IgG along the basement membrane zone. Localization of immune deposits to the lamina and sublamina densa was confirmed by immunoelectron microscopy.
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PMID:Bullous systemic lupus erythematosus with cutaneous mucinosis and leukocytoclastic vasculitis. 803 11

A 38-year-old woman with systemic lupus erythematosus presented to the dermatology department with diffuse nodules and plaques on her face, trunk and extremities. A lesional biopsy revealed large dermal mucin deposits. Papulonodular dermal mucinosis is characterized histologically by diffuse dermal mucin without the classic epidermal or inflammatory changes seen in lupus erythematosus (LE). This rare variant of LE should be distinguished from other cutaneous mucinoses.
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PMID:Papulonodular dermal mucinosis in systemic lupus erythematosus. 806 39

A case presentation of a patient with SLE associated with papulonodular mucinosis, generalized lymphadenopathy and splenomegaly is reported, along with a review of the literature. This 24 year old woman had biopsy-proven, skin lesions consistent with dermal mucinosis and a non-homogeneous immunofluorescence pattern. Serum anti-nuclear and anti-DNA antibodies were present and a mesangioproliferative glomerulonephritis was documented. The patient responded dramatically to standard therapy with prednisolone. The nature of the mucin deposits and the pattern of immunofluorescence deposition in the skin are discussed based on the findings reported in the literature.
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PMID:[A case of lupus associated with papulo-nodular mucinosis]. 807 67

A wide variety of cutaneous manifestations of lupus erythematosus have been reported. Among them, papulonodular eruptions produced by dermal mucin deposition have been recognized as nodular cutaneous lupus mucinosis (NCLM), and many cases have been reported in Japan. A 21-year-old woman with systemic lupus erythematosus (SLE) showed papulonodular eruptions on her back. A skin specimen from the nodule revealed mucin deposition (hyaluronic acid) in the dermis. NCLM should be recognized as a clinical form associated with SLE. The pathogenesis of the mucin deposition in NCLM is still uncertain.
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PMID:Nodular cutaneous lupus mucinosis: report of a case and review of previously reported cases. 880 51

We report a patient with the typical lesions of diabetic scleredema. Histological findings of the involved skin were thickening of the dermis, depositions of mucins, and fibrosis. Biochemical analysis revealed an increase in glycosaminoglycans in the involved skin as well as in the cutaneous lupus mucinosis. Mucinous materials were composed of hyaluronic acid.
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PMID:Diabetic scleredema: a case report and biochemical analysis for glycosaminoglycans. 906 4

Perniosis is a term applied to cold-induced painful or pruritic erythematous or violaceous acral papular or nodular lesions. We examined 39 skin biopsies from 38 patients who presented with acral purpuric lesions, suggesting a diagnosis of perniosis clinically or pathologically. The presence of a systemic or extracutaneous disease was established in 17 patients, including 5 with systemic lupus erythematosus (SLE), 3 with antiphospholipid antibodies, in 1 in whom there was underlying HIV disease, 2 with viral hepatitis, 2 with rheumatoid arthritis (RA), 2 with cryofibrinogenemia, 1 with hypergammaglobulinemia, 1 with iritis, and 1 with Crohn's disease. In the other 21 patients, the clinical presentations prompted further studies in 12, which showed a positive antinuclear antibody (ANA) in 10. A diagnosis of idiopathic perniosis (IP) was rendered in all 21 of these patients including those in whom a positive ANA was discovered, based on the absence of any other serological markers, signs, or symptoms indicative of a specific systemic disease complex; many had Raynaud's phenomenon, small joint arthralgias, atopy, or a family history of either connective tissue disease or Raynaud's disease. The histopathology of IP comprised a superficial and deep angiocentric lymphocytic infiltrate with papillary dermal edema and lymphocytic exocytosis directed to retia and acrosyringia. A few cases showed a mild vacuolopathic or lichenoid interface dermatitis, adventitial dermal mucinosis, lymphocytic eccrine hidradenitis, vascular ectasia, and thrombosis confined to dermal papillae capillaries. The biopsies from patients with iritis, RA, and Crohn's disease showed a granulomatous vasculitis and a granuloma annulare-like tissue reaction. The biopsies from the patients with SLE, cryofibrinogenemia, primary antiphospholipid antibody syndrome, and hypergammaglobulinemia shared a similar histopathology comprising an interface dermatitis, superficial and deep angiocentric and eccrinotropic lymphocytic infiltrates, vascular ectasia, and dermal mucinosis with prominent involvement of the eccrine coil. Many cases did not show features of IP, namely papillary dermal edema, thrombosis of dermal papillary capillaries, and lymphocytic exocytosis into the retia and acrosyringia. There was frequent vascular fibrin deposition involving reticular dermal vessels. The latter two variables were statistically significant discriminators between IP and in perniotic lesions observed in the setting of underlying systemic disease. With respect to the latter, some cases occurred in the setting of cold exposure and were designated by us as "secondary perniosis" (SP), whereas others showed no specific association with cold exposure and were designated as perniotic mimics (PMs) based exclusively on the gross and microscopic morphology of the lesions.
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PMID:Idiopathic perniosis and its mimics: a clinical and histological study of 38 cases. 910 49

We describe a 23-year-old Japanese man with systemic lupus erythematosus (SLE) who developed massive cutaneous mucinosis. He was diagnosed with SLE when he was 11 years old. Prednisolone therapy (30 mg/day) was initiated and reduced to 10 mg/day 3 months later; the SLE had been well-controlled with this dose of prednisolone for 12 years. However, infiltrated erythematous plaques developed on the middle-lateral area of his back at 17 years old and progressed to erythematous and elastic soft tumorous masses over 20 cm in diameter at 23 years old. Biopsies of the lesions on the nape revealed massive mucin deposition. Topical injection with hyaluronidase decreased the lesion. This cutaneous mucinosis can be distinguished clinically and histopathologically from papular and nodular mucinosis associated with SLE. The present case might be an unusual clinical variant of cutaneous mucinosis associated with SLE.
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PMID:Massive cutaneous mucinosis associated with systemic lupus erythematosus. 934 48

We report a 38-year-old man systemic lupus erythematosus who presented with an acute onset of paraplegia and urinary retention. The man had a 12-year history of nodular cutaneous mucinosis and arthralgia. In 1994, he was admitted to our hospital with a sudden onset of weakness and numbness of the right leg followed by an emergence of similar symptoms in the left leg. His elder sister had died at 16 years of age after suffering from systemic lupus erythematosus for 6 years. On examination, the patient had skin rash on his chest, back, head, forehead, and extremities. The neurological examination revealed that his tongue deviated to the right on protrusion. The muscle power was reduced to 2-3/5 in the right leg and to 4/5 in the left leg. The sensory disturbance was noted in the lower extremities with predominant involvement of the right leg. Reflexes were increased in the right biceps, triceps, both patellas, and Achilles tendons. Babinski sign was noted bilaterally. Urinary retention and constipation were also noted. The results of the blood cell count and hepatic and renal function tests were normal. Serum levels of C-reactive protein and complements (C3, C4, CH50) were also normal. Serological examinations showed increased anti-DNA antibody (14 U/ml, [normal, < 6]). Antinuclear antibody was positive at a titer of 1:1380. CSF study showed an increased protein concentration of 83 mg/dl and an IgG level of 14 mg/dl with a normal number of cells. MR images revealed a T1-low, T2-high signal lesion at the upper part of the left ventral medulla. MR images of the brain and spinal cord were normal. The patient was diagnosed as having SLE. High-dose intravenous methylprednisolone (1 g/day) pulse treatment that was started 25 days after the onset of neurological symptoms, produced partial relief. Our case presented with paraplegia with a focal lesion in the left upper ventral part of the medulla on MR images. The incidence of male SLE is low, and paraplegia is a rare complication of SLE. Thus, the medullary lesion in SLE observed in our case appears to be rare. SLE should be considered as a cause of acute onset paraplegia or myelopathy.
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PMID:[A man with systemic lupus erythematosus presenting with spastic paraplegia]. 936 89

Cutaneous lupus mucinosis (CLM) is a rare variant of lupus erythematosus eruptions. Our 5 cases with CLM were reviewed. All were men with systemic lupus erythematosus (SLE). CLM occurred as asymptomatic cutaneous papules, nodules, or plaques on the trunk, upper and lower extremities, and face. Histopathology of CLM mainly revealed abundant mucin deposits among splayed collagen bundles throughout the dermis. However, some CLM lesions showed discoid lupus erythematosus-like epidermal and dermal changes and/or lupus profundus. Vasculitis was also revealed in the CLM lesions of 2 cases. The pathogenesis of CLM may be closely related to its two important features, the male preponderance and the association with SLE. Vasculopathy may also be involved in the development of CLM.
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PMID:Cutaneous lupus mucinosis: a review of our cases and the possible pathogenesis. 940 52

A 38-year-old female with systemic lupus erythematosus presented with abdominal pain, diarrhea and iron-deficient anemia. Computed tomogram showed a 2 x 4 cm inhomogeneous lesion of the right adnexa. An unusual mass was identified extending from the appendiceal orifice at colonoscopy, and an 8 cm tubular appendix, apparently prolapsed into the cecum, was identified at celiotomy. An appendectomy with cecectomy was performed. On cut section, mucin was extruded from the lumen of the appendix. A mucinous neoplasm of the appendix with mucinous dissection to the serosal surface was reported at the time of frozen section. No gross ovarian pathology or peritoneal implants were noted. Cystadenoma with associated mucocele formation was verified by permanent histology. Mucocele of the vermiform appendix is a rare condition associated with neoplastic transformation in approximately 75% of all cases. Benign mucinous cystadenoma of the appendix should be differentiated from cystadenocarcinoma by frozen section at the time of celiotomy to ensure appropriate treatment. While systemic lupus erythematosus can lead to cutaneous mucinosis, an association with mucinous cystadenoma of the appendix has not been previously reported. Surveillance for metachronous colonic neoplasms is warranted in patients diagnosed with a mucinous neoplasm of the appendix.
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PMID:Mucinous cystadenoma of the appendix in a patient with systemic lupus erythematosus. 992 68


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