Lupus erythematosus associated with erythema multiforme-like lesions in identical twins.
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Twenty-two skin biopsies have been split and each half examined either by the immunoperoxidase technique on paraffin-fixed tissue or by both immunoperoxidase (IP) and immunofluorescence (IF) technique on snap-frozen tissue. All twenty-two specimens gave positive results by frozen IF techniques, nineteen out of twenty-two (86%) were positive by frozen IP techniques and sixteen out of twenty-two (72%) were positive by IP on paraffin-fixed material. If retrospective immunopathology is required on a specimen and only paraffin-fixed material is available a positive result may be expected in the majority of cases.
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The prevalence and pattern of mucosal involvement in 121 patients with lupus erythematosus (LE) was investigated. Fifty-three patients had systemic LE (SLE) and 68 patients had chronic cutaneous LE (CCLE). Twenty-one per cent (11/53) of patients with SLE and 24% (16/68) of patients with CCLE had signs of mucosal involvement, but the pattern of involvement differed in the two groups. Nasal mucosal lesions were a feature in 2% (I/53) of patients with SLE and 9% (6/68) of patients with CCLE. Hyperkeratotic lichen planus-like plaques on buccal mucosa and the palate occurred in 9% (6/68) of patients with CCLE and 4% (2/53) of patients with SLE. Episcleritis occurred in 9% (5/53) of patients with SLE and not seen in CCLE. Erythematous plaques on the lower eyelids were present in 6% (4/68) of patients with CCLE and these were associated with conjunctival scarring in two patients. Vulval lesions were present in 5% (2/42) of female patients with CCLE. Oral plaques may occur when the disease is relatively quiescent elsewhere. The prevalence of mucosal involvement in lupus is underestimated as the lesions may be asymptomatic.
The prevalence and clinical significance of subepithelial immunoglobulin and complement deposition (the lupus band) were examined in the uninvolved sun-protected skin of the forearm, the uninvolved sun-protected lip mucosa and sun-protected bulbar conjunctival mucosa in systemic lupus erythematosus (SLE) and chronic cutaneous lupus erythematosus (CCLE). In SLE, linear deposition of an immunoreactant at the BMZ was detected in 32% (6/19) of skin biopsies; 21% (4/19) of lip mucosal biopsies and 42% (5/12) of conjunctival biopsies. There was no significant difference in the sensitivity of the test at different sites in SLE and no correlation between a positive test in skin, lip or conjunctiva and clinical mucosal involvement. In CCLE, linear deposition of an immunoreactant at the BMZ was found in 3% (1/32) of skin biopsies; 3% (1/29) of lip mucosal biopsies and 50% (10/20) of conjunctiva and clinical mucosal involvement. In the conjunctiva, IgG was present in all but one of the biopsies and was the only immunoreactant in 90% (9/10) of positive CCLE biopsies and 60% (3/5) of positive SLE biopsies. In lupus erythematosus immunoreactants may be deposited in the basement membrane zone beneath non-keratinizing mucosal surfaces of the lip and the eye as well as the skin. In CCLE, the test may be positive in conjunctiva when skin and lip are negative.
BACKGROUND: Thalidomide is an anti-inflammatory and immunomodulatory agent with proven efficacy in several refractory inflammatory skin conditions including photoexacerbated skin diseases. The effects of thalidomide on ultraviolet (UV)-induced cutaneous damage in humans have not been extensively studied. We describe the results of minimal erythema dose (MED) testing in nonlesional skin of three patients with chronic cutaneous lupus erythematosus (CCLE) before and after treatment with thalidomide. OBJECTIVES: To determine whether thalidomide treatment provides clinical and histological evidence of photoprotection from acute UV injury. METHODS: MED testing was performed in nonlesional skin of three patients with CCLE before and after treatment with thalidomide. Skin biopsy specimens were taken from MED sites for in situ immunochemistry. RESULTS: In each patient, the MED to UVB irradiation was significantly higher while the patient was receiving thalidomide treatment than in the absence of thalidomide, suggesting a systemic photoprotective effect. Thalidomide treatment had no significant effect on markers of apoptosis including sunburn cell formation and terminal deoxynucleotidyl transferase-mediated biotinylated deoxyuridine triphosphate nick end labelling, which identifies single-strand breaks in DNA. CONCLUSIONS: Thalidomide inhibits acute UVB erythema at 24 h after exposure, as a 100-mg daily dose of this drug for 4 weeks conveyed a sun protection factor of 1.56 to > 4.0. We conclude that inhibition of UVB-induced inflammation may, in part, explain the therapeutic benefits of this agent on photosensitive diseases.
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Primary anetoderma (PA) has occasionally been described in association with lupus erythematosus (LE). The present study was performed to elucidate a possible causal link between PA and LE by the use of direct and indirect immunofluorescence (IF) methods. Two patients with PA were studied. Biopsy specimens were obtained from early inflammatory and atrophic anetoderma lesions and from the exposed and unexposed uninvolved skin of each patient. The pattern of immune deposits observed in one patient was indistinguishable from that which is often seen in systemic LE, and in the other patient from that which may be observed in chronic cutaneous LE. The direct IF study also showed fibrillar immune deposits in the dermis that resembled elastic fibres morphologically. The indirect IF study, however, failed to demonstrate anti-elastic fibre antibodies in the patients' sera. The results of this study and a review of the literature suggest that some cases of PA have direct IF findings similar to those of either chronic cutaneous or systemic LE. However, these findings, along with the serological findings, are insufficient to establish a diagnosis of LE in most of these PA cases.
The involvement of oral, conjunctival and genital mucosa in lupus erythematosus (LE) has recently been described. Reports of nasal involvement have been sporadic. A prospective study was performed to assess the prevalence of nasal symptoms and signs in LE and to identify the incidence of deposition of immunoreactants in the nasal mucosa of patients with LE. Thirty-six patients with LE were studied; 21 had non-specific nasal symptoms and 12 had evidence of chronic inflammatory changes in the mucosa of the nasal cavity or in the vestibule (septal perforation, inflamed mucosa, vestibulitis). Immunoreactants were found in only 4 patients and did not correlate well with clinical evidence of disease. Nasal mucosal involvement in LE is underestimated and often overlooked in the assessment of such patients.
An indirect immunoperoxidase technique employing a specific anti-human T lymphocyte antiserum has been used to identify T lymphocytes in cutaneous tissue sections of various benign and malignant dermatoses. Skin biopsies from patients with histologically confirmed lichen planus, discoid lupus erythematosus, psoriasis, actinic keratosis, squamous cell carcinoma, Bowen's disease, keratoacanthoma, malignant melanoma, inflamed serborrhoeic warts, sarcoidosis, follicular lymphoma and multicentric reticulohistiocytosis were examined. T lymphocytes were found to be the predominant cell type in the cutaneous infiltrates of the majority of these dermatoses. However, only few T lymphocytes were identified in sarcoidosis where they tended to be restricted to the periphery of the epithelioid cell granulomas. Similarly, in the cutaneous infiltrates of follicular lymphoma and in a patient with multicentric reticulohistiocytosis, few T lymphocytes were identified.
Canine discoid lupus erythematosus (DLE) and mucocutaneous pyoderma (MCP) have overlapping clinical and histopathological changes, often making diagnosis difficult. Histopathological features of 27 nasal planum biopsies were scored to determine whether DLE and MCP were histopathologically distinguishable. Long-term follow-up, enabling assessment of clinical diagnoses, was available on 15 cases; 11/15 cases were immunomodulatory responsive (ImR) and 4/15 were antibiotic responsive (AbR). Clinical diagnosis, determined by response to treatment for 15/27 cases, was not predictable based on scoring of histopathological features. Distinct histopathological patterns were observed: 2/11 ImR cases had a lymphocyte-rich interface dermatitis. All other cases had the same histopathological changes: a band-like diffuse superficial plasmacytic to lymphoplasmacytic dermatitis +/- focal basal cell damage, but different clinical diagnoses (4/4 AbR, 9/11 ImR). German shepherd dogs/crosses were over-represented (44.4% of the cases) and tended to have more multifocal lesions (41.7% vs. 26.7% of all other breeds). Longer duration of disease was associated with a preponderance of plasmacytic infiltrate (P = 0.026).
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In dermatology, patients with nodosum leprosum and discoid lupus erythematosus are benefited by clofazimine therapy. Its use in other autoimmune diseases has to be considered. Patients with lupus pernio may respond to this regime, but further prolonged trials will have to be undertaken.
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