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Case report on a 50-year-old man suffering from discoid lupus erythematosus since about 20 years. The development of a tumor of the left lower leg within a l. e. area, which turned out to be a squamous-cell carcinoma, had been noticed since two years. One year after tumor excision our patient died of multiple metastases. This case demonstrates the rare formation of a squamous-cell-carcinoma "in erythematode ".
A patient with the discoid lupus erythematosus and lichen planus overlap syndrome has profound depression of serum C4 concentration associated with substantial mixed cryoblobulinemia. A family study failed to disclose evidence of a familial hypocomplementemia, cryoglobulinemia, or a dermatologic condition. Immunologlobulin, but no complement, was detected at the site of the skin abnormality. This case illustrates an immune-complex disorder with a mixed cryoglobulinemia that is related to immunoglobulin deposition in the skin.
Acetylator phenotype was measure in 58 patients presenting to a skin clinic with discoid lupus erythematosus (DLE) and in 51 normal healthy subjects. Twenty seven of the patients with DLE were found to have evidence of systemic lupus erythematosus (D+SLE). Frequency of slow acetylator phenotype was 58% in all DLE patients, 52% in those with D+SLE and was no different from the 57% in controls. The distribution of acetylator phenotypes within the groups with DLE and those with D+SLE was similar to controls. Severity of DLE was assessed as number of skin lesions and median lesion count was 11.5 in slow acetylators and 10 in fast acetylators but in D+SLE median lesion count was 22 in slow acetylators and 12 in fast acetylators, and there was a significant inverse relationship between lesion count and rate of acetylation; scores for systemic involvement showed no relationship. We conclude that there is no difference in the frequency or distribution of slow acetylator phenotype between normal subjects and patients with DLE with or without SLE but that actual rate of acetylation may determine severity of expression of the disease in slow acetylators.
Systemic and discoid lupus erythematosus (LE) have both been reported in association with psoriasis. Four additional patients who had systemic LE are reported. All of them had a high titer of antinuclear antibodies (ANAs) and were SS-A(Ro)-negative. Only one patient exhibited photosensitivity. SS-A(Ro) antibodies do not appear to be specific markers for the coexistence of LE and psoriasis, and screening for them in all psoriatics prior to ultraviolet B phototherapy is not recommended. Pitfalls in the management of patients with systemic LE and psoriasis are discussed.
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The prevalence of different antinuclear antibodies was examined in systemic lupus erythematosus, rheumatoid arthritis, Sjögren's syndrome, progressive systemic sclerosis, dermatomyositis, discoid lupus erythematosus, and mixed connective tissue disease. Antibody to Sm antigen (a nuclear acidic protein) was found almost exclusively in serums of patients with systemic lupus erythematosus, suggesting that this antibody might be a "marker" antibody for this disease. However, antibodies to native DNA and to nuclear ribonucleoprotein were found in a variety of systemic rheumatic diseases. In systemic lupus erythematosus, antibody to native DNA was present in high titer but in other rheumatic diseases in low titer. Antibody to nuclear ribonucleoprotein occurred in certain other systemic rheumatic diseases besides the mixed connective tissue disease syndrome. These studies showed that there were distinct profiles of antinuclear antibodies in certain systemic rheumatic diseases, characterized by the presence or absence of certain antibodies and by differences in their mean titers.
Biopsy specimens of skin lesions from three patients with discoid lupus erythematosus and six patients with systemic lupus erythematosus contained the membrane attack complex, which comprises C5b through C9, as well as immune complexes at the dermal-epidermal junction. The basilar epithelium in these areas was vacuolated and edematous, and the dermis contained an inflammatory infiltrate. In contrast, 19 of 29 specimens of normal-appearing skin from patients with discoid or systemic lupus erythematosus showed only immune complexes at the dermal-epidermal junction, without the membrane attack complex. The other 10 specimens, all from patients without cutaneous involvement, showed neither immune complexes nor membrane attack complexes. These data suggest that immune complexes within skin lesions selectively generate the assembly of the membrane attack complex, which mediates membrane injury. A synergistic interaction of immune complexes and cofactors may be required to activate complement in areas of skin that are predisposed to tissue injury.
Lupus erythematosus-like syndromes have been reported as an adverse effect of anti-tumour necrosis factor-alpha therapy. We report the case of a patient with rheumatoid arthritis who developed a discoid lupus erythematosus-like eruption after treatment with infliximab. The rash consisted of diffuse scaly erythematous plaques on the face, trunk and extremities, and occurred in the context of elevated anti-nuclear and anti-double-stranded DNA antibody titres. Direct immunofluorescence of lesional skin showed linear deposition of IgG, IgM and C3. The lesions resolved completely after the discontinuation of infliximab and with the use of anti-malarial therapy. We discuss the clinical, histological and immunohistochemical features of this case and review the literature with respect to the incidence of lupus erythematosus-like syndromes in patients receiving tumour necrosis factor-alpha antagonists.
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The authors describe a case of hypertrophic erythematous lupus in a 64- year-old male. Warty elements appeared in patches typical of discoid lupus erythematosus, confined almost exclusively to the limbs. The reported cases of this uncommon variety are reviewed, and the clinical, histopathological and differential diagnostic features are discussed.
A case of chronic granulomatous disease (CGD) in a 32-year-old female with two episodes of opportunistic infections is described. At the age of 29 the patient was suspected to be a carrier of X-linked CGD on the basis of discoid lupus erythematosus-like skin lesions. No respiratory burst activity, as measured by phorbol myristate acetate stimulated superoxide production, was observed in isolated neutrophils of the patient. Membrane-rich fractions elicited no superoxide production in the presence of NADPH. The neutrophil content of cytochrome b-245 was within normal range. Family investigations revealed neither cellular abnormalities nor any history of skin diseases or opportunistic infections in first degree relatives. The parents of the patient were first cousins. On the basis of family history and the in-vitro assessment of neutrophil function, the patient is believed to have autosomal recessive CGD. The presented case illustrates that lupus erythematosus-like skin lesions are not restricted to female carriers of X-linked CGD, but may also be found in the autosomal recessive type of the disease.
Sunlight is a well-established factor in the induction and exacerbation of lupus erythematosus. Although experimental reproduction of lupus erythematosus lesions with wavelengths shorter than 320 nm was demonstrated previously, the effect of wavelengths longer than 320 nm was not investigated adequately. In this study we show that the action spectrum of lupus erythematosus reaches into the UVA region. A total of 128 patients with lupus erythematosus underwent phototesting with the use of polychromatic UVB and long-wave UVA. Subsets of the disease consisted of discoid lupus erythematosus (n = 86), subacute cutaneous lupus erythematosus (n = 22), and systemic lupus erythematosus (n = 20). Skin lesions clinically and histologically compatible with lupus erythematosus were induced in 64% of patients with subacute cutaneous lupus erythematosus, 42% of patients with discoid lupus erythematosus, and 25% of patients with systemic lupus erythematosus. The action spectrum of the induced lesions was within the UVB range in 33% of patients, in the UVA range in 14%, and in the UVB and UVA range in 53%. In positive test reactions patchy dark erythema and urticarial plaques developed within a few days. In some patients typical discoid lesions persisted for months.
Thirty-three patients with chronic discoid lupus erythematosus (DLE) who had received dapsone for 1-27 months were reviewed. The dapsone treatment gave excellent results in 8 patients (24%), some effect in 8 patients (24%), while no response was seen in 17 patients (52%). Of the patients who had earlier been treated with hydroxychloroquine (HCQ), 76% responded excellently to the treatment. Of 6 patients who received a combined dapsone and HCQ therapy, 2 responded well. Six patients (18%) terminated the dapsone treatment because of side effects, none of which were serious. It is concluded that dapsone might be an alternative or a supplement to HCQ in the treatment of DLE, when the latter causes adverse reactions or fails to be effective. However, we find that HCQ remains the drug of choice when systemic treatment is needed.
Our paper summarizes the clinical and photobiological studies on lupus erythematosus (LE) performed at the Department of Dermatology, University of Düsseldorf, during the last 5 years. A total of 190 LE cases were examined and classified according to the various cutaneous forms of LE. Except for lupus erythematosus tumidus (LET), we found a female predominance in all other groups. Discoid lupus erythematosus (DLE) is the most common variant of cutaneous LE. Subacute cutaneous lupus erythematosus (SCLE), a less common type, is characterized by the presence of Ro-antibodies, although this marker does not correlate with the degree of photosensitivity. The rare LET is an extremely photosensitive form of LE, which clearly prefers the male sex.
The child of a woman with discoid lupus erythematosus (DLE) had lesions of DLE develop at the age of 2 months. The original lesions persisted and new lesions continued to develop to date; the child is now 28 months old. In a second child, now 11 months old, a probable lupus erythematosus rash developed at 1 week of age.
On the basis of LE cases treated at the Department of Dermatology, Düsseldorf University, during the last few years, we present the various forms of cutaneous lupus erythematosus (CLE). 72% of the patients showed discoid lupus erythematosus (DLE), whereas disseminated discoid LE (DDLE) and lupus panniculitis were found in 3% each. Lupus erythematosus tumidus (LET), as well, must be regarded as exceptional. Subacute cutaneous LE (SCLE) and systemic LE (SLE) showed nearly similar frequency (10 and 12%, resp.). Bullous LE is also very rare and must be considered a variant of SLE. The various forms of cutaneous LE can be differentiated according to clinical presentation and histopathology. Direct immunofluorescence, in contrast, has but limited diagnostic value, except with lesions on the scalp. Exact classification of cutaneous LE is the more essential, as it implies considerable therapeutic and prognostic consequences for the patient.
Lichenoid dermatosis is a pattern description of a variety of cutaneous lesions which primarily affect the dermoepidermal junction. Involvement of skin appendages has been restricted to hair follicles in lichen planopilaris and discoid lupus erythematosus. Sweat gland involvement has not been described in the four common members of this group, namely, lichen planus, discoid lupus erythematosus, fixed drug eruptions and erythema multiforme, although structural abnormalities have been reported in graft-versus-host disease. In a detailed morphological study of 59 cases, including lichen planus (12), discoid lupus erythematosus (18), fixed drug eruption (14) and erythema multiforme (15), 78% (47/59) showed sweat, gland abnormalities. The abnormalities included vacuolation of cell cytoplasm, with and without lymphocytic infiltration, apoptosis of basal cells and basal cell hyperplasia of the excretory ducts which predominantly affected the portion of the duct adjoining the acrosyringium. The portion of the duct close to the secretory gland was only involved in continuity and the secretory glands were unaffected. These abnormalities of the sweat gland mostly constitute primary involvement by the disease process in contrast to structural abnormalities secondary to fibrosis.