Incidence of IgA vasculitis in the adult Slovenian population.

BACKGROUND: IgA vasculitis (IgAV) is assumed to be uncommon in adults. OBJECTIVES: To determine the incidence rate of histologically proven IgAV in the adult Slovenian population. METHODS: A retrospective chart review of adult patients diagnosed with IgAV was performed at the departments of rheumatology, nephrology, infectious diseases and dermatovenereology at an integrated secondary/tertiary university teaching hospital. In order to avoid missing miscoded cases, the Institute of Pathology, University of Ljubljana, Slovenia, provided a list of all patients with an IgAV-compatible histological pattern on biopsy. The annual incidence rate of histologically proven IgAV was calculated. RESULTS: Eighty-one new cases of IgAV were identified from June 2010 to June 2013. The estimated annual incidence rate of IgAV was 5·1 per 100,000 adults [95% confidence interval (CI) 3·4-7·4]; in men it was 6·1 per 100,000 (95% CI 3·9-10·6) and in women it was 3·7 per 100,000 (95% CI 1·8-6·8). CONCLUSIONS: Although we only included histologically proven cases of IgAV, the annual incidence rate of 5·1 per 100,000 adults is 3-6-times higher than previously reported.

Scleromyxedema with subcutaneous nodules: successful treatment with thalidomide and intravenous immunoglobulin.

Scleromyxedema is a rare cutaneous mucinosis, usually presenting with generalized papular eruption and sclerodermoid induration, monoclonal gammopathy and systemic manifestations. An atypical clinical presentation with cutaneous and subcutaneous nodules has been reported rarely. In recent years, intravenous immunoglobulin (IVIg) appears to be the therapy of choice for scleromyxedema. Treatment experiences in atypical manifestations with mucinous nodules are limited to sporadic reports. We report the case of male patient with atypical scleromyxedema without underlying paraproteinemia, presenting with generalized papular and sclerodermoid skin eruption and multiple nodular mucinous lesions on the fingers and face as well as on the eyelids, and associated systemic symptoms. Complete regression of all cutaneous lesions and extracutaneous symptoms with sustained remission was achieved by combined treatment with thalidomide and IVIg.

The surgical treatment of hypertrophic intertriginous Darier's disease.

Darier's disease is a rare autosomal-dominant genodermatosis characterised by persistent hyperkeratotic papules and plaques in seborrhoeic areas. The flexural areas of the body are especially difficult to treat, and they are often resistant to current topical and systemic treatment strategies. To our knowledge, this article provides the first description of a successful surgical treatment of a severely debilitating, hypertrophic Darier's disease of intergluteal area, resistant to oral retionids. A 25-year-old male patient was successfully treated with the use of tissue expanders and diseased skin excision. There are no signs of recurrence of the disease in the perianal region 6 months postoperatively, and there is a good result in functional as well as cosmetic terms. The formation of new skin on the excision site was observed, and there were no evident signs of infection. These encouraging results suggest that a patient who responds poorly to conventional treatment in a specific area should be presented to the plastic surgeon at an earlier point in time. Therefore, unnecessary hospitalisations, antibiotic treatments, dose-related adverse effects of oral retionids can be avoided, and eventually a long-term result can be achieved.

Tubulo-interstitial involvement in lupus nephritis with emphasis on pathogenesis.

Glomerular lesions in lupus nephritis have been extensively studied in recent decades, but much less attention has been paid to the tubulo-interstitial compartment. The aim of this study was to contribute to the understanding of the pathogenesis of tubulo-interstitial lesions in lupus nephritis by analysing their incidence, character, and their associations. One hundred and ninety kidney biopsies of 190 patients fulfilling American Rheumatology Association (ARA) criteria of systemic lupus erythematosus (SLE) were examined by traditional light, immunofluorescence and electron microscopy. Interstitial inflammatory infiltration and tubulo-interstitial immune deposits concurred in 72 cases (37.9%). Their frequency was the highest in WHO class IV lupus glomerulonephritis. By multivariate analysis, the intensity of interstitial inflammatory infiltration correlated best with the percentage of renal corpuscules with extracapillary crescents and the extent of interstitial fibrosis. On immunohistochemical assessment, the inflammatory infiltrate was found to be composed of CD45RO positive T lymphocytes (191.3/mm2), CD68 positive macrophages (101.7/mm2) and CD45RA positive B lymphocytes (17.2/mm2). For all cell types the median value was higher in cases with extracapillary crescents, and did not correlate with presence and intensity of tubulo-interstitial immune deposits. Infiltration showed the tendency of periglomerular distribution, especially around glomeruli showing extracapillary proliferation and destruction of the capsular basal membrane. Rare S100 positive cells were only found in the interstitium. Tubulo-interstitial lesions estimated semiquantitatively correlated with the degree of proteinuria. Our findings suggest that tubulo-interstitial deposits do not play a major role in the pathogenesis of tubulo-interstitial lesions. The formation of interstitial cell infiltrates appears to be greatly influenced by the development of extracapillary crescents, perhaps by direct transmission of the severe inflammatory process to the adjacent interstitium. The composition of the infiltrate, including antigen presenting cells may signalize an additional involvement of cell-mediated immune mechanisms acting against so far hypothetical tubular epithelial neoantigens.

[Significance of histopathologic analysis of skin lesions in scleromyxedema. Light microscopy, electron microscopy, immunohistochemistry and immunofluorescence microscopy]. / Bedeutung der histopathologischen Analyse der Hautläsionen bei Skleromyxödem. Lichtmikroskopie, Elektronenmikroskopie, Immunhistochemie und Immunfluoreszenzmikroskopie.

Scleromyxedema (SM) may be considered as a possible disease entity in the differential diagnosis of scleroderma. Clinical data and the results of light, immunohistochemical, immunofluorescence and electron microscopic study of skin biopsies taken from a 53-year old patient with SM are reported. In the patient with SM in which abnormal serum paraprotein was not identified, the skin biopsy showed mucinous material in the dermis and proliferation of fibroblasts accompanied by mild dermal sclerosis. Immunofluorescence showed scanty granular IgG along the epidermal basement membrane and IgG and C1q focally along the connective tissue fibres in the dermis of clinically involved skin. In addition to clinical findings, detailed skin biopsy studies including contemporary techniques can contribute to the diagnosis of the disease.

The significance of histologic analysis of skin lesions in porphyria cutanea tarda. Light microscopy, electron microscopy, immunohistochemical and immunofluorescence analysis.

Porphyria cutanea tarda (PCT) is one of several entities in the differential diagnosis of scleroderma. We report a 62-year-old man with PCT diagnosed since two decades. Clinical data and the results of light microscopy, immunohistochemistry, immunofluorescence and electron microscopy of skin biopsies are presented. The biopsy revealed sclerosis of the dermis mainly due to increased collagens I and III, and accumulation of collagen IV, which had caused the vessel wall to thicken. Immunofluorescence for detection of immune reactants was negative. It was concluded that the histomorphology of PCT of long duration may be similar to that of scleroderma. Nevertheless, in addition to clinical findings, detailed skin biopsy studies including contemporary techniques can contribute to the differentiation of these diseases.