Thalidomide treatment of subacute cutaneous lupus erythematosus.
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Biomedical subjects
Publications and source records attributed to W R Faber.
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Seven patients who developed a characteristic pruritic rash in late pregnancy are described. Clinically, the lesions consisted of erythematous urticarial papules and plaques, with vesicles in some cases. The eruption started in the striae on the abdomen and spread over the thighs and in most cases also over the arms and buttocks. Biopsy showed lymphocytic vasculitis with a varying admixture of eosinophils and only minor epidermal changes. Immunofluorescence examination of six cases showed C3 deposition among the dermoepidermal junction in only one case. The lymphocytes, identified by a specific anti-T-cell serum, appeared to be mostly T-cells. The nomenclature of pruritic skin disease in late pregnancy is confusing, but the term 'late prurigo of pregnancy' seems appropriate for this condition.
Five patients with a histopathologically confirmed diagnosis of pityriasis lichenoides were treated with PUVA or irradiated with a light source emitting UVB and UVA, without prior intake of psoralens. All patients showed a good response to treatment. Long-term follow up showed that patients remained free of lesions during a period of 20 to 36 months; 3 patients had a recurrence of the disease, though less extensive than before, after 25, 23, and 23 months, respectively.
This paper reports an immunofluorescence (IF) study of the skin of 16 patients with chronic discoid localised lupus erythematosus and 5 patients with cutaneous disseminated lupus erythematosus. In the majority of the cases (18), we have found immunoglobulin and complement deposits not only along the dermoepidermal junction, but also in the blood vessel walls. In these deposits there was a predominance of IgM. It appears that an immune complex vasculitis in the skin can be found in patients from the entire lupus erythematosus spectrum. The discovery of immunoglobulins and complement deposits in the blood vessel walls of the involved skin of patients with discoid lupus erythematosus, suggests that it can be considered as an important (and even characteristic) feature of the disease.
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A 39-year-old man is described who has been suffering from psoriasis for many years. Because of the extensive involvement of the skin he was treated with PUVA therapy, initially with a good result. A second course of PUVA treatment was unsuccessful. Histopathological examination of the (persisting) skin lesions at this time showed clear granulomatous infiltration, in addition to psoriatic changes. Based on history, serological and dark-field examination, a secondary syphilis was diagnosed. The possible influence of PUVA therapy is discussed.
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A 50-year-old woman with autoimmune thyroiditis had an eruption resembling pyodermite végétante as described initially by Hallopeau. Direct immunofluorescence microscopy studies indicated deposition of IgG and C3 in the intercellular area of the epidermis. Circulating IgG antibodies to the intercellular areas of stratified epithelial tissue were demonstrable on indirect immunofluorescence microscopy. Treatment with prednisone and azathioprine resulted in the disappearance of vetetating lesions and in healing, with postinflammatory hyperpigmentation. Immunofluorescence microscopy studies, in an early stage of the disease, are of great value in the diagnosis of pyodermite végétante of Hallopeau. Our findings support the view that pyodermite végétante of Hallopeau belongs immunologically to the pemphigus group.
A case of cicatricial pemphigoid (CP) with a rare primary localization on the mucosal surfaces of the anus and the vulva and on perianal skin, associated with long-term therapy with clonidine, is described. Direct immunofluorescence investigation of lesional skin suggests possible complement mediated tissue injury in between basement membrane and epidermal basal cells. The possibility is discussed that drug induced metabolic disturbance (cyclic AMP-system) at a local level at the site of the basal cells might lead to the formation of immunogens and be one of the initiating factors in subepidermal bullous immune dermatoses. It is concluded that in cases of cicatricial pemphigoid the use of drugs must be considered as one of the aetiological possibilities.
Nine biopsies from skin lesions of 5 patients with pityriasis lichenoides acuta and three biopsies from skin lesions of 3 patients with pityriasis lichenoides chronica were examined by means of the direct immunofluorescence technique. IgM deposits along the dermoepidermal junction were found in only two biopsies. In the majority of bioipsies, complement (C3) deposits were found along the dermo-epidermal junction and in the vessel walls. Immunoglobulin and C3 deposits were not found concomitantly in the vessel walls.
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A 19-year-old girl presented with cutaneous lupus erythematosus and hypothyroidism, and developed a thyrotoxic crisis when chloroquine therapy was discontinued.
The results of treatment of the group of leprosy patients at the lepromatous side of the leprosy spectrum registered at the Department of Dermatology of the University of Amsterdam in the years 1950-1976 were studied. The average duration of treatment to obtain bacteriologically negative skin biopsies in patients who were untreated at the time of registration, was 5 years. A substantial number of patients suffered a relapse; the main reasons for these relapses were discontinuation of treatment and DDS treatment in low dosage.
The effects of repeated injections of transfer factor over a period of 20 weeks were investigated in fourteen bacteriologically positive patients at the lepromatous side of the leprosy spectrum. All patients showed negative (0 mm induration) skin tests to M. leprae antigens (i.e. leprolin and lepromin). Of these patients, seven were treated with transfer factor with a total of 9 units (1 unit being equivalent to 5 x 10(8) lymphocytes) and seven with a placebo. Maintenance treatment with clofazimine was continued. Transfer factor was prepared from the lymphocytes of donors who showed positive skin tests to M. leprae antigens (i.e. leprolin greater than or equal to 12 mm induration, average 15.5 mm or lepromin greater than or equal to 8 mm induration, average 13.6 mm), as well as a positive lymphocyte transformation in vitro to M. leprae (the average transformation being higher than the average transformation of lymphocytes of tuberculoid leprosy patients). No differences were found between the two groups as regards the clinical course of the disease, the histopathological and bacteriological evaluation of skin biopsies, changes in skin test reactivity to various antigens (i.e. lepromin, leprolin, PPD, Mumps, C. albicans, Tr. rubrum and Varidase), as well as the lymphocyte transformation in vitro to various mitogens (i.e. PHA, PWM, Con A) and antigens (i.e. M. leprae, leprolin, PPD, BCG, Mumps, C. albicans, Trichophyton and Varidase). No evidence was found to suggest that transfer factor is a valuable adjuvant in the treatment of lepromatous leprosy patients or that it increases cell-mediated immune reactivity towards M. leprae.
Recently, there has been a sharp increase in the number of leprosy patients in The Netherlands, due to an increased immigration from Surinam where leprosy is hyperendemic. The patients registered at the Dermatological Department of the University of Amsterdam during the years 1972-1976 were reviewed. It was found that 28% of the untreated patients had recently suffered a relapse. A substantial percentage took the medication irregularly or discontinued treatment prematurely and quite a few patients required other types of treatment besides chemotherapy, such as surgical treatment and physiotherapy.
A case is reported of a patient with a bullous eruption with in vivo linear deposition of IgA and complement (C3) along the basement membrane zone of perilesional skin (DIF method). Some data presented here are in favour of the concept that cases of linear in vivo deposition of IgA alone, or in combination with other IF findings, might be classified as bullous pemphigoid (BP). Clearing of the lesions due to oral prednisone therapy was accompanied by disappearance of complement deposition, while IgA deposition remained unchanged. Some aspects of tissue injury in this case are briefly discussed.
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