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Cutaneous Ulcers as Initial Presentation of Localized Granulomatosis with Polyangiitis: A Case Report and Review of the Literature.

Nasir N, Ali SA, Mehmood Riaz HM - Case Rep Rheumatol (2015)

Bottom Line: Conclusion.In the absence of typical manifestations, granulomatosis with polyangiitis (GPA) is indeed a diagnostic challenge to the physician.Atypical manifestations like unexplained recurrent fever and cutaneous ulcers nevertheless call for keeping a low threshold for the diagnosis of GPA as the disease can initially present in localized form before heralding into a generalized disease.

View Article: PubMed Central - PubMed

Affiliation: Section of Internal Medicine, Department of Medicine, The Aga Khan University Hospital, P.O. Box 3500, Stadium Road, Karachi 74800, Pakistan.

ABSTRACT
Background. Granulomatosis with polyangiitis (GPA) is an ANCA associated small vessel vasculitis characterized by necrotizing granulomatous inflammation involving the upper and the lower respiratory tract and the kidneys. The disease has a broad clinical spectrum that ranges from limited/localized involvement of a single organ system to a generalized systemic vasculitis that affects several organs with evidence of end organ damage. Atypical forms of the disease have been recognized with or without respiratory tract involvement with a long protracted course before manifesting as generalized disease. Case Presentation. We describe a 57-year-old woman who presented with recurrent fever and cutaneous ulcers on her legs who was diagnosed to have granulomatosis with polyangiitis (GPA) after an extensive evaluation which excluded infectious, other vasculitides, connective tissue disease and malignant etiologies. Conclusion. In the absence of typical manifestations, granulomatosis with polyangiitis (GPA) is indeed a diagnostic challenge to the physician. Atypical manifestations like unexplained recurrent fever and cutaneous ulcers nevertheless call for keeping a low threshold for the diagnosis of GPA as the disease can initially present in localized form before heralding into a generalized disease.

No MeSH data available.


Related in: MedlinePlus

Ultrasound upper abdomen. Multiple hypoechoic tiny lesions on both lobes, few of them cystic in appearance.
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fig2: Ultrasound upper abdomen. Multiple hypoechoic tiny lesions on both lobes, few of them cystic in appearance.

Mentions: The remainder of the examination was unremarkable. Her complete blood count revealed normocytic, normochromic anemia with normal white cell and platelet count. Her biochemistry showed normal kidney and liver function. No active sediment was present on urinalysis. Her three sets of blood cultures and one urine culture reported no growth. The test for latent tuberculosis by interferon gamma release assay (IGRA) was also negative. Serology for Brucella abortus and melitensis also came negative. Tests for chronic viral hepatitis B and C were also normal. Serological tests for connective tissue disease were negative as was the serum angiotensin converting enzyme level. Other relevant investigations are outlined in Table 1. An upper abdominal ultrasound showed hypoechoic, cystic lesions scattered across both lobes of the liver (Figure 2). A subsequent CT scan of the abdomen with contrast showed multiple oval hypoattenuating lesions in both lobes of the liver (Figure 3).


Cutaneous Ulcers as Initial Presentation of Localized Granulomatosis with Polyangiitis: A Case Report and Review of the Literature.

Nasir N, Ali SA, Mehmood Riaz HM - Case Rep Rheumatol (2015)

Ultrasound upper abdomen. Multiple hypoechoic tiny lesions on both lobes, few of them cystic in appearance.
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4664794&req=5

fig2: Ultrasound upper abdomen. Multiple hypoechoic tiny lesions on both lobes, few of them cystic in appearance.
Mentions: The remainder of the examination was unremarkable. Her complete blood count revealed normocytic, normochromic anemia with normal white cell and platelet count. Her biochemistry showed normal kidney and liver function. No active sediment was present on urinalysis. Her three sets of blood cultures and one urine culture reported no growth. The test for latent tuberculosis by interferon gamma release assay (IGRA) was also negative. Serology for Brucella abortus and melitensis also came negative. Tests for chronic viral hepatitis B and C were also normal. Serological tests for connective tissue disease were negative as was the serum angiotensin converting enzyme level. Other relevant investigations are outlined in Table 1. An upper abdominal ultrasound showed hypoechoic, cystic lesions scattered across both lobes of the liver (Figure 2). A subsequent CT scan of the abdomen with contrast showed multiple oval hypoattenuating lesions in both lobes of the liver (Figure 3).

Bottom Line: Conclusion.In the absence of typical manifestations, granulomatosis with polyangiitis (GPA) is indeed a diagnostic challenge to the physician.Atypical manifestations like unexplained recurrent fever and cutaneous ulcers nevertheless call for keeping a low threshold for the diagnosis of GPA as the disease can initially present in localized form before heralding into a generalized disease.

View Article: PubMed Central - PubMed

Affiliation: Section of Internal Medicine, Department of Medicine, The Aga Khan University Hospital, P.O. Box 3500, Stadium Road, Karachi 74800, Pakistan.

ABSTRACT
Background. Granulomatosis with polyangiitis (GPA) is an ANCA associated small vessel vasculitis characterized by necrotizing granulomatous inflammation involving the upper and the lower respiratory tract and the kidneys. The disease has a broad clinical spectrum that ranges from limited/localized involvement of a single organ system to a generalized systemic vasculitis that affects several organs with evidence of end organ damage. Atypical forms of the disease have been recognized with or without respiratory tract involvement with a long protracted course before manifesting as generalized disease. Case Presentation. We describe a 57-year-old woman who presented with recurrent fever and cutaneous ulcers on her legs who was diagnosed to have granulomatosis with polyangiitis (GPA) after an extensive evaluation which excluded infectious, other vasculitides, connective tissue disease and malignant etiologies. Conclusion. In the absence of typical manifestations, granulomatosis with polyangiitis (GPA) is indeed a diagnostic challenge to the physician. Atypical manifestations like unexplained recurrent fever and cutaneous ulcers nevertheless call for keeping a low threshold for the diagnosis of GPA as the disease can initially present in localized form before heralding into a generalized disease.

No MeSH data available.


Related in: MedlinePlus