Palpable Purpura Research Articles

Characterization of non‐IgA vasculitis: Demographic, clinical, and treatment‐related features in a retrospective analysis of 28 biopsy‐confirmed cases from a German university hospital

AbstractNon‐IgA vasculitis is a rare disease that belongs to the group of small‐vessel vasculitides. Due to nomenclature and classification changes introduced in 2018, there are few published data under this name. The aim of this study is to characterize non‐IgA vasculitis as an independent vasculitis entity in terms of demographic, clinical, and treatment‐related features. A retrospective data analysis of patients with biopsy‐confirmed non‐IgA vasculitis treated at the Department of Dermatology at the University Medical Center Hamburg‐Eppendorf between January 1, 2018, and December 31, 2022, was performed. A total of 28 patients with non‐IgA vasculitis were included; 53.6% (15/28) were women and 42.9% (12/28) were older than 71 years. Previous infection as a possible triggering factor was found in 42.6% (12/28) of the cases. Palpable purpura was the most common skin finding (78.6%, 22/28) and 28.6% patients (8/28) had skin lesions above the waist. On direct immunofluorescence, C3 (89.3%, 25/28) was the most frequent deposition, followed by fibrinogen (71.4%, 20/28) and IgM (53.6%, 15/28). Hospitalization was required in 85.7% (24/28), with a mean hospital stay of 9.4 ± 4.1 days. No fatal courses were reported. This study is the first characterization of non‐IgA vasculitis based on patient cases from Germany and contributes to a better understanding of non‐IgA vasculitis as an independent entity. Non‐IgA vasculitis primarily affects older patients of both sexes, with most cases having an identifiable trigger. Our results indicate that cutaneous manifestations often extend beyond the lower legs. Treatment is usually required in the inpatient setting and requires a longer stay than other dermatological conditions. With proper treatment, the disease is not expected to be fatal.

Characteristics of cutaneous manifestations in immunoglobulin a vasculitis and their relationships with system involvement and treatment needs.

The characteristics of cutaneous manifestations in IgAV patients may be helpful in predicting the course of the disease. The skin lesion type, distribution and persistence of cutaneous manifestations in IgAV are associated with system involvement. In addition, the need for intensive therapy increases in the presence of vesicles/bullae, necrosis/ulcer, rash spreading over the buttocks and persistent rash in IgAV. • Palpable purpura localized to the lower extremities is the expected cutaneous manifestation of immunoglobulin A vasculitis. • The type, distribution and duration of rash in IgAV have effects on theimmunoglobulin A vasculitis disease course. • Genital tract involvement is more common in patients with necrosis/ulcers. • Gastrointestinal tract involvement and genital tract involvement are more commonin patients with rash spreading above the buttocks. • Renal involvement is more common in patients with persistent rash.

Abstract 4121816: Viral Venture: A Case of Myocarditis and IgA Vasculitis from Adenovirus Infection

Case Description: A 19-year-old man presented to the emergency department for 3 days of shortness of breath, progressive bilateral lower extremity rash, generalized fatigue, and diffuse joint following an earlier visit to urgent care for pharyngitis symptoms. Physical exam was notable for tachycardia, pain with active wrist and ankle range of motion, pharyngeal erythema, cervical adenopathy, and confluent erythematous palpable purpura and petechiae noted on bilateral lower extremities with scattered lesions on his thighs, arms, and back. Laboratory workup notable for markedly elevated high-sensitivity troponin to 3398 ng/L, and elevated CRP. Further workup was notable for normal urinalysis, negative streptococcus antigen, negative ANCA antibodies, ANA, Hepatitis B and C, and cryoglobulins. Imaging and cardiac workup were notable for normal left ventricular function and cardiac MRI notable for late gadolinium enhancement in the anteroapical mid-myocardial region suggestive of myocarditis. Skin biopsy ultimately revealed immunodeposition of IgA on superficial capillaries compatible with a diagnosis of IgA vasculitis. The patient was started on steroids for IgA vasculitis with rapid improvement in his symptoms and he was discharged home in good condition. Discussion: Recognizing the severe complications of viral infection can lead to prompt treatment and prevention of progression to severe end organ damage. Our case highlights rarely reported cardiac involvement of IgA vasculitis secondary to adenovirus infection based on laboratory and imaging diagnosis with cardiac MRI. To date, only 16 cases of cardiac involvement in IgA vasculitis have been reported. This case adds to the limited body of published cases describing this presentation of IgA vasculitis and is also consistent with the results of prior cases suggesting that an immunosuppressive regimen including glucocorticoids may help to prevent disease progression and induce remission in cases with cardiac involvement without the need for endomyocardial biopsy.

Community-Associated MRSA—Not So Innocent Sibling per Se: A Case Report

Background: Methicillin-resistant S. aureus (MRSA) is a major healthcare burden and is classified as healthcare-associated (HA-MRSA) and community-associated (CA-MRSA). While HA-MRSA is clinically feared, CA-MRSA is often considered less pathogenic. This case report highlights the serious course of illness due to CA-MRSA infection and provides a treatment strategy for the management of such cases. Case description: A 41-year-old male presented with fever and breathlessness for five days. Upon admission, he was provided empirical treatment for atypical infections and vasopressor support for hypotension. His condition deteriorated, necessitating ventilator support. Although the initial tracheal Bio Fire test indicated MRSA, his clinical manifestations did not match MRSA pneumonia symptoms; however, CA-MRSA was confirmed within 12 h. Skin legions developed within 16 h and progressed gradually from ecchymosis, petechial, and palpable purpura to bullous lesions over 72 h. The antibiotic regimen was modified and optimized with the addition of Clindamycin, Vancomycin, and Meropenem–Colistin. Owing to high IL-6 levels, dual vasopressor support, and acute kidney failure, he was started on early (within 12 h) continuous renal replacement therapy (CRRT) with CytoSorb filter (for 3 days) for cytokine removal. IL-6 levels decreased after two days of CytoSorb use. Subsequently, the patient stabilized with reduced dependence on vasopressor and ventilator assistance. Discussion: Early diagnosis of CA-MRSA and management with CRRT using CytoSorb may help improve patient outcomes. To our knowledge, this is the first report of clinical management of CA-MRSA with CytoSorb therapy in India that resulted in positive outcomes.

Complete renal response of IgA vasculitis with steroid and mycophenolate mofetil in an adult HIV patient.

IgA vasculitis in adult HIV patients is rare and not well understood. Data on treatment of IgA vasculitis associated with HIV is sparse and limited to case reports. Highly active antiretroviral therapy (HAART) is the mainstay therapy for inducing remission. We report a case of a 41-year old HIV patient who presented with palpable purpura, melaena and oedema and was diagnosed with IgA vasculitis with severe renal (crescentic glomerulonephritis), gastrointestinal and skin involvement. He was treated with immunosuppressive drugs and HAART which led to remission of disease. This case is unusual due to the presence of crescentic glomerulopathy and the requirement of immunosuppression for remission induction.

A unique and rare presentation of Henoch-Schonlein purpura: a case report

Henoch-Schönlein purpura (HSP), or IgA vasculitis, is a common small vessel vasculitis in children, typically presenting with palpable purpura, abdominal pain, arthritis, and renal involvement. However, atypical presentations can delay diagnosis and management. A 12-year-old girl presented with multiple episodes of loose stools and abdominal pain. The initial diagnosis focused on acute gastroenteritis, and she was treated symptomatically. Subsequently, she developed urticarial rashes with breathing difficulty, complicating the diagnosis. Despite symptomatic treatment, she experienced severe abdominal pain, hematemesis and melena. Further investigations revealed antral gastritis, duodenitis, and cystitis. On the 14th day of admission, the patient developed characteristic palpable purpura on the lower limbs. Skin biopsy confirmed leukocytoclastic vasculitis with IgA deposits, consistent with HSP. The patient recovered with supportive care and remained symptom-free on follow-up. This case underscores the importance of considering HSP in pediatric patients with atypical presentations, such as gastrointestinal symptoms and urticaria without initial purpura. Early recognition and appropriate management are essential to prevent complications and ensure favourable outcomes. The variability in HSP presentations necessitates a high index of suspicion for timely diagnosis and treatment.

Хірургічні ускладнення пурпури Шенляйн-Геноха в дітей

Henoch-Schönlein purpura is the systemic pathology that affected the small vessels of the skin, gastrointestinal tract, kidneys, and joints. Typical clinical manifestation of Henoch-Schönlein purpura include palpable purpura, abdominal pain syndrome, arthralgia, renal involvement, and, in some children, externa genitalia involvement. The aim of this study was to present the own experience in the management of three children with the surgical complications of Henoch-Schönlein purpura. The own experience includes three children with Henoch-Schönlein purpura, particularly two children with ileum intussusception (both of 3 years old) and one patient of 4 years old with the syndrome “acute scrotum”. Gastrointestinal involvement may appear in most of patients with Henoch-Schönlein purpura and intussusception is the most common complication, which require the urgent treatment. Appearance of typical palpable purpura followed the abdominal pain syndrome and ultrasonography is the main method of diagnostic in this group of patients. Ileum and ileocolic are the common types of intussusception in children with Henoch-Schönlein purpura. The main method of treatment in case of ileum intussusception is surgical reduction, while in case of ileocolic intussusception non-surgical reduction can be applied. Besides the typical manifestation of Henoch-Schönlein purpura, in some children could be the scrotal involvement, which developing after appearance of palpable purpura, and rare caused by inflammation or torsion of testis or hydatid. It is believed that patients with Henoch-Schönlein purpura and scrotal involvement have more severe clinical course when compared with patients with abdominal manifestation, which agreed with our observation. Conclusions. In children older than one year, with the abdominal pain, with or without bloody stools, and ultrasonographic signs of intussusception, it is necessary consider about possibility the presence of Henoch-Schönlein purpura in these patients. In children with Henoch-Schönlein purpura despite the corticosteroids’ treatment, could be developed the syndrome of “acute scrotum”, which required emergent surgery. The research was carried out in accordance with the principles of the Declaration of Helsinki. The research protocol was approved by the Local Ethics Committee of the institution mentioned in the work. Informed consent of the patients was obtained for the research. No conflict of interests was declared by the authors.

Immunoglobulin A vasculitis: The clinical features and pathophysiology.

Palpable purpura, gastrointestinal symptoms, joint involvement, and renal disease characterize immunoglobulin A vasculitis (IgAV). Renal involvement ranging from mild proteinuria to severe nephritic or nephrotic syndrome highlights the importance of monitoring kidney function in patients with IgAV. Recognizing these key features is crucial for early diagnosis and appropriate management to prevent long-term complications related to kidney disease. However, the pathogenesis of IgAV remains unclear. Disease mechanisms involve various factors, including the interplay of aberrantly glycosylated IgA, anti-endothelial cell antibodies, and neutrophils following infection triggers, which are the main pathogenic mechanisms of IgAV. Insights from cases of IgAV related to Coronavirus disease 2019 have offered additional understanding of the connection between infection and IgAV pathogenesis. This review provides a valuable resource for healthcare professionals and rheumatology researchers seeking a better understanding of the clinical features and pathophysiology of IgAV.

Red flags for clinical suspicion of eosinophilic granulomatosis with polyangiitis (EGPA)

BackgroundEosinophilic granulomatosis with polyangiitis (EGPA), is a rare ANCA-associated systemic vasculitis. Its overlapping features with other vasculitic or eosinophilic diseases, and the wide and heterogeneous range of clinical manifestations, often result in a delay to diagnosis. ObjectiveTo identify red flags that raise a suspicion of EGPA to prompt diagnostic testing and to present an evidence-based clinical checklist tool for use in routine clinical practice. MethodsSystematic literature review and expert consensus to identify a list of red flags based on clinical judgement. GRADE applied to generate a strength of recommendation for each red flag and to develop a checklist tool. Results86 studies were included. 40 red flags were identified as relevant to raise a suspicion of EGPA and assessed by the experts as being clinically significant. Experts agreed that a diagnosis of EGPA should be considered in a patient aged ≥6 years with a blood eosinophil level >1000 cells/µL if untreated and >500 cells/µL if previously treated with any medication likely to have altered the blood eosinophil count. The presence of asthma and/or nasal polyposis should reinforce a suspicion of EGPA. Red flags of asthma, lung infiltrates, pericarditis, cardiomyopathy, polyneuropathy, biopsy with inflammatory eosinophilic infiltrates, palpable purpura, digital ischaemia and ANCA positivity, usually anti-myeloperoxidase, among others, were identified. ConclusionThe identification of a comprehensive set of red flags could be used to raise a suspicion of EGPA in patients with eosinophilia, providing clinicians with an evidence-based checklist tool that can be integrated into their practice.

Storia di una porpora timida e infida

The author describes a case of Henoch-Schönlein purpura in a 4-year-old child who, before showing cutaneous signs (the classic palpable purpura on the lower limbs), developed abdominal signs (pain, vomiting and loss of appetite).

#823 Effect of dapsone on the treatment of refractory IgA vasculitis

Abstract Background and Aims IgA vasculitis (IgAV), previously known as Henoch-Schönlein purpura, is a systemic IgA-mediated vasculitis of the small vessels commonly seen in children. The natural history of IgAV is generally self-limiting. However, one-third of patients experience symptom recurrence and a refractory course. We performed this study to examine the use of dapsone (4,4'-sulfonyldianiline or diaminodiphenyl sulfone) in refractory IgAV cases. Method A literature search of PubMed databases retrieved 16 articles published until May 31, 2023. A total of 38 patients with IgAV were recruited. Results A palpable purpura persisted in nearly all the patients with refractory course, followed by joint pain (25 of 38 patients; 69.2%). Hematuria and proteinuria were noted in 11 (28.9%) and 7 (18.4%) of the patients, respectively. Treatment response within 1-2 days was obtained in 12 of 38 patients (31.6%), compared to within ≥3 days in 22 patients (57.9%). Relapse after treatment discontinuation was reported in 21 patients (55.3%) but not in 11 patients (28.9 %). Twelve of the 38 patients (31.6%) reported adverse effects of dapsone, including arthralgia (5.3%), rash (5.3%), dapsone hypersensitivity syndrome (2.6%), methemoglobinemia (21%), and hemolysis (15.8%). These findings indicate that dapsone may have a beneficial effect on refractory IgAV. Conclusion It is observed that in spite of several adverse effects, dapsone treatment exerts a beneficial effect in IgAV patients with prolonged cutaneous manifestation refractory to corticosteroid. Close renal monitoring may be needed to discover renal involvement of IgAV. Multicenter randomized placebo-controlled trials are necessary to determine the standard dosage of dapsone at initial or tapering of treatment in IgAV patients.

P022 Unmasking the storm: hemophagocytic lymphohistiocytosis in systemic lupus erythematosus - a case series

Abstract Background/Aims Hemophagocytic lymphohistiocytosis (HLH), a critical illness of uncontrolled immunological activity and hyperinflammation, manifests as primary/familial or secondary form. Secondary HLH can be of multi-faceted etiology including infections, cancer, autoimmune and immunodeficiencies. HLH in context of systemic lupus erythematosus (SLE) is a rare entity and mirrors the clinical picture of SLE flare, adding to the diagnostic dilemma. Methods Our objective is to consider HLH as a key differential in unidentified autoimmune diseases. We present a trio of cases of undiagnosed SLE manifesting as HLH. Results A 13-year-old male presented with fever, rash, oral ulcers, and jaundice for a month with passage of dark coloured urine for last seven days. On examination, he had generalized lymphadenopathy, erythematous maculopapular rashes, palpable purpura, non-scarring alopecia, and hepatosplenomegaly. The labs revealed pancytopenia (Hb 9.8g/dL, WBC 2000/mm3, Platelets 23000/mm3), deranged creatinine (1.6mg/dL), transaminitis and hyperbilirubinemia, hyper-ferritinemia (2420µg/dL), hypertriglyceridemia (280mg/dL), hypofibrinogenemia (142mg/dL), and nephrotic range proteinuria (3.7g/24h). Diagnosis of SLE was confirmed by high titres of antinuclear antibody (ANA) and anti-double stranded DNA (dsDNA) and anti-Smith positivity. Renal biopsy showed class II lupus nephritis and acute tubular necrosis. Bone marrow biopsy revealed hemophagocytosis and the diagnosis of SLE complicated by HLH was made. A 16-year-old girl presented with fever and headache for three weeks, worsening over last ten days with irritable behaviour for one day, and respiratory distress that necessitated endotracheal intubation. The presence of pancytopenia (9.1/1940/41000), transamintis, hyper-ferritinemia (4040µg/dL), hypertriglyceridemia (250mg/dL), and low fibrinogen (133mg/dL) in laboratory evaluation raised a compelling suspicion of HLH confirmed by hemophagocytosis on marrow biopsy. Cerebrospinal fluid analysis was unremarkable, however MRI detected features of vasculitis with vasculitic infarcts. Direct Coomb’s test was positive. Autoimmune profiling unveiled positive results for ANA, anti-dsDNA, and anti-Smith antibodies consistent with SLE. A 21-year-old female presented with fever and arthralgias for two months. Investigations were suggestive of pancytopenia (7/1050/36000), raised ESR (126mm/hr) and CRP (54 mg/dL), elevated ferritin (4320 µg/dL), lactate dehydrogenase (1068U/L) and triglycerides (439mg/dL). The marrow biopsy was negative for hemophagocytosis while her soluble interleukin - 2 receptor levels were elevated (2740 U/ml). ANA and dsDNA were positive in significant titres, complement levels were low and Coombs test was positive, thereby confirming the primary diagnosis of SLE. All patients received methylprednisolone pulse along with cyclophosphamide in case 1 and IVIG in case 2 and 3, resulting in remarkable improvement. Conclusion With a prevalence of 0.9-4.6%, HLH secondary to SLE is an uncommon entity. The diagnosis of HLH related to SLE is challenging since the symptoms overlap, however findings of hyperferritinemia, hypofibrinogenemia, and hypertriglyceridemia favour HLH over SLE. Once HLH is proven, immunological testing for SLE is warranted to prevent diagnostic delay. Early treatment is needed to combat such a fulminant life-threatening illness. Disclosure R. Mishra: None. A. Rohatgi: None. S. Anuradha: None. N. Kumar: None. S. Singhla: None. S.T. Mathews: None. S. Kumar: None. P. Gautam: None.

Glomerulonephritis as a renal manifestation in a patient with systemic sclerosis overlapped with anti-neutrophil cytoplasmic antibody-associated vasculitis

Introduction and Importance: Systemic sclerosis (SSc) is a systemic immune disorder that may overlap with other rheumatologic disease; however, overlapping with antineutrophil cytoplasmic antibody-associated vasculitis is rare. Case Presentation: A 28-year-old Syrian male patient with SSc diagnosed according to the American College of Rheumatology/European League against Rheumatism 2013 criteria with a disease duration of 4 years, was admitted to the hospital complaining of palpable purpura in the lower limbs and hemoptysis and later, a rise in creatinine level. Laboratory tests showed high levels of perinuclear antineutrophil cytoplasmic antibodies (p-ANCA). The renal biopsy results were consistent with the diagnosis of glomerulonephritis. He was treated with methylprednisolone, cyclophosphamide, and rituximab, as he was diagnosed with SSc overlapping antineutrophil cytoplasmic antibody-associated vasculitis. Clinical Discussion: SSc most commonly renal manifestations are proliferative vasculopathy leading to scleroderma renal crisis. However, other types of renal involvement were also reported in SSc patients with comorbid autoimmune diseases such as glomerulonephritis and signs of concurrent vasculitis. SSc may overlap with rheumatoid arthritis, systemic lupus erythromatosus, polymyositis/dermatomyositis (PM/DM), and Sjogren Syndrome. Overlapping with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis is mentioned in rare cases. The authors reported a rare case of overlapping SSc with antineutrophil cytoplasmic antibody-associated vasculitis with renal involvement. Conclusion: The authors revealed a rare case of overlapping SSc with antineutrophil cytoplasmic antibody-associated vasculitis with renal involvement. In SSc, renal involvement as glomerulonephritis is infrequent and should be detect in other rheumatologic disease such as systemic lupus erythematosus or antineutrophil cytoplasmic antibody-associated vasculitis.

Epidermodysplasia verruciformis arising in a female with systemic lupus erythematosus: a rare case from Syria

Introduction and importance: Epidermodysplasia verruciformis is a rare autosomal recessive genodermatosis. Clinical manifestations might be helpful in the diagnosis of this disease. However, the final diagnosis is made after a genetic and histological study. Acquired epidermodysplasia verruciformis is a form of epidermodysplasia verruciformis described in patients with compromised cell-mediated immunity. Case presentation: A 42-year-old female with a history of a pain and itch on the soles and palms started a year ago. There were multiple flat papules on the dorsal hands, scarring alopecia, malar rash, oral ulcers, Raynaud phenomenon, and palpable purpura. A histological examination confirmed the diagnosis of epidermodysplasia verruciformis. Clinical discussion: Epidermodysplasia verruciformis is an uncommon disease that affects the immune system. The coexistence of systemic lupus erythematosus and epidermodysplasia verruciformis is rarely reported in the medical literature. This paper reports a rare case in which these two diseases have coexisted. Conclusion: This publication aims to document this rare case and highlight the ideal criteria in diagnosing and treating epidermodysplasia verruciformis.

Ulnar Nerve Palsy as the Initial Presentation of Small Vessel Vasculitis With a Negative Antineutrophil Cytoplasmic Antibody Level

Small-vessel vasculitis is a rare disease entity that affects the arteries, arterioles, capillaries, and venules of the circulatory system. Small vessel vasculitis can cause severe small vessel inflammation and multisystem involvement. The respiratory tract and kidneys are the most commonly affected organs, and occasionally neurological manifestations can occur. But neurological manifestation as the initial presentation without other system involvement is very rare. We report a case of a 16-year-old previously well female patient presenting with right lower limb distal muscle weakness associated with malaise, lethargy, and multiple joint pains for 2 weeks. On examination, she had palpable purpura below the knees with right sided ulnar nerve palsy. Her antineutrophil cytoplasmic antibody and antinuclear antibody testing was negative. A skin biopsy showed vasculitis involving small vessels. She was started on Prednisolone 60mg daily, followed by Mycophenolate Mofetil 500mg twice daily, and physiotherapy. She had a good clinical response to treatment over time.

Acral palpable purpura with rapidly progressing multiorgan involvement

Acral palpable purpura with rapidly progressing multiorgan involvement

A study on clinico- epidemiological profile & histopathological profile of cutaneous vasculitis

: The study aimed to analyse the potential etiological factors, clinical presentation, past medical history & laboratory findings in patients with biopsy-proven cutaneous vasculitis to better understand the disease and identify the disease classifications. The objective of this study was to establish a database of the clinic-epidemiological profile and histopathological features of cutaneous vasculitis in a tertiary hospital in Eastern Nepal.: A Descriptive cross-sectional study was planned to analyse and study the clinic-epidemiological profile and histopathological features of cutaneous vasculitis in Nepal. This study has reviewed the facts which were published earlier to determine the current scenario by vast study of statistics and derivation of facts. Proper examination of data was made to evaluate with final conclusion. The study included sample size of 30 consecutive patients with biopsy-proven cutaneous vasculitis attending the Dermatology outpatient department and admitted to the dermatology ward at B.P. Koirala Institute of Health Science, Dharan. : The study population of 30 patients with biopsy-proven cutaneous vasculitis, with an average age of 32.96 years, and a female-to-male ratio of 1.3:1. The majority of patients (66.67%) had lesions lasting less than 6 weeks, and acute disease was observed in 86.66% of the cases. The most common symptoms were itching (73.66%) and arthralgia (66.67%). Palpable purpura was the predominant cutaneous manifestation (70%), mainly distributed on the legs and ankles (93.33%). Elevated ESR was the most common laboratory abnormality (73.33%). Histopathological examination revealed leukocytoclastic vasculitis in 97% of cases, with predominantly neutrophilic infiltrates. Henoch-Schoenlein purpura (HSP) was the most common classification (43.33%), followed by urticarial vasculitis (26.67%).: This study provides valuable insights into the clinic-epidemiological profile and histopathological features of cutaneous vasculitis in the Nepalese population. It highlights the diverse clinical presentation and laboratory abnormalities associated with the condition, as well as potential etiological factors and disease classifications. The findings contribute to improved understanding, diagnosis, and management of cutaneous vasculitis. Further studies with larger cohorts are needed to validate and expand upon these findings.

Panuveitis and palpable purpura in a patient with interstitial Pasteurella multocida pneumonia

Aims/Purpose: We describe a case of panuveitis and palpable purpura in a patient with Pasteurella multocida pneumonia.Methods: A 75‐year‐old pseudophakic woman with type‐2 diabetes and chronic obstructive pulmonary disease complained of lower limb palpable purpura and right vision loss of five‐day duration. On admission, right visual acuity was hand motion. Slit‐lamp examination disclosed severe conjunctival and perikeratic reaction, corneal oedema, 3‐mm hypopyon with heavy fibrin accumulation and blood clots, and synechiae to the IOL.Results: Chest computed tomography revealed interstitial pneumonia. Sputum culture yielded P. multocida. Amoxicillin/clavulanic tablets (1gx3/daily) and topical and systemic steroids were given. Later on, fluorescein angiography showed multiple areas of choroiditis bilaterally. With the above‐mentioned therapy, there was full recovery of the skin, lung, and eye lesions.Conclusions: This report emphasizes that P. multocida infection should be considered in patients with intraocular inflammation, palpable purpura, and interstitial pneumonia. A multidisciplinary approach is necessary for a correct diagnosis and management.

Trombocitopenia primária imune com púrpura palpável: um relato de caso

Immune thrombocytopenia is a relatively common bleeding disorder characterized by isolated destruction of platelets by autoantibodies, with a typically acute onset, benign course and self-limiting in most cases. The aim of this report is to present a rare case of Primary Immune Thrombocytopenia (ITP) with palpable purpura, an uncommon manifestation in this disease and little described in the literature. This is a 12-year-old female adolescent who presented with palpable purpura, ecchymosis and oral cavity bleeding associated with thrombocytopenia and anemia. Initial investigation excluded autoimmune diseases, vasculitis, lymphoproliferative diseases and coagulation disorders, in addition to the myelogram showing typical findings of ITP: hypercellular bone marrow with megakaryocytic hyperplasia and decreased thrombocytogenesis. Due to the diversity of clinical presentation and the possibility of atypical manifestations, the diagnosis is often challenging, as in the case of this patient in which the main finding on physical examination was the presence of palpable purpura.

Thymic mucosa-associated lymphoid tissue lymphoma in a patient with Sjögren’s syndrome with cutaneous vasculitis

Introduction. The association between Sj?gren?s syndrome (SS) and the development of lymphoma is well known. The prevalence of lymphoma in patients with SS is 5%. Mucosa-associated lymphoid tissue (MALT) lymphoma is the most common lymphoma type in patients with SS. It is common for MALT lymphoma to develop in the stomach, while it is extremely rare in the thymus. Case report. We present a 61-year-old Caucasian male patient with primary SS, cutaneous vasculitis, and thymic MALT lymphoma. The patient had a two-year history of diffuse cutaneous palpable purpura on legs, intermittently enlarged left parotid gland, and dry mouth. The results of Schirmer?s test were positive, labial salivary glands biopsy revealed a focus score ? 1, serology testing showed positive anti-Ro/SS-A and anti- La/SS-B antibodies, while skin biopsy findings revealed leukocytoclastic vasculitis. Diagnosis of primary SS with extraglandular cutaneous manifestations was confirmed. Cryoglobulinemia (Cg) and monoclonal gammopathy (MG) were detected, which increased the suspicion of hematological malignancy, and additional diagnostic procedures were performed. Computed tomography of the chest revealed an enlarged, multicystically altered anterior mediastinal mass. A thymectomy was performed through video-assisted thoracic surgery. Histological findings of the tissue confirmed the presence of tumor tissue consistent with MALT lymphoma in the thymus. Induction therapy with pulse doses of glucocorticoids was applied for three days, which was continued with medium doses of the drug. The doses were gradually reduced, and hydroxychloroquine was introduced. This has shown to be an effective therapy against features of SS. Postoperative local radiotherapy was performed. Conclusion. In SS patients with CV and in the presence of Cg and MG, attention should also be paid to the eventual development of MALT lymphoma, including the rare localization in the thymus.