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Discoid lupus erythematosus and cicatrizing conjunctivitis: clinicopathologic study of two cases.

BACKGROUND: Discoid lupus erythematosus (DLE) demonstrates both cutaneous and mucosal manifestations. Mucosal involvement is typically limited to the oral and anogenital mucosa. Conjunctival involvement in DLE is rare, especially in the absence of accompanying cutaneous disease. OBJECTIVE: We describe the clinical and immunopathologic features of two cases of cicatrizing conjunctivitis due to DLE. METHODS: In each patient, the clinical, immunopathologic, and serologic features were reviewed and the results recorded. RESULTS: Both patients presented with cicatrizing conjunctivitis suggestive of mucous membrane pemphigoid. Patient 1 had no history of typical DLE skin lesions. Patient 2 had a history of cutaneous and oral DLE prior to presentation. Histologic, electron microscopic, and direct immunofluorescence examination confirmed the diagnosis DLE in both patients. CONCLUSION: In patients presenting with cicatrizing conjunctivitis, DLE should be considered in the differential diagnosis. Performing appropriate investigations to distinguish conjunctival DLE from other autoimmune diseases with conjunctival involvement such as mucous membrane pemphigoid is critical in selecting an appropriate treatment regimen, in offering an accurate prognosis, and in monitoring for signs and symptoms of disease progression.

Adult↗

Pimecrolimus 1% cream for the treatment of discoid lupus erythematosus.

OBJECTIVES: To determine the safety and efficacy of pimecrolimus cream on lesions of discoid lupus erythematosus. METHODS: In an open-label phase II trial, patients with discoid lupus were treated with pimecrolimus 1% cream twice daily for 8 weeks. We assessed skin involvement with a clinical severity score, quality of life, patient improvement and toxicity. The changes were documented by skin biopsy at baseline and at the end of treatment. RESULTS: Ten patients with a mean age of 34 +/- 17 yr and disease duration of 3 yr (range 1-8) were studied; 90% were female and 40% had received prior topical or systemic therapy without response. In all patients, improvement of skin damage was observed after therapy. A significant decrease of 52% was observed in the mean +/- s.d. clinical severity score, from 6.1 +/- 1.4 before treatment to 2.9 +/- 1.5 after treatment (P = 0.005). Quality of life score (0 = no effect, 100 = maximum effect on quality of life) showed a mean improvement of 46%, from 42.8 +/- 23.1 before to 23 +/- 16.5 after treatment (P = 0.008). According to the patients' assessment of the response to treatment, 50% qualified as marked improvement, 40% moderate and 10% slight improvement. The treatment was well tolerated; adverse reactions consisted of minimal erythema and pruritus, which resolved without any further action. CONCLUSIONS: Our data suggest that pimecrolimus cream for discoid lupus erythematosus seems to be a safe and clinically effective option. However, this was an open and uncontrolled study, and double-blind, placebo-controlled studies are needed.

Adolescent↗

Chronic discoid lupus erythematosus in Thailand: direct immunofluorescence study.

BACKGROUND: Studies of chronic discoid lupus erythematosus (DLE) lesions by direct immunofluorescence (DIF) were heterogeneous with respect to classes of immunoglobulins and sites where these were deposited. Most of the studies were done in the USA and European countries. MATERIALS AND METHODS: To obtain representative data from Asiatic countries, we analyzed the direct immunofluorescent abnormalities of 100 DLE lesions in Thai patients who were diagnosed on the basis of clinical and histologic criteria. RESULTS: Granular deposits at the dermoepidermal junction (DEJ) were detected in 90% of cases. The common immunoreactants at the DEJ were IgG (63%) and IgM (47%). The deposits were usually combinations of various classes of immunoglobulins, mostly IgG (53%) and IgM (41%). Deposits of IgG and IgM alone at the DEJ were observed in 12% and 8%, respectively. Deposits at colloid bodies, dermal blood vessel walls, and epidermal nuclei were sometimes also seen. CONCLUSIONS: The DIF test of skin biopsy specimens is diagnostically significant in chronic DLE. Our study in Thai patients showed that the most common deposit was a combination of various classes of immunoglobulins, mostly IgG and often IgM as well as C3, and occurred at the DEJ of the involved area.

Adolescent↗

Cell renewal in discoid lupus erythematosus.

Cell proliferation has been evaluated in four patients with discoid lupus erythematosus after in vivo incorporation of tritiated thymidine. Numerous epidermal cells are in S phase or in mitosis. The acceleration of cell renewal is paralleled by a shortening of the transit time of completely differentiating keratinocytes. Remodellings of the vascular belt are evidenced by the labeling of many endothelial cells. There is no sign of lymphoblastic transformation in the dermis since the mononuclear infiltrate remains unlabeled.

Adult↗

[Squamous cell carcinoma in discoid lupus erythematosus foci].

Development of squamous cell carcinomas in lesions of discoid lupus erythematosus (DLE) has been observed extremely rarely. This paper reports on two patients with squamous cell carcinomas in DLE-lesions that had preexisted for decades. Because this complication is observed so rarely, individual predisposing factors are discussed.

Carcinoma, Squamous Cell↗

Discoid lupus erythematosus presenting as asymmetric posterior blepharitis.

PURPOSE: To describe the ophthalmic findings of patients with discoid lupus erythematosus. METHOD: We describe two women who originally were thought to have asymmetric posterior blepharitis; however, the involved eyelid also had an erythematous, scaly cutaneous lesion. RESULT: In both patients, histology and immunofluorescence studies performed on cutaneous biopsy specimens established the diagnosis of discoid lupus erythematosus. CONCLUSIONS: It is important to diagnose discoid lupus of the eyelids because misdiagnosis can delay treatment and thus lead to deformities of the eyelid margin. Misdiagnosis can also lead to a complicated full-thickness eyelid biopsy and delay the diagnosis of systemic lupus erythematosus.

Adult↗

A clinical study of 32 patients with oral discoid lupus erythematosus.

Thirty-two patients (26 females, six males) with discoid lupus erythematosus of the oral mucosa have been studied. Patients with signs of dissemination were classified according to accepted criteria. Four patients had systemic lupus erythematosus as well as oral discoid lesions. Twenty-one patients had discoid skin lesions. Eleven patients had oral discoid lesions without involvement of the skin. The time of onset of the oral discoid lesions rnaged from 6 to 75 years with a mean of 41 years. The mean druation of the oral lesions was 4.2 years. Symptoms from the oral discoid lesions were present in 75% of the patients. The clinical appearance varied and some lesions were difficult to differentiate from oral leukoplakia or from the lesions seen in oral lichen planus. The oral discoid lesions were most often seen on the buccal mucosa, the gingiva, labial mucosa and vermilion border. The oral lesions were infected by yeasts in more than half of thepatients.

Adolescent↗

Acetylator polymorphism in discoid lupus erythematosus.

Acetylator phenotype was determined, using sulphamethazine, in 37 patients with histologically confirmed discoid lupus erythematosus, who were free from visceral damage, and in 157 normal control subjects. Twenty patients (54%) and 90 control subjects (57.4%) were slow acetylators (p not significant). Acetylator polymorphism appears not to be related to the risk of developing pure cutaneous discoid lupus erythematosus.

Acetylation↗

[Spinocellular cancer at the site of chronic discoid lupus erythematosus].

A case of squamous cell carcinoma is presented that developed within a tense, fibrotic scar caused by chronic discoid lupus erythematosus. The carcinoma appeared in a woman of 60 years of age who has had chronic discoid lupus erythematosus on the scalp for 30 years. According to the literature, the frequency of this type of malignant transformation is estimated to be within the same range as the incidence of squamous cell carcinomas arising within tense, fibrotic scars, that is ranging between 0.1% and 2.6%. Therefore, no additional carcinoma-promoting factors of different origin specific for chronic discoid lupus erythematosus could be found.

Alopecia↗

Hyperkeratotic nail discoid lupus erythematosus evolving towards systemic lupus erythematosus: therapeutic difficulties.

Nail changes occur in about 25% of systemic lupus erythematosus (SLE) cases. Onycholysis has been reported as the most frequent abnormality in SLE. Nailbed hyperkeratosis may be observed in both SLE and discoid lupus erythematosus (DLE). Involvement of the nail apparatus in DLE is extremely uncommon and never restricted to it. We report on a patient in whom the clinical features on the proximal nailfold were similar to those observed on the skin of a patient with typical DLE. This has, to the best of our knowledge, not yet been reported. The patient also exhibited a very distinctive prominent subungual hyperkeratosis. Interestingly, the patient developed biological alterations suggesting a systematization of the disease. Only a combination of systemic corticoids, retinoids and antimalarials was able to achieve nail improvement and this partial resistance to therapy may be explained by the very unusual subungual hyperkeratosis.

Dermatologic Agents↗

Cutaneous calcinosis in localized discoid lupus erythematosus.

Cutaneous calcinosis is reported in the lesions of a woman with long-standing discoid lupus erythematosus. Calcium deposits were found in the middle and deep parts of the dermis surrounded by homogeneous collagen staining positively with Alcian blue solution. In contrast to the relative frequency of this event in patients with scleroderma or dermatomyositis, calcinosis cutis occurs very rarely in lupus erythematosus. To the best of our knowledge, this is the first report of calcinosis cutis in discoid lupus erythematosus.

Adult↗

[Familial chronic discoid lupus erythematosus].

We report on two patients (father and son) suffering from familial Lupus erythematosus chronicus discoides which could be proved clinically, histologically, and immunologically. Investigations on HLA revealed A-11, A-24, and B-35 for the father and A-24, B-13, and C-4 for the son.

Adult↗

T lymphocytes and mononuclear phagocytes in the skin infiltrate of systemic and discoid lupus erythematosus and Jessner's lymphocytic infiltrate.

T Lymphocytes and mononuclear phagocytes were measured quantitatively with the histochemical acid alpha-naphthyl acetate esterase (ANAE) method from paraffin sections of skin affected by systemic and discoid lupus erythematosus and by Jessner's lymphocytic infiltrate. The composition of the patchy cutaneous mononuclear cell infiltrates was similar in these three disorders.

Histocytochemistry↗

Childhood discoid lupus erythematosus: report of five new cases and review of the literature.

Discoid lupus erythematosus (DLE) is an uncommon disease in childhood. In this paper we present five new cases of childhood DLE. Two of them are identical twin brothers, who developed similar lesions during an interval of 5 years. This is in favour of the hypothesis that both genetic factors and somatic mutations, due to environmental factors, are implicated in the pathogenesis. A review of the English language literature is also presented. In order to have better epidemiological data on this disease, all cases of childhood DLE, including those published in non-English literature and those not yet published, should be placed together and analysed.

Biopsy, Needle↗

Discoid lupus erythematosus and carrier status of X-linked chronic granulomatous disease.

Discoid lupus erythematosus (DLE)-like lesions and recurrent aphthous-like stomatitis have often been described in carriers of X-linked chronic granulomatous disease (CGD). The capacity of the polymorphonuclear leucocytes to reduce nitroblue tetrazolium (NBT) after stimulation with phorbol myristate acetate (NBT test), a function of normal oxidative metabolism, was determined in 34 patients with DLE of whom 17 also suffered from recurrent stomatitis. The NBT test turned out to be normal in all 34 patients, indicating that none of them were carriers of X-linked CGD. In spite of the negative results of this study it is recommended that all female patients suffering from DLE in combination with recurrent aphthous-like stomatitis are screened by means of the NBT test, because this examination is simple and inexpensive, and because of the importance of identifying carriers of CGD with a view to genetic guidance.

Adolescent↗