scholarly journals A RARE PRESENTATION OF GRANULOMATOSIS WITH POLYANGIITIS MIMICKING CERVICAL CANCER

2019 ◽  
Author(s):  
ANA JULIA FURBINO DIAS BICALHO ◽  
DEBORAH LOBATO GUIMARAES ◽  
HENRIQUE VALLADAO PIRES GAMA ◽  
MOISES SALGADO PEDROSA ◽  
DANIELA DE SOUZA BRAGA ◽  
...  
Keyword(s):  
Cervical Cancer ◽  
Granulomatosis With Polyangiitis ◽  
Rare Presentation

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 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.

scholarly journals Sensory-Motor Polyneuropathy and Digital Ischemia: A Rare Presentation of Granulomatosis with Polyangiitis

2021 ◽  
Vol 2021 ◽  
pp. 1-6
Author(s):  
Wasundara Wathurapatha ◽  
B. G. A. Rathnamali ◽  
Upul Dissanayake
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Renal Involvement ◽  
Immunosuppressive Treatment ◽  
Lower Limbs ◽  
Rare Presentation ◽  
Clinical Criteria ◽  
Sensorimotor Polyneuropathy ◽  
Digital Ischemia ◽  
Sensory Motor ◽  
Peripheral Sensory

Granulomatosis with polyangiitis (GPA) typically presents with upper or lower respiratory tract symptoms and/or with renal involvement. Although it can affect the peripheral nervous system frequently, with mononeuritis multiplex being the most common pattern, the occurrence of peripheral sensory-motor polyneuropathy as a presenting manifestation is distinctly rare. Prevalence of digital gangrene is also extremely rare in GPA. We describe a 46-year-old woman presenting with severe peripheral sensorimotor polyneuropathy affecting bilateral lower limbs preceded by a purpuric skin rash and multiple painful ulcers confined to the lower limbs. She had evidence of digital ischemia affecting multiple toes and dry gangrene of the left 4th toe. Diagnosis of GPA was made based on skin biopsy, positive ANCA serology, and clinical criteria. She made a good recovery following aggressive immunosuppressive treatment with methylprednisolone and cyclophosphamide and was maintained on prednisolone and azathioprine. This case highlights the importance of suspecting GPA in a patient presenting with sensorimotor polyneuropathy and/or digital ischemia even in the absence of more classic presenting features and underlies the necessity of accurate differential diagnosis in evaluating a case of peripheral neuropathy.

scholarly journals Rare presentation of granulomatosis with polyangiitis

2019 ◽  
Vol 12 (2) ◽  
pp. e227218
Author(s):  
Adam Hines ◽  
Vered Bello ◽  
Asma Iftikhar ◽  
Hadi Zein
Keyword(s):  
Inflammatory Markers ◽  
Granulomatosis With Polyangiitis ◽  
Channel Blocker ◽  
Outpatient Setting ◽  
Blood Cell Count ◽  
Rare Presentation ◽  
Lower Extremity Weakness ◽  
Intravenous Steroid ◽  
Lost To Follow Up

A 62-year-old man with no pertinent medical history presented with lower extremity weakness and worsening distal fingertips and toe cyanosis/gangrene. In the outpatient setting, he was initially being treated for Raynaud’s phenomenon with a calcium channel blocker. On presentation, the patient had elevated inflammatory markers and white blood cell count. Serum vasculitis panel (proteinase-3 antibody) supported the diagnosis of granulomatosis with polyangiitis. His hospital course was complicated by ischaemic stroke and a diagnosis of mononeuritis multiplex in his lower extremities. After initiating therapy with intravenous steroid and rituximab, his symptoms overall improved including cyanotic fingertips/toes. His inflammatory markers and leucocytosis also improved. Outpatient follow-up consisted of further rituximab infusions and unrelated umbilical hernia incarceration which required surgery. He was found incidentally to have subsegmental pulmonary emboli which most likely occurred during the initial presentation prior to his diagnosis. The patient moved out of state and was lost to follow-up.

scholarly journals Use of Plasmapheresis and Immunosuppressants to Treat Diffuse Alveolar Hemorrhage in a Patient with Granulomatosis with Polyangiitis

Medicina ◽  
2019 ◽  
Vol 55 (7) ◽  
pp. 378
Author(s):  
Yasar Sattar ◽  
Ammu Thampi Susheela ◽  
Waqas Ullah ◽  
Norina Usman ◽  
Fnu Zafrullah
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Hemodynamic Instability ◽  
Acute Onset ◽  
Diffuse Alveolar Hemorrhage ◽  
Alveolar Hemorrhage ◽  
Severe Anemia ◽  
Rare Presentation ◽  
Pulmonary Manifestations ◽  
Remarkable Recovery ◽  
Pulse Steroids

Granulomatosis with polyangiitis (GPA) is a systemic granulomatous inflammatory disease characterized by small-to-medium vessel vasculitis due to Central Anti-Neutrophil Cytoplasmic Antibody (C-ANCA). GPA commonly involves the lungs and the kidneys. Among the pulmonary manifestations, diffuse alveolar hemorrhage (DHA) is a rare presentation of GPA that can present with hemoptysis leading to acute onset of anemia and hemodynamic instability. An active diagnostic workup including serologic titer of C-ANCA, imaging, intensive care, and aggressive immunosuppression is the key to DAH management. We report a case of DAH secondary to GPA that presented with hemoptysis leading to severe anemia, initially resuscitated symptomatically and started on plasmapheresis with pulse steroids and cyclophosphamide. Timely diagnosis and management led to a remarkable recovery of the pulmonary symptoms and imaging findings of DAH.

scholarly journals Prostatitis, a rare presentation of granulomatosis with polyangiitis, successfully treated with rituximab and prednisone

2018 ◽  
pp. bcr-2018-225379
Author(s):  
Muhammad Saad Shaukat ◽  
Anthony J Ocon ◽  
Ruben A Peredo ◽  
Birju D Bhatt
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Rare Presentation

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.

Sign in / Sign up

Export Citation Format

Share Document