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A young healthy gardener became febrile with abdominal pain, nausea, vomiting and diarrhoea followed by palpable purpura, mostly on the legs and buttocks with associated arthralgia. Dehydration, azotemia and hyponatraemia resolved with fluid replacement. Tests demonstrated acute pancreatitis, hepatitis, thrombocytopenia, microscopic haematuria and proteinuria. He improved with doxycycline, but bipedal pitting oedema and punctate rash involving the soles/hands appeared. Microbiological tests revealed positive IgM and IgG serology for rickettsiae spotted fever. Skin biopsy of the purpura confirmed leucocytoclastic vasculitis, positive for Rickettsiae conorii by PCR amplification. Palpable purpura is a rare important manifestation of Mediterranean spotted fever (MSF), due to either secondary leucocytoclastic vasculitis or associated Henoch-Schonlein purpura (HSP), which best explains the distribution of the rash, arthralgia, gastrointestinal symptoms, and microhaematuria not usually seen in R. conorii infections. Likewise, the patient’s acute pancreatitis may be interpreted as a rare presentation of HSP or a seldom-encountered feature of MSF.

Made available in DSpace on 2022-05-01T09:31:07Z (GMT). No. of bitstreams: 0 Previous issue date: 2021-01-01 Cutaneous involvement is one of the most common extraintestinal manifestations of inflammatory bowel disease (IBD). More commonly, pyoderma gangrenosum and erythema nodosum are noted, but psoriasis, aphthous stomatitis, Sweet's syndrome, and vasculitis may also occur. Leukocytoclastic vasculitis (LCV) is a rare cutaneous manifestation, characterized by the appearance of palpable purpura, urticaria, and ulcer-necrotic lesions predominantly in the lower extremities that improve with immunosuppressive therapy. In this case, we report a patient with CD and LCV. We also searched the literature on the diagnosis and treatment of LCV in patients with CD. Female, 31, presented with diarrhea containing mucus and blood, abdominal pain, arthralgia, and enanthematous plaques and ulcers with a hematinic background in the lower extremities. The results of the colonoscopy were compatible with CD and skin biopsy showed signs of LCV. Systemic autoimmune disease and primary vasculitis were ruled out. The patient received treatment with a systemic corticosteroid and the skin lesions improved. Outpatient treatment with antitumor necrosis factor therapy was initiated to promote skin healing and IBD clinical remission. As LCV is a rare manifestation of IBD, it is necessary to distinguish this dermatopathy from other systemic vasculitis. The engagement of a multidisciplinary team is essential for the correct diagnosis and management. São Paulo State University (Unesp) Medical School Clinica Universidad de Los Andes São Paulo State University (Unesp) Medical School

Rarely, patients with systemic lupus erythematosus (SLE) develop bullous eruptions, a disease called bullous SLE in a narrow sense that has autoantibodies against type VII collagen. We describe an unusual case in which a patient with SLE developed extensive bullae on her lower extremities. Histologically, the bullous lesions were suggestive of leukocytoclastic vasculitis with deposition of C3 within blood vessel walls. Immunoblot analyses and enzyme-linked immunosorbent assays were negative for anti-type VII collagen antibodies. We initially considered bullous SLE, but eventually made a diagnosis of secondary vasculitis in SLE. The oral prednisolone dose was increased, and the vesiculobullous lesions resolved. The clinical presentations of cutaneous vasculitis in SLE include palpable purpura, petechiae, papulonodular lesions, and livedo reticularis. Bullous lesions seem to be uncommon. Physicians need to be aware that extensive bullae can occur as a result of secondary vasculitis in SLE, even if the patient does not exhibit high disease activity.

Objective: Henoch Schönlein Purpura also known as IgA vasculitis is the most common form of pediatric vasculitis and renal involvement is responsible for the mortality and long-term morbidity. We aimed to describe the epidemiological, clinical, and laboratory characteristics of patients with IgAV and analyze the predicting factors associated with renal involvement. Materials and Methods: This study included188 children diagnosed with IgA vasculitis. Demographical, and clinical data were retrospectively reviewed from the patient files. Results: Of the 188 IgA vasculitis patients, 51.6% were female. The mean±SD age at diagnosis was 8.49±3.35 years, and 66% of them were diagnosed before 10 years of age. All the patients had palpable purpura, 35.6% had arthritis, 34.6% had gastrointestinal system involvement, 12.2% had renal disorders, at the time of diagnosis. Besides 23(12.2%) patients presented with renal involvement, 42(22%) patients developed renal involvement at follow-up. Patients under 10 years of age had significantly more arthritis, patients over 10 years of age had significantly more renal involvement. Among laboratory work-up, erythrocyte sedimentation rate levels were found significantly higher in patients with renal involvement. In multivariate analysis, the occurrence of renal involvement was not associated with any of the defined demographic and clinical characteristics of the disease. Although erythrocyte sedimentation rate levels showed a higher risk ratio, it has only borderline significance. Conclusion: Although IgA vasculitis is a self-limiting disease, renal involvement can cause serious complications. In the presented study, being older than 10 years of age and having high levels of erythrocyte sedimentation rate at the time of diagnosis could serve as a possible predictor of renal involvement.

What is known and objective Primary myelofibrosis (PMF) is characterized by myeloid cell proliferation and prominent bone marrow fibrosis. Ruxolitinib, a selective inhibitor of JAK 1 and 2, significantly reduces constitutional symptoms and spleen size compared with placebo, and has significant clinical benefits in patients with myelofibrosis. The most common haematological side effects are thrombocytopenia and anaemia, and the most common non-haematological side effects are grade 1-2 diarrhoea and pyrexia. Leukocytoclastic vasculitis is small vessel vasculitis, characterized histopathologically by immune complex-mediated vasculitis of the dermal capillaries and venules in the lower extremities, which can be seen as palpable purpura. Although the cause is 50% idiopathic, the aetiology of leukocytoclastic vasculitis can be collected under many headings. Case summary The case is here presented of a patient with PMF who developed leukocytoclastic vasculitis after ruxolitinib treatment. Ruxolitinib was discontinued as the lesions were thought to be drug-related and all skin lesions disappeared approximately 2 months after termination of the drug. When the ruxolitinib treatment was restarted at the same dose (2 × 15 mg), the skin lesions recurred. The drug dose was reduced to 1 × 15 mg, and the rashes disappeared. Currently, the patient has no active complaints and is being followed up with ruxolitinib 1 × 15 mg without any complications. What is new and conclusion To the best of our knowledge, leukocytoclastic vasculitis due to ruxolitinib is extremely uncommon. This case report can be considered to contribute to the literature of this rare event.

Approximately 20% of renal cell carcinoma (RCC) is diagnosed because of paraneoplastic manifestations. RCC has been associated with a large variety of paraneoplastic syndromes (PNS), but it is rarely associated with PNS vasculitis. We present a case of a previously healthy male who presented with systemic vasculitis; bitemporal headaches, diplopia, polyarthritis, palpable purpura, tongue lesion, peri-orbital edema, scleritis, chondritis and constitutional symptoms. He was subsequently found to have oligometastatic RCC. Both his primary lesion and site of oligometastasis were treated with stereotactic radiotherapy (SBRT) and resulted in the resolution of his vasculitis, as well as sustained oncologic response. This is the first case to demonstrate that effective sustained treatment for PNS vasculitis due to oligometastatic RCC is possible with SBRT.

Objectives Cutaneous IgM/IgG vasculitis (IgM/IgG V) is characterized by leukocytoclastic vasculitis histologically and by IgM‐ or IgG‐ perivascular deposition. However, clinical differences between IgM/IgG V and skin‐limited IgA vasculitis (IgA V) have not been fully clarified. In the present study, we assessed the clinical and histopathological differences between IgM/IgG V and IgA V. Methods We examined 14 patients with IgM/IgG V and 24 patients with skin‐limited IgA V. The cases associated with systemic diseases were excluded. Clinical findings, laboratory data, and histological findings were compared between IgM/IgG V and IgA V groups. Results Livedo lesions appeared more often, and palpable purpura less often in IgM/IgG V. Localization of the lesions was more limited to the lower legs in IgM/IgG V. In laboratory data, there were no statistical differences in peripheral blood cell counts, IgG, IgA, and IgM levels between the two groups. Histopathological findings showed less infiltration of neutrophils, CD8+ T cells, and CD3+ T cells, and nuclear dusts in IgM/IgG V. Systemic steroids were administered significantly less frequently in IgM/IgG V cases, but the recurrence rate did not differ. Conclusions Based on these results, IgM/IgG V may be a clinically and histologically different entity from IgA V. However, further studies are necessary to define the differences between them more clearly.

Background Purpura in inpatients commonly leads to dermatologic consultation. The differential diagnosis is broad and algorithms are intricate. Objective We evaluated inpatient consultations for complex purpura to document the most common diagnoses and to validate the true diagnostic utility of histopathology, clinical morphology, and distribution. Methods We reviewed a case series of 68 inpatients during a 4-year period with a dermatologic consultation for purpura and biopsy findings of vasculitis or microvascular occlusion. Results Key features of complex purpura are nonbranching (round) versus branching (retiform) morphology, dependent versus acral or generalized distribution, and leukocytoclastic vasculitis versus microvascular occlusion (with emphasis on depth of involvement). Dependent nonbranching purpura with only superficial vessels involved by leukocytoclastic vasculitis was most often due to IgA vasculitis or cutaneous single-organ small-vessel vasculitis. In contrast, deeper involvement by leukocytoclastic vasculitis was suggestive of systemic disease (eg, antineutrophil cytoplasmic antibody–associated vasculitis). Branching purpura was concerning, with greater than 90% sensitivity and specificity for microvascular occlusion and associated high mortality (≈50%). The majority of patients who died had acral branching lesions. Limitations Small sample size, inpatients at a tertiary care center, and retrospective nature are some limitations. Conclusion Nonbranching dependent purpura corresponded to leukocytoclastic vasculitis, with the most common diagnoses being IgA vasculitis or skin-limited small-vessel vasculitis; patients with deep involvement often had systemic diseases. In this series, branching purpura was due to microvascular occlusion rather than medium-vessel vasculitis, and had associated high mortality.

Haemorrhagic bullous form of IgA vasculitis (IgAV), or Schonlein-Henoch purpura, is an unusual presentation of the disease in paediatric patients (

espanolLa forma hemorragico-ampollosa de la vasculitis IgA (VIgA) o purpura de Schonlein-Henoch, es una de las presentaciones menos frecuentes de la enfermedad en pacientes pediatricos ( Se presenta el caso de una nina de 5 anos que resulta muy representativo. Fue diagnosticada de VIgA hemorragico-ampollosa, desarrollando lesiones ampollosas 4 dias despues de la aparicion de las lesiones purpuricas. En ningun momento preciso tratamiento antiinflamatorio ni inmunosupresor, resolviendose las lesiones 14 dias despues, sin complicaciones significativas. EnglishHaemorrhagic bullous form of IgA vasculitis (IgAV), or Schonlein-Henoch purpura, is an unusual presentation of the disease in paediatric patients ( We present the case of a 5-year-old girl that is very representative. She developed palpable purpura and four days later the skin lesions evolved into blistering lesions. She did not receive any anti-inflammatory nor immunosuppressive treatment and the lesions spontaneously subsided within 14 days. She did not develop any extracutaneous nor systemic involvement.

Hepatitis C-induced mixed cryoglobulinemia leading to rapidly progressive gangrene, necessitating amputations, is a rare presentation. We describe a case of a 55-year-old man with untreated chronic hepatitis C virus (HCV) presenting with arthralgia and palpable purpura, which rapidly progressed to life-threatening gangrene of all extremities requiring amputations in the setting of mixed cryoglobulinemia. Treatment for HCV was initiated which led to the arrest of gangrene progression and the patient's survival. Patients with HCV-induced cryoglobulinemia should be closely monitored and started on early therapy with direct-acting antiviral therapy to prevent progression of vasculitis to gangrene. Universal screening for HCV can aid in early diagnosis and treatment to prevent devastating consequences.

Leukocytoclastic vasculitis (LCV) is a histopathologic description of a common form of small vessel vasculitis (SVV), that can be found in various types of vasculitis affecting the skin and internal organs. The leading clinical presentation of LCV is palpable purpura and the diagnosis relies on histopathological examination, in which the inflammatory infiltrate is composed of neutrophils with fibrinoid necrosis and disintegration of nuclei into fragments (“leukocytoclasia”). Several medications can cause LCV, as well as infections, or malignancy. Among systemic diseases, the most frequently associated with LCV are ANCA-associated vasculitides, connective tissue diseases, cryoglobulinemic vasculitis, IgA vasculitis (formerly known as Henoch–Schonlein purpura) and hypocomplementemic urticarial vasculitis (HUV). When LCV is suspected, an extensive workout is usually necessary to determine whether the process is skin-limited, or expression of a systemic vasculitis or disease. A comprehensive history and detailed physical examination must be performed; platelet count, renal function and urinalysis, serological tests for hepatitis B and C viruses, autoantibodies (anti-nuclear antibodies and anti-neutrophil cytoplasmic antibodies), complement fractions and IgA staining in biopsy specimens are part of the usual workout of LCV. The treatment is mainly focused on symptom management, based on rest (avoiding standing or walking), low dose corticosteroids, colchicine or different unproven therapies, if skin-limited. When a medication is the cause, the prognosis is favorable and the discontinuation of the culprit drug is usually resolutive. Conversely, when a systemic vasculitis is the cause of LCV, higher doses of corticosteroids or immunosuppressive agents are required, according to the severity of organ involvement and the underlying associated disease.

This study aims to analyze the clinical characteristics of juvenile primary Sjogren’s syndrome (pSS) with cutaneous involvement. We investigated the clinical and immunological characteristics of 37 children with pSS. All the patients met the 2012 American College of Rheumatology Classification Criteria for Sjogren’s syndrome. The cutaneous involvement presented in 19 children (excluding drug eruption), 16 (84.2%) female patients, and 3 (15.8%) male patients, with a mean age of 11 ± 2.68 years, 17 of whom (89.5%) had cutaneous lesions as the first symptom, with a median time of 12 months (1 day to 4 years) before the diagnosis of pSS. The cutaneous lesions included 12 cases of palpable purpura (63.2%), 5 cases of urticaria (26.3%), 2 cases of xeroderma (10.5%), 1 case of skin ulcer, 1 case of erythema nodosum, 1 case of livedo reticularis, 1 case of Raynaud’s phenomenon, and 1 case of hard erythema. Children with cutaneous lesions had a higher prevalence of articular involvement (42.1% vs. 11.1%, P = 0.016), fever (47.4% vs. 5.6%, P = 0.004), ESR > 50 mm/h (47.4% vs. 11.1%, P = 0.016), and a lower prevalence of thrombocytopenia (0% vs. 27.8%, P = 0.013) and methylprednisolone pulse treatment (0% vs. 13.5%, P = 0.013), compared with pSS without cutaneous involvement. More than half (51.3%) of the children with juvenile pSS presented with cutaneous lesions; the main cutaneous involvement was palpable purpura. Children with cutaneous lesions were more likely to have fever and arthritis, were more likely to have stronger inflammatory response, and were less likely to have serious complications. In many cases, the cutaneous lesion could be the first symptom of juvenile pSS, which could easily lead to a misdiagnosis. The possibility of pSS should be considered for children with skin lesions such as palpable purpura and urticaria, and further examinations should be carried out.

Immunoglobulin A (IgA) vasculitis is characterized by small vessel vasculitis involving immune complexes and IgA deposition. The development of heparin-induced thrombocytopenia (HIT) during IgA vasculitis is extremely rare. An 87-year-old man presented with general fatigue, leg edema, purpura, arthritis and renal disease. He was diagnosed with IgA vasculitis and was admitted to our hospital. Hemodialysis with heparin was initiated thrice a week on post-admission Day 11. On Day 21, during hemodialysis, the pressure in the dialysis circuit increased and the dialysis was interrupted. On Day 24, the platelet count rapidly decreased to 18 000/μl. The patient was diagnosed with HIT after testing positive for HIT antibodies; heparin was discontinued at this time. Precautions must be taken against the onset of HIT when initiating hemodialysis in patients with IgA vasculitis.

Henoch-Schonlein Purpura (HSP) is the most common childhood systemic vasculitis. It is characterized by leucocytoclastic vasculitis and IgA deposition in the skin, joints, gastrointestinal tract, and kidneys, and typically manifests with purpura, arthritis/arthralgia, abdominal pain and nephritis. About 70% of children with HSP develop abdominal pain from submucosal haemorrhage and bowel wall oedema. These may result in gastrointestinal complications such as intussusception and may impact adversely on outcome. We report a case of HSP in a 7-year-old female who had presented with a recurring course of palpable purpura restricted to both lower limbs, painful swelling of the right knee joint, as well as severe abdominal pains which later became associated with passage of bloody stools. She subsequently had an exploratory laparotomy for sonographically confirmed intussusception following which she was referred to the haematology clinic on account of persistence of purpuric limb lesions post-surgery. This report highlights HSP as the commonest childhood systemic vasculitis. Its prognosis in childhood is generally excellent, with a self-limited course in most cases. Gastrointestinal complications such as intussusception, may, however, impact adversely on outcomes, while renal involvement may progress to chronic kidney disease.

Background: Henoch-Schonlein Purpura (HSP), or IgA vasculitis, is the most common vasculitis in children and is characterized by four symptoms: palpable purpura with no concurrent thrombocytopenia or coagulopathy, arthritis/ arthralgias, abdominal pain, and renal disease. HSP does occur in older patients at a much lower frequency. Objective: To discuss the atypical presentation of HSP in an elderly adult and the increased nephrogenic risks that are involved in this patient group. Case Presentation: We present the history, physical findings, laboratory results, and the clinical course of a 74-year-old Caucasian male with an atypical presentation of HSP. Our patient presented with severe gastrointestinal and cutaneous symptoms which occurred after the onset of abdominal pain. Biopsies demonstrated leukocytoclastic vasculitis in the skin and acute inflammation and hemorrhage in the gastrointestinal tract. The diagnosis was confirmed as IgA vasculitis following the renal biopsy. The patient’s symptoms improved with the administration of methylprednisolone. Conclusion: In adults with HSP there is greater concern regarding renal involvement and a greater risk of developing end-stage renal disease compared to children. The importance of recognizing HSP in the elderly is imperative for early diagnosis and appropriate treatment to limit renal damage.

Leukocytoclastic vasculitis (LCV) is a small-vessel vasculitis that presents with palpable purpura. The most common identified triggers of LCV are infection or exposure to a new medication. Herpes zoster (HZ) occurs as a result of the reactivation of the varicella-zoster virus (VZV). VZV is able to infect endothelial cells directly, producing a spectrum of vasculitides, including that of large and medium vessels. We report a case of a patient with synchronous HZ infection and segmental small-vessel vasculitis.

Polyarteritis nodosa (PAN) belongs to the group of medium vessel vasculitis. Two subtypes of the disease are classically distinguished — systemic variant and cutaneous variant. Systemic PAN is a potentially life-threatening disease, while cutaneous subtype is usually characterized by mild course. The review aims to summarize the symptomatology, diagnostics and treatment of PAN and to draw attention to the dermatological manifestations of the disease. Skin changes develop in 28–60% of patients with systemic PAN. The most common manifestations include palpable purpura, livedo reticularis and inflammatory nodules, and less commonly - urticarial lesions, transient erythema, distal necrosis, superficial phlebitis and splinter haemorrhages. Skin lesions in cutaneous PAN are typically located on the lower extremities, and less frequently — on the upper extremities or trunk. Painful nodules, livedo reticularis and ulcerations are the most frequent manifestations of cutaneous PAN. Ulcerations usually heal leaving ivory-white stellate scars ( atrophie blanche ) surrounded by telangiectasias. The diagnosis of both systemic and cutaneous subtype of the disease is based on diagnostic criteria. Management of systemic PAN consists of induction of remission and maintenance therapy. The choice of therapeutic option should be based on the severity of the disease and comorbidities, especially hepatitis B virus infection. Cutaneous PAN is characterized by a benign course with frequent relapses. First-line treatment usually consists of non-steroidal anti-inflammatory drugs, colchicine and dapsone. Considering frequent development of skin lesions in the course of PAN, knowledge of the clinical presentation, diagnosis and treatment of the condition is an important part of the everyday dermatological practice.

We present the case of a 17-year-old male patient with ulcerative colitis (UC) under infliximab therapy and a family history of IgA vasculitis (IgAV). During a maintenance infliximab session, he developed palpable purpura, abdominal pain, and hematochezia. Computed tomography and endoscopy revealed findings compatible with gastrointestinal manifestations of IgAV. He was successfully treated by intravenous prednisolone and did not develop recurrence of IgAV during subsequent infliximab infusions.

Immunoglobulin A vasculitis (IgAV) is a type of vasculitis affecting small vessels with deposition of immune complexes consisting of IgA and complement component 3. IgAV involves the skin, gastrointestinal (GI) tract, joints, and kidneys. Adult patients have higher risks of gastrointestinal tract involvement than children. To investigate the risk factors of the GI tract involvements in adult IgAV patients, we enrolled 29 adult (aged ≥ 20 years) Japanese patients recently (from 2013 to 2019) histopathologically diagnosed with IgAV and classified them into the GI lesion (+) group and the GI lesion (−) group. All patients presented with purpura on the lower extremities; moreover, GI lesion (+) patients presented significantly more with extensive purpura on the upper extremities, and low levels of factor XIII activity (≤70%) than gastrointestinal lesion (−) patients (87.5% vs 28.6% [P = .004]; odds ratio [OR], 17.5; 95% confidence interval [CI], 2.4 to 366], and 57.1% vs 14.3% [P = .04]; OR, 8; 95% CI, 1.06 to 83.9, respectively). There was no significant difference between the two groups in the populations with extensive purpura on the trunk, arthralgia, hematuria, proteinuria, or elevated serum levels of C‐reactive protein or IgA. Widespread purpura on the upper extremities accompanied by a low factor XIII activity is a suggestive factor for severe GI lesions in adult IgAV patients.

Polyarteritis nodosa (PAN) is a necrotizing vasculitis. The clinical manifestations are determined by the location of the compromised arteries. Cutaneous PAN can present as nodular lesions similar to erythema nodosum, palpable purpura, livedo reticularis, and ulceration. It often affects the lower limbs but other anatomical sites can also be involved. However, concomitant facial edema is an extremely rare manifestation. It has been more than 20 years since the last case report describing this unusual presentation of PAN. Furthermore, our patient is the first case presenting with hemifacial edema fluctuating every second or third day due to PAN confirmed by skin biopsy.

Immunoglobulin A (IgA)-mediated leukocytoclastic vasculitis is a cutaneous small-vessel vasculitis characterized by skin findings of palpable purpura. It may occur secondary to infections, neoplasms, drugs, and systemic conditions, although it is most commonly idiopathic. A known, but rare, trigger for IgA vasculitis is alcohol consumption. We present a case of a man with IgA vasculitis associated with alcohol use and review the literature on alcohol-associated vasculitis. Although rarely reported, alcohol-associated IgA vasculitis is an important entity to consider for appropriate diagnosis and management of such patients.

We report a case of polycythaemia vera (PV) associated with IgA vasculitis. A 45-year-old man was admitted for evaluation of abdominal pain and palpable purpura. IgA vasculitis was diagnosed, and oral prednisolone therapy (30 mg/day) was initiated. On day 6, the patient developed left hemiparesis, and magnetic resonance imaging revealed acute cerebral infarction. Bone marrow biopsy results and the identification of a Janus kinase 2 (

IgA vasculitis, also called Henoch-Schonlein purpura (HSP), is a form of cutaneous small-vessel vasculitis characterized by palpable purpura favoring the lower extremities, arthritis, nephritis, and IgA deposition within postcapillary venules of the skin and mesangium. In children, respiratory infections are well known to precede HSP. In adults, infectious endocarditis is a rare but potentially fatal trigger for HSP. We report the subtle presentations of subacute endocarditis in 2 adults with cutaneous IgA vasculitis.

Leukocytoclastic vasculitis (LCV) is a small-vessel vasculitis that most commonly affects the postcapillary venules in the skin. It classically presents with purpuric macules that progress to palpable purpura on the bilateral shins 7–10 days after an inciting medication or infection, or in the setting of connective tissue disease, malignancy, or inflammatory bowel disease. Up to 50% of cases have no identifiable cause. Lesions on the buttocks, abdomen, upper extremities, and face are uncommon, as are bullae and ulcers. We present a rare case of bullous LCV manifesting as grouped vesicles on the face and body mimicking varicella-zoster infection.

Cryoglobulins are immunoglobulins that undergo reversible precipitation at cold temperatures. Monoclonal type-I cryoglobulinaemia is the least frequent and is associated to hematological diseases such as multiple myeloma, Waldenström’s macroglobulinaemia, chronic lymphocytic leukaemia and lymphoma. We describe the case of a 60-year-old female patient, who suffered from burning pain in her feet for ten months before her admission. The patient presented intermittent distal cyanosis that progressed to digital ischaemia. She also reported paresthesia in her hands, difficulty in writing, and a 26-kg-weight loss. At the physical examination, it was identified livedo reticularis, palpable purpura, and painful ecchymotic lesions in her calves and feet. Moreover, peripheral pulses were palpable and symmetrical. It was observed an atrophy of the right first dorsal interosseous and both extensor digitorum brevis, as well as a distal bilateral apalesthesia and allodynia. Both Achilles reflexes were absent. Laboratory tests revealed anemia, high erythrosedimentation rate and C-reactive protein. Serum protein electrophoresis showed a monoclonal IgG-Kappa gammopathy. The results also evidenced the presence of Bence-Jones proteinuria. The bone marrow biopsy revealed less than 10% of plasma cells, and skin biopsy informed leukocytoclastic vasculitis. The patient was treated with high-dose intravenous steroids and cyclophosphamide. The treatment showed that the skin lesions had improved, pain disappeared and motor deficit stopped its progression.

Henoch-Schönlein purpura (HSP) is a small vessel systemic vasculitis. Typical symptoms include palpable purpura, joint pain, and abdominal pain. Most cases improve after a few weeks, not requiring any treatments other than symptom control. Acute abdomen resulting from vasculitis is very rare and should be treated as a surgical emergency. We report the case of a 9-year-old boy with acute gangrenous appendicitis as the first manifestation of HSP.

Immunoglobin A (IgA) vasculitis (IgAV), formerly called the Henoch-Schönlein purpura (HSP), is a small vessel vasculitis, characterized by IgA1-dominant immune deposition at diseased vessel walls. IgAV is the most common form of vasculitis in children; typical symptoms include palpable purpura, arthritis or arthralgia, abdominal pain, and hematuria or proteinuria. Galactose-deficient IgA1 is detected in the tissues of the kidney and skin in patients with IgAV; it forms immune complexes leading to subsequent immune reactions and injuries. This report provides the recent advances in the understanding of environmental factors, genetics, abnormal innate and acquired immunity, and the role of galactose-deficient IgA1 immunocomplexes in the pathogenesis of IgAV.

A 23-year-old man presented to the acute assessment unit with acute-onset haematuria within 24 hours of receiving his second dose of the Pfizer-BioNTech COVID-19 vaccine. He had been diagnosed with IgA vasculitis 8 months previously. IgA vasculitis is an autoimmune condition characterised by palpable purpura affecting the lower limbs, abdominal pain, arthralgia and renal disease. He was diagnosed with an acute exacerbation of IgA vasculitis and was discharged with oral prednisolone. Reactivation or first presentation of IgA vasculitis is a rare but increasingly recognised complication of COVID-19 vaccination. This is an important new differential in the assessment of patients with haematuria following COVID-19 vaccination.

Small vessel vasculitis with anti-proteinase antibodies 3 is an atypical clinical presentation of tuberculosis. The authors present the case of a 47-year-old male patient, with palpable purpura and palmoplantar hemorrhagic blisters, with subsequent dissemination. He presented severe pulmonary symptoms with cavitation, fever, hemoptysis, and high levels of anti-proteinase 3. Histopathological assessment of the skin revealed small vessel vasculitis; pulmonary histopathology showed granulomas with caseation. Bronchoalveolar lavage was positive for alcohol-acid-fast bacilli. In countries with a high prevalence of tuberculosis, the presence of autoantibodies in a patient with vasculitis, fever, and pulmonary cavitation requires investigation of infectious causes.

Henoch-Schonlein purpura (HSP), also known as immunoglobulin A (IgA) vasculitis, is a small-vessel vasculitis characterized by IgA deposits in various organs in the body producing a unique constellation of symptoms. This disease predominantly affects the skin (palpable purpura), joints (arthritis/arthralgia), gut (abdominal pain), and kidneys (nephritic syndrome-IgA nephropathy [IgAN]). The pathogenesis of HSP in children is usually secondary to an immune reaction after viral infections. In adults, few cases of HSP/IgA vasculitis have been reported secondary to altered metabolism of IgA in patients with alcoholic liver cirrhosis. Here, we report an unusual case of HSP/IgA vasculitis. The patient presented with signs of alcoholic liver cirrhosis with abdominal pain and ascites along with a lower extremity purpuric rash. The patient had significant findings of liver cirrhosis with radiographic evidence of cirrhotic liver with esophageal varices and splenorenal shunt and elevated serum ascites albumin gradient. Urinalysis revealed proteinuria with microscopic hematuria, further evaluated with a kidney biopsy. Microscopic analysis revealed focal segmental endocapillary and extracapillary proliferative glomerulonephritis with focal necrotizing features, consistent with IgAN/HSP nephritis. Treatment was initiated with high-dose steroids and cyclophosphamide infusions. Alcohol-induced endotoxin release and inflammation lead to high amounts of circulating IgA due to increased intestinal permeability and reduced hepatic clearance. Further disease development is caused by IgA deposits in affected organs (skin and kidney in our case). We hypothesize that the development of disease for the patient was secondary to altered IgA processing in decompensated alcoholic cirrhosis.

We present a case of diffuse skin immune reaction, diagnosed as cutaneous small-vessel vasculitis, following assessment of tubal patency by contrast ultrasound, which appears to be the first reported case of hypersensitivity reaction to sonographic tubal patency testing, based on a literature search. A 32-year-old woman presented with non-thrombocytopenic palpable purpura the day after assessment of tubal patency by two-/three-dimensional hysterosalpingo-foam sonography (HyFoSy) using ExEm® Foam. During real-time ultrasound, the observer identified flow in only the right tube when using saline with air as contrast medium; however, the same observer identified flow in both tubes after injecting ExEm Foam and the woman left the clinic without any complications. The next day, the patient was admitted with a complaint of a red-purple skin rash noticed the same morning, associated with moderate leg pain. Slow-motion analysis of the recorded videos and three-dimensional ultrasound datasets showed previously unnoticed venous intravasation of ExEm Foam into the myometrial vessels. Palpable purpura is typically found in vasculitis as a result of extravasation of red cells outside the inflamed blood vessel. This previously unreported side effect of tubal patency testing by HyFoSy, its potential rare organ consequences, as well as unknown consequences of venous intravasation by foam, should be included in the informed consent prior to the examination. Copyright © 2019 ISUOG. Published by John Wiley & Sons Ltd.

Background: The incidence of Henoch-Schonlein purpura (HSP), the most common systemic vasculitis in children, is steadily increasing. It is an immunoglobulin A (IgA) mediated systemic small-vessel vasculitis. HSP is now referred to as IgA vasculitis as per the Chapel Hill Consensus Conference on the Nomenclature of Systemic Vasculitides in 2012. Objective: The objective of the study was to study the clinical profile, pattern of organ involvement, treatment modalities, and outcomes of HSP. Materials and Methods: A retrospective study was done from January 2016 to June 2018 using the medical records of our institute. During the study period, 27 children were diagnosed with HSP. The age, gender, clinical presentation, and laboratory data including complete blood count, C-Reactive protein, erythrocyte sedimentation rate, and complete urinalysis, USG abdomen, and recurrence were studied. Results: The ratio of male:female was 3:1. Palpable purpura was present in all the patients and was the initial presentation reported in most of the cases. Arthritis was seen in 44% and abdominal pain in 66% of the cases. Renal manifestations in the form of hematuria were reported in 22% and proteinuria in 11% of the patients. Recovery with conservativemanagement was seen in two patients, whereas others required steroids. Conclusion: HSP is a common vasculitis seen in childhood and it might have the risks for various complications.

Cryoglobulinemia is a condition in which circulating cryoprecipitate immune complexes are detected in serum. The cryoglobulin concentration above 50 mg/l is considered diagnostically significant for the statement of cryoglobulinemia. The production of cryoglobulins, as a rule, is a consequence of the underlying disease, which requires etiological evaluation. The diagnosis of cryoglobulinemic vasculitis (CV) is based on laboratory detection of serum cryoglobulinemia in combination with characteristic clinical signs and symptoms. The main clinical manifestations include common symptoms (severe fatigue, unexplained fever with or without weight loss), skin lesions (orthostatic palpable purpura, necrotic ulcers), joints (arthritis, arthralgia), peripheral nervous system (mononeuritis, polyneuritis) and kidney (membranoproliferative glomerulonephritis). Given the many signs and symptoms, a patient with CV rarely turns primarily to a rheumatologist. First contact doctors are often family doctors or dermatologists, along with the fact that the awareness of doctors of other specialties about this pathology is insufficient. The article presents modern data on the etiological factors, CV types and variants of the clinical course and treatment. CV classification criteria are highly informative and available to practitioners. Treatment of CV remains a challenge due to serious specific target organ damage and sometimes life-threatening manifestations. In secondary cryoglobulinemia, treatment of the underlying disease is crucial. In case of CV on the background of mixed cryoglobulinemia, the treatment strategy is based on antiviral, anti-inflammatory and immunosuppressive therapy. The therapy goals for CV include reducing the immunoglobulin level and removing the antigen. The first goal can be achieved with immunosuppressants, while the second goal depends on whether the antigen is known or not. With CV associated with HCV, antiviral therapy reduces the number of antigens. However, in autoimmune diseases, the potential antigen is usually not recognized, and only non-specific immunosuppressants are used.