High dose intravenous immunoglobulin (IVIG) in dermatomyositis: clinical responses and effect on sIL-2R levels.

An open study was conducted to identify and investigate dermatomyositis patients who benefit from IVIG treatment, based on dermatological criteria, myositis-related symptoms and immune/inflammatory parameters. 19 patients (16 females and three males, ages 31-84) suffered from dermatomyositis, and 4/19 patients had paraneoplastic dermatomyositis. We monitored the disease activity by documenting the clinical symptoms, recording muscle-related parameters (electromyography, serum creatine kinase, histopathology), and by determining circulating autoantibodies and serum levels of IL-6, sIL-2R, sTNF-a-R, sICAM-1, and sCD8. 7/19 patients responded to IVIG. They had severe skin but only moderate muscle involvement, no autoantibodies, and no malignancy. IVIG-nonresponders had severe skin and muscle disease, concomitant with autoantibodies and/or malignancy. sIL-2R levels were initially elevated in all patients but reverted to normal in IVIG-responders only. Creatine kinase-levels and other parameters did not correlate with disease activity and/or treatment response. IVIG is effective in selected dermatomyositis patients. sIL-2R serum levels appear to be useful predictors of IVIG-induced treatment response and disease activity.

Immunohistochemistry with monoclonal antibody against Candida albicans mannan antigen demonstrates cutaneous Candida granulomas as evidence of Candida sepsis in an immunosuppressed host.

We report the occurrence of invasive Candida albicans infection with disseminated cutaneous Candida granulomas in a patient with aplastic anaemia after viral hepatitis. Fungal elements in a skin biopsy specimen were detected by PAS stain and identified as Candida sp. by immunohistochemistry directed against the C. albicans mannan surface antigen. Based on rapid diagnosis of Candida granuloma and by Candida-positive cultures of blood and swabs, systemic treatment with liposomal amphotericin B led to survival of the patient.

[Established and new therapeutic approaches in dermatomyositis, polymyositis and overlapping syndromes]. / Etablierte und neue Therapiestrategien bei Dermatomyositis, Polymyositis und Uberlappungssyndromen.

An overview is presented on current antiinflammatory, cytostatic/cytotoxic and immunomodulatory treatments for immunologically mediated inflammatory myopathies with skin involvement. High-dose intravenous immunoglobulin (IVIG) therapy is discussed in the context of recent findings regarding pathogenic mechanisms of autoimmune diseases.

[Primary antiphospholipid antibody syndrome in pregnancy--successful pregnancy outcome after intravenous immunoglobulin administration]. / Das primäre Antiphospholipid-Antikörper-Syndrom (APAK) in der Schwangerschaft--erfolgreicher Schwangerschaftsverlauf nach intravenöser Immunglobulingabe (IVIG).

Primary antiphospholipid antibody syndrome is characterized by intrauterine fetal death and arterial and/or venous thrombosis. The clinical course of a 37-year-old pregnant woman with a history of eight previous miscarriages is presented. By application of intravenous immunoglobulin (Venimmun Behringwerke Marburg/Lahn) a successful pregnancy could be accomplished. Diagnosis, immunologic pathomechanisms, possible treatment modalities as well as the obstetric management of this autoimmune disease will be discussed.

Bullous pemphigoid induced by PUVA therapy.

An 80-year-old psoriatic patient developed a blistering eruption during oral PUVA therapy. The diagnosis of bullous pemphigoid (BP) was established by routine histopathology, which demonstrated subepidermal blistering, and direct immunofluorescence, which revealed linear deposits of IgG, IgM and C3 along the basement membrane zone. Indirect immunofluorescence using normal human split skin revealed binding of IgG antibodies to the epidermal side, thus confirming a subepidermal blistering disorder. These proteins were identified by the immunoblotting technique as BP antigens I and II. Clinically, the lesions could be reproduced by phototesting using topical 8-methoxypsoralen. Again, the histopathological and immunopathological findings were consistent with the diagnosis of PUVA-induced BP. To the best of our knowledge, this is the first report demonstrating psoriasis-associated BP, in which the clinical diagnosis of BP is confirmed by immunoblotting analysis. The exact role of UV light in precipitating bullous lesions, particularly the question whether UV light may represent an unspecific epidermal injury leading to further attraction of autoantibodies to the basement membrane zone, as suggested recently by an experimental study in rodents, remains to be clarified in future studies.

Association between chronic cutaneous lupus erythematosus and HLA class II alleles.

CCLE, a disease entity at the benign end of the lupus spectrum, is characterized by marked photosensitivity and skin lesions in sun-exposed areas. The histopathology of lesions resembles hypersensitivity type IV reactions. We have asked whether an association between class II alleles and CCLE exists. RFLP analysis of HLA-DQA genes revealed a Taq I HLA-DQA1 allelic restriction fragment overrepresented in a group consisting of 26 patients as compared to healthy control individuals. This result was corroborated by typing with oligonucleotide probes. The presence of the DQA1*0102 allele in the patients' group led to a relative risk of 4.57, with a statistical significance of p < 0.05 after correction for 36 comparisons. Although not statistically significant, it is interesting that all patients possess in at least one of their HLA-DQA1 alleles a nucleotide sequence coding for the amino acid glutamine at position 34 of the DQ alpha molecule. The expected frequency of these alleles in the control population amounts to 82%. The HLA-DRB1*16 allele, which is found in linkage disequilibrium with the HLA-DQA1*0102 allele, is also observed at an increased frequency in the patient's group, though the association was not significant after correction for the number of comparisons. However, no associations of CCLE with alleles at the HLA-DPB1 locus was found. The association of CCLE with certain HLA class II alleles points to an involvement of HLA-DQ and/or -DR molecules in the pathogenesis of the disease. Alternatively, genetic loci in linkage disequilibrium may code for elements which contribute to the development of CCLE.(ABSTRACT TRUNCATED AT 250 WORDS)

[Schoenlein-Henoch purpura associated with chronic post-traumatic osteomyelitis]. / Purpura Schönlein Henoch assoziiert mit chronischer posttraumatischer Osteomyelitis.

In Schönlein-Henoch purpura, a distinctive form of leucocytoclastic vasculitis, immune complex deposits in vessel walls characteristically contain IgA. Complement activation, neutrophil chemotaxis and the subsequent destruction of vessel walls make up the pathologic substrate behind the clinical symptoms, which are characterized by palpable purpura lesions, renal and gastrointestinal symptoms and arthralgias. Viral infections, malignant neoplasms and drugs, but especially streptococcal infections, are incriminated as possible causes of the disease. A case of Schönlein-Henoch purpura associated with chronic osteomyelitis is described. It is note-worthy insofar as there is only one similar case reported in the literature.

Accumulation of gamma delta T cells in chronic cutaneous lupus erythematosus.

Monoclonal antibodies (moAbs) that recognize common or variable determinants of the gamma delta T-cell receptor (TcR) were used to assess gamma delta T-cell distribution on biopsy specimens and/or peripheral blood leukocytes (PBL) from 30 patients suffering from chronic cutaneous lupus erythematosus (CCLE). CD3+/gamma delta TcR + T cells were evaluated in 15 biopsies from patients with CCLE lesions, their numbers varying from 0.5 to 15.0% of all intralesional CD3 +T cells present. In all specimens from lesional skin gamma delta TCR+T cells were BB3 + and/or Ti gamma A +, indicating predominant use of the V gamma 2/V delta 2 phenotype. In the CCLE lesions the intraepidermal V gamma 2/V delta +T cells were observed in close vicinity to the damaged basal keratinocyte (KC) layer, and also randomly scattered among the densely packed inflammatory infiltrate in the dermis. In contrast to the immunohistologic findings, no numerical increase of gamma delta TcR+T cells could be observed among PBL from 28 of 30 CCLE patients. Only one CCLE patient being treated with hydroxychloroquine for two months had 15% CD3 +/gamma delta TcR+T cells among the PBL. Based on the immunohistologic findings one may infer that in CCLE, a skin-restricted form of LE, V gamma 2/V delta 2 +T cells expand extrathymically to an as yet unknown stimulus. One may also propose that these gamma delta T cells--based on their cytotoxic capacity--may contribute to the epidermal damage. It remains to be determined whether the extrathymic expansion of V gamma 2/V delta 2 +Tells occurs within lesional skin or in the periphery within subsequent recruitment into skin lesions. The results obtained by fluorescence-activated cell sorter analysis favor the first possibility.

Cyclosporin A in combination with photochemotherapy (PUVA) in the treatment of psoriasis.

Forty patients with relapsing plaque psoriasis involving more than 20% body surface were treated either with cyclosporin A (CyA) plus PUVA or the retinoid etretinate plus PUVA (RePUVA). They initially received either CyA (2 weeks) or etretinate (1 week) alone and then PUVA was given concomitantly until complete remission. The patients were monitored over a period of 6 months and any relapse recorded. With each combined treatment regimen, CyA plus PUVA and RePUVA, the patients cleared within comparable periods of time (mean treatment period of 5.3 vs. 4.7 weeks after initiation of therapy and 3.3 vs. 3.7 weeks after initiation of PUVA). However, the cumulative UVA dose required for clearance (110.9 J/cm2 vs. 62.1 J/cm2 (P less than 0.05)) and the incidence of severe and early relapses were significantly higher in the CyA cohort. Within 6 months severe relapses had occurred in 58% of CyA plus PUVA but only in 15% of RePUVA-treated patients (P less than 0.001). This suggests that the CyA plus PUVA regimen as performed in this study is less effective than RePUVA.

[Wound myiasis. Facultative myiasis]. / Wundmyiasis. Fakultative Myiasis.

Two cases of traumatic myiasis, one in Bowen carcinoma and one in chronic leg ulcer, are described. The maggots isolated were reared to the adult stage; they proved to be Calliphoridae of the genus Lucilia (presumably L. sericata). The various forms of myiasis and the species involved in such parasitism are described, and the therapeutic efficiency of proteolytic enzymes and the necrolytic potency of maggots are briefly discussed.