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PubMed1981

Discoid lupus erythematosus-like lesions and stomatitis in female carriers of X-linked chronic granulomatous disease.

The skin and oral mucosa were studied in an unselected series of carriers of x-linked chronic granulomatous disease, a hereditary condition in which phagocytic cells display a pronounced functional defect. Three carriers had discoid lupus erythematosus (DLE)-like skin lesions which histopathologically were consistent with DLE of the hypertrophic and profundus type. Four patients had experienced photosensitivity in childhood. Seven patients had recurrent aphthous-like stomatitis which should be distinguished from the recurrent aphthous stomatitis seen in otherwise healthy individuals. The remarkably high incidence of DLE-like symptoms in heterozygous carriers might be related to the presence of mixed populations of defective and normal phagocytes. The variable expression of skin symptoms may be related to uneven distribution of abnormal and normal phagocytes. Female patients with these clinical symptoms, especially the combination of DLE-like skin lesions and aphthous-like stomatitis, should be suspected of being carriers of chronic granulomatous disease and studies of phagocyte function in vitro should be performed, since the diagnosis of the carrier state is of utmost importance for genetic counselling before pregnancy.

Adult↗
PubMedDate not supplied

Chronic cutaneous lupus erythematosus--clinical spectrum.

The typical clinical manifestation of chronic cutaneous lupus erythematosus (CCLE) is the chronic discoid lupus erythematosus. This form is characterized by inflammatory, scarring lesions mainly involving the head or neck, but also elsewhere, mostly on the photoexposed areas. The typical lesions in chronic discoid lupus erythematosus appear as round or oval erythematous plaques with scales and follicular plugging. These plaques are well demarcated and spread peripherally. On the face, they can form a butterfly-like rash involving the cheeks and nose. The long-standing lesions are atrophic and scarring, sometimes with hypo-, and hyperpigmentation and teleangiectasias. In 50% of the patients, there is a widespread skin involvement. This form is considered to be the typical clinical picture of CCLE.

Chronic Disease↗
PubMed1993

Pathomechanisms of photosensitive lupus erythematosus.

This review discusses the mechanisms involved in different photosensitive lupus syndromes: acute cutaneous lupus erythematosus, chronic cutaneous (discoid) lupus erythematosus, subacute cutaneous lupus erythematosus, and neonatal lupus erythematosus. It is proposed that there are three principal determinants of photosensitivity in lupus: 1) susceptibility to UVR-induced release of epidermal and dermal cytokines; 2) susceptibility to UVR-induced release or translocation of sequestered antigens in the epidermis or dermis; and 3) different specific immunologic effector mechanisms, activated by cytokines and directed against discrete epidermal targets. Several characteristics of photosensitive lupus are discussed in detail: autoantibody specificities, autoantigen translocation, induction of epidermal intercellular adhesion molecule-a (ICAM-1), vascular activation, cytokine release and T-cell activation, and clinical phototesting. The role of antibodies to the extractable nuclear antigens Ro and La and the relationship to subacute cutaneous lupus erythematosus (SCLE) and neonatal lupus erythematosus is discussed in detail, and a model of SCLE is proposed.

Animals↗
PubMed1993

The effect of hormonal changes on cutaneous disease in lupus erythematosus.

The behaviour of cutaneous disease in systemic lupus erythematosus (SLE) and discoid lupus erythematosus (DLE), under the influence of various hormonal states, was studied in 68 patients. In 28 pregnancies, cutaneous disease was essentially unchanged. In a total of 57 patients whose lupus erythematosus (LE) had been diagnosed prior to the menopause, 20% described a premenstrual cutaneous exacerbation. Only three patients had taken an oestrogen-containing contraceptive. The duration of oral contraceptive treatment before the onset of lupus varied: 1 month in a patient presenting with the acute malar rash of SLE, 2 months in a patient who presented with annular weals and later developed systemic features, and 12 months in a patient who developed generalized DLE. Thirty-three patients were menopausal at the time of the study; 4% had noticed a perimenopausal cutaneous flare. There was no deterioration in the skin of the five patients on hormone replacement therapy.

Adolescent↗
PubMed2004

Prevalence of skin diseases in Ibadan, Nigeria.

AIM: To study the pattern of skin diseases in patients attending the skin clinic of the University College Hospital, Ibadan, Nigeria, and to compare our findings with studies performed earlier in the same clinic. PATIENTS AND METHODS: The study involved 1091 new patients who had attended the skin clinic of the University College Hospital, Ibadan, Nigeria, between January 1994 and December 1998. The patients were examined by the authors, and laboratory investigations were ordered when necessary to make a diagnosis. RESULTS: An increased prevalence of eczema, idiopathic pruritus, urticaria, connective tissue diseases, and fixed drug eruptions was observed. Infections, such as scabies, candidiasis, and tinea versicolor, had also increased. Pyoderma, leprosy, onchocerciasis, and dermatophytoses showed a decline. Psoriasis was uncommon, although there was a slight increase in prevalence. Vitiligo and alopecia were stable. Cutaneous tuberculosis, such as lupus vulgaris, was rare. CONCLUSIONS: Allergic conditions have increased; connective tissue disorders, such as systemic lupus erythematosus, scleroderma, and discoid lupus erythematosus, have also increased. Cutaneous disorders associated with human immunodeficiency virus infection, such as seborrheic dermatitis, have increased. Health workers need to be educated on the management and treatment of these conditions, and should be advised to refer patients to appropriate health facilities when necessary.

Female↗
PubMed2003

Lupus mucinosis: a case report and review of cutaneous lupus.

Lupus mucinosis (LM) is a rare disorder found only in patients with systemic lupus erythematosus (SLE) or discoid lupus erythematosus (DLE). We describe a patient with lupus mucinotic nodules as the initial presenting sign of SLE. We further discuss other forms of cutaneous lupus and contrast them with our findings.

Ankle↗
PubMedDate not supplied

Clinical studies of antibodies binding polyriboadenylic acid in systemic lupus erythematosus.

Antibodies of polyriboadenylic acid (poly rA) are demonstrated by filter radioimmunoassay in 75% of patients with systemic lupus erythematosus (SLE), in 54% of patients with discoid lupus erythematosus, and in only 0-7% of normal controls and patients with rheumatoid arthritis or Sjögren's syndrome. These antibodies are distinct from antibodies to single- and double-stranded DNA and double-stranded RNA. Poly rA binding is associated with IgM and IgG serum fractions. Because poly rA may have a role in the transcription of mRNA in mammalian cells and viruses, antibodies to poly rA may be important clues to virologic and immunogenetic mechanisms in the pathogenesis of SLE.

Antibodies↗
PubMed1990

[Lichen planus/lupus erythematosus overlap syndrome].

A patient, showing features of discoid lupus erythematosus (DLE) of the face and of lichen planus (LP) on the arms, is reported. The histopathology showed a typical LP on the dorsum of the hand and a DLE on the face, but direct immunofluorescence in both cases was more suggestive of LP.

Biopsy↗
PubMed1987

A family survey of lupus erythematosus. 1. Heritability.

First degree relatives and spouses of 36 patients with systemic lupus erythematosus (SLE) and 37 with discoid lupus erythematosus (LE) were assessed using the ARA criteria. They were compared with relatives and spouses of patients with other rheumatic and related complaints. Definite SLE was present in 3.9% of relatives of SLE probands, 2.6% of discoid relatives and 0.3% of controls. Discoid LE was diagnosed in 0.6% of SLE and 3.5% of discoid families compared with 0.5% of controls. None of the spouses of LE probands had SLE or discoid LE. The data gave the best fit for a polygenic inheritance with a heritability of 66 +/- 11% for SLE and 44 +/- 10% for discoid LE. Genetic factors are thus less important in SLE and discoid LE than in generalized osteoarthritis, spondylitis or gout with heritabilities of 90, 72 and 90%, respectively.

Arthritis, Rheumatoid↗
PubMed1982

Chronic cutaneous lupus erythematosus. Clinical, laboratory, therapeutic, and prognostic examination of 62 patients.

Chronic discoid lupus erythematosus (DLE) is a common condition. Sixty-two patients with biopsy-proved, active DLE were observed and their conditions were analyzed for clinical, laboratory, and therapeutic data. Fifty-six patients had disease limited to the skin-26 localized and 30 widespread (above and below the neck). At the time of follow-up examination, active disease was present in 32 patients, 28 of whom had widespread DLE. Six patients had DLE as a manifestation of systemic LE (SLE). In four patients, the DLE preceded the development of SLE. Laboratory abnormalities were substantially more common in patients with widespread DLE than in patients with localized DLE. An analysis of therapeutic results in this series confirmed the beneficial effects of intralesional corticosteroids and antimalarial agents and demonstrated relatively poor responsiveness to topical or oral corticosteroids.

Adolescent↗