Does a Subset of Localized Chronic Fibrosing Vasculitis Represent Cutaneous Manifestation of IgG4-Related Disease/a Histologic Pattern of IgG4-Related Skin Disease? A Reappraisal of an Enigmatic Pathologic Entity.

Localized chronic fibrosing vasculitis (LCFV) is a rare cutaneous fibroinflammatory and vasculitic process of poorly defined etiology. Furthermore, controversy remains as to whether LCFV represents a primary pathologic process or a histologic pattern. The current case documents a 52-year-old male patient with a scrotal mass and clinical history of a retroperitoneal mass as well as a previously resected tumor of the right submandibular salivary gland displaying morphologic features of eosinophilic angiocentric fibrosis. Histologic examination of the resected scrotal mass revealed a tumefactive lesion characterized by focally storiform fibrosis, obliterative phlebitis, tissue infiltration by IgG4-positive plasma cells, and leukocytoclastic vasculitis. Apart from the leukocytoclastic vasculitis, the scrotal lesion demonstrated characteristic morphologic features of an IgG4-related disease (IgG4-RD). In recognition of the combined histologic findings of both LCFV and IgG4-RD in the scrotal mass, it was postulated that a subset of LCFV cases might represent cutaneous manifestations of IgG4-RD or a new histologic pattern of IgG4-related skin disease (IgG4-RSD). The literature analysis of previously reported LCFV cases appeared to lend credence to this hypothesis. Pathologists should be aware of this new histologic pattern of IgG4-RSD as judicious consideration for additional studies might potentially detect an unexpected systemic IgG4-RD in the patient, particularly in cases of LCFV displaying storiform fibrosis associated with plasma cell infiltrate.

Primary Surgical Treatment of Erythema Elevatum Diutinum.

Erythema elevatum diutinum (EED) is a rare skin disease caused an Arthrus-type immunological reaction to antigen with immune complex deposition in the cutaneous microvasculature, which leads to tissue damage secondary to the effects of complement and leukocytes. It presents as brown or red cutaneous nodules, papules, or plaques, often on the extensor surfaces of the hands, knees, or elbows. Onset usually occurs in the fourth to sixth decades but possibly younger in patients with human immunodeficiency virus. Medical treatment is usually successful; however, surgical treatment can be used when chemotherapy fails. We present a case of a 29-year-old man with EED treated with excision and skin grafting.

Propylthiouracil-Associated Leukocytoclastic Necrotizing Cutaneous Vasculitis: A Case Report and Review of the Literature.

The purpose of this case report and review of the literature is to provide an exploration of the clinical symptoms, diagnosis, prevention, and management of propylthiouracil (PTU)-associated vasculitis in the intensive care setting. A PubMed search of the available literature was conducted using the MeSH search terms "propylthiouracil" and "vasculitis." The literature search returned 121 articles. Twenty-five were excluded because they were not in English. Fifty-nine case reports or case studies describing PTU-associated vasculitis were included. Data extracted from each case study included patient age, sex, autoimmune markers, laboratory tests, length of time on PTU, treatment for vasculitis, and patient outcomes. The authors reviewed 128 cases of PTU-associated vasculitis. The majority were women (8.8:1 F:M ratio), and the most common presenting symptoms were rash (51.6%), fever (46.9%), and arthralgia (43.8%). In addition to discontinuing PTU, the most common treatment was steroids (71.9%). Eight patients (6.3%) progressed to end-stage renal disease; two (1.6%) required intubation for respiratory failure; and five (3.9%) died of various organ systems failure related to vasculitis development. A high index of suspicion for vasculitis should be maintained, especially when presented with skin manifestations in the presence of PTU therapy. Screening with myeloperoxidase-antinuclear cytoplasmic antibodies is most sensitive. Positive screening should prompt a thorough clinical investigation. In cases of severe skin manifestations, the focus should be on aggressive wound care. Our case report is unique, not only in the size and extent of cutaneous involvement, but also as the first description of mortality secondary to cutaneous manifestations.

Leukocytoclastic vasculitis associated with perforated diverticular disease.

Leukocytoclastic vasculitis (LV) is a small vessel vasculitis associated with infections, connective tissue disease, malignancies and, rarely, gastrointestinal conditions. An association between LV and acute diverticulitis has not previously been reported. LV may be localised to the skin as a purpuric rash or might manifest with systemic involvement, such as of the joints, gastrointestinal tract or kidneys. Management of LV can be medical or surgical, based on the degree of systemic involvement. We present the case of a 56-year-old man with a 2-year history of a purpuric rash associated with diarrhoea, who presented acutely with abdominal pain. Imaging studies revealed sigmoid diverticulitis with a pericolic collection. Operative findings were purulent peritonitis secondary to perforated diverticular abscess, which was treated with a Hartmann's procedure. Postoperatively, the purpuric rash resolved rapidly on treatment with antibiotics and steroids. Histopathology of the resected bowel and skin punch biopsy confirmed sigmoid diverticulitis and LV, respectively.

Levamisole-induced necrosis of skin, soft tissue, and bone: case report and review of literature.

This represents the largest case of skin necrosis related to levamisole, a common cocaine contaminant, requiring closure with skin grafts, and is the only case resulting in nasal amputation, central upper lip excision, extremity bone necrosis, and above knee amputation. The case report is followed by a review of the literature. Unique considerations for the full-thickness necrosis induced by levamisole vasculitis are highlighted, including antibody level monitoring, need for multiple excisions, timing of skin grafting, and potential for soft tissue and bone necrosis as well. A 54-year-old man presented to an outside facility with fever, generalized weakness, and agranulocytosis, with a history of cocaine use 3 weeks before. After admission, he developed generalized violaceous lesions and an elevated p-antineutrophilic cytoplasmic antibody and was diagnosed with disseminated vasculitis and agranulocytosis secondary to levamisole-contaminated cocaine exposure. On transfer to the authors' facility, 52% TBSA was involved with violaceous, nonblanching lesions, which progressed to full-thickness necrosis. Local wound care continued until necrotic areas fully demarcated and progressive necrosis stabilized, and skin grafting for closure was not performed until antibody levels normalized. Current treatment of levamisole-induced skin rash or necrosis focuses on discontinuation of levamisole. As demonstrated by this case, extensive necrosis secondary to levamisole-induced vasculitis can be successfully treated with multiple excisions until necrosis stabilizes, and then, split-thickness autografts may be applied. In areas with poor vascular supply or areas with poor functional prognosis, amputation may ultimately be required.

Necrotizing peripheral vasculitis/vasculopathy following the use of cocaine laced with levamisole.

The objective of this study was to describe a novel presentation of peripheral vasculitis associated with levamisole-adulterated cocaine. Cocaine abuse is widespread in the United States with 5.3 million people using cocaine in 2008. Over the past decade, drug enforcement officials have noticed the presence of levamisole in confiscated cocaine samples as an adulterant. Known side effects of cocaine-related levamisole ingestion have included agranulocytosis and a cutaneous acral purpura that is histopathologically characterized by a mixture of inflammation (vasculitis) and occlusion (vasculopathy). A 54-year-old man who nasally ingested cocaine laced with levamisole developed widespread necrotic/purpuric skin lesions on approximately 20% of his body with an acral accentuation. These lesions were complicated by multiple areas of sloughing and necrosis. He was initially treated with topical silver sulfadiazine dressing changes but progressed to require debridement and split-thickness skin grafting. Peripheral vasculitis/vasculopathy with severe necrosis resembling Coumadin necrosis is a relatively recently recognized sequelae from levamisole-adulterated cocaine use.

Rupture of a pseudo aneurysm of the abdominal aorta in a patient with human immunodeficiency virus infection.

Human immunodeficiency virus (HIV) infection has an impact on all systems of the body, including the cardiovascular system. A 54-year-old man presented with abdominal pain. Enhanced computed tomography revealed rupture of a pseudoaneurysm of the abdominal aorta. After surgery, the patient tested positive for HIV. Histological examination of the resected aorta showed leukocytoclastic vasculitis, a characteristic feature of HIV-related vasculitis.

[Cutaneous leukocytoclastic vasculitis and renal carcinoma]. / Vasculitis leucocitoclástica cutánea y carcinoma renal.

The association between leukocytoclastic vasculitis and solid tumors like renal carcinoma has been infrequently described. We present the case of a 76-year-old woman who began with skin lesions on the legs that were clinically and histologically diagnosed as leukocytoclastic vasculitis. During analysis of the symptoms, she was diagnosed with renal carcinoma. After the tumor was excised, the vasculitis lesions disappeared, with no recurrence during the follow-up period.

Isolated colonic leukocytoclastic vasculitis causing segmental megacolon: report of a rare case.

We report the case of a 44-year-old white man who presented with progressively worsening crampy abdominal pain and distention. Deterioration of his clinical picture along with leukocytosis and radiographic evidence of severe colonic dilation rendered exploratory laparotomy necessary. Greatly distended and inflamed transverse and descending colon were evident and an extended left colectomy was performed. Characteristic changes of leukocytoclastic vasculitis in the serosal and muscular layers of the resected colon were demonstrated at histopathologic examination. Systemic leukocytoclastic vasculitis, usually coexisting with Henoch-Schonlein purpura, commonly affects the small bowel with clinical evidence of ischemia or bleeding. Colon involvement is infrequently reported in the context of systemic disease. Isolated colonic leukocytoclastic vasculitis without extraintestinal manifestations is rare. A previously unreported case of localized leukocytoclastic vasculitis of the left colon resulting in the impressive presentation of megacolon, without the presence of any precipitating factor or associated systemic disease is presented here, with an overview of the related literature.

Successful renal transplantation in a child with ANCA-associated microscopic polyangiitis.

Crescentic glomerulonephritis (CGN) is a clinicopathologic entity which is characterized by severe renal dysfunction of rapid onset with glomerular crescents. Type III CGN is associated with the absence of glomerular immune complex deposition (pauci-immune) and is associated with antineutrophil cytoplasmic antibody (ANCA). Microscopic polyangiitis and idiopathic pauci-immune necrotizing glomerulonephritis (NCGN) are strongly associated with ANCA directed against myeloperoxidase (anti-MPO). We describe here an unusual pediatric patient with MPO-ANCA-associated rapidly progressive glomerulonephritis (RPGN), emphasizing the management and outcome of the disease.

Laparoscopically assisted treatment of acute abdomen in systemic lupus erythematosus.

The incidence of abdominal pain in patients with systemic lupus erythematosus (SLE) is very high. Most patients do not require surgical treatment (serositis). Some cases such as appendicitis, perforated ulcer, cholecystitis or, rarely, intestinal infarction are surgical. Differential diagnosis is difficult, partly because noninvasive examinations do not provide enough evidence to rule out a diagnosis. On the other hand, in patients with SLE who have acute abdomen, it is dangerous to delay surgery by attempting conservative therapy. In fact, a better survival rate has been associated with early laparotomy. We report a case of acute abdomen in a patient affected by SLE, in which the diagnostic problem was solved by means of laparoscopy and the treatment was laparoscopically assisted. A 45-year-old woman with a 25-year history of SLE was admitted with abdominal pain and fever. Her physical examination revealed a painful right iliac fossa with rebound tenderness. Her WBC count was normal. Abdominal x-ray, ultrasonography, paracentesis, and peritoneal lavage did not provide a diagnosis. A diagnostic laparoscopy was performed, showing segmentary small bowel necrosis. The incision of the umbilical port site was enlarged to allow a small laparatomy, and a small bowel resection was performed. The histopathologic finding was "leucocytoclasic vasculitis, with infarction of the intestinal wall." The patient recovered uneventfully. In conclusion, this case report shows that emergency diagnostic laparoscopy is feasible and useful for acute abdomen in SLE. Currently, this diagnostic possibility could be considered the technique of choice in these cases, partly because, when necessary, it also can allow for mini-invasive treatment therapy.

Fournier's gangrene in hypersensitivity vasculitis.

Fournier's gangrene in IgE positive hypersensitivity vasculitis demands concurrent high dose immunosuppression, radical surgery and intensive treatment of septic sequelae. Even extended autologous skin grafting is possible with immunosuppression. A case of fulminant necrotizing infection of the genitalia, perineum and abdominal wall during the acute generalization phase of IgE positive hypersensitivity vasculitis is described. Apart from the radical surgical approach, medical treatment demands a different strategy than in classical Fournier's gangrene. The favorable outcome in a 21-year-old man after several months of intensive care could be realized only by close cooperation of internal specialists, urologists and plastic surgeons.