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PubMed1988

[Chilblain lupus erythematosus].

Chilblain lupus erythematosus is a chronic form of lupus erythematosus that is seen predominantly in women and first manifests itself during cold weather as painful purplish acral swellings. These symptoms and possible progression to systemic lupus erythematosus are discussed with regard to two cases. Patients with recurrent or persistent "chilblains" should be investigated histologically and serologically as well as by immunofluorescence.

Aged↗
PubMed1997

Approaches to the management of systemic lupus erythematosus.

Systemic lupus erythematosus is a difficult disease to study with a variable disease course characterized by exacerbations and remissions. A variety of biologic agents are under investigation as potential treatments for systemic lupus erythematosus, either in murine disease models or in clinical trials. These products are designed to specifically interfere with the following immunologic processes: T-cell activation and T-cell-B-cell collaboration, production of anti-dsDNA antibodies, deposition of anti-dsDNA antibody complexes, complement activation, and deposition, and cytokine activation and modulation. More aggressive interventions include gene therapy and stem-cell transplantation. Recently developed immunomodulators have been studied in patients with systemic lupus erythematosus 2'-Chlorodeoxyadenosine, mycophenolate mofetil, and leflunomide. Additional innovative pharmaceutical treatments include the mild androgen dehydroepiandrosterone, estrogen antagonists, including tamoxifen and selective estrogen receptor modulators, and the prolactin inhibitor bromocriptine. Other promising pharmaceutical interventions include products designed to inhibit synthesis of the proinflammatory mediators: prostaglandins, leukotrienes, and nitric oxide. Although previously regarded as an indication to be avoided in the development of new therapeutics, enthusiasm for studying systemic lupus erythematosus in clinical trials now exists. A variety of biologic and pharmaceutical agents offer promise as potential therapies. As with rheumatoid arthritis, development of these products will benefit from active involvement of rheumatologists and efforts to develop international consensus regarding trial methodology and outcome measures.

Animals↗
PubMed1991

Immunohistochemical detection of proliferation and differentiation in discoid lupus erythematosus.

Discoid lupus erythematosus lesions show hyperkeratosis and atrophy, which may reflect abnormal epidermal proliferation, differentiation, or both. In this investigation, markers for epidermal proliferation, differentiation and inflammation were studied in cutaneous lesions of discoid lupus erythematosus. Frozen sections of biopsy specimens from 20 patients were examined immunohistochemically regarding Ki-67 staining and keratin 16 expression (parameters for proliferation), and the expression of keratin 10, involucrin, and filaggrin (parameters for differentiation). The inflammatory infiltrate was characterized with the use of antibodies against T lymphocytes, monocytes/macrophages, and Langerhans cells. With these markers, epidermal proliferation was found to be increased in discoid lupus erythematosus. Keratin 10 expression, a marker for early differentiation, showed the pattern of normal skin. Involucrin and filaggrin, markers for terminal differentiation, were expressed already in the lower part of the stratum spinosum, whereas in normal skin these markers were restricted to the stratum granulosum and the upper layers of the stratum spinosum, and the stratum granulosum and stratum corneum, respectively. Infiltrate analysis revealed the well-established picture. We conclude that in cutaneous lesions of discoid lupus erythematosus, hyperproliferation is combined with normal early differentiation and premature terminal differentiation of keratinocytes.

Adult↗
PubMed2003

Diagnosis of systemic lupus erythematosus.

Systemic lupus erythematosus is a multisystem inflammatory disease that is often difficult to diagnose. Before the diagnosis can be established, four of 11 clinical and laboratory criteria must be met. Antinuclear antibody titer is the primary laboratory test used to diagnose systemic lupus erythematosus. Because of the low prevalence of the disease in primary care populations, the antinuclear antibody titer has a low predictive value in patients without typical clinical symptoms. Therefore, as specified by the American College of Rheumatology, this titer should be obtained only in patients with unexplained involvement of two or more organ systems. Patients with an antinuclear antibody titer of 1:40 and characteristic multiorgan system involvement can be diagnosed with systemic lupus erythematosus without additional testing; however, patients with an antibody titer of 1:40 who fail to meet full clinical criteria should undergo additional testing, including tests for antibody to double-stranded DNA antigen and antibody to Sm nuclear antigen. While an antinuclear antibody titer of less than 1:40 usually rules out systemic lupus erythematosus, patients with persistent, characteristic multisystem involvement may be evaluated for possible antinuclear antibody-negative disease.

Algorithms↗
PubMed1986

Large vessel occlusion and gangrene in systemic lupus erythematosus and "lupus-like" disease. A report of six cases.

Six patients are presented, all of whom had systemic lupus erythematosus or a "lupus-like" disease and who developed major thromboses with gangrene of the extremities. Four of the 6 patients had circulating antiphospholipid antibodies at some point during the course of their illness. These serological markers, which have been associated with a tendency to thrombosis, may have contributed to the development of gangrene in these patients. Histological examination of affected arteries in 4 patients did not show any evidence of vasculitis. One patient in whom antiphospholipid antibodies were negative showed healing vasculitis on histology.

Adult↗
PubMed1990

[Chronic lupus erythematosus, subacute cutaneous lupus].

The cutaneous manifestations of lupus erythematosus (LE) are extremely varied, and they predominate among the clinical signs of chronic LE and subacute cutaneous LE. In chronic LE, typical lesions are found in regions exposed to sunlight and consist of erythema, hyperkeratosis and skin atrophy. The skin of patients with subacute cutaneous LE is also highly sensitive to light; the lesions consist of an annular or polycyclic or sometimes psoriasiform erythemato-squamous eruption observed mainly in uncovered areas. These dermatological forms are part of the wide spectrum of "lupus". Cutaneous manifestations are often associated with systemic signs of varying type and severity and with immunological abnormalities, such as antibodies to Ro in subacute cutaneous LE, which act as markers of the disease.

Adult↗
PubMed1984

Oral discoid lupus erythematosus. II. Skin lesions and systemic lupus erythematosus in sixty-six patients with 6-year follow-up.

Sixty-six patients with oral discoid lesions and either discoid (DLE) or systemic (SLE) lupus erythematosus were followed during an average period of 6 years (range, 0.1 to 25 years). At time of initial examination, thirty-seven patients (56%) had discoid skin lesions and the remaining 29 (44%) had isolated oral discoid lesions. Among the latter, seven patients developed discoid skin lesions during the observation period. Ten of the sixty-six patients (15%) had SLE at the time of initial examination, and SLE developed in another five (8%) who had DLE initially. In six of fifteen SLE patients the oral discoid lesions were the first manifestation of SLE. Presence of ulceration of the oral discoid lesions at time of initial examination was of predictive value with regard to the presence or development of SLE. It is concluded that patients with oral discoid lesions should be seen at least yearly in order to secure an early diagnosis of eventually developing signs of cutaneous DLE and SLE. Furthermore, no connection was noted between the presence of skin lesions and the development or presence of SLE.

Adult↗
PubMed1984

[Drug-induced lupus erythematosus syndrome. Pathogenesis and relation to systemic lupus erythematosus].

The drug-induced lupus erythematodes syndrome is a heterogeneous group of exogenically caused transitory autoimmunopathies . Essential knowledge has been got concerning the pathomechanism of the syndromes evoked by hydralazine, procainamide, diphenylhydantoin and Venopyronum . Though there are analogies to the systemic lupus erythematosus, we must take into consideration a diversity of diseases. Antibodies against histone have a certain diagnostic as well as pathogenetic relevance.

Diagnosis, Differential↗
PubMed2002

Follicular lupus erythematosus: a new cutaneous manifestation of systemic lupus erythematosus.

We report the clinical, histopathological and immunological features of follicular erythema and petechiae in a 30-year-old Japanese woman with systemic lupus erythematosus (SLE). Histology showed this eruption to constitute a cutaneous manifestation of SLE. To our knowledge, this is the first reported case of follicular erythema and petechiae in association with SLE. Accordingly, we propose that this rare eruption be termed 'follicular lupus erythematosus'.

Adult↗
PubMed2003

Bullous eruption: a manifestation of lupus erythematosus.

Bullous lupus erythematosus (BSLE) is a rare subset of systemic lupus erythematosus (SLE), often associated with autoimmunity to type VII collagen. Generally, patients with BSLE meet the criteria for SLE as defined by the American College of Rheumatology. We present a case of a 17-year-old adolescent girl who presented with a vesiculobullous eruption without detectable type VII collagen antibodies and without full criteria for SLE. Differential staining was characteristic for lupus erythematosus (LE), suggesting her eruption is related to LE. We review the spectrum of bullous disease in patients with LE and discuss the pathogenesis and histology of these eruptions, as well as current therapeutic options.

Adolescent↗
PubMed2000

Lupus erythematosus tumidus--a neglected subset of cutaneous Lupus erythematosus: report of 40 cases.

BACKGROUND: Lupus erythematosus tumidus (LET) is characterized clinically by erythematous, succulent, edematous, nonscarring plaques in sun-exposed areas. Results of histological examination show perivascular and periadnexal lymphocytic infiltration and interstitial mucin deposition. The main differential diagnoses are polymorphous light eruption, Jessner's lymphocytic infiltration of the skin, reticular erythematous mucinosis, and pseudolymphoma. Since its first description in 1930, LET has been documented rarely in the literature, and its clinical importance has not been fully appreciated. OBSERVATIONS: We characterized 40 patients with clinical and histological features of LET observed at our department from 1984 through 1998. The onset of the disease clustered in summer because of sun exposure, and 28 (70%) of the patients showed a remarkable photosensitivity confirmed by results of provocative phototesting. A complete resolution of the skin lesions was seen after systemic therapy with antimalarials and, in some cases, with local corticosteroids or spontaneously without any treatment. In 4 (10%) of the patients, antinuclear antibodies were detected; however, there was no evidence of underlying systemic involvement in any of the patients. CONCLUSIONS: Our data constitute the largest number of patients with LET collected until now. The clinical picture, extreme photosensitivity, histological findings, and effective treatment with antimalarials are so characteristic that LET should be considered as a separate entity and differentiated from other variants of cutaneous LE. Arch Dermatol. 2000;136:1033-1041

Adolescent↗
PubMed1993

Neonatal lupus erythematosus.

Neonatal lupus erythematosus (NLE) is an autoimmune disease whose major findings are subacute cutaneous lupus erythematosus (SCLE) skin lesions and congenital heart block. Babies have maternal anti-Ro/SSA, anti-La/SSB, or anti-U1RNP autoantibodies. Anti-Ro/SSA are the predominant autoantibodies, having been found in about 95% of cases. The autoantibodies pass through the placenta from mother to child. Skin disease resolves at about the time that maternal autoantibodies can no longer be detected in the baby. NLE therefore provides the strongest clinical evidence that autoantibodies are involved in at least some manifestations of lupus erythematosus, but there is as yet no definitive evidence implicating autoantibodies in the disease process. Skin disease usually begins after birth, is transient, and does not result in scarring. Cardiac disease begins in utero, and the heart block is almost always permanent. Many babies require pacemakers, and about 10% die from complications related to cardiac disease. In some cases, transient liver disease or thrombocytopenia have been observed. Individuals who had NLE usually have healthy childhoods but may develop autoimmune disease in adulthood. Whether the later development of autoimmune disease is a common or an unusual event is not yet known. Mothers of babies with NLE may be asymptomatic initially, but with time usually develop symptoms of autoimmune disease. The most typical constellation of symptoms in our group of approximately 30 mothers of babies with NLE is that of Sjögren's syndrome. Most babies exposed to anti-Ro/SSA autoantibodies during gestation will not develop NLE. There is no test to determine prospectively which babies will be affected. Treatment during gestation is still controversial and, if attempted, should be reserved for fetuses with potentially life-threatening disease. Treatment after birth consists of topical management for skin disease and pacemaker implantation, if necessary, for heart block. Systemic steroids may be given for serious internal disease.

Adult↗
PubMed1988

[Bullous systemic lupus erythematosus].

Bullous lupus erythematosus (BLE) is an uncommon variant of systemic lupus erythematosus (SLE). We present a 23-year-old man with SLE, who developed a bullous eruption associated with a flare of the systemic disease. Differential diagnosis and problems of diagnosis are discussed.

Adult↗