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Thalidomide: use and possible mode of action in reactional lepromatous leprosy and in various other conditions.

The literature concerning the use and possible mode of action of thalidomide in reactional lepromatous leprosy and in various other conditions is reviewed. Although it has no action against the leprosy bacillus, its value in the treatment of the adverse reactions in this type of leprosy is well established, many leprologists considering it to be superior to any other drug for this purpose. Its efficacy in actinic prurigo is also impressive, and there are reports suggesting benefit in discoid lupus erythematosus. By contrast, its reported action in a number of other conditions, including severe aphthous stomatitis, Behçet's syndrome, pyoderma gangrenosum, nodular prurigo, and postherpetic neuralgia, needs confirmation in a larger number of cases, backed in some instances by clinical trial. The mechanism of action of this drug may be related to (1) anti-inflammatory effects, particularly an inhibition of neutrophil chemotaxis, (2) immunosuppressive effects, or (3) effects on neural tissue. Furthermore, structure-activity studies may allow separation of these and other possible effects. This review is in no way intended to lend support to the indiscriminate use of a potentially hazardous drug in various diseases of unknown cause, but rather to draw attention to a number of conditions in which the drug has been found effective. The further judicious use of thalidomide or a nonteratogenic analogue, with careful observation of results, may contribute to knowledge of the underlying pathology in some of these conditions, and possibly also to the mechanism of action of the drug itself.

Anti-Inflammatory Agents↗

Toxic psychosis: a complication of antimalarial therapy.

A case is reported of toxic psychosis in a patient with a diagnosis of discoid lupus erythematosus treated with hydroxychloroquine. The dose slightly exceeded the maximum recommended dose for 10 days because of a pharmacy error. Since more antimalarials are being used for various diseases, attention is called to this possible complication with a subtle onset.

Adult↗

HLA and genetic predisposition to lupus erythematosus and other dermatologic disorders.

Human leukocyte antigen (HLA) associations with different clinical and serologic subsets of lupus erythematosus are providing important clues to genetic predisposition and pathogenesis. The evolving complexity of the HLA-D region is described, and currently recognized HLA-region associations with systemic lupus erythematosus, subacute cutaneous lupus erythematosus, homozygous C2-deficient lupus, Sjögren's syndrome, and the neonatal lupus syndrome are reviewed. The striking relationship between the Ro/SSA-La/SSB antibody responses and HLA-DR2 and DR3 are emphasized. Other dermatologic conditions associated with HLA are also noted.

Adult↗

Lupus profundus in children: treatment with hydroxychloroquine.

Discoid lupus erythematosus and lupus profundus, rare in children, are described in two young girls, one with onset at 3 1/2 years of age, the other at 8 years of age. Unusual nodules of the face that ultimately healed with atrophy and hyperpigmentation showed histologic and immunofluorescent confirmation of lupus erythematosus. These patients, the second and third in the English literature to be treated for childhood lupus profundus with antimalarials, responded successfully to hydroxychloroquine. No systemic involvement was found.

Child↗

Successful treatment of hypertrophic lupus erythematosus with isotretinoin.

A patient with systemic lupus erythematosus had the additional finding of hypertrophic lupus erythematosus. The lesions cleared with an 11-week course of isotretinoin alone. She has remained without recurrence for 9 months. This is the first reported case of total resolution of hypertrophic lupus erythematosus with a short course of isotretinoin.

Adult↗

Immunopathologic study of lichenoid skin diseases: correlation between HLA-DR-positive keratinocytes or Langerhans cells and epidermotropic T cells.

Skin biopsy specimens from 24 patients with different lichenoid skin diseases that had been proved histologically were studied immunohistologically. Marked differences were noted in the number of OKT6+/S100+ Langerhans cells within the epidermis and dermis in the lesional skin between lichen planus (and its related disease) and lupus erythematosus; in the former these cells were increased in number; in the latter they were decreased in number compared with those in uninvolved perilesional skin. Human lymphocyte antigen (HLA)-DR expression on keratinocytes was observed not only in lichenoid skin diseases but also in control cases without epidermal involvement. In the two cases of systemic lupus erythematosus, the uninvolved perilesional skin also show weak and focal HLA-DR reactivity in the basal layer. HLA-DR+ keratinocytes could play an important role at least in the perpetuation of epidermal cell damage mediated by T cells.

Epidermis↗

Rowell's syndrome. Report of a case.

We describe a patient with discoid lupus erythematosus who developed annular lesions of the thigh and chilblainlike lesions of the fingers matching those described in the original reports of Rowell's syndrome. The patient also had circulating anti-Ro(SS-A) antibodies whose similarity to the anti-Sj-T antibodies found in the original Rowell's syndrome cases has been recently claimed. A review of the literature suggests that most of the cases of Rowell's syndrome described thus far in fact may be cases of coincidental association of lupus erythematosus and erythema multiforme.

Antibodies, Antinuclear↗

Treatment of severe cutaneous lupus erythematosus with a chimeric CD4 monoclonal antibody, cM-T412.

BACKGROUND: Monoclonal CD4 antibodies are among the most potent immunomodulatory agents in various experimental models of autoimmune disease, including murine lupus erythematosus. OBJECTIVE: The aim of this study was to evaluate the toxicity and therapeutic efficacy of a chimeric monoclonal CD4 antibody, cM-T412, in patients with cutaneous lupus erythematosus (LE). METHODS: Five patients with severe cutaneous LE lesions received intravenously a total of 275, 400, or 475 mg of cM-T412 in single doses of 20 to 50 mg during a period of 5 to 8 weeks. RESULTS: CD4 antibody treatment induced a long-lasting decrease in disease activity. It resulted in healing of LE skin lesions, a reconstituted responsiveness to conventional treatment, or both. Despite a substantial depletion of circulating CD4+ T lymphocytes, no clinical signs of immunosuppression were noted. CONCLUSION: Monoclonal CD4 antibodies should be considered as a novel treatment for the management of severe cutaneous LE.

Adjuvants, Immunologic↗

Significance of the anti-Ro (SS-A) antibody in evaluation of patients with cutaneous manifestations of a connective tissue disease.

The anti-Ro(SS-A) antibody is arguably the most important antibody determination except for antinuclear antibodies in evaluation of patients suspected of having lupus erythematosus. During the past 25 years, studies have established the importance of this antibody in the evaluation of patients with atypical lupus erythematosus, who have a photosensitive dermatitis as the presenting sign. The purpose of this review is to demonstrate the utility and the necessity of this antibody determination in the evaluation of all patients with cutaneous manifestations who are suspected of having a connective tissue disease.

Antibodies, Antinuclear↗

Recently described feline dermatoses.

This article discusses the etiology, clinical signs, diagnosis, and treatment of several diseases. Feline immunodeficiency, virus infection, cryptococcosis, dermatophyte pseudomycetomas, demodicosis, Sézary-like syndrome, and discoid lupus erythematosus in cats are reviewed.

Animals↗

Collagen vascular diseases and radiation therapy: a critical review.

PURPOSE: Although many oncologists have the impression that patients with collagen vascular disease tolerate radiotherapy less well than other patients, until now this was never described in a review article. METHODS AND RESULTS: The principal objective was to determine whether patients with collagen vascular diseases have a greater risk of severe radiation therapy complications, than those without a collagen vascular disease. However, most of the publications found on this topic are short anecdotal case reports of patients with increased toxicity after radiation. Consequently, the true incidence of these side effects is unknown. CONCLUSIONS: Unless further studies on this subject are reported, each radiation oncologist should be cautious in treating these patients.

Adult↗

Systemic lupus erythematosus in childhood.

Systemic lupus erythematosus is a disease of immune dysregulation that strikes approximately 1 in 2000 individuals. The usual patient is a young woman of child-bearing age; however, this illness affects patients of all ages, ethnic backgrounds, and both sexes. Twenty percent of all cases of lupus are diagnosed during the first two decades of life. Perhaps the most essential point in treating a child with lupus is to be aware and concerned about how to deliver treatment to a patient in the middle of their physical, intellectual, and emotional development.

Adolescent↗

Primary presentation of spontaneous jugular vein thrombosis to the otolaryngologist--in three different pathologies.

An otolaryngologist is conversant with the potential of middle-ear disease leading to lateral sinus thrombosis and subsequent jugular vein thrombosis, although this is becoming an increasingly rare event. Spontaneous jugular vein thrombosis from non-otological causes, however, is not well described in the otolaryngology literature. We present three cases of jugular vein thrombosis who presented primarily to the Otolaryngology department each attributable to a different pathology including discoid lupus erythematosus, antiphospholipid syndrome and metastatic malignancy. Awareness needs to be increased of this condition in the differential diagnosis of acute neck swelling and neck pain.

Adult↗