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cutaneous necrotizing vasculitis

Joshua Mandrell, Christina L Kranc
Levamisole is an immunomodulatory drug that was previously used to treat various medical conditions, including parasitic infections, nephrotic syndrome, and colorectal cancer. Over the last few years, increasing amounts of levamisole have been used as an adulterant in cocaine. Levamisole-cut cocaine has become a concern because it is known to cause a necrotizing purpuric rash, autoantibody production, and life-threatening leukopenia. Mixed histologic findings of vasculitis and thrombosis are characteristic of levamisole-induced purpura...
August 2016: Cutis; Cutaneous Medicine for the Practitioner
Lara El Khoury, Nabil Zeineddine, Richard Felix, Mark Goldstein
Levamisole is an antihelminthic drug banned by the US Food and Drug Administration (FDA) in 2000 because of its dangerous side effects. Over the past few years, it has been identified as an adulterant in cocaine and reported to cause cutaneous vasculitis in cocaine users. The health burden of levamisole is serious since it is estimated that over 5 million Americans use cocaine and that 70% of the cocaine used in the USA contains levamisole. In this paper we report the case of a 23-year-old female cocaine user that presented with purpuric rash and skin necrosis, found to have positive c-ANCA and anti-proteinase 3 antibodies...
2016: Case Reports in Rheumatology
Fatma Alibaz-Oner, Matthew J Koster, Cynthia S Crowson, Ashima Makol, Steven R Ytterberg, Carlo Salvarani, Eric L Matteson, Kenneth J Warrington
OBJECTIVES: Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis of medium-sized visceral vessels. However, cutaneous arteritis (CA) and gastrointestinal (GI) vasculitis are forms of single organ vasculitis having indistinguishable histopathological findings from PAN. The aim of this study was to evaluate and compare the clinical characteristics, treatment, and outcomes of patients with systemic PAN, CA and GI vasculitis. METHODS: Retrospective cohorts were assembled of patients with PAN, CA and GI vasculitis between 1980 and 2014...
August 26, 2016: Arthritis Care & Research
A Torrelo, L Noguera-Morel, A Hernández-Martín, D Clemente, J M Barja, L Buzón, D Azorín, A A de Jesús, J C López-Robledillo, I Colmenero, H Kutzner, R Goldbach-Mansky, L Requena
BACKGROUND: Recurrent panniculitis in children with lipoatrophy has been loosely described and reported under different names, but has never been systematically evaluated by immunohistochemical stains. OBJECTIVE: To depict the profile of children with recurrent idiopathic panniculitis. METHODS: Study of clinical, histopathological and immunohistochemical features in five cases with recurrent idiopathic panniculitis. RESULTS: Five children with repeated attacks of painful subcutaneous nodules in association with fever, malaise and abdominal pain or arthralgia, with subsequent lipoatrophy were reviewed...
August 12, 2016: Journal of the European Academy of Dermatology and Venereology: JEADV
Tatsuro Okano, Sora Takeuchi, Yoshinao Soma, Koya Suzuki, Sachiko Tsukita, Akihiro Ishizu, Kazuo Suzuki, Tamihiro Kawakami
We measured both serum anti-phosphatidylserine-prothrombin complex (anti-PSPT) antibodies and anti-moesin antibodies, as well as various cytokines (interleukin [IL]-2, IL-4, IL-5, IL-10, IL-13, IL-17, granulocyte macrophage colony-stimulating factor, γ-interferon, tumor necrosis factor-α) levels in polyarteritis nodosa (PAN) patients with cutaneous manifestations. All patients showed the presence of a histological necrotizing vasculitis in the skin specimen. They were treated with i.v. cyclophosphamide pulse therapy (IV-CY) and prednisolone therapy or steroid pulse therapy...
June 27, 2016: Journal of Dermatology
Toshitaka Kizawa, Yuko Yoto, Miyako Mizukami, Takeshi Tsugawa, Takako Takeuchi, Hotaka Kamasaki, Yasue Ishii-Osai, Toshiharu Yamashita, Kazushige Nagai, Tsukasa Hori, Hiroyuki Tsutsumi
Cutaneous polyarteritis nodosa (CPAN) is characterized by a necrotizing vasculitis of small and medium-sized arteries in the skin, which can be associated with fever, arthralgia, myalgia, and neuropathy, but, unlike polyarteritis nodosa (PAN), there is no visceral involvement. CPAN is rare in childhood. We report two siblings who developed CPAN during childhood. Interestingly, both had Mediterranean fever gene (MEFV) mutation, i.e. heterozygous E148Q. They also shared HLA-A24, -DR15 alleles. Simultaneous occurrence of MEFV mutation and HLA alleles with CPAN has never been reported in Japan...
June 14, 2016: Modern Rheumatology
Yi-Wei Justin Liu, Sangeeta Mutnuri, Sarah Batool Siddiqui, Geoff Richard Weikle, Olajumoke Oladipo, Niharika Ganti, Robert E Beach, Marjan Afrouzian
OBJECTIVES: The issue of levamisole-adulterated cocaine is emerging as a rapidly growing public health concern due to an increasing number of reports describing its role in cutaneous vasculitis and agranulocytosis. Of note, levamisole is recognized as a contaminant in 69% of the cocaine used within the United States. METHODS: We describe a patient who was a chronic cocaine user and developed systemic vasculitis characterized by polyarthralgia, bullous skin lesions, agranulocytosis, and antineutrophil cytoplasmic antibody-positive rapidly progressive glomerulonephritis...
May 2016: American Journal of Clinical Pathology
Najeeba Riyaz, Sarita Sasidharanpillai, Cherukattil Hazeena, Karumathil P Aravindan, Chettithodi Sivasankaran Bindu, Krishnakumari Nair Silpa
Recurrent cutaneous necrotizing eosinophilic vasculitis is a rare entity described by Chen et al. It has a benign course without any systemic involvement. However, often long-term treatment with systemic steroids is required. The exact etiopathogenesis remains unknown. We report a female patient, who presented with recurrent pruritic purpuric papules and plaques affecting different body parts of long duration. Disease was well controlled with low dose systemic steroids, but invariably recurred on its withdrawal...
March 2016: Indian Journal of Dermatology
G de Cambourg, A Mahé, S Banea, C Moulinas, G Blaison
INTRODUCTION: Granulomatosis with polyangiitis is a systemic and necrotizing vasculitis, and cutaneous involvement is uncommon. We report two cases of skin ulceration mimicking a pyoderma gangrenosum, and revealing granulomatosis with polyangiitis. CASE REPORTS: We report two patients who presented with atypical cutaneous ulcerations, with a chronic course. These wounds were large ulcerations with abrupt edges, with purulent and hemorrhagic exudates. The first hypothesis was a pyoderma gangrenosum, but the biopsies were not specific...
September 2016: La Revue de Médecine Interne
Hafiz Abdul Moiz Fakih, Emmanuel Elueze, Rajiv Vij
BACKGROUND: Cryoglobulinemia is a cold-reactive autoimmune disease. It is of distinctive importance in cardiac surgery because of the use of hypothermic cardiopulmonary bypass (CPB). Cryoglobulins, which activate at variable levels of hypothermia, can cause precipitation during surgery leading to possibly severe leukocytoclastic or necrotizing vasculitis, clinically manifested as ischemic events, such as cutaneous ulcerations, glomerulonephritis, arthritis, or peripheral neuropathies among the most reported associated comorbidities...
2016: Journal of Community Hospital Internal Medicine Perspectives
Armando De Virgilio, Antonio Greco, Giuseppe Magliulo, Andrea Gallo, Giovanni Ruoppolo, Michela Conte, Salvatore Martellucci, Marco de Vincentiis
Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis predominantly targeting medium-sized arteries. PAN is a rare form of vasculitis, and the precise frequency of this disease is difficult to determine. The major environmental factor associated with PAN is HBV infection. The pathogenesis of "idiopathic PAN" remains enigmatic, although the clinical responses to immunosuppressive therapy support the concept that immunological mechanisms play an active pathogenic role. The spectrum of disease ranges from involving a single organ to polyvisceral failure...
June 2016: Autoimmunity Reviews
Qing Huang, Yu-lan Liu
The male patient reported here presented as gangrene and central diabetes insipidus (CDI), who had characteristics of vasculitis. The patient complained about polydipsia and polyuria half a year ago, and then developed tingling, pain and blackish discoloration of some fingers and toes 3 month ago. He also had Raynaud's phenomenon. After admission, his laboratory examination showed the rise of erythrocyte sedimentation rate, C-reactive protein, immunoglobulin, β2-glycoprotein I and the activity of rheumatoid factors, lupus anticoagulant test...
December 18, 2015: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
Noreen Nasir, Syed Ahsan Ali, Hafiz Mohammed Mehmood Riaz
Background. Granulomatosis with polyangiitis (GPA) is an ANCA associated small vessel vasculitis characterized by necrotizing granulomatous inflammation involving the upper and the lower respiratory tract and the kidneys. The disease has a broad clinical spectrum that ranges from limited/localized involvement of a single organ system to a generalized systemic vasculitis that affects several organs with evidence of end organ damage. Atypical forms of the disease have been recognized with or without respiratory tract involvement with a long protracted course before manifesting as generalized disease...
2015: Case Reports in Rheumatology
Lan-Huong Thai, Saskia Ingen-Housz-Oro, Bertrand Godeau, Luc Rethers, Pierre Wolkenstein, Nicolas Limal, Virginie Papillon, Jean Kapfer, Olivier Chosidow, Nicolas Ortonne
Kikuchi-Fujimoto disease (KFD) is a rare and benign disorder that usually occurs in young adults with enlarged lymph nodes containing infiltrate of cytotoxic T cells and nuclear debris. It can be a manifestation of systemic lupus erythematosus (SLE) although the strength of this association has varied among studies. Although specific KFD cutaneous lesions are well described, pure cutaneous lesions have never been reported. We studied a series of patients prospectively entered into a database between 2007 and 2014 with skin biopsies showing diffuse or localized inflammatory infiltrates reminiscent of cutaneous KFD, without lymph-node-related KFD...
November 2015: Medicine (Baltimore)
María Alejandra Matteoda, Paola Cecilia Stefano, Marcela Bocián, María Marta Katsicas, Josefina Sala, Andrea Bettina Cervini
Polyarteritis nodosa is a rare vasculitis in children characterized by necrotizing inflammation in small and medium size arteries. It is classified into systemic and cutaneous PAN according to the presence of systemic symptoms or visceral involvement. We describe the case of a 14-year-old girl with cutaneous Polyarteritis nodosa with an atypical clinical presentation.
May 2015: Anais Brasileiros de Dermatologia
Alexandre Moretti de Lima, Pedro de Freitas Silva Torraca, Sheila Pereira da Rocha, Carmelia Matos Reis Santiago, Fabio Humberto Ribeiro Paes Ferraz
The granulomatosis with polyangiitis, initially known as Wegener's granulomatosis, is a small and medium vessels vasculitis. It's classic form presents a triad: necrotizing granuloma of respiratory tract, necrotizing cutaneous vasculitis and glomerulonephritis. This vasculitis has cytoplasmic antineutrophil antibodies as signal. This work illustrates a case, of multisystemic rare disease, in which the segment and treatment were considered satisfactory for symptoms remission.
May 2015: Anais Brasileiros de Dermatologia
B Grygiel-Górniak, M Puszczewicz
Granulomatosis with polyangiitis (GPA) is an autoimmune disease which has a variable clinical presentation and usually progresses from a localized to a generalized form over the course of weeks to years. Histopathologically, it is a necrotizing systematic vasculitis that can cause sino-nasal, pulmonary, renal, ocular, and cutaneous manifestations. Diagnostic workup should include serologic, radiologic, endoscopic and histopathological examination. Autoantibody c-ANCA may be used as a marker of disease activity and individual follow-up...
July 2015: European Review for Medical and Pharmacological Sciences
Tomohiro Terai, Mitsushige Sugimoto, Satoshi Osawa, Ken Sugimoto, Takahisa Furuta, Shigeru Kanaoka, Mutsuhiro Ikuma
Ulcerative colitis is occasionally complicated by dermatological disorders presenting as extra-intestinal manifestations, including erythema nodosum and pyoderma gangrenosum. Sweet's syndrome is considered to be a rare cutaneous disease in patients with ulcerative colitis. To date, only 17 cases of Sweet's syndrome complicating ulcerative colitis have been reported in the English literature. Here, we report a case of a 41-year-old male who had been suffering from ulcerative colitis for 20 years. He was admitted to hospital with hematochezia, diarrhea and fever, and painful erythematous nodules on the face and arms...
June 2011: Clinical Journal of Gastroenterology
Flauberto Sousa Marinho, Rodrigo Pirmez, Renata Nogueira, Tullia Cuzzi, Celso Tavares Sodré, Marcia Ramos-e-Silva, Marcia Silva
The authors report a case of sensorimotor polyneuropathy, diffuse cutaneous hyperpigmentation, skin sclerodermiform thickening and papular lesions in the infraclavicular and abdominal region. Besides weight loss, there were diabetes mellitus and hypothyroidism. The alterations were consistent with POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy and Skin changes) syndrome, which is a rare systemic disease with monoclonal proliferation of plasmacytes and slow progression. Cutaneous alterations are present in 68% of patients with diffuse cutaneous hyperpigmentation, plethora and acrocyanosis...
January 2015: Case Reports in Dermatology
Amber L Nolan, Kuang-Yu Jen
UNLABELLED: Rheumatic manifestations of cocaine have been well described, but more recently, a dramatic increase in the levamisole-adulterated cocaine supply in the United States has disclosed unique pathologic consequences that are distinct from pure cocaine use. Most notably, patients show skin lesions and renal dysfunction in the setting of extremely high perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA). Unexpectedly, antibodies to myeloperoxidase, the typical target of p-ANCA, are relatively low if at all present...
2015: Diagnostic Pathology
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