Use of granulocyte and monocyte adsorption apheresis in dermatology (Review).

Adsorptive granulocyte and monocyte apheresis (GMA) is an extracorporeal treatment that selectively removes activated myeloid lineage leukocytes from peripheral blood. This technique consists of a column with cellulose acetate beads as absorptive leukocytapheresis carriers, and was initially used to treat ulcerative colitis. A literature search was conducted to extract recently published studies about the clinical efficacy of GMA in patients with different skin disorders, reporting information on demographics, clinical symptoms, treatment and clinical course. Dermatological diseases, in which GMA has been performed, include generalized pustular psoriasis, pyoderma gangrenosum, palmoplantar pustular psoriasis, Behcet's disease, Sweet's syndrome, adult-onset Still's disease, impetigo herpetiformis, reactive arthritis, acne and hidradenitis suppurativa syndrome, cutaneous allergic vasculitis and systemic lupus erythematosus. In most patients, GMA was started after the failure of conventional therapeutic options and it was helpful in the majority of cases. Based on the information summarized, GMA could be considered a valid non-pharmacological treatment option for patients with several dermatological conditions, which are difficult to treat with other pharmacological preparations.

IgA nephropathy with leucocytoclastic vasculitis.

Leucocytoclastic vasculitis is a rare type of allergic disease caused by immune complexes. IgA nephropathy is a glomerulopathy characterized by recurrent episodes of gross haematuria or microscopic haematuria and IgA deposition in the glomerular mesangial region. IgA nephropathy complicating leucocytoclastic vasculitis is rare documented. We present a case of IgA nephropathy in a 47-year-old woman with leucocytoclastic vasculitis and discuss the clinical and pathological data, aiming to promote the diagnosis and treatment of this specific clinical manifestation.

A Case of Tuberculosis-related Leukocytoclastic Vasculitis Presenting With Peripheral Neuropathy.

Tuberculous granulomatous vasculitis is commonly associated with meningitis and retinitis. We describe a 39-year-old male, with a history of pulmonary tuberculosis (TB) who presented with progressive weakness, pain, tingling and numbness in the bilateral lower extremities. Significant atrophy and weakness of the lower extremities were evident along with absent reflexes. Nerve conduction studies and electromyography showed severe axonal polyneuropathy and denervation on the lower extremities. Nerve biopsy demonstrated small vessel leukocytoclastic vasculitis without any granuloma formation. Muscle biopsy was consistent with denervation and atrophy with target fiber changes. Tuberculosis-related vasculitis causing peripheral neuropathy is extremely rare and our case is unique in manifesting this presentation.

A phosphatidylinositol 3-kinase inhibitor strongly suppressed pulmonary vascular remodeling of allergic vasculitis in a murine model.

OBJECTIVES: We investigated the effects of pan-class I PI3K inhibitor, ZSTK474 on vascular remodeling using a murine model of allergic vasculitis with eosinophil infiltration. METHODS: C57BL/6 mice were sensitized with OVA. The positive controls were exposed to aerosolized OVA daily for 7 days. The other group of mice were administered ZSTK474 (30 mg/kg, p.o. daily) in parallel with daily exposure to aerosolized OVA for 7 days. On the 3rd and 7th day, bronchoalveolar lavage (BAL) was performed and the lungs were excised for pathological analysis. Cell differentials were determined and the concentrations of IL-4, IL-5, IL-13 and TGF-ßin BAL fluid were measured. RESULTS: The total cell numbers and eosinophil numbers in BALF were greatly reduced in the ZSTK474-treated group on the 3rd and 7th day after exposure to OVA. The numbers of total white blood cells and eosinophils in the peripheral blood were significantly reduced in the ZSTK474-treated group on the 3rd and 7th day after exposure to OVA. The concentrations of IL-4, IL-5, and IL-13 in BAL fluids were also reduced significantly on the 3rd day in the ZSTK474-treated group. The concentrations of TGF-ß in BAL fluids were also reduced significantly on the 3rd and 7th day in the ZSTK474-treated group. The pathological scores reduced significantly in the ZSTK474-treated group compared to the control group. CONCLUSION: The PI3K inhibitor, ZSTK474 suppressed pulmonary vascular remodeling in the murine model of allergic vasculitis with eosinophil infiltration. PI3K signal transduction may have a critical role in the immunological process that induces allergic vasculitis.

Síndrome de Churg-Strauss (vasculite sistêmica alérgica) / Syndrome of Churg-Strauss

Os autores apresentam uma revisão de literatura sobre a síndrome de Churg-Strauss, uma vasculite secundária de médios e pequenos vasos, de baixa incidência, que vem ganhando importância, principalmente depois de relatos de casos após uso de inibidor de leucotrienos, medicação indicada para tratamento de manutenção de asma moderada e grave, isoladamente ou em associação, portanto de uso cada vez mais frequente.Vasculites são entidades de relativa ocorrência na prática clínica, sendo as mais frequentes as secundárias ao uso de drogas (sulfas, aspirina, penicilinas etc.), aos agentes infecciosos (vírus da hepatite B e C etc.) e em menor número às doenças difusas do tecido conjuntivo (lúpus) e diagnosticá-las corretamente e em tempo hábil, para tratamento ou encaminhamento ao especialista, é primordial para o sucesso da terapêutica e prognóstico.A SCS é uma vasculite alérgica, sistêmica, com características próprias, passível de ser identificada e tratada, através de exames e medicamentos acessíveis. Apresenta uma forte associação com asma, que normalmente precede o acometimento de outros órgãos e costuma se manifestar após a suspensão de corticosteroide. Apresenta alteração característica à histopatologia, caracterizada por infiltrado perivascular de eosinófilos, bem como eosinofilia, normalmente acima de 10%, no hemograma. Costuma responder bem ao uso de corticosteroides, isolados ou em associação com outras drogas, como a ciclofosfamida nos casos refratários.

A Case of Allergic Vasculitis Associated with Chronic Active Hepatitis / 대한피부과학회지

Hepatitis B virus is of growing clinical importance. This virus not only produces acute and chronic liver disease but also produces a variety of dermatologic syndromes. A 77-year-old man with erythematous urticarial-like papulonodules had a chronic active hepatitis. And microscopic findings were compatible with allergic vasculitis. We reported a case of allergic vasculitis associated with chronic active hepatitis and reviewed literatures briefly.