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In two black patients, squamous cell carcinoma of the lip developed within lesions of discoid lupus erythematosus (DLE). The association of DLE and squamous cell carcinoma is unusual in blacks, as is the anatomical site involved in these cases. Enhanced metastatic potential may be present when squamous cell carcinoma arises in DLE of the lips.
HLA-A, B, C and Bf typing was performed in 55 cases of Discoid Lupus Erythematosus (DLE). When both the sex and age of the patient at the onset of the disease were taken into consideration (group I under 40 years, group II over 40), the following increases in antigen frequency were observed: group I: A2 in women, B5, A10 in men; group II: Aw19.2 in women, B8 in both sexes. Nevertheless, if the probability is multiplied by the number of antigens tested, these results are no longer significant.
Four patients with concurrent, chronic, progessive, localized scleroderma and discoid lupus erythematosus were studied; the condition originated as linear scleroderma in three of them. Three of the four patients were young females at the onset of the first skin disease. Dermatopathologic study confirmed the scleroderma and lupus erythematosus (LE). Direct immunofluorescence showed a positive band test in three cases. Unusual serological results included a positive LE clot test in three cases, a positive extractable nuclear antigen test in one case, and a negative antinuclear antibody test on repeated occasions in all four cases. Rare cutaneous disease similar to systemic, "mixed," or "overlap" connective tissue disease exists and offers an opportunity to study unusual immunologic and pathological events in both scleroderma and LE.
Periorbital edema associated with lupus erythematosus is not frequently reported. To our knowledge, periorbital edema from increased dermal mucin has not been reported with any form of lupus. We present a patient with discoid lupus exhibiting periorbital edema from massive mucinosis.
A comparative immunohistochemical study was performed on skin biopsies from 10 patients with lichen planus and 10 patients with discoid lupus erythematosus (DLE). A panel of antibodies against T lymphocytes (UCHL-1, OPD-4, CD8, CD43), B lymphocytes (L-26), granulocytes (Leu-M1), activation markers (Ki-1, LN-3), macrophages, fibroblasts and dendritic cells (FXIIIa, S-100, Mac-387, KP-1, vimentin), endothelial cells (CD34), and epithelial cells (epithelial membrane antigen) was employed using a peroxidase-anti-peroxidase technique. The recently released CD8 antiserum required microwave antigen retrieval of formalin-fixed, paraffin-embedded tissue to label lymphocytes. The results showed many similarities in the lymphocyte subsets and macrophages between lichen planus and discoid lupus erythematosus. The most important differences between the two conditions were statistically significant increases in the number of S-100+ cells in the epidermis and dermis, FXIIIa+ cells in the dermis and CD34+ vessels within the inflammatory infiltrate in lichen planus.
A 39-year-old man presented with a 4-month history of unilateral blepharitis that did not respond to conventional treatment. Punch biopsy confirmed the diagnosis of discoid lupus erythematosus. Unilateral blepharitis as the only presenting sign of discoid lupus erythematosus is uncommon but should be considered in the differential diagnosis in patients with asymmetric blepharitis.
A 70-year-old woman developed dark reddish blue pigmentation in scarred areas of discoid lupus erythematosus after taking clofazimine intermittently over a period of 10 years. Although light microscopy of routinely processed tissue failed to define the cause of the pigment, fluorescent microscopy showed vivid red deposits concentrated around larger vessels within the dermis. These deposits were shown to correspond to birefringent red clofazimine crystals on fresh frozen sections. Although the hyperpigmentation may clinically resemble melanin, biopsy specimens from our patient revealed a loss of melanin pigment in lesional skin, suggesting a primary role for clofazimine in producing the color changes observed.
BACKGROUND: Low-dose thalidomide therapy (median dose 100 mg/day, 50-200 mg/day) in chronic discoid lupus erythematosus was studied with regard to efficacy, tolerance, and toxicity in 22 patients. Intense contraceptive precautions were taken in women patients of childbearing age. METHODS: An open uncontrolled trial was conducted. Age, the total drug intake, disease duration, extent/severity, and adverse reactions were studied with regard to the final clinical outcome. The follow-up duration was 1.8 years (range 1 month to 3 years). RESULTS: With the exception of age (inverse correlation, P < 0.01), the parameters studied did not influence the final clinical amelioration: complete responders numbered 54.5%, partial responders 22.7%, and 13.6% were withdrawn from the trial with complaints of intolerance. The initial (first month) clinical response correlated significantly with the final one (P < 0.01). Drowsiness (40.9%) and somnolence (18.2%) were the most common side-effects, without affecting seriously the daily life of the participants. No case of real neurotoxicity was confirmed. Relapses occurred within 39.4 +/- 21.4 days after drug withdrawal, presenting a milder clinical picture. CONCLUSIONS: In the context of a predictable final outcome, low-dose thalidomide therapy is effective as an alternative choice in cases resistant to the usual treatment.
Scarring alopecia is a very frequent feature of chronic discoid lupus erythematosus (DLE). So far in the literature, only clinic-pathologic features or histopathologic-immunopathologic traits of DLE scarring alopecia (DLESA) have been reported. We describe the most significant features of clinical morphology, histopathology, serum and tissue immunopathology of 36 DLESA patients (41.9% of all our scarring alopecia patients). Clinically, 33.3% presented a single lesion and 52.7% presented multiple lesions of scarring alopecia, while 13.8% exhibited a picture resembling Pseudopelade of Brocq, with the classic 'footprints in the snow' appearance. The most frequent morphologic features were sclero-atrophy (80.5%) and erythema (63.8%). The main histopathologic aspects appeared to be fibrosis (100%), follicular hyperkeratosis (91.4%), epidermal atrophy (88.5%), lymphocytic infiltrate (88.5%), thickened basement membrane (77.1%) and basal vacuolar degeneration (74.2%). Antinuclear antibodies were present in 42.8% of patients and antigastric mucosa, antithyroid and anticardiolipin antibodies in 17-21% of patients. A positive lupus band test was demonstrated in 81.8% of cases and perivascular deposit in 30.3% of patients. Histopathology alone allowed a correct diagnosis only in 68.5% of cases; in the other cases, the diagnosis was assessed also taking into account immunopathologic findings. Our study defines the clinic, histopathologic and immunopathologic features of DLESA patients and points out that a multiparametric approach is mandatory to assess the diagnosis of DLESA.
In a long-term follow-up of 52 patients with oral lesions of discoid lupus erythematosus (DLE) 8 patients showed a gradual transition of the typical DLE lesions to lesions which closely resemble leukoplakia. Histopathologically and immunopathologically the leukoplakia-like lesions generally showed features similar to those in leukoplakias not preceded by DLE. Ultrastructurally, the leukoplakia-like lesions showed the presence of cytoplasmic tubular structures in vascular endothelium in two out of three patients examined. The leukoplakia-like stage is considered to be an inactive or scar-like stage, which may be the mucous membrane counterpart of the atrophic scars following DLE lesions of the skin. A primary examination of a DLE patient presenting oral lesions at the leukoplakia-like stage may result in an incorrect diagnosis. Therefore, when investigating patients with leukoplakias, they should be questioned on previous or present skin lesions as well as symptoms of systemic lupus erythematosus.
An evaluation of fluocinolone acetonide applied topically in the treatment of discoid lupus erythematosus is presented. Bilateral comparison of 0.025% fluocinolone acetonide cream to the cream base alone demonstrated the effectiveness of this program. In 59 patients an attempt was made to control this disease with topical fluocinolone acetonide as their only treatment. Forty three patients could be controlled in this fashion. Eleven patients responded partially, but for optimal response required a systemic supplement. Five patients did not show any improvement following topical fluocinolone acetonide applications. Noteworthy, is a group of 37 patients followed through two summer seasons. Twenty-four of these patients could be controlled with topical applications but reapplication the following summer was required. Three patients in this group cleared and remained clear. Six patients followed through two summers required a systemic supplement, while four in this group did not respond to therapy.
The authors present the data of biochemical studies of lipid peroxidation in 63 patients with discoid lupus erythematosus, validating the use of antioxidants combined with prednisolone and delagil in the treatment of such patients.
We have seen a 54-year-old patient with discoid lupus erythematosus and biopsy-proven vasculitis with granular deposits of complement and immunoglobulins in small subcutaneous vessels of clinically normal skin. Serologically, neither antinuclear antibodies nor antibodies against double-stranded DNA, Sm-Ag nor ribonucleoprotein could be demonstrated whereas the immunofluorescence test for antimitochondrial antibodies was positive during the whole observation period of 15 months. The further characterisation of the antimitochondrial antibodies by a newly described radio-immuno-assay demonstrated that the antibodies are directed against the primary biliary cirrhosis specific M2-antigen of mitochondria. So far, the patient, however, does not show any clinical or laboratory signs of primary biliary cirrhosis and a liver biopsy showed no evidence of that disease. It is discussed whether there is a coincidence of different autoimmunopathies or if the findings are symptoms of a single disease. Follow-up study might solve this open question.
A description is given of the clinical and histologic characteristics of chronic discoid lupus erythematosus. At the same time the authors describe the results of the cytologic study carried out in keeping with the Papanicolau technique. Although no specifically indicated in the disease under consideration, the cytological method, added to the clinical study, enables a prompt presumptive diagnosis to be arrived at. However, under no circumstances does it exclude a biopsy study.
Jessners lymphocytic infiltration of the skin (14 cases) and discoid lupus erythematosus (13 cases) were studied and the lymphoid infiltrates in the dermis were compared in the two conditions, using a standard immunoperoxidase technique. Mouse monoclonal antibodies were used to identify T helper lymphocytes, T suppressor lymphocytes and, using the antibody Leu 8, "immunoregulatory lymphocytes". It was shown that the proportions of Leu 8 positive cells was significantly different in the two conditions. The average percentage of Leu 8 positive lymphocytes in the dermal infiltrate found in the cases of Jessner's was 65% (range 40-80%) whereas the average percentage in the cases of discoid LE was 15% (range 2-30%). This observation is further evidence that Jessner's lymphocytic infiltration and chronic discoid lupus erythematosus should be regarded as separate entities.
Squamous cell carcinoma (SCC) is a rare late sequel of chronic discoid lupus erythematosus (CDLE). We report two cases of SCC developing in Chinese patients with CDLE. The first patient had prior biopsies from the same site that showed viral warts and the second patient had multiple histologically confirmed viral warts around the vicinity of the previously excised SCC. In this paper, we emphasize the need to be suspicious of warty lesions on skin afflicted by CDLE, and repeat biopsies should be performed if there is failure to respond to conventional therapy.
Anti-DNA antibodies have been detected previously in patients with chronic discoid lupus erythematosus (DLE) despite the absence of overt systemic manifestations. 27 patients with DLE were followed up 3 years after the detection of anti-DNA antibodies in 7. None had developed other features of systemic lupus erythematosus. We conclude that the occasional finding of anti-DNA antibodies in patients with DLE does not predict those likely to develop systemic disease.
In this study 8 patients with alopecia due to chronic discoid lupus erythematosus (CDLE), 10 patients with traumatic alopecia (TA), and 12 patients with male pattern baldness (MPB) were treated with the punch graft method (PGM). Stastistical evaluations of the results and factors influencing the results were careied out. The mean survival rate of hairs was 72% in CDLE, 97% in TA, and 104% in MPB. The mean number of hairs in a 4 mm graft before transplantation in 30 patients and 752 grafts was found to be 18.2. This is far more than the number reported in previous publications concerning PGM owing probably to the difficulties of counting several hairs simultaneously emerging from the same pilary canal when the hair is cut to a length of 1--2mm preoperatively. For this purpose the author has developed a special photographical magnifying system. The number of hairs surviving transplantation in MPB in a 4 mm graft was about 19 hairs per graft, which is superior to previous reports. This is possibly due to some modifications of the procedure made by the author. In a control series done with the same technique as that often seen in the literature, about 50% inferior results were obtained. These results were however similar to those reported in the literature. Consequently the modifications of the procedure have been successful. Only one article with a series of 12 patients with TA treated with PGM was found in the literature. The hair survival results of the author exeeded the reported results by about 100%. The size, hardness, and elevation of the scars of the donor holes were checked statistically after various treatments. It was found that the diameter of the scar is the same regardless of type of treatment but that a signifcantly greater risk of hard and elevated scars is taken if the bald grafts are put back in the donor holes. No complications were seen in this series. PGM is a safe method that gives good hair survival results on transplanted hairs in MPB and TA, but the results vary in CDLE. In spite of the good results with PGM the author emphasizes that, although the therory of the method is simple, both the preoperative judement and the performance with its practical difficulties and many cosmetic problems is complex and requires a lot of psychological insight, practice and experience before correct preoperative judgement and a maximally good cosmetic result can be achieved.