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PubMed2005

Systemic sclerosis with unusual panniculitis and overlying discoid lupus erythematosus-like lesions.

A 33-year-old men presented with systemic sclerosis accompanied by both unusual panniculitis and overlying discoid lupus erythematosus (DLE)-like skin changes on the left buttock. The lesion did not completely match either lupus erythematosus profundus or morphea profunda but featured clinical and pathological findings halfway between the two entities. No case report of co-existence of systemic sclerosis and morphea profunda was found in the literature, but there are three case reports of patients with systemic sclerosis and lupus erythematosus profundus. One of the three cases had DLE-like changes on the surface of the lesion, a clinical picture similar to our case. We speculated that the DLE-like changes in the overlying skin were due to local tissue ischemia. The accompanying panniculitis in our case was considered to be unique and somehow different from morphea profunda or lupus erythematosus profundus.

Adult↗
PubMed1987

Natural killer cell activity of peripheral blood mononuclear cells from patients with various forms of lupus erythematosus.

Natural killer (NK) cell activity of peripheral blood mononuclear cells from 28 patients with cutaneous discoid lupus erythematosus, seven with subacute cutaneous lupus erythematosus, and 17 with systemic lupus erythematosus was studied using a 4 h 51Cr release assay using K-562 cells as target cells. NK cell activity was found to be markedly decreased not only in patients with systemic lupus erythematosus but also in a proportion of cases of subacute cutaneous lupus erythematosus and cutaneous discoid lupus erythematosus (57% and 39% respectively). There was a relationship between the activity and severity of LE and the decrease in NK cell activity.

Adult↗
PubMed2002

Comparison of three monoclonal and three polyclonal antibodies in the immunohistochemical diagnosis of canine autoimmune skin diseases.

The aim of this study was to evaluate the utility of three monoclonal antibodies (mAbs), and two anticanine IgG and one anticanine IgM polyclonal antibodies (pAbs) for the immunohistochemical diagnosis of canine autoimmune skin diseases. Skin biopsies from 11 cases of pemphigus (7 foliaceus, 3 vulgaris and 1 erythematosus), 12 cases of discoid lupus erythematosus (DLE) and 12 cases of chronic hyperplasic dermatitis were used. The CA4E7 mAb (IgG1 + IgG2) showed similar sensitivity, but higher specificity and lower background than the two anti-IgG pAbs for the immunohistochemical diagnosis of pemphigus and DLE. The CA4F1 mAb (IgG2) and CA3H1 mAb (IgG2) showed moderate and low interepithelial reactivity, respectively, in autoimmune skin diseases, but strong staining of the cytoplasm of plasma cells of the inflammatory infiltrates. These results suggest that the CA4E7 mAb may be valuable in the immunohistochemical diagnosis of such disorders.

Animals↗
PubMed1986

Discoid lupus erythematosus-like lesions in an autosomal form of chronic granulomatous disease.

Chronic granulomatous disease (CGD) is characterized by a bactericidal defect involving the oxidative metabolism of polymorphonuclear leukocytes (PML) and is most often transmitted as an X-linked trait. The cutaneous features of this disorder include infections and lupus-like rashes. These have been described in female carriers as well as in males with the disease. Two cases of siblings presenting an autosomal form of CGD syndrome, with lupus-like cutaneous manifestations, are reported here.

Adolescent↗
PubMedDate not supplied

[Lupus profundus].

Seven cases of lupus erythematosus profundus are described (chronic discoid lupus erythematosus with athrophic panniculitis) with dissemination of one of them. All patients were females. The authors stress their opinion that it should not be called as lupus profundus, the cases of lupus erythematosus disseminated with erythema nodosum neither the cases of L.E.S. preceded or associated with the so called Weber-Christian's type of panniculitis. Six of the cases had athrophic alopecia of scalp by lupus discoid.

Adolescent↗
PubMed1979

Isomorphic skin reaction with DNCB in SLE and DLE.

DNCB sensitization was studied in 76 cases of systemic and 44 of discoid lupus erythematosus and in 101 immunosuppressed and tumor patients as controls, as well as in 40 contact-sensitized eczematous and 115 unselected patients. Sensitization of patients with discoid LE and that of mixed patients are of the same order, while it is lower in the systemic LE group. The highest incidence was found in the polysensitized positive control group; the lowest in the immunosuppressed and tumor patients. DNCB sensitization showed clear age dependency in the different groups. At the site of the DNCB exposure, isomorphic reactions were found in 43% of discoid and 25% of systemic LE patients. Histological and immunofluorescence studies supported the findings of the clinical picture, i. e. the appearance of isomorphic reactions at the site of the exposure cannot be related either to the intensity of the early reaction or with the developing intensity.

Adult↗
PubMed1990

Discoid lupus erythematosus-like lesions in carriers of X-linked chronic granulomatous disease.

A questionnaire was sent to 16 carriers of the X-linked cytochrome-b558 negative variant of chronic granulomatous disease (CGD). Of the 15 who answered the questionnaire and from data of one additional case, 70% reported recurrent aphthous stomatitis and 63% had recurrent skin eruptions. Five of the carriers (31%) had clinically discoid lupus erythematosus (DLE), although the histopathology was not typical and the immunofluorescence findings were negative. The infiltrate in the lesional skin of three of these patients was analysed using monoclonal antibodies against CD3, CD4, CD8, CDIa, HLA-DR, CDIc, interdigitating cells, B lymphocytes and cells of the monocyte/macrophage lineage. The immunophenotype of the infiltrating cells resembled that found in discoid lupus erythematosus.

Adolescent↗
PubMed1989

Interferon response to dipyridamole in lupus erythematosus patients.

Studies in patients with autoimmune disorders strongly support a role for interferon (IFN) in the disease process. In the present study we investigated the in vivo production of alpha-IFN in lupus erythematosus patients after stimulation with dipyridamole, recently characterized as an alpha-IFN inducer in mice and humans. Levels of IFN were measured in serum samples from 22 patients with systemic lupus erythematosus (SLE) and 12 patients with discoid lupus erythematosus (DLE) before and 24 h after dipyridamole administration. IFN activity was assayed on human and bovine cells in parallel. Initial serum concentrations of alpha-IFN in SLE patients were markedly elevated. The percentage of DLE positive responders to dipyridamole induction was about twice as high as that found for SLE. Studies of factors responsible for IFN production in lupus erythematosus might result in better understanding of the relationship between DLE and SLE.

Dipyridamole↗
PubMedDate not supplied

Serological levels of apoptotic bodies, sFAS and TNF in lupus erythematosus.

In our study we have investigated the presence of apoptotic bodies, soluble FAS receptor and TNF (tumor necrosis factor) in three clinical forms of lupus erythematosus. Determinations were performed in attack period of: systemic lupus erythematosus (SLE) for 20 patients, 20 patients with subacute cutaneous lupus erythematosus (SCLE), 20 patients with chronic discoid lupus erythematosus (DLE). Determinations were performed by ELISA (for apoptotic bodies, kit Boehringer, normal values 400-800 mU), (for sFAS, kit R&D Systems, normal values 4500-17000 pg/ml) (for TNF, ELISA kit R&D Systems, normal values 0.4-3.6 pg/ml). Results in SLE: apoptotic bodies were increased in 16 cases (980-1030); sFAS in 18 cases (17000-24000 pg/ml) TNF was increased in all 20 cases (40-140 pg/ml). In SCLE with multiple cutaneous lesions and without internal organs disturbance the apoptotic bodies were increased in 10 cases (960-1030 pg/ml), sFAS in 9 cases (17000-22000 pg/ml), and TNF alpha in 9 cases. In DLE, apoptotic bodies were increased in 2 patients (980-1010 pg/ml), sFAS in 3 patients (17000-20000 pg/ml) and TNF in 2 patients (20-40 pg/mil). Investigated values were slightly correlated with immune parameters (anti dsDNA antibodies), but they were correlated with the presence of renal disturbances or extension of cutaneous lesions. We consider that the presence of increased apoptotic bodies as a result of peripheral mononuclear cells apoptosis appear as a nauto-limiting mechanism in a pathological immune response. The increase of sFAS in lupus patients serum might be interpreted as an alteration of apoptosis respectively a deficit in apoptosis which has as a first consequence the persistence of B and T lymphocytes, activated, in the pathogen immune response.

Apoptosis↗
PubMed2005

Lupus erythematosus in children: a report of six cases.

The clinical features of childhood discoid lupus erythematosus (DLE) are similar to those of adult DLE in presentation and chronic course. However, children have a particularly high levels of transition to systemic disease. Systemic lupus erythematosus (SLE) is the most common rheumatic disease associated with significant morbidity and mortality in children. This is a retrospective study reporting all cases of childhood lupus erythematosus observed in the dermatology department of Habib Thameur Hospital over a 14-year period. From 1989 to 2003, six cases of childhood lupus erythematosus are included, three patients with discoid lupus erythematosus (2 girls, 1 boy), and three patients with systemic lupus erythematosus (2 boys, 1 girl). The mean age of onset was 12 years (range 10-16 years). Skin manifestations were localized in sun exposed areas in both discoid and systemic lupus erythematosus. Photosensitivity was noted in all cases. The diagnosis was confirmed by histopathologic examination, direct immunofluorescence, and immunologic findings. Treatment included sun avoidance, oral hydroxychloroquine, and topical and systemic steroids. An average follow-up time was 18.1 months (1-96 months). The severity of onset of SLE is usually greater in children than adults. We note that lupus erythematosus is not a static disease and progression from DLE to SLE is possible.

Adolescent↗