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PubMed1979

Abnormal laboratory test results and their relationship to prognosis in discoid lupus erythematosus. A long-term follow-up study of 92 patients.

Investigation of 92 patients with discoid lupus erythematosus, manifested initially by localized cutaneous lesions only, showed abnormal laboratory test results for 57 patients (62%) on admission and for 62 patients (67.4%) on review 16 to 20 years later. Patients with discoid lesions confined to the head and neck (DLE) showed fewer laboratory abnormalities than those patients with disseminated lesions involving trunk and limbs (disseminated discoid lupus erythematosus [DDLE]). Systemic lupus erythematosus (SLE) eventually developed in six (6.5%) of the patients, and all had shown persistent multiple abnormal laboratory findings from the beginning. Complete remission occurred in 46.7%. A persistent positive antinuclear factor of either speckled or homogeneous pattern with a titer greater than 1:50, leukopenia, thrombocytopenia, or a false-positive Wassermann reaction indicated those patients who may progress to DDLE or SLE.

Antibodies, Antinuclear↗
PubMed1998

Postmenopausal estrogen replacement therapy and the risk of developing systemic lupus erythematosus or discoid lupus.

OBJECTIVE: There is evidence that estrogens play a role in the etiology of systemic lupus erythematosus (SLE), but this has not yet been shown for discoid lupus. We examined the association of postmenopausal estrogen use with the development of SLE and discoid lupus. METHODS: We did a case-control evaluation, using the UK based General Practice Research Database. We analyzed 41 cases with SLE, 34 cases with discoid lupus, and 295 age, sex and practice matched controls, and estimated relative risk estimates (odds ratios) in relation to estrogen exposure duration as well as total cumulative dose and estrogen type (alone or combined with progestogens). RESULTS: While short term estrogen exposure was not associated with increased risk, the risk of developing SLE (adjusted OR 2.8; 95% CI 0.9-9.0) or discoid lupus (adjusted OR 2.8; 95% CI 1.0-8.3) was significantly increased among current users who were exposed for 2 or more years. The adjusted RR estimate comparing longer term estrogen users and nonusers for all cases (SLE and discoid lupus combined) was 2.8 (95% CI 1.3-5.8; p < 0.01). A difference was found between longterm users of estrogens alone (OR 5.3; 95% CI 1.5-18.6) and those who used estrogens combined with progestogens (OR 2.0; 95% CI 0.8-5.0), compared to nonusers. CONCLUSION: Our findings suggest that longer term use of postmenopausal estrogens plays a role in the etiology of both SLE and discoid lupus. There is a suggestion that progestogens may reduce the effect of estrogens on these autoimmune disorders.

Aged↗
PubMed1985

T-cell subsets in lesions of systemic and discoid lupus erythematosus.

In 6 patients with untreated systemic lupus erythematosus (SLE) in the progressive stage, and in 6 with discoid lupus erythematosus (DLE), an analysis of inflammatory infiltrates was performed in situ using the avidin-biotin-peroxidase complex (ABC) method with monoclonal antibodies. In all patients, over 75% of the infiltrates reacted with the pan T-cell antibody OKT3, but only sporadically with that of B-cell OKB7. In addition, a large number of the infiltrates were OKIal-positive, indicating that they were in an activated state. Many OKT8-positive cells were seen infiltrating the epidermis especially in the vicinity of basal keratinocytes. Staining for T-cell subsets revealed that the proportion of OKT8-positive cells (suppressor/cytotoxic) was from 2 to 3 fold higher than that of OKT4-positive cells (helper/inducer) in lesions of SLE. On the contrary, in DLE, a predominance of OKT4-positive cells (the OKT4/OKT8 ratio was from 1:1 to 3:1) was observed. Thus, our results provide further evidence that these 2 main types of LE show quite contrary findings on immunohistochemical analysis of T-cell subsets, and that besides the humoral immune mechanism, the cell-mediated immune mechanism may be involved in the pathogenesis of these disorders.

Antibodies, Monoclonal↗
PubMed1988

Squamous-cell carcinoma of the scalp arising in lesions of discoid lupus erythematosus.

Squamous-cell carcinoma may arise in scars of chronic discoid lupus erythematosus. Although there have been 19 cases reported previously, detailed histopathologic features of this entity have not been recorded. We report a patient with extensive chronic discoid lupus erythematosus involving the scalp with subsequent development of multiple squamous-cell carcinomas. The tumors were locally aggressive with recurrences and invasion into the underlying skull and dura. The patient died of respiratory failure 4 1/2 years after initial surgical treatment. There was no clinical evidence of metastasis. Squamous carcinoma arising in discoid lupus erythematosus can be regarded as a low-grade carcinoma. Although about 20% of patients developed local recurrences and metastasis developed in about 30%, fatality occurred in only two patients (10.5%). Pertinent literature is reviewed, and the histopathologic findings, differential diagnosis, and biologic behavior of this tumor are discussed.

Aged↗
PubMed1984

Discoid lupus erythematosus involving the eyelids.

Of two patients with discoid lupus erythematosus with eyelid involvement, one, a 42-year-old man, had a one-year history of persistent periorbital edema and a violaceous discoloration as the sole manifestation. Although treatment with corticosteroids and antihistamines failed to produce improvement, the patient had an excellent clinical response to systemic hydroxychloroquine therapy. Histologic examination with immunofluorescent staining demonstrated deposition of immunoglobulins at the dermoepidermal junction. The second patient, a 37-year-old woman, had a hyperpigmented lesion on her forearm as the initial symptom but later developed similar lesions elsewhere, including her lower eyelid. Treatment with hydroxychloroquine again produced an excellent response.

Adult↗
PubMed2005

Discoid lupus erythematosus masquerading as chronic blepharoconjunctivitis.

PURPOSE: To recognize discoid lupus erythematosus (DLE) as a treatable cause of chronic blepharoconjunctivitis. DESIGN: Retrospective observational case series. PARTICIPANTS: Records of 5 patients with biopsy-proven DLE were reviewed. METHODS: Clinical and pathology records were examined. MAIN OUTCOME MEASURES: Patients' clinical and histopathological characteristics and response to treatment were assessed. RESULTS: Clinical features included meibomian gland dysfunction, blepharitis, chalazia, trichiasis, madarosis, conjunctivitis, chronic eyelid edema, and eyelid plaques. Histopathology showed hyperkeratotic epithelium, degeneration of the basal cell layer, and a perivascular lymphocytic infiltrate. There was delayed diagnosis in all cases, ranging from 4 months to 25 years. All of the patients responded to systemic hydroxychloroquine therapy. CONCLUSIONS: Heightened awareness of eyelid DLE may lead to earlier detection and specific therapy for this chronic disorder.

Adult↗
PubMed1997

NAT2 genotyping and efficacy of sulfasalazine in patients with chronic discoid lupus erythematosus.

Sulfasalazine is an effective agent for chronic discoid lupus erythematosus (CDLE) but the response to treatment is considerably variable between patients and is also unpredictable. The reason for this might relate to differences in metabolism of the drug which is extensively acetylated by the polymorphic enzyme N-acetyltransferase 2 (NAT2). To test this possibility, the N-acetylation phenotype of eleven patients with CDLE and treated by standard doses of sulfasalazine was retrospectively determined by genotyping. A clear-cut difference in the outcome of treatment was observed according to whether the patients were slow acetylators (SA) or rapid acetylators (RA). Eight out of 11 patients responded to treatment with a complete or marked remission of the disease. Seven of them were RA. The three other patients who did not respond at all to the drug were SA. In addition, SA seem to be more prone to toxic events. These findings strongly suggest that the genetic polymorphism of NAT2 is responsible for differences in the response to sulfasalazine in patients with CDLE. Therefore, candidates for sulfasalazine therapy should be genotyped to identify those patients who might benefit from the drug.

Acetylation↗
PubMed1983

Thalidomide in the treatment of sixty cases of chronic discoid lupus erythematosus.

The therapeutic effect of thalidomide in chronic discoid lupus erythematosus (CDLE) was studied in sixty patients who were followed up for 2 years. In fifty-four patients (90%) a complete or marked regression of the disease was observed, but when the thalidomide was stopped, thirty out of forty-one (71%) patients relapsed. Patients undergoing a second course of thalidomide treatment again responded well. Nine of the patients in whom the disease recurred after successful treatment with thalidomide and who had been unresponsive to intermittent treatment with antimalarials, showed a good response to a second or third course with thalidomide. Mild side-effects were common and 25% of patients complained of slight to moderate polyneuritic symptoms. Since electroneurological examinations had not been performed before the thalidomide therapy, the frequency of neurological side-effects cannot be accurately calculated but we recommend neurological examinations before and periodically during thalidomide treatment. Thalidomide is a very effective drug in CDLE, but in most cases it exerts its effect only whilst treatment is continued. Its use should be restricted to patients resistant to topical steroids and systemic antimalarials.

Chronic Disease↗
PubMedDate not supplied

Histopathology and immunofluorescence of discoid lupus erythematosus in children.

The histopathology and immunofluorescence of discoid lupus erythematosus (DLE) are well documented in adults, but in children they have not been studied systematically. We reviewed nine skin biopsy specimens from six children with DLE. Only three specimens showed the well-developed epidermal changes seen in lesions of adults with DLE, and in the other six, these changes were either mild or absent. All nine specimens showed some degree of vacuolar alteration of the basal layer, ranging from focal and mild to severe. They all had a moderate to heavy lymphocytic dermal infiltrate in a perivascular and periadnexal distribution. Direct immunofluorescence performed on five specimens of lesional skin was weakly positive for IgM or IgG in three cases, negative in one, and strongly positive for IgM, IgG, IgA, and C3 in one. This study indicates that the range of histologic and immunofluorescence features in children with DLE is similar to that in adults. The histologic findings are distinctive enough to be useful in establishing the diagnosis, although clinicopathologic correlation is required.

Adult↗
PubMed1993

Systemic sclerosis (scleroderma) associated with discoid lupus erythematosus.

Six patients with systemic sclerosis (SS) and discoid lupus erythematosus (DLE) were studied to determine whether such cases have some common clinical and laboratory findings. DLE preceded SS in all cases. Three patients had diffuse scleroderma with lung and esophagus involvements and the others limited scleroderma. Three patients had anti-topoisomerase-I and antiribonucleoprotein antibodies, 2 had either of them and the remaining anticentromere antibodies. Four had DLE located on the scalp, leading to alopecia. The other 2 had DLE on the face and extremities. No case fulfilled criteria for systemic lupus erythematosus (SLE). The present cases with SS and DLE, but without SLE, indicate that this type of systemic-cutaneous collagen disease overlap does exist and may be not so rare.

Adult↗
PubMed1981

Dermal mucinosis in discoid lupus erythematosus. Report of two cases.

Two patients with discoid lupus erythematosus (DLE) had lesions exhibiting prominent atrophy and telangiectasia. At the time of biopsy, the skin specimens were grossly mucinous. Light and immunofluorescence microscopy findings confirmed the diagnosis of DLE in both cases. Abundant mucin deposition throughout the dermis was also seen microscopically in both cases. Although secondary mucinosis is known to occur in LE, cases with massive mucinous change, ie, those reported herein, have apparently not been recorded. Mucinosis is not considered important in the histologic diagnosis of LE but may create some confusion with other mucinous disorders.

Adult↗
PubMed1978

Local excision in the treatment of oral discoid lupus erythematosus.

Oral lesions in eight patients with discoid lupus erythematosus (DLE) were treated with local excision. Ten well-demarcated oral lesions were excised and a primary closure was obtained following the excision. Six patients out of seven became symptom-free following the treatment. The median observation time was 1.0 year. The follow-up examination showed erythema in one case and recurrence in one case at the operation site. In the remaining eight operation sites, a soft scar with no sign of recurrence was seen. These results indicate that the method should be investigated further in order to obtain long-term results. Local excision of oral discoid lesions may be a valuable therapeutic supplement in some cases of DLE.

Adult↗
PubMed1999

Hypertrophic discoid lupus erythematosus of the conjunctiva.

PURPOSE: To report the ophthalmic manifestations of hypertrophic discoid lupus erythematosus of the conjunctiva. METHOD: Case report and review of biopsy results. RESULTS: A 58-year-old woman with a history of chronic blepharoconjunctivitis presented with an unusual raised conjunctival lesion. Previous biopsy slides were reviewed and interpreted as diagnostic of discoid lupus erythematosus, hypertrophic or verrucous type. Both blepharoconjunctivitis and the raised conjunctival lesion resolved with hydroxychloroquine therapy. CONCLUSIONS: A raised conjunctival mass in the context of refractory blepharoconjunctivitis should elicit suspicion for discoid lupus erythematosus. The hypertrophic variant of this disease can affect the conjunctiva.

Biopsy↗