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Discoid lupus erythematosus associated with a primary immunodeficiency syndrome showing features of non-X-linked hyper-IgM syndrome.

Hyper-IgM (HIM) syndrome is a rare primary immunodeficiency disorder. Approximately 120 cases have been described in the literature world-wide. Features of HIM include low serum IgG, a very low IgA with normal or high IgM levels. Autoimmune phenomena are recognized associations but connective tissue disorders have so far not been described in HIM patients. We report the case of a 19-year-old Indian woman with an immunodeficiency syndrome characteristic of non-X-linked HIM who developed discoid lupus erythematosus. Anti-double-stranded DNA antibodies were negative. Antibodies to extractable nuclear antigens were positive for Ro and nRNP, with evidence that they were of both IgG and IgM class. Treatment with hydroxychloroquine and topical steroids was successful.

Adult↗

Retinitis pigmentosa and discoid lupus erythematosus.

A 41 year old male is presented who suffers from both advanced retinitis pigmentosa and active discoid lupus erthematosus. A possible association between the two pigmenting disorders is discussed as well as the treatment of the discoid lupus with potentially retinotoxic hydroxychloroquine.

Adult↗

[Soluble interleukin 2 receptor as activity parameter in serum of systemic and discoid lupus erythematosus].

The evaluation of disease activity in systemic lupus erythematosus (SLE) is important for selection of the appropriate therapeutic regimen. In addition to the clinical picture, various laboratory parameters are taken into account. However, no validated criteria for the evaluation of the disease activity in SLE have yet been established. Recently, serum levels of soluble interleukin-2 receptor (sIL-2R) have been proposed as a potential parameter for disease activity in SLE. However, the studies reported on this subject so far have focused mainly on certain subsets of the disease, and the evaluation of the disease activity was based on a very limited number of parameters. In the present study, we determined serum levels of sIL-2R in 23 patients with SLE and 30 patients with discoid LE (DLE). Evaluation of disease activity in SLE was based on a comprehensive scale which considered numerous clinical signs and laboratory parameters. In SLE, serum levels of sIL-2R showed a better correlation with disease activity than all the other parameters investigated, including proteinuria, erythrocyte sedimentation rate, serum globulin concentration, titre of antibodies against double-stranded DNA, serum albumin concentration, serum complement levels and white blood cell count. For the first time, we report on elevated serum levels of sIL-2R in DLE, which also correlated with disease activity.

Adolescent↗

Clinical, serologic and immunogenetic studies in patients with chronic cutaneous (discoid) lupus erythematosus who have verrucous and/or hypertrophic skin lesions.

Verrucous and/or hypertrophic lesions occasionally occur in patients with lupus erythematosus. It has been suggested that these patients have skin disease which is characterized by chronicity and resistance to therapy other than intralesional corticosteroids. We studied 8 patients with chronic cutaneous (discoid) lupus erythematosus and 1 patient with papulosquamous subacute cutaneous lupus erythematosus with verrucous and/or hypertrophic lesions. Serologic, immunogenetic, and clinical data were gathered to confirm the distinctiveness of this subset and to seek specific laboratory correlates.

Adrenal Cortex Hormones↗

Periorbital oedema and erythema as a manifestation of discoid lupus erythematosus.

Periorbital oedema and erythema are a rare cutaneous manifestation of discoid erythematosus (DLE) which is well known to ophthalmologists but less to dermatologists. We report the case of a 74-year-old female patient with periorbital oedema and erythema. Since swelling of the eyelid is common in the dermatological practice, dermatologists should be aware of the differential diagnosis and its occurrence in patients with DLE.

Aged↗

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↗

[Lupus erythematosus discoides or lichen planus (author's transl)].

A patient with well-defined erythematous lesions with some atrophy on the scalp and face and lichen planus papules on the limbs and sacral region is reported. The histopathological features and the deposits of immunoglobulins in the cutaneous lesions confirm the presence of both diseases. The difficulties of the differential diagnosis are due to the interaction between lupus erythematosus chronicus discoides and lichen planus probably occurring in the same patient.

Diagnosis, Differential↗

Oral discoid lupus erythematosus. III. A histopathologic study of sixty-six patients.

A detailed histopathologic study was conducted blindly on biopsy specimens from 66 patients with oral discoid lesions (oral DLE) mixed in random order with biopsy specimens from control groups of 25 cases of oral lichen planus (LP), 25 cases of oral leukoplakia, 13 cases with an uncertain diagnosis termed DLE? LP? and 7 cases of galvanic lesions related to amalgam fillings. Fifty-two histopathologic variables were examined, and a ranking was calculated for the variables that provided the best discrimination between the subgroups of clinically typical oral DLE and LP and between typical DLE and leukoplakia, respectively. Histopathologic variables not previously described in oral DLE but characteristic of this disease were identified: multinucleated epithelial cells containing three or more nuclei and the presence of epithelial islands in the connective tissue. Patients with systemic lupus erythematosus (SLE) in addition to oral DLE showed generally the same histopathologic features as those without SLE. The inflammatory infiltrates were slighter and more diffuse among the SLE patients as compared to the non-SLE patients. The simplest possible combination of histopathologic variables diagnostic for oral DLE consisted of the following five: (1) hyperkeratosis with keratotic plugs, (2) atrophy of the rete processes, (3) deep inflammatory infiltrate, (4) edema in the lamina propria, and (5) thick patchy or continuous PAS-positive juxtaepithelial deposits. This combination is suggested as new histopathologic criteria for oral DLE. These criteria were tested among clinically atypical cases of DLE and the other groups of mucosal lesions, with a sensitivity of 92% and a specificity of 96% against both LP and leukoplakia for the presence of two or more of the five criteria.

Biopsy↗

[Extracorporeal photopheresis in therapy-refractory disseminated discoid lupus erythematosus].

Lupus erythematosus (LE) is a disease with a wide spectrum of cutaneous and systemic manifestations. Discoid LE (DLE) is the most common form of cutaneous LE; the disseminated form of DLE is rare. We report an encouraging response to treatment with extracorporeal photopheresis (ECP) in a single patient with disseminated DLE who did not respond to conventional therapy. To the best of our knowledge this is the first successful use of ECP in the management of such a patient. Extracorporeal photopheresis is a therapeutic modality that has been under investigation for more than 12 years. Although originally developed for the treatment of cutaneous T-cell lymphoma, ECP has recently been used for the management of autoimmune diseases including systemic scleroderma, pemphigus vulgaris and SLE, as well as prevent organ rejection in patients with cardiac or kidney transplants and graft versus host disease after bone marrow transplantation.

Dose-Response Relationship, Drug↗

[Morphological studies of leukocyte long-term culture. 1. A new in vitro phenomenon in the diagnosis of discoid lupus erythematosus].

A new routine-method is presented for the morphokinetic documentation of autoimmunologically induced processes in vitro via leukocyte long-term culture (LLC). The prominent rosette-sheeped autologous lymphocytes in deposition at central granulocytes are well defined. These autologous rosette-formations in LLC rank very high in diagnosis of autoimmundiseases, since this phenomenon is positive already, while the results of current methods in routine-serumexaminations are still negative. Presenting a case of discoid Lupus Erythematodes this well developed method is represented in early recognition of dermatological autoimmundiseases, while cellular cinetics are discussed.

Adult↗

Virus-like structures in lupus erythematosus discoides.

Biopsy specimens from typical skin lesions of DLE patients were studied by means of electron microscopy. Virus-like structures were discovered in the cytoplasm of fibrocytes, endothelial cells and keratinocytes. After prolonged chloroquine treatment a decrease in the frequency and size of these structures was observed.

Chloroquine↗

Thalidomide for treatment of severe generalized discoid lupus lesions in two patients with systemic lupus erythematosus.

We describe 2 patients with systemic lupus erythematous whose widespread discoid lupus erythematosus was unresponsive to systemic steroids and antimalarial agents. They showed dramatic improvement to thalidomide at a dose of 300 mg/d, with maximum benefit achieved within 15 weeks of therapy. Dosages of 50 to 100 mg/d were effective in maintaining remission for 1 year. However, thalidomide-induced neuropathy was observed in both cases.

Adult↗

[Lupus erythematosus discoid-like dermatosis in a carrier of septic granulomatosis].

We present a clinically atypical case of discoid lupus erythematosus in the mother of a boy with chronic granulomatous disease. In this disorder, the phagocytes are unable to produce superoxide anion to degrade incorporated microorganisms. In addition to a discoid lupus erythematosus-like skin disease, recurrent stomatitis aphthosa, hidradenitis suppurativa and Raynaud phenomenon are markedly associated with heterozygote carriers of chronic granulomatous disease. Based on this conspicuous association, diverse models concerning the pathogenesis of lupus erythematosus are discussed.

Adult↗