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)

LE panniculitis is an uncommon but distinctive subset of LE. It may develop in patients with discoid LE or SLE or may occur as an isolated phenomenon. The typical clinical presentation is that of multiple indurated nodules or plaques (or both), often associated with lipoatrophy, there being a predilection for the proximal extremities and trunk. Because the clinical and histologic findings of LE panniculitis overlap with those of other connective tissue diseases, evaluation of patients suspected of having LE panniculitis should include a complete history and physical examination as well as serologic studies, determination of peripheral blood counts, and tests of renal function. A deep excisional biopsy rather than punch biopsy should be performed for diagnosis. The characteristic histologic pattern includes hyaline necrosis of fat; lymphoid nodules, often with germinal centers; and lymphocytic lobular panniculitis. Direct immunofluorescence testing of skin may help confirm the diagnosis in patients who have less than classic histologic features. LE panniculitis tends to have a chronic course marked by recurrent nodules or plaques (or both). Antimalarial agents, with or without courses of systemic steroids, are beneficial in most patients.
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PMID:Lupus erythematosus panniculitis. 267 35

We present two Japanese cases of involutional lipoatrophy. The first case is that of a 30-year-old woman, who first appeared at our hospital complaining of a localized, well-demarcated depression, approximately 3 x 4 cm in size, normal to slightly erythematous in coloration, on the lateral side of the left upper arm (Fig. 1a). The condition was asymptomatic, and she had noticed this anomaly a month prior to consultation. She received intramuscular injections of corticosteroids of unknown dosage at the affected site for the treatment of allergic rhinitis 4 months prior to her present consultation. The second patient, a 23-year-old woman, appeared at our hospital complaining of a similar macule 4 x 4 cm in size, which she noticed several weeks prior to her most recent consultation. She had no history of injury or injection at the site before the development of the condition (Fig. 1b). She had been under treatment for atopic dermatitis since early childhood and was treated only with topical applications of white petrolatum containing 2% salicylic acid for the past several years. In order to rule out the possibility of acquired partial lipodystrophy associated with localized scleroderma, lupus profundus and the other connective tissue diseases, a histological examination was performed for both patients. Histopathological analysis of the region exhibited a well-defined fat lobule composed of numerous small adipocytes (Fig. 1c) embedded in hyaline connective tissue. Edema and dilated capillaries were noticeable in the subcutaneous tissue surrounding the area. Inflammatory cells were not prominent, although mononuclear cells were observed in both patients. No epidermal change was seen in either patient. Direct and indirect immunofluorescence studies revealed no deposits of immunoreactants in the skin of either patient. Immunohistochemical studies with the antibody against macrophage (anti-CD68 antigen; DAKO.) showed that positive cells were scattered around blood vessels and shrunken lipocytes in the subcutaneous tissues (Fig. 1d). Most of these cells in the fat lobules were also positive for mucin stains such as Alcian blue. No abnormal findings came to light in the ordinary hematological and blood chemistry examinations of both patients. The autoantibody screening tests using antinuclear, anti-DNA, anticentromere, and anti-Scl-70 antibodies were negative in both patients.
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PMID:Two Japanese cases of localized involutional lipoatrophy. 1201 Mar 46

A 54-year-old woman developed partial lipodystrophy on the left side of her face. She had been suffering from systemic lupus erythematosus (SLE) since 1985 when she was 45 years old, and she had been treated with 30 mg/day of oral prednisolone as an initial dose. Partial lipodystrophy appeared on her left lower jaw in 1994 when the SLE was inactive, and the dose of prednisolone was reduced to 5 mg/ 3 days. Gradually, the lipodystrophy spread toward her left cheek and her left forehead without any preceding skin symptoms. Histological examination showed a loss of fat tissue and mild lymphocytic infiltrations mainly around cutaneous appendages and vessels in the dermis and subcutaneous tissue. The dose of prednisolone was increased to 10 mg/day and the lesions stopped spreading. Such partial lipodystrophy is distinct from lipoatrophy of lupus profundus.
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PMID:Partial lipodystrophy in a patient with systemic lupus erythematosus. 1207 27

Lipoatrophia semicircularis, a rare entity which presents as atrophic cutaneous indentations exclusively on the anterior thighs of women, is thought to result from physical trauma. Localized lipoatrophies are common following injection with drugs and occur in patients with collagen disease. We report a 10-year-old girl who developed semicircular lipoatrophy on the anterior thighs after treatment of systemic lupus erythematosus (SLE) with subcutaneous injections of methotrexate. Until now, subcutaneously administered methotrexate has not been reported to cause lipoatrophy. Other possible causes include underlying autoimmune disease, the predisposition for females to be affected, and local trauma, also from the injections, at the specific body site. We suggest that subcutaneous injections with methotrexate on the anterior thighs should be avoided or monitored closely in female patients with SLE.
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PMID:Semicircular lipoatrophy in a child with systemic lupus erythematosus after subcutaneous injections with methotrexate. 1238 2

Discoid lupus erythematosus (DLE) is a rare disorder in childhood, with 22 cases reported in the English-language literature. Less than 2% of patients with DLE have an onset before 10 years of age. We describe two children with DLE and lupus profundus with an onset of the disease at the ages of 11 and 15 years and focus on likely histopathologic differences between DLE in children and in adults. Histopathologic characteristics for childhood DLE might be an intense periadnexal and perivascular infiltrate extending into the interstitium and subcutaneous tissue consisting of lymphocytes, histiocytes, eosinophilic granulocytes, and plasma cells and lacking epidermal atrophy. The diagnosis of DLE in our two patients was established by clinicopathologic correlation based on clinical presentation, histologic and immunofluorescent findings in skin biopsy specimens, and the absence of clinical and laboratory evidence of systemic involvement. Therapy with antimalarials resulted in reduction of the skin lesions, but in one patient severe lipoatrophy occurred.
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PMID:Discoid lupus erythematosus and lupus profundus in childhood: a report of two cases. 1265 12

An 11-year-old girl presented with a one-year history of multiple, hard, slightly painful subcutaneous nodules on her right cheek, upper arms, and buttock. Histology of a skin biopsy specimen showed a lobular panniculitis. Laboratory studies revealed positive ANA, anti-double strand DNA, and elevated muscle enzymes. She was diagnosed as having lupus panniculitis. During hydroxychloroquine treatment, erythema over knuckle joints developed. These unusual clinical and laboratory findings of panniculitis associated connective tissue diseases made it difficult to make a precise diagnosis. We report this unusual case of lupus panniculitis with combined features of dermatomyositis resulting in severe lipoatrophy.
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PMID:Lupus panniculitis with combined features of dermatomyositis resulting in severe lipoatrophy. 1549 20

We report a 58-year-old Afro-Caribbean woman who presented with more than 20 dermatofibromas on the body particularly on the back, arms and legs. These developed spontaneously over the course of 5 years. She also had a long-standing 5 x 2-cm area of lipoatrophy on the right upper arm and a 2-year history of several inflammatory subcutaneous nodules developing on the upper chest and left breast. These were confirmed histologically as lupus profundus. Apart from a mild arthritis, she had no other markers for systemic lupus erythematosus and was systemically well. Multiple dermatofibromas are rare. There are around 30 reports of multiple dermatofibromas associated with systemic diseases. More than half of these cases were associated with systemic lupus erythematosus, with or without systemic steroid therapy and about one-third were associated with HIV infection. Although the mechanism is unknown, it appears that multiple dermatofibromas are associated with autoimmune diseases or altered immune states. This is the first case of multiple dermatofibromas associated with lupus profundus. The knowledge of such associations may contribute to the understanding of the pathogenesis of dermatofibromas, which is as yet unknown.
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PMID:Multiple dermatofibromas associated with lupus profundus. 1572 36

Lipoatrophy and localized panniculitis have been described as rare complications of daily subcutaneous glatiramer acetate injections for the treatment of relapsing-remitting multiple sclerosis (MS). We describe the biopsies from two MS patients in a single neurologist's practice who developed clinical lesions of lipoatrophy at the sites of subcutaneous glatiramer acetate injections. These biopsies showed a lobular panniculitis with lipoatrophy that more closely resembled lupus panniculitis than previous reports of localized panniculitis at glatiramer acetate injection sites. In one case, the area of clinical lipoatrophy continued to enlarge for 6 months after stopping glatiramer acetate therapy, before stabilizing at its current size for the last 8 months. Injection site reactions to glatiramer acetate should be considered in the differential diagnosis of biopsies that show a lupus panniculitis-like appearance. Our observations indicate that glatiramer acetate induced panniculitis is common and may continue to progress after therapy has stopped. In this single neurologist's practice, 64% of the patients receiving daily glatiramer acetate injections had clinical evidence of lipoatrophy or panniculitis. Of 100 consecutive patients receiving therapy for MS between February and November 2006, 14 patients were on glatiramer acetate, 9 of whom had clinical lipoatrophy.
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PMID:Lobular panniculitis at the site of glatiramer acetate injections for the treatment of relapsing-remitting multiple sclerosis. A report of two cases. 1833 2

Lupus profundus is a rare lupus-specific skin lesion with skin biopsies exhibiting lobular lymphocytic infiltration and destruction of subcutaneous fat tissue. In this report, a CT scan was effective in demonstrating both the presence and the extent of inflammation of lupus profundus in two patients with systemic lupus erythematosus (SLE). Case 1 was a 30-year-old woman developing erythema with subcutaneous induration on the upper arms during the quiescent phase of SLE. A skin biopsy confirmed a diagnosis of lupus profundus. A CT scan of the right upper arm demonstrated a high density area (HDA) of the subcutis under the erythema: a finding consistent with lupus profundus. Case 2 was a 28-year-old woman recently diagnosed with SLE. She also developed a skin ulcer on the right hip. A CT scan of the hip revealed an HDA and lipoatrophy of the subcutis around the ulcer: these findings were compatible with lupus profundus. Treatment with high-dose prednisolone improved the illness in the both cases. A CT scan is a useful and convenient imaging modality for confirming the diagnosis of lupus profundus.
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PMID:Clinical utility of computed tomographic scanning for the evaluation of lupus profundus in two patients with systemic lupus erythematosus. 1883 69

We observed 5 patients with subcutaneous panniculitis-like T-cell lymphoma (SPTCL) who were unusual, in that they also exhibited features of lupus erythematosus (LE). This observation is in keeping with a recent study that reported an increased rate of autoimmune disease, including systemic lupus erythematosus (SLE), among patients with SPTCL. In all cases, attributes indicating SPTCL included an infiltrate of lymphocytes with pleomorphic nuclei involving subcutaneous lobules exhibiting a cytotoxic T-cell (CD3/CD8/betaF1) immunophenotype. Additionally, a high proliferation rate and a monoclonal T-cell receptor-gamma gene rearrangement were observed in most cases. The manifestations of LE in these patients included a spectrum of clinical and histopathological abnormalities. Clinical manifestations of LE included, in some patients, morphologic evidence of lupus erythematosus panniculitis (LEP) with subcutaneous nodules that healed with lipoatrophy on the face. In addition, all the patients exhibited serologic and/or extracutaneous end-organ abnormalities seen in patients with SLE, with 2 patients having sufficient findings to meet American College of Rheumatology criteria for SLE. Histopathological evidence of LE included vacuolar change at the dermal-epidermal interface in 3 patients, 2 of whom also showed interstitial deposition of mucin in the reticular dermis. One of these patients also had findings of LEP in the subcutaneous lobules with clusters of CD20 B cells partially arranged within germinal centers. In 2 patients in which neither the epidermis nor dermis was available for review, histopathological features of LE included, in one patient, a few small clusters of CD123 plasmacytoid dendritic cells within the adipose tissue and, in the other patient, a positive direct immunofluorescence test (lupus band) on clinically uninvolved and lesional skin. Our study shows that some patients show overlap between SPTCL and LE. We suspect that these patients may suffer from both diseases concomitantly. Furthermore, patients with LE, particularly LEP, should be monitored for evolution into SPTCL with biopsy of any subcutaneous lesion that is not typical of LEP. Additionally, screening for cutaneous LE and SLE could be considered in patients with SPTCL.
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PMID:Subcutaneous panniculitis-like T-cell lymphoma with overlapping clinicopathologic features of lupus erythematosus: coexistence of 2 entities? 1959 Apr 24


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