scholarly journals Pyoderma gangrenosum-like ulceration as a presenting feature of pediatric granulomatosis with polyangiitis

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Rotem Semo Oz ◽  
Oluwakemi Onajin ◽  
Liora Harel ◽  
Rotem Tal ◽  
Tomas Dallos ◽  
...  
Keyword(s):  
Pyoderma Gangrenosum ◽  
Granulomatosis With Polyangiitis ◽  
Skin Lesions ◽  
Response To Treatment ◽  
Proteinase 3 ◽  
Newly Diagnosed ◽  
Rare Presentation ◽  
Case Presentation ◽  
Appropriate Treatment ◽  
Systemic Symptoms

Abstract Background Granulomatosis with polyangiitis (GPA) is an anti-neutrophilic cytoplasmic antibody-associated vasculitis affecting small to medium-sized vessels and involves most commonly the kidneys and the respiratory tract. Skin involvement can be seen in up to 50% of children with GPA and is the initial presenting symptom in 7.7%. Pyoderma gangrenosum (PG)-like ulcers are rarely described as a skin manifestation in GPA and very few cases have been reported previously in children. Case presentation We describe 3 new pediatric cases of GPA with PG-like ulcerations. The median age at first symptom was 15 years. Two patients had PG-like ulceration as their initial presentation; additional symptoms eventually led to the diagnosis of GPA 2–24 months later. In 1 case, proteinase 3 (PR3) was negative when first tested, but converted to positive when systemic symptoms emerged; in the other 2 cases PR3 was positive at presentation. All 3 patients had prominent facial lesions. None of the patients responded to treatment with antibiotics or medications commonly used to manage PG, including corticosteroids and cyclosporine. All patients had excellent responses to rituximab. An electronic database literature review was performed and 4 previously reported cases were identified. We assessed the clinical characteristics, serology, and response to treatment of the previously reported and our newly diagnosed cases. Conclusion PG-like ulceration is a rare presentation of pediatric GPA which may precede classic systemic GPA symptoms. The predominance of facial ulcer, granulomatous and neutrophilic inflammation on skin biopsy and lack of response to PG treatments are characteristic of GPA-associated PG-like ulcers. Our review suggests that treatment with rituximab may be needed to improve the skin lesions. Recognizing that PG-like ulcerations can occur in pediatric GPA may result in timely diagnosis, appropriate treatment and improved prognosis.

scholarly journals Pyoderma Gangrenosum-like ulceration as a presenting feature of pediatric Granulomatosis with Polyangiitis

2020 ◽  
Author(s):  
Rotem Semo Oz ◽  
Oluwakemi Onajin ◽  
Liora Harel ◽  
Rotem Tal ◽  
Tomas Dallos ◽  
...  
Keyword(s):  
Pyoderma Gangrenosum ◽  
Granulomatosis With Polyangiitis ◽  
Skin Lesions ◽  
Response To Treatment ◽  
Newly Diagnosed ◽  
Rare Presentation ◽  
Case Presentation ◽  
Appropriate Treatment ◽  
The Mean ◽  
Systemic Symptoms

Abstract Background: Granulomatosis with polyangiitis (GPA) is an anti-neutrophilic cytoplasmic antibody-associated vasculitis affecting small to medium-sized vessels and involves most commonly the kidneys and the respiratory tract. Skin involvement can be seen in up to 50% of children with GPA and is the initial presenting symptom in 7.7%. Pyoderma gangrenosum (PG)-like ulcers are rarely described as a skin manifestation in GPA and very few cases have been reported previously in children. Case presentation: We describe 3 new pediatric cases of GPA with PG-like ulcerations. The mean age at first symptom was 15 years. Two patients had PG-like ulceration as their initial presentation; additional symptoms eventually led to the diagnosis of GPA 2-24 months later. In 1 case, proteinase 3 (PR3) was negative when first tested, but converted to positive when systemic symptoms emerged, in the other 2 cases PR3 was positive at presentation. All 3 patients had prominent facial lesions. None of the patients responded to treatment with antibiotics or medications commonly used to manage PG, including corticosteroids and cyclosporine. All patients had excellent responses to rituximab. An electronic database literature review was performed and 4 previously reported cases were identified. We assessed the clinical characteristics, serology, and response to treatment of 4 previously reported and newly diagnosed cases. Conclusion: PG-like ulceration is a rare presentation of pediatric GPA which may precede classic systemic GPA symptoms. The predominance of facial ulcer, granulomatous and neutrophilic inflammation on skin biopsy and lack of response to PG treatments are characteristic of GPA-associated PG-like ulcers. Our review suggests that treatment with rituximab may be needed to improve the skin lesions. Recognizing that PG-like ulcerations can occur in pediatric GPA may result in timely diagnosis, appropriate treatment and improved prognosis.

Can we effectively predict the occurrence of cerebral edema in children with ketoacidosis in the course of type 1 diabetes? – case report and literature review

2020 ◽  
Vol 33 (2) ◽  
pp. 319-322
Author(s):  
Krzysztof Jeziorny ◽  
Arleta Waszczykowska ◽  
Dobromiła Barańska ◽  
Agnieszka Szadkowska ◽  
Wojciech Młynarski ◽  
...  
Keyword(s):  
Type 1 Diabetes ◽  
Cerebral Edema ◽  
Diagnostic Methods ◽  
Newly Diagnosed ◽  
Cns Disorders ◽  
Case Presentation ◽  
Non Invasive ◽  
Appropriate Treatment ◽  
Life Threatening

AbstractBackgroundCerebral edema (CE) is one of the most serious complications of diabetic ketoacidosis (DKA) and can result in central nervous system (CNS) disorders and even lead to death of the patient.Case presentationWe present the case of a 11-year-old boy with severe DKA in the course of newly diagnosed type 1 diabetes (T1D). The delay in the diagnosis of DKA and some therapeutic problems contributed to the development of CE and direct life-threatening conditions. Early diagnosis of CE development in the course of DKA using non-invasive methods such as pachymetry or transorbital ultrasound seems to be a very important prognostic factor.ConclusionsThis case highlights the importance of appropriate treatment according to the newest recommendations and presents the usefulness of new diagnostic methods to assess the risk of CE in children with newly diagnosed T1D.

scholarly journals Peripheral Ulcerative Keratitis with Pyoderma Gangrenosum

2015 ◽  
Vol 2015 ◽  
pp. 1-4
Author(s):  
Adrián Imbernón-Moya ◽  
Elena Vargas-Laguna ◽  
Antonio Aguilar ◽  
Miguel Ángel Gallego ◽  
Claudia Vergara ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Pyoderma Gangrenosum ◽  
Early Recognition ◽  
Skin Lesions ◽  
Unusual Event ◽  
Peripheral Ulcerative Keratitis ◽  
Appropriate Treatment ◽  
Autoimmune Conditions ◽  
Ulcerative Keratitis ◽  
Peripheral Cornea

Pyoderma gangrenosum is an unusual necrotizing noninfective and ulcerative skin disease whose cause is unknown. Ophthalmic involvement in pyoderma gangrenosum is an unusual event. Only a few cases have been reported, from which we can highlight scleral, corneal, and orbital cases. Peripheral ulcerative keratitis is a process which destroys the peripheral cornea. Its cause is still unknown although it is often associated with autoimmune conditions. Pyoderma gangrenosum should be included in the differential diagnosis of peripheral ulcerative keratitis. Early recognition of these manifestations can vary the prognosis by applying the appropriate treatment. We introduce a 70-year-old woman who suffered pyoderma gangrenosum associated with peripheral ulcerative keratitis in her left eye. The patient’s skin lesions and peripheral keratitis responded successfully to systemic steroids and cyclosporine A.

scholarly journals Large Pyoderma Gangrenosum-Like Ulcers: A Rare Presentation of Granulomatosis with Polyangiitis

2014 ◽  
Vol 2014 ◽  
pp. 1-4 ◽  
Author(s):  
Basheer Tashtoush ◽  
Roya Memarpour ◽  
Yasmin Johnston ◽  
Jose Ramirez
Keyword(s):  
Pyoderma Gangrenosum ◽  
Granulomatosis With Polyangiitis ◽  
Leukocytoclastic Vasculitis ◽  
Systemic Vasculitis ◽  
Rare Presentation ◽  
Serological Tests ◽  
Cutaneous Manifestations ◽  
Wide Range ◽  
Common Skin ◽  
Nasal Septum Perforation

Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis (WG), is a rare systemic vasculitis that classically manifests as necrotizing granulomas of the upper and lower respiratory tract, kidneys, and blood vessels; however, it may affect any organ system, including the skin. Cutaneous manifestations occur in up to 45% of patients during the disease course, and are the presenting feature in 9% to 14% of patients. The most common skin lesion specific to GPA is palpable purpura, with the histopathologic correlate of leukocytoclastic vasculitis. However, a wide range of clinical and histologic features may be seen. We herein report a case of a previously healthy 52-year-old Caucasian man who presented with multiple progressively enlarging painful ulcers on his face, upper extremities, back, and abdomen over a two-month period. Skin biopsies revealed pyoderma gangrenosum-like features. Serological tests were positive for PR3/c-ANCA. Six months later, the patient developed recurrent episodes of sinusitis associated with nasal bleeds and eventually nasal septum perforation. Despite aggressive treatment with Cyclophosphamide and steroids over one year, the patient had persistent nonhealing large ulcers and developed multiple lung nodules with cavitary lesions.

scholarly journals Could COVID-19 Trigger Presentation or Exacerbation of Vasculitides? (A 14-Year-Old Girl with Newly Diagnosed GPA After COVID-19 Infection)

2021 ◽  
Vol In Press (In Press) ◽  
Author(s):  
Seyed Reza Raeeskarami ◽  
Seyyed Hosein Mousavi ◽  
Mohammad Ashouri ◽  
Parvin Akbariasbagh ◽  
Raheleh Assari ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Pediatric Population ◽  
Multiple Organ ◽  
Newly Diagnosed ◽  
Gi Tract ◽  
Case Presentation ◽  
Anca Associated Vasculitis ◽  
Organ Systems ◽  
Necrotizing Glomerulonephritis ◽  
St Elevation

Introduction: Granulomatosis with polyangiitis (GPA) is a kind of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), which involves small-to-medium-sized vessels. Besides, GPA usually involves the upper and lower respiratory tract, and also causes necrotizing glomerulonephritis. The involvement of the heart and gastrointestinal (GI) tract in GPA is unusual, and these are atypical places of this vasculitis. Case Presentation: A 14-year-old girl with a newly diagnosed GPA after afflicting with COVID-19 infection presented with ST-elevation Myocardial Infarction (MI), GI perforation, and intracranial hemorrhage. Conclusions: Although GPA is rare in the pediatric population, it might occur in this age group and could involve multiple organ systems.

scholarly journals Spontaneously Regressing Neonatal Oral Aphthous Ulceration of the Palate

2021 ◽  
Vol 2021 ◽  
pp. 1-3
Author(s):  
Sandara Wayangi Madurapperuma ◽  
Andra Hennadige Heshan Malinga Jayaweera ◽  
Ruwan Duminda Jayasinghe
Keyword(s):  
Clinical Presentation ◽  
Rare Presentation ◽  
Diagnostic Dilemma ◽  
Nasogastric Feeding ◽  
Case Presentation ◽  
Uncommon Presentation ◽  
Appropriate Treatment ◽  
Aphthous Ulceration ◽  
Mild Fever ◽  
Ongoing Infection

Background. Neonatal oral aphthous ulceration of the palate also known as Bednar’s aphthae is not an uncommon presentation. They clinically present as spontaneously regressing, shallow, and symmetrical ulcers on the posterior palate of newborns from 2 days up to 6 weeks of age. Case Presentation. We, herein, report a case of a one-month-old baby girl who presented with an ulcer in the posterior palate and intermittent mild fever. The patient was admitted and monitored in the ward. Haematologic investigations disclosed features of ongoing infection. Nasogastric feeding was commenced to avoid any irritation of the ulcer, and glycerine was applied on the ulcer. Antibiotic therapy was continued because of the intermittent mild fever. The lesion healed spontaneously within one week, and fever subsided afterwards. Currently, the patient is faring healthily without any complications. Conclusion. Although Bednar’s aphthae is not a rare presentation, clinicians are often met with a diagnostic dilemma due to the alarming clinical presentation of this condition. Therefore, it leads to overinvestigation and overtreatment. With this case report, we would like to highlight the importance of being aware of this condition to provide the patients with the appropriate treatment.

scholarly journals Lennox–Gastaut Syndrome: A Prospective Follow-up Study

2017 ◽  
Vol 08 (02) ◽  
pp. 225-227 ◽  
Author(s):  
Bhanu Pratap Rathaur ◽  
Ravindra Kumar Garg ◽  
Hardeep Singh Malhotra ◽  
Neeraj Kumar ◽  
Praveen Kumar Sharma ◽  
...  
Keyword(s):  
Valproic Acid ◽  
Seizure Frequency ◽  
Epileptic Encephalopathy ◽  
Response To Treatment ◽  
Newly Diagnosed ◽  
Follow Up Study ◽  
Appropriate Treatment ◽  
Disability Status ◽  
Ischemic Encephalopathy

ABSTRACT Objectives: Lennox–Gastaut syndrome is a catastrophic epileptic encephalopathy. In Lennox–Gastaut syndrome, seizures are resistant to pharmacological treatment. In this prospective study, we evaluated the clinical features, neuroimaging, and response to treatment. Materials and Methods: Forty-three consecutive newly diagnosed patients of Lennox–Gastaut syndrome were enrolled in the study. Baseline clinical assessment included seizure semiology, seizure frequency, electroencephalography, and neuroimaging. Patients were treated with combinations of preferred antiepileptic drugs (sodium valproate [VPA], clobazam [CLB], levetiracetam [LVT], lamotrigine [LMT], and topiramate [TPM]). Patients were followed for 6 months. The outcome was assessed using modified Barthel index. Results: Tonic and generalized tonic-clonic forms were the most common seizures types. Features suggestive of hypoxic-ischemic encephalopathy (37.2%) were most frequent neuroimaging abnormality. Neuroimaging was normal in 32.6% of patients. With a combination valproic acid (VPA), CLB, and LVT, in 81.4% of patients, we were able to achieve >50% reduction in seizure frequency. Eleven (25.58%) patients showed an improvement in the baseline disability status. Conclusions: A combination of VPA, CLB, and LVT is an appropriate treatment regimen for patients with Lennox–Gastaut syndrome.

scholarly journals Pretibial myxedema in a euthyroid patient: a case report

2021 ◽  
Vol 14 (1) ◽  
Author(s):  
Rediet Ambachew ◽  
Tizita Yosef ◽  
Aklilu M. Gebremariam ◽  
Lishan Demere ◽  
Theodros Aberra ◽  
...  
Keyword(s):  
Case Reports ◽  
Skin Lesions ◽  
Thyroid Function Tests ◽  
Thyroid Disorder ◽  
Rare Presentation ◽  
Case Presentation ◽  
Normal Thyroid Function ◽  
History Of ◽  
Euthyroid Patient ◽  
Pretibial Myxedema

Abstract Background Pretibial myxedema also known as localized myxedema, thyroid dermopathy, or infiltrative dermopathy and rarely as localized mucinosis is an infrequent manifestation of Graves’ disease. It can appear before, during, or after the thyrotoxic state. Euthyroid pretibial myxedema is a rare presentation with few case reports in the literature. This case highlights the importance of considering pretibial myxedema when characteristic skin lesions are observed in a euthyroid patient. Case presentation A 72-year old male Ethiopian patient with a very rare presentation of biopsy-proven pretibial myxedema in a euthyroid state without history of thyroid disease and absence of thyroid autoimmune markers. Resolution of skin lesion was achieved after topical corticosteroid application. Conclusion Absence of history of thyroid disorder and normal thyroid function tests should not exclude the diagnosis of pretibial myxedema.

scholarly journals MO309SARS COV2 INFECTION COMPLICATING CRYOGLOBULINEMIC VASCULITIS WITH RENAL INVOLVEMENT

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
Hanene Bouafif ◽  
Hajji Mariem ◽  
Samia Barbouch ◽  
Hedri Hafedh ◽  
Imen Gorsane ◽  
...  
Keyword(s):  
Hemorrhagic Cystitis ◽  
Renal Involvement ◽  
Skin Lesions ◽  
Response To Treatment ◽  
Case Presentation ◽  
Nasal Septal Perforation ◽  
History Of ◽  
Peripheral Arterial ◽  
Inflammatory Syndrome

Abstract Background and Aims Cryoglobulinaemic vasculitis (CV) leading to clinically apparent skin lesions, and in some cases also to internal organ involvement such as renal involvement. The SARS Cov2 infection in this field is very serious and in the case of our patient the outcome was fatal. The aim of this work is to highlight the particularities of CV in this case Case presentation We report a case of a 62-year-old woman followed since 2014 for CV type 1 IgG Lambda revealed by vascular purpura and nasal septal perforation. the etiological assessment was negative. Hepatitis C virus serology was negative. A corticosteroid therapy at a dose of 1 mg/kg/day was started with partial improvement. Then the cyclophosphamide at a dose of 150 mg/d was added with a fairly good clinical result but stopped for hemorrhagic cystitis. Chlorominophene was started at a dose of 6 mg/d and then reduced to 4 mg/d for leuconeutropenia but the peripheral arterial manifestations (acrocyanosis of the limbs and ears) were not treated. The evolution was marked by the appearance of renal failure (creatinine 482.33 umol/l) associated with proteinuria at 1.32 g/24h. An outbreak of cryoglobulinemia was suspected. Plasma exchange was discussed as a therapeutic alternative and the patient was scheduled for a renal biopsy. However, the patient became febrile with the appearance of a dry cough and the presence of a biological inflammatory syndrome (CRP at 72). A SARS-CoV-2 PCR was completed which was positive. Therefore, she was put on antibiotic therapy (Azythromycin and Cefotaxime) associated with vitamin therapy and anticoagulant treatment. The patient was stable in terms of respiration and hemodynema, but her renal function worsened and progressed well under hydration. In addition, she presented with a slippage syndrome and she was died Conclusion The natural history of CV is not predictable and strongly depends on concomitant diseases and complications and response to treatment. The SARS Cov2 infection can complicate its evolution. There is no association has been described between them to our knowledge

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