Nodular vasculitis associated with propylthiouracil therapy.

Cutaneous vasculitis is a rare complication of propylthiouracil therapy. We describe a patient who presented with lower extremity subcutaneous nodules, hemorrhagic bullae, and necrotic ulcers during treatment with this agent. Examination of a skin biopsy specimen revealed nodular vasculitis. This type of vasculitis has not been described in association with propylthiouracil therapy before. Nodular vasculitis not associated with the drug is characterized by a chronic protracted course, whereas in the patient reported the lesions rapidly regressed when use of the drug was discontinued.     

Systemic nodular panniculitis in a patient with alpha-1 antitrypsin deficiency (PiSS phenotype)

The association of alpha-1 antitrypsin deficiency (PiZ phenotype) with systemic nodular panniculitis has been well documented. Despite reports of cases of systemic nodular panniculitis associated with other alpha-1 antitrypsin deficiency phenotypes, it is still not known if this association is fortuitous rather than causal. We report a case of systemic nodular panniculitis associated with alpha-1 antitrypsin deficiency (PiSS phenotype), with clinicopathological features similar to those reported in cases associated with the PiZ phenotype.     

Leucocytoclastic vasculitis following nodular scabies

We describe the case of an 80-year-old man who came to our observation with a widespread form of nodular scabies. During therapy purpuric dermatitis developed and was diagnosed as leucocytoclastic vasculitis based on histological findings. Blood chemistry revealed raised levels of circulating immunocomplexes. We report this case because of the association of an unusual form of scabies with a complication only rarely associated with this disease.     

Red fingers syndrome and recurrent panniculitis in a patient with chronic hepatitis C

Cases of red fingers syndrome and nodular vasculitis in patients with hepatitis C have rarely been described. Both red fingers syndrome and nodular vasculitis are characterized by microscopic vasculitis, and it has been hypothesized that hepatitis C plays a role in the pathogenesis of cutaneous disorders such as vasculitis, especially in cryoglobulinaemic vasculitis. We describe the case of a 75-year-old woman diagnosed with chronic hepatitis C who presented with nodular lesions on her right thigh and red fingers syndrome. A skin biopsy taken from a nodule showed infiltration of the vessel walls by mononuclear cells and septal and lobular panniculitis. The diagnosis of nodular vasculitis was established. In our opinion, red fingers syndrome and nodular vasculitis might be related to a vascular reaction of immune-mediated mechanisms induced by hepatitis C.     

Nodular Vasculitis That Developed during Etanercept (Enbrel) Treatment in a Patient with Psoriasis

Nodular vasculitis was introduced by Montgomery for cases of erythema induratum-like lesions that were not associated with tuberculosis. Nodular vasculitis has been associated with both nontuberculous infections and noninfectious conditions. However, there has been no report on the development of nodular vasculitis during tumor necrosis factor-α inhibitor treatment. A 28-year-old man visited our clinic for the treatment of severe psoriasis with a 20-year history. Subcutaneous injection of etanercept (25 mg, twice weekly) was started. One year later, erythematous nodules developed on his lower leg. A skin biopsy showed lobular panniculitis with extensive necrosis and vasculitis. To exclude latent tuberculosis, an assay specific for Mycobacterium tuberculosis antigens was performed, with a negative result. After stopping etanercept under the diagnosis of nodular vasculitis associated with etanercept, the lesions gradually disappeared, leaving depressed scars in 3 months. There has been no recurrence after 6 months of follow-up.     

Antineutrophil cytoplasmic antibodies crescentic allograft glomerulonephritis after sofosbuvir therapy

Antineutrophil cytoplasmic antibodies (ANCA) are well known to be associated with several types of vasculitis, including pauci-immune crescentic glomerulonephritis, a form of rapid progressive glomerular nephritis (RPGN). ANCA vasculitis has also been reported after administration of propylthiouracil, hydralazine, cocaine (adulterated with levimasole), allopurinol, penicillamine and few other drugs. All previously reported cases of drug-associated ANCA glomerulonephritis were in native kidneys. Sofosbuvir is a new and effective drug for hepatitis C virus infection. Here, we report a case of ANCA vasculitis and RPGN following sofosbuvir administration in a kidney transplant recipient. It also represents the first case of drug-associated ANCA vasculitis in a transplanted kidney. Further drug monitoring is necessary to elucidate the degree of association and possible causal effect of sofosbuvir and perinuclear ANCA vasculitis.     

Hypocomplementemic urticarial vasculitis in mixed connective tissue disease

Urticarial vasculitis is characterized clinically by urticaria-like skin lesions and histologically by leukocytoclastic vasculitis. It may be idiopathic or associated with various conditions such as infections, hematologic disorders, drugs, and connective tissue diseases, primarily systemic lupus erythematosus; an association with mixed connective tissue disease (MCTD) has rarely been reported. We present a case of hypocomplementemic urticarial vasculitis in a patient with MCTD that responded to hydroxychloroquine after a period of corticosteroid dependence.     

Nodular Dermal Allergide of Gougerot

The authors present the clinical, histological and laboratory findings in a case of cutaneous necrotizing vasculitis which fits the picture of Gougerot's nodular dermal allergide. They discuss the classification of dermal vasculitis, and the reasons for regarding it as a hypersensitivity reaction.     

Livedo (livedoid) vasculitis and the factor V Leiden mutation: additional evidence for abnormal coagulation.

We report the case of a patient with livedo vasculitis associated with the factor V Leiden mutation. This association provides additional support for abnormalities of coagulation in patients with this disorder. The spectrum of platelet, coagulation, and fibrinolytic disorders reported with livedo vasculitis is reviewed.     

Cocaine-related Stevens-Johnson syndrome

We report the case of a 26-year-old student with erosive skin changes on his oral and genital mucosa and skin as well as on his thighs twice after cocaine snorting. The second of these two episodes was documented histologically, and other underlying causes of disease were excluded. A diagnosis of recurring Stevens-Johnson syndrome was made. Treatment was initiated with bed rest, systemic prednisone at 1 mg/kg body weight and liquid nutrition. Mucosal changes resolved within 4 days. On follow-up the patient remained free of symptoms while abstaining from the particular cocaine street mix in question. Cocaine has been involved in seizures, headaches, ischemic strokes, intracerebral hemorrhage, cerebral vasculitis, Churg-Strauss vasculitis, palpable purpura and Henoch-Sch?nlein vasculitis. Bullous skin disease in association with cocaine has been reported in 1 patient. To our knowledge, we report the first case of Stevens-Johnson syndrome associated with cocaine snorting.     

Granuloma necrosante extravascular de Churg-Strauss

—Cutaneous involvement occurs in twothirds of patients with allergic granulomatosis of Churg-Strauss (Churg-Strauss syndrome).We report the case of a 37-year-old woman with an extravascular necrotizing granuloma of Churg-Strauss associated to Churg-Strauss syndrome. Despite its characteristic clinical features, three biopsy especimens were needed to establish the diagnosis.Extravascular necrotizing granuloma of Churg-Strauss consists of a palisaded granuloma with basophilic degeneration of collagen, neutrophilic infiltration and leukocytoclastic debris. It was first reported as an especific histopathologic finding in Churg-Strauss syndrome, but it has also been described in association to other systemic vasculitis, connective tissue diseases and lymphoproliferative diseases.     

Cutaneous vasculitis,myelodysplasia and relapsing polychondritis

BACKGROUND: Relapsing polychondritis is a rare autoimmune disease, characterized by recurrent inflammation of cartilaginous tissues. In some cases, many other tissues can be involved. CASE REPORT: We describe the case of a 64 year-old man with relapsing polychondritis, whose first symptoms were papulonodular and mucosal aphthous lesions. The skin biopsy revealed vasculitis without leukocytoclasic features. The diagnostic of relapsing polychondritis was made only two years later, thanks to recurrent auricular chondritis. Since the beginning, the disease was associated with a myelodysplasia. DISCUSSION: Dermatological manifestations are noticed in less than 50 p. 100 of cases during the evolution of relapsing polychondritis. An association with a myelodysplasia has already been reported, and it could be a particular form of relapsing polychondritis. Myelodysplasic syndromes are more frequently associated with relapsing polychondritis than with other vasculitis. It must be sought especially when cutaneous and mucosal manifestations exist.     

Anaplastic Large Cell Lymphoma Masquerading as an Infectious Syndrome

BACKGROUND: Nodular vasculitis is a disorder that can be associated with several disease states, including infection, medications, malignancy, and collagen vascular disorders. The pathogenesis is unknown, but may be related to immune complex deposition, leading to subsequent vascular damage. METHODS: We report a case of nodular vasculitis occurring in a 40‐year‐old African‐American woman with end‐stage liver disease and a 3 month history of ulcerating lesions on her lower extremities. Although these lesions were both clinically and histologically consistent with erythema indratum, all studies directed at determining a mycobacterial etiology were negative. RESULTS: Biopsy of the skin lesions revealed a neutrophil‐predominant vasculitis in the setting of a lobular panniculitis. The histopathology of the liver biopsies, and the patient's clinical course were consistent with auto‐immune cholangitis. A course of oral prednisone was initiated, which resulted in improvement of the lower extremity lesions. CONCLUSION: In this case report we document a case of nodular vasculitis occurring in the setting of auto‐immune cholangitis. The pathogenesis of this condition remains obscure, however possible causes include immune complex deposition or a delayed‐type hypersensitivity reaction.     

Cryptococcus‐like changes in the setting of vasculitis

Cutaneous vasculitis has many underlying causes, and the clinical and histological findings often overlap. Inflammatory vasculitis can mimic infection; however, distinction is critical for the timely institution of appropriate therapy. We present two patients who had generalized polymorphous eruptions whose cutaneous pathology showed vasculitis with unusual haloed yeast‐like cells within the inflammatory infiltrate, mimicking Cryptococcus. The unusual cells stained negatively with Gomori methenamine silver and periodic acid‐Schiff fungal stains, but positively for CD68 and had cytoplasmic reactivity with antibody to myeloperoxidase (MPO). Both patients had positive serum anti‐MPO antibodies. The first patient experienced a rapidly fatal course, whereas the second patient improved with prompt initiation of systemic corticosteroids. Interestingly, the second case had prior biopsy showing Sweet syndrome with crypotoccoid‐appearing cells. Cryptococcoid cells have been described previously in association with neutrophilic dermatoses, but not in the setting of vasculitis as was seen in our patients. Our cases add to the existing literature on crypotoccoid mimickers, and are the first to be reported in association with vasculitis.     

E选择素和P选择素在结节性血管炎皮损中的表达

目的 探讨E选择素、P选择素在结节性血管炎中的意义。方法 分别检测70例结节性血管炎及24例正常皮肤组织中E选择素、P选择素的表达情况,比较两组间E选择素、P选择素表达的差异以及在结节性血管炎中两者之间的相互关系。结果 70例结节性血管炎E选择素表达均为阳性,以中度阳性（++）为主;24例正常皮肤组织均为阴性;两组比较,差异有统计学意义（P < 0.01）。70例结节性血管炎P选择素表达均为阳性,以强阳性（+++）为主;24例正常皮肤P选择素表达仅有2例弱阳性,其余为阴性,两组比较,差异有统计学意义（P < 0.01）。结节性血管炎中E选择素组与P选择素组比较,两者表达相关性好（P < 0.01）。对患者年龄、性别比较,E选择素、P选择素表达无统计学差异（P > 0.05）。结论 E选择素、P选择素的表达与结节性血管炎有一定的相关性,且在急性期两者相互关联。     

Malignant acanthosis nigricans and endometrioid adenocarcinoma of the parametrium: the search for malignancy

Malignant acanthosis nigricans is recognized as a cutaneous sign of internal malignancy, usually an adenocarcinoma. Although cases of malignant acanthosis nigricans have been associated with cervical, ovarian, and endometrial neoplasms, we describe a case with a rarely if ever reported association, endometrioid adenocarcinoma of the parametrium.     

Clofazimine-induced enteropathy in treatment-resistant nodular vasculitis

We report a chronic case of nodular vasculitis that responded to oral clofazimine 300 mg daily. The condition had previously responded to moderate dose oral prednisolone, 50 mg daily, but would recur with weaning. Multiple corticosteroid-sparing agents were trialled, however these were either ineffective or poorly tolerated. The introduction of clofazimine enabled prednisolone dose reduction, not achieved with other agents, to 22.5 mg daily, and was associated with complete suppression of disease activity. Unfortunately the patient developed a clofazimine-induced enteropathy and the treatment was ceased after almost 2 years of therapy. Cessation of clofazimine was associated with a flare of the condition. Clofazimine should be considered as a corticosteroid-sparing agent in resistant cases of nodular vasculitis. Clinicians should be aware of clofazimine-induced enteropathy as a potentially serious complication of the therapy.     

Nodular vasculitis - a novel cutaneous manifestation of autoimmune colitis

Panniculitis in association with autoimmune colitis (AC) is mostly confined to reports of erythema nodosum (EN) and Crohn's disease. We expand the repertoire of panniculitides associated with autoimmune bowel disease through a unique case report of nodular vasculitis (NV) that developed in parallel fashion to flares of ulcerative colitis (UC). A 51-year-old woman with a clinical history of surgically treated UC developed lesions on the lower legs that were initially diagnosed and treated as EN. Two years later, during a clinical exacerbation of UC, she developed similar lesions that on biopsy examination showed features consistent with NV. While treatment with antibiotics resulted in amelioration of both bowel symptoms and leg lesions, cessation of the same resulted in recurrence of both. This report implies an association between NV and UC.     

Coronavirus disease 2019-associated immunoglobulin A vasculitis/Henoch–Schönlein purpura: A case report and review

Immunoglobulin A (IgA) vasculitis or Henoch–Schönlein purpura is a predominantly pediatric disease occurring after a triggering viral or bacterial infection. Conversely, drug exposure is the most common inciting event in adult cases of IgA vasculitis. Recently, data has suggested a temporal association between coronavirus disease 2019 (COVID-19) and the development of IgA vasculitis in children and adults. Here, we describe a case of IgA vasculitis with nephritis in a 70-year-old man with COVID-19 and perform a comprehensive review of eight reported cases of suspected COVID-19-associated IgA vasculitis. When compared to classical IgA vasculitis, COVID-19-associated IgA vasculitis exclusively affects males (p < 0.00002) and is more common in adults (p < 0.005). Among cases of COVID-19-associated IgA vasculitis, adult cases were associated with significantly more arthralgia than pediatric cases (p = 0.04). In cases where skin biopsy was obtained, direct immunofluorescence (DIF) was negative for IgA in 50% of cases; thereafter, kidney biopsy DIF was positive for IgA in all cases. With this study, we provide support for an association between IgA vasculitis and severe acute respiratory syndrome coronavirus 2 infection and provide clinical information differentiating its manifestations from classical IgA vasculitis.     

Erythema induratum (nodular vasculitis) associated with Crohn's disease: a rare type of metastatic Crohn's disease

We herein report a patient with erythema induratum/nodular vasculitis (EI/NV) associated with Crohn's disease (CD), which is considered to be a rare type of metastatic CD. A 54-year-old woman, who had a history of CD, presented with erythematous nodules on her legs. The histopathological features of the skin biopsy revealed a granulomatous, mixed septal and lobular panniculitis, which was characterized by many discrete epithelioid cell granulomas (necrobiotic/necrotizing-type and sarcoidal type), necrosis of the adipocytes, and granulomatous phlebitis in the muscular wall of a subcutaneous vein. A review of the pertinent literature and the presented case suggested the following: (1) panniculitis associated with CD may be either an erythema nodosum type or an EI/NV type; (2) so far, the reported cases of metastatic CD or granulomatous vasculitis in CD rarely presented with granulomatous panniculitis without dermal involvement, and most cases showed histopathological features that were similar to or indistinguishable from those of EI/NV; and (3) the finding of granulomatous vasculitis (especially the presence of discrete epithelioid cell granulomas involving the veins or venules) may be a characteristic feature of EI/NV associated with CD, in contrast to the finding of acute vasculitis, which is typically present in patients with EI/NV due to causative factors other than CD.