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Dermatologic Manifestations of Granulomatosis With Polyangiitis (Wegener Granulomatosis) 

  • Author: Meera Eisa Ali AlMatrooshi; Chief Editor: Dirk M Elston, MD  more...
 
Updated: May 12, 2016
 

Granulomatosis with Polyangiitis

Granulomatosis with polyangiitis (formerly known as Wegener granulomatosis) is a multisystem disease characterized by a disseminated necrotizing inflammation involving the small blood vessels and the surrounding tissue. The vast majority of patients are positive for antineutrophil cytoplasmic antibodies, which are hypothesized to play a major role in the pathogenesis of the disease.[1]

Clinical manifestations are widely variable, depending on the stage of the disease and the organs affected. The respiratory tract and the kidneys are almost always affected. Patients usually present with nonspecific symptoms, which include fever, malaise, weakness, arthralgia, anorexia, and weight loss.[2]

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Types of Skin Involvement

Of patients with the disease, 30-46% present with dermatologic manifestations.[2] These manifestations include palpable purpura, papules, vesicles, subcutaneous nodules, plaques, and ulcers, which may resemble pyoderma gangrenosum.[3] Rarely, digital gangrene may develop owing to involvement of the digital arteries.[4] Splinter hemorrhages may also occur, confusing the diagnosis with endocarditis.[5] In 13% of patients, cutaneous symptoms are the initial manifestation of the disease.[3] They rarely dominate the clinical picture though; instead, they parallel disease activity in other organs. Skin lesions are usually indicative of an active systemic disease.[6] They are typically located on the lower extremities, but they can also manifest on the face, upper extremities, and the extensor surfaces of the joints. Oral and nasal ulcerations may also occur.[3] Dermatologic manifestations may be treated with topical steroids or surgery in cases of severe tissue damage due to fibrosis or necrosis.[7]

See the images below.

Several necrotic, purpuric, and blistering papulesSeveral necrotic, purpuric, and blistering papules and plaques on the hands.
Necrotic, purpuric, and blistering plaque on the wNecrotic, purpuric, and blistering plaque on the wrist.
Large ulceration of the pharynx covered with a denLarge ulceration of the pharynx covered with a dense necrotic membrane.
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Histopathological Findings

A wide spectrum of histopathologic features may be seen when examining a skin biopsy specimen. Many biopsy specimens reveal nonspecific patterns, but the most common finding is leukocytoclastic vasculitis, which presents clinically as palpable purpura.[8] Other common histological findings include perifollicular and dermal granulomatous inflammation and palisaded neutrophilic and granulomatous inflammation.[9] Necrotizing granulomatous inflammation has been reported, but it is rarely seen in skin biopsy specimens; it is usually found in biopsies from medium-sized vessels.[9]

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Diagnosis

Histopathological examination is required to make a reliable diagnosis of granulomatosis with polyangiitis.[5] Measuring antineutrophil cytoplasmic antibody titers may also help in diagnosing patients who exhibit cutaneous lesions with histological findings showing leukocytoclastic vasculitis. Positive serological tests for these autoantibodies support the suspicion for granulomatosis with polyangiitis. If the cutaneous lesions are the only clinical manifestation without any other organ involvement, patients should be screened regularly for signs of systemic involvement.[8]

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Differential Diagnosis

Disorders to be considered in the differential diagnosis of cutaneous granulomatosis with polyangiitis (Wegener granulomatosis) lesions are listed below.

Other vasculitides include the following:

  • Polyarteritis nodosa
  • Microscopic polyangiitis
  • Churg-Strauss syndrome
  • Henoch-Schönlein purpura
  • Mixed cryoglobulinemia
  • Goodpasture syndrome
  • Giant cell arteritis

Infections include the following:

  • Mycobacterial diseases
  • Fungal infections (histoplasmosis, blastomycosis, coccidioidomycosis)
  • Streptococcal pneumonia with glomerulonephritis

Malignancies include the following:

  • Nasopharyngeal carcinoma
  • Hodgkin disease
  • Non-Hodgkin lymphoma
  • Angiocentric lymphoma (lymphomatoid granulomatosis)
  • Castleman disease

Granulomatous disorders include the following:

  • Sarcoidosis
  • Berylliosis

Systemic autoimmune conditions include the following:

  • Systemic lupus erythematosus
  • Rheumatoid arthritis
  • Relapsing polychondritis[5]
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Contributor Information and Disclosures
Author

Meera Eisa Ali AlMatrooshi United Arab Emirates (UAE) University College of Medicine and Health Scienes

Disclosure: Nothing to disclose.

Coauthor(s)

Hassan I Galadari, MD Assistant Professor of Dermatology, Faculty of Medicine and Health Sciences, United Arab Emirates (UAE) University, UAE

Hassan I Galadari, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, American Medical Student Association/Foundation, American Society for Dermatologic Surgery

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: American Medical Association, Alpha Omega Alpha, Association of Military Dermatologists, American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Phi Beta Kappa

Disclosure: Nothing to disclose.

Jeffrey P Callen, MD Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, American College of Rheumatology

Disclosure: Received income in an amount equal to or greater than $250 from: XOMA; Biogen/IDEC; Novartis; Janssen Biotech, Abbvie, CSL pharma<br/>Received honoraria from UpToDate for author/editor; Received honoraria from JAMA Dermatology for associate editor and intermittent author; Received royalty from Elsevier for book author/editor; Received dividends from trust accounts, but I do not control these accounts, and have directed our managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various trust accounts include some pharmaceutical companies and device makers for i inherited these trust accounts; for: Celgene; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble; Amgen.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Christen M Mowad, MD Professor, Department of Dermatology, Geisinger Medical Center

Christen M Mowad, MD is a member of the following medical societies: Alpha Omega Alpha, Noah Worcester Dermatological Society, Pennsylvania Academy of Dermatology, American Academy of Dermatology, Phi Beta Kappa

Disclosure: Nothing to disclose.

David Hensley, MD Mullanax Dermatology Associates, Arlington Memorial Hospital

David Hensley, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Pennsylvania Medical Society, Southern Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

Acknowledgements

Lindsey Ann Dohse, MD Resident Physician, Department of Dermatology, Geisinger Health System

Lindsey Ann Dohse, MD is a member of the following medical societies: Alpha Omega Alpha

Disclosure: Nothing to disclose.

References
  1. Kasper D, Fauci A, Hauser S, Longo D, Jameson J, Loscalzo J, et al. The Vasculitis Syndromes. Harrison's Principles of Internal Medicine. 19th ed. New York, NY: McGraw-Hill; 2015.

  2. Ferri F. Granulomatosis with Polyangiitis (Wegener’s Granulomatosis). Ferri's Clinical Advisor. Elsevier; 2016. 555-56.

  3. Wolff K, Johnson R, Saavedra A.P. The Skin in Immune, Autoimmune, and Rheumatic Disorders. Fitzpatrick's Color Atlas and Synopsis of Clinical Dermatology. 7th ed. New York, NY: McGraw-Hill; 2013.

  4. Avcin T, Singh-Grewal D, Silverman ED, Schneider R. Severe digital ischaemia in a child with Wegener's granulomatosis. Scand J Rheumatol. 2007 Jan-Feb. 36 (1):78-80. [Medline].

  5. Stone JH. Granulomatosis with Polyangiitis (Wegener Granulomatosis). Current Diagnosis & Treatment in Rheumatology. 3rd ed. New York, NY: McGraw-Hill; 2013.

  6. Francès C, Du LT, Piette JC, Saada V, Boisnic S, Wechsler B, et al. Wegener's granulomatosis. Dermatological manifestations in 75 cases with clinicopathologic correlation. Arch Dermatol. 1994 Jul. 130(7):861-7. [Medline].

  7. Hernandez-Rodriguez J, Hoffman GS, Koening CL. Surgical interventions and local therapy for Wegener's granulomatosis. Curr Opin Rheumatol. 2010 Jan. 22(1):29-36. [Medline].

  8. Comfere NI, Macaron NC, Gibson LE. Cutaneous manifestations of Wegener's granulomatosis: a clinicopathologic study of 17 patients and correlation to antineutrophil cytoplasmic antibody status. J Cutan Pathol. 2007 Oct. 34 (10):739-47. [Medline].

  9. Clarke LE, Clarke JT, Helm KF. The Vasculopathic Pattern. Color Atlas of Differential Diagnosis in Dermatopathology. Jp Medical Ltd; 219.

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Large ulceration of the pharynx covered with a dense necrotic membrane.
Necrotic, purpuric, and blistering plaque on the wrist.
Several necrotic, purpuric, and blistering papules and plaques on the hands.
 
 
 
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