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[Köbner phenomenon in lupus erythematosus].

In several cases of lupus erythematosus (SLE, SCLE, DLE), the Koebner phenomenon could be seen in the apparently normal skin of patients following traumas, scratching effects, operation scars, contact dermatitis, pressure from sock tops, application of liquid nitrogen, exposure to strong sun light, etc. The interval between exposure to such provocation factors and appearance of the isomorphic reactions varied from case to case. In SLE patients receiving systemic treatment with glucocorticosteroids, the Koebner phenomenon could not be found, while it was detected in patients with generalized DLE who had never received any systemic glucocorticoid hormones. It was not seen in patients with localized DLE. There was no direct relationship between the appearance of humoral autoantibodies and onset of the Koebner phenomenon. With regard to the histopathogenesis of the Koebner phenomenon, it is thought that disturbances of the local cellular repair processes and of the cytokine network, with expression of adhesion molecules, might be involved. The Koebner phenomenon may be a valuable model for the investigation of lupus erythematosus.

Adult↗

Cutaneous warts in patients with lupus erythematosus.

The aim of this study was to determine the frequency of cutaneous warts (CW) in patients with lupus erythematosus (LE) and evaluate the effect of immunosuppressants on the appearance of CW. Fifty-eight patients with LE, 74 with rheumatoid arthritis (RA), and 105 healthy individuals were interviewed and examined for the presence of CW. All were questioned to find out whether their CW developed before or after onset of the disease or during treatment. Cutaneous warts occurred considerably more often in patients with LE than in healthy control and RA patients ( P<0.003 and P<0.04, respectively). The presence of CW did not correlate with the taking of immunosuppressive drugs. The findings suggest that the high prevalence of CW in patients with LE is probably due to defects in some immune mechanisms, independently of immunosuppressive drugs.

Adolescent↗

Kikuchi-Fujimoto's necrotizing lymphadenitis in association with discoid lupus erthematosus: a case report.

BACKGROUND: Kikuchi-Fujimoto's necrotizing lymphadenitis (KFNL) is a rare, benign, self-limited condition characterized by constitutional symptoms, lymphadenopathy, and skin lesions. OBJECTIVE: We report a case of KFNL in a 43-year-old East Indian woman with a ten-year history of discoid lupus erythematosus (DLE) of the scalp and a three-month history of a erythematous plaque on the left nasal bridge, cervical lymphadenopathy, and fever. Skin biopsy samples were taken from the face and lymph node. RESULTS: Histopathological examination of the skin revealed a mixed infiltrate of inflammatory cells, nuclear dust, and histiocytes phagocytosing nuclear debris in the reticular dermis. The lymph node showed interfollicular liquefactive necrosis, immunoblasts, and a similar cellular infiltrate as the skin. The non-necrotic areas demonstrated follicular hyperplasia. These pathological changes are associated with a diagnosis of KFNL. CONCLUSIONS: KFNL is reported in association with systemic lupus erythematosus, but only two other cases of systemic KFNL in association with DLE exist in the literature. This case is unique in that the patient presented with cutaneous and systemic KFNL in the setting of longstanding DLE.

Female↗

A histopathologic study of oral mucosal lupus erythematosus.

Specimens representing 17 cases of each of oral mucosal lupus erythematosus (LE) and lichen planus were examined under the light microscope to establish a set of histopathologic criteria that would distinguish between the two. Statistical analysis showed that significant differences in histopathology exist between the two diseases. A classic case of LE was found to exhibit the following characteristics: vacuolization of keratinocytes, patchy PAS-positive deposits subepithelially, edema in the lamina propria, PAS-positive thickening of blood vessel walls, and the presence of a severe or perivascular inflammatory cell infiltrate. A number of other less significant alterations were also identified. It is concluded from this study that oral LE in most cases is characteristic enough to provide a definitive diagnosis at the light microscopic level.

Adult↗

Serologic profiles as immunologic markers for different clinical presentations of lupus erythematosus.

The clinical and laboratory features of 55 patients with lupus erythematosus (LE), grouped on the basis of six nuclear immunofluorescent pattern results commonly encountered in this disease were examined. Serologic profiles of antinuclear antibodies (ANA), anti-DNA and anti-ENA results can serve as immunologic markers in LE for a benign subset and two other groups with a different incidence of certain clinical characteristics. The large speckle-like thready pattern without antibodies to DNA or ENA is an immunologic marker for a benign LE subset, with generalized skin lesions with or without joint involvement only. Significant levels of the anti-DNA antibodies with the shrunken peripheral, peripheral, or leukocyte-specific ANA with a particulate pattern are markers for severe systemic involvement. The thready pattern with antibodies to ENA (Sm antigen) and leukocyte-specific ANA without a particulate pattern, with or without antibodies to DNA or ENA, indicate less severe systemic disease.

Adolescent↗

The relationship between Sm and RNP antigens.

The relationship between the antigenic proteins of Sm and RNP is not clear. To further clarify their relationship, we examined sera found monospecific by counterimmunoelectrophoresis (CIEP) for anti-Sm or anti-RNP with the more sensitive techniques of immunoblotting, radioimmunoprecipitation, or enzyme immunoassay (EIA). The same eight unique problems were precipitated by both anti-Sm and anti-RNP in radioimmunoprecipitation. They had molecular weights (MWs) of 11, 13, 17, 18, 24, 26, 28, and 68 kDa. The 17, 18, and 28 kDa bands were more intense with anti-RNP. Immunoblotting with anti-Sm and anti-RNP also recognized similar proteins with MWs of 14, 17, 25, 28, 29, 30, 36, 38, and 68 kDa. Anti-Sm resulted in more intense 14, 28, 29, and 30 kDa bands, while anti-RNP gave maximum intensity of the 14, 36, 38, and 68 kDa bands. The band intensity pattern differences were more easily appreciated with immunoblotting than with radioimmunoprecipitation. RNase, heat, and urea caused a similar diminution of antigen reactivity with both anti-Sm and anti-RNP on immunoblotting, but eliminated immunoprecipitability only of RNP on immunodiffusion. The great similarities between Sm and RNP suggest several possibilities: Anti-Sm and anti-RNP antibodies coexist in the same patients; and the more sensitive techniques of immunoblotting and radioimmunoprecipitation detect both precipitating and nonprecipitating antibodies while only precipitating antibodies are detected by immunodiffusion. Sm and RNP may represent different determinants on the same macromolecular complex. Sm and RNP may be cross-reacting determinants on distinct molecules.

Adolescent↗

Tertiary syphilis of the face.

We report a case of noduloulcerative tertiary syphilis that had gone misdiagnosed as discoid lupus erythematosus for many years. This case is noteworthy for (1) a Jarisch-Herxheimer-like reaction, (2) a dramatic response to penicillin therapy despite the presence of long-standing disease, and (3) posttreatment granulomas of possible hypersensitivity origin.

Diagnosis, Differential↗

Neonatal lupus erythematosus in Japan.

BACKGROUND: Although more than 200 cases of neonatal lupus erythematosus (NLE) have been reported, the prognosis, clinical characteristics, and genetic background of the patients are still obscure. Their symptoms seem to vary in different races. OBJECTIVE: We had an opportunity to see two clinically different types of NLE. It is important to define the clinical characteristics in Japanese cases and to compare them to caucasian NLE cases reported earlier. METHODS: Sixty Japanese infants with NLE and their mothers reported in Japan were investigated and compared with cases reported from other countries. RESULTS: Japanese cases were highly associated with anti-SS-A/Ro, anti-SS-B/La, anti-ribonuclear protein, and anti-DNA antibodies. A low frequency of congenital heart block was noted but 8.3% of the cases progressed to systemic lupus erythematosus. HLA-DRw12 was a significantly relative risk in NLE. CONCLUSION: The clinical characteristics of Japanese NLE patients were different from those of caucasian patients reported in the literature.

Adult↗

Deposition of membrane attack complex in cutaneous lesions of lupus erythematosus.

BACKGROUND: The membrane attack complex (MAC; C5b-9) localizes in the basement membrane zone (BMZ) of cutaneous lesions from patients with lupus erythematosus (LE) and has been implicated in the pathogenesis of this disease. OBJECTIVE: Our purpose was to compare the frequency of MAC deposition with that of immunoglobulin and C3 deposition (lupus band). METHODS: We studied 56 skin biopsy specimens from 42 patients with LE by direct immunofluorescence. RESULTS: MAC was deposited in a granular pattern at the BMZ in 29 of 38 biopsy specimens (76%) from lesional skin; 5 of 38 specimens (13%) had focal or weak segmental deposition and 4 of 38 (11%) were negative. In contrast, IgG, IgM, IgA, and C3 were detected in 16 of 38 specimens (42%), 30 of 38 (79%), 4 of 38 (11%), and 22 of 38 (58%), respectively. None of the uninvolved skin biopsy specimens was MAC positive, although 4 of 18 (22%), 5 of 18 (28%), 1 of 18 (6%), and 2 of 18 (11%) were positive for IgG, IgM, IgA, and C3, respectively. CONCLUSION: MAC deposition in lesional skin appears to be a relatively sensitive and specific marker for cutaneous LE and may be useful as an adjunct to the "lupus band" test.

Basement Membrane↗

Pimecrolimus 1% cream for cutaneous lupus erythematosus.

Topical treatment of cutaneous lupus erythematosus usually includes potent glucocorticosteroids. However, prolonged use causes adverse side effects including skin atrophy as the foremost concern. In contrast to glucocorticosteroids, the anti-inflammatory and immunosuppressive macrolactam pimecrolimus has no atrophogenic potential. Affected areas of 11 patients with different forms of lupus erythematosus were treated with pimecrolimus 1% cream under semiocclusive conditions twice daily for 3 weeks. Skin involvement before and after therapy was assessed by means of a clinical score. In all patients, significant regression of skin lesions was observed after therapy (P <.001). This was an open and uncontrolled study on a limited number of cases. We suggest that pimecrolimus 1% cream could be an efficacious and safe treatment option for cutaneous lupus erythematosus.

Administration, Cutaneous↗