Quality of life and satisfaction with life in SLE patients—the importance of clinical manifestations

To assess the correlation between quality of life (QoL) and satisfaction with life (SL) in SLE patients and correlate both with clinical symptoms of the disease. The study was performed in 83 patients. QoL was assessed by Short Form 36, and SL was assessed by the Satisfaction with Life Scale. Clinical manifestations presented at the time of examination were taken into consideration. SLE patients assessed their QoL and SL as rather low. Those with photosensitivity as well as neurological symptoms presented lower QoL in particular domains, while those with renal manifestation of SLE assessed their QoL as higher. Similar observations were made for SL only in relation to neurological symptoms. Moreover, our findings show that although SL is a part of QoL, both these parameters should be distinguished in order to fully assess the state of the patient

Pozatwarzowy ziarniniak twarzy – opis przypadku

Wprowadzenie: Ziarniniak twarzy (granuloma faciale) jest nieczęstospotykaną, przewlekłą chorobą skóry. Charakteryzuje się występowaniemna twarzy brunatnoczerwonych grudek, guzków i ognisk naciekowych.Wyjątkowo ziarniniaka spotyka się poza twarzą. Cel pracy: Przedstawienie chorego, u którego granuloma faciale był zlokalizowanywyłącznie poza twarzą. Opis przypadku: U 56-letniego, ogólnie zdrowego mężczyzny w ostatnich15 latach stopniowo ukazywały się bezobjawowe, pojedynczegrudki i ograniczone, brunatnoczerwone, koliste nacieki, o średnicy1–4 cm. Wykwity były zlokalizowane na plecach, barku, brzuchu i zalewą małżowiną uszną. Wymagały różnicowania z chłoniakiem, lymphocytoma,mięsakiem Kaposiego oraz sarkoidozą. W badaniu histopatologicznymstwierdzono obraz wskazujący na ziarniniaka twarzy, copozwoliło na ustalenie rozpoznania granuloma faciale o lokalizacji pozatwarzowej.Po 6-miesięcznej terapii dapsonem i takrolimusem stosowanymmiejscowo wykwity ustąpiły, pozostawiając jedynie przebarwienia. Wnioski: W różnicowaniu przewlekłych zmian skórnych typu brunatnoczerwonychgrudek, guzków oraz wyniosłych nacieczonych ognisk,niezależnie od ich umiejscowienia, należy brać pod uwagę rozpoznaniepozatwarzowego ziarniniaka twarzy. W diagnostyce decydująceznaczenie ma badanie histopatologiczne. Korzystnym leczeniem jestogólne stosowanie dapsonu oraz miejscowo takrolimusu

Pseudoporphyria secondary to renal failure

Introduction . Pseudoporphyria is a rare disease associated with chronic renal failure. Symptoms of pseudoporphyria may develop in response to UV exposure and medications. The literature reports cases of pseudoporphyria in patients infected with hepatis C virus, HIV and undergoing dialysis therapy. Objective . Presentation of the case of a patient with pseudoporphyria and uraemic pruritus, and overview of therapeutic management. Case report . A 64-year-old male patient, who had been on dialysis for chronic renal failure secondary to type 2 diabetes for the past 11 years, presented with tense bullae located on sun-exposed skin which had persisted for the previous 5 months. The patient was diagnosed with pseudoporphyria, and treatment was prescribed including N-acetylcysteine, chloroquine, paroxetine and mianserin. An improvement in the patient’s clinical condition and a regression of pruritus were achieved. Conclusions . Patients with renal failure may develop symptoms of pseudoporphyria requiring differentiation from porphyria cutanea tarda

Mediators of Mast Cells in Bullous Pemphigoid and Dermatitis Herpetiformis

Bullous pemphigoid (BP) and dermatitis herpetiformis (DH) are skin diseases associated with inflammation. However, few findings exist concerning the role of mast cells in autoimmune blistering disease. Skin biopsies were taken from 27 BP and 14 DH patients, as well as 20 healthy individuals. Immunohistochemistry was used to identify the localization and mast cell expression of TNFα and MMP9 in skin lesions and perilesional skin. The serum concentrations of TNFα, MMP9, chymase, tryptase, PAF, and IL-4 were measured by immunoassay. TNFα and MMP9 expression in the epidermis and in inflammatory influxed cells in the dermis was detected in skin biopsies from patients. Although these mediators were found to be expressed in the perilesional skin of all patients, the level was much lower than that in lesional skin. Increased serum PAF levels were observed in BP patients. Mast cells may play an essential role in activating inflammation, which ultimately contributes to the tissue damage observed in BP and DH. Our findings suggest that differences in the pattern of cytokine expression directly contribute to variations in cellular infiltration in DH and BP