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

Lupus erythematosus profundus (panniculitis) is an unusual clinical variant of lupus erythematosus in which the lesions occur in deeper corium and panniculus. The diagnosis usually causes difficulty. Both reported cases are similar and concern young men with subcutaneous nodules appearing on the face and leaving depressed areas. Histological examination reveals a marked infiltrate in the dermis especially around the skin appendages, reaching the deep dermis and the fat tissue; this infiltarte is composed mainly of lymphocytes and histiocytes. Some moderate epidermal changes are present: liquefaction degeneration of the basal cell layer, epidermal atrophy, thickening of basement membrane (P. A. S.). Direct immunofluorescence of the lesions showed, in one case IgG deposits at the dermo-epidermal junction. Ultrastructural findingd are similar in both cases and may be an important point of diagnosis for isolated forms: -- tubular structures "paramyxo-virus-like-inclusions" are present in dermal infiltrate, endothelial cells and sometimes in keratinocytes of the basal layer; -- the basal lamina has many folds and villous projections;-- vacuoles and concentric lamellar formations are observed inside keratinocytes. No clinical or immunological signs of systemic lupus were found. Antimalaric drugs clear up the nodules.
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PMID:[Lupus erythematosus profundus: two cases with ultrastructural study]. 67 84

A 4 year old boy with lupus panniculitis since the third month is presented. Typical discoid lupus erythematosus lesions were also present. There were no signs of systemic involvement. Lupus panniculitis is an uncommon subtype of cutaneous lupus erythematosus. Our case is very unusual as the lesions developed already 3 month after birth. Clinical manifestation, therapy and histopathology of lupus panniculitis are discussed.
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PMID:[Lupus erythematodesus profundus]. 395 91

Lupus panniculitis usually affects the hypodermis and the dermis profundus (Irgang's lupus profundus). In our case, the systemic lupus erythematosus was preceded by an intraabdominal panniculitis, occurring as a voluminous tumor. Histological examination showed the usual picture of panniculitis, but there were also severe fibrinoid necrotic lesions, which should have alerted us. Lupus must be included among the etiologies of intraabdominal panniculitis.
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PMID:[Pseudo-tumorous panniculitis of the mesentery. An unusual initial stage of acute lupus erythematosus in a 10-year-old girl]. 630 53

Lupus panniculitis is a rare variant of cutaneous lupus erythematosus. Two typical cases demonstrate the association of lupus panniculitis with signs of discoid lupus erythematosus and systemic lupus erythematosus. Clinically it is characterised by subcutaneous nodules or plaques, which may ulcerate leaving depressed atrophic scars. Histologically the lesions show lobular panniculitis. A positive immunofluorescent band test with deposition of IgG, IgM and C3 at the dermal-epidermal junction and blood vessel walls helps to confirm the diagnosis. The knowledge of the clinical features and histopathology of the disease is important because lupus panniculitis may precede systemic lupus erythematosus by some years.
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PMID:[Kaposi-Irgang lupus erythematosus profundus. Lupus erythematosus panniculitis]. 815 Jun 15

Lupus erythematosus profundus (LEP) is an unusual variant of cutaneous lupus erythematosus (CLE)that is characterized by chronic, recurrent inflammation of the subcutaneous tissue leading to fibrosis. It is found in the settings of both discoid and systemic lupus erythematosus. Generalized forms are extremely rare. We present a case of generalized LEP associated with genetic partial C4-deficiency.
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PMID:Generalized lupus erythematosus profundus in a patient with genetic partial deficiency of C4. 1042 34

Lupus erythematosus profundus is a rare manifestation of lupus. We conducted a study to observe the epidemiologic and clinical aspects of lupus erythematosus profundus, its development into other types of lupus or systemic disease, and its response to treatment. Six patients were followed for variable periods. We conclude that lupus erythematosus profundus is a benign form of lupus, distinguishable by systemic manifestations, clinical evolution and resolution, and heterogeneity in clinical manifestations. A skin biopsy is necessary to make an accurate diagnosis.
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PMID:Lupus erythematosus profundus: case reports. 1141 17

Lupus panniculitis has been reported to occur with a frequency of 2-3% in patients with lupus erythematosus (LE). It is most often reported in association with lesions of discoid LE. We describe a patient with subacute cutaneous LE who developed calcified nodules that were histopathologically consistent with lupus panniculitis. She was treated with a combination of chloroquine and diltiazem with a good therapeutic response. The addition of diltiazem may be beneficial in patients with calcified nodules of lupus panniculitis.
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PMID:Calcifying lupus panniculitis in a patient with subacute cutaneous lupus erythematosus: response to diltiazem and chloroquine. 1155 Sep 87

Lupus panniculitis is characterized by a T-cell lymphocyte infiltration of the fat, with fibrosing and cicatricial progression. The lesions are predominantly found on the head and the upper part of the body; they are more frequent in young women. Diagnosis is based on the integration of the clinical and histological data, which renders a deep cutaneous biopsy indispensable. Histopathology is the key to the differential diagnosis, which essentially includes subcutaneous T-cell lymphoma. The lupus panniculitis lesions can be isolated or, more often, integrated within a known lupus disease, usually of good prognosis. Treatment relies above all on synthetic anti-malarials, occasionally associated with local or general corticosteroids.
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PMID:[Lupus panniculitis]. 1579 39

Lupus panniculitis is an unusual immunological disease that characteristically affects the subcutaneous fat and occurs in 2% of patients with systemic lupus erythematosus. We report a case of lupus panniculitis involving the breast, which represents a very uncommon location. Mammographically, it presented as a suspicious irregular mass involving the subcutaneous fat pad with skin thickening. High echogenicity constituted the most relevant sonographic finding. To the best of our knowledge, the magnetic resonance (MR) features have not been previously described. High signal intensity was found on both T1- and T2-weighted precontrast MR images. A dynamic contrast-enhanced study revealed a suspicious focal mass with irregular margins and rim enhancement, with a type 3 time-signal intensity curve. Differential diagnosis with carcinoma and fat necrosis and the value of core biopsy are discussed.
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PMID:Lupus panniculitis involving the breast. 1593 81

Lupus erythematosus profundus or lupus panniculitis is a rare clinical variant of lupus erythematosus, which involves the deep dermis and subcutaneous fat. Diagnosis may be difficult in cases with isolated involvement. Further manifestations of lupus erythematosus may thus be essential for diagnosis, which depends on the clinical picture, histopathology and a positive lesional lupus band test. We report a severe, mutilating case of lupus panniculitis, which responded well to thalidomide.
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PMID:Facets of lupus erythematosus: panniculitis responding to thalidomide. 1807 56


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