Dermatologic Manifestations of Granulomatosis With Polyangiitis (Wegener Granulomatosis) 

Updated: May 12, 2016
  • Author: Meera Eisa Ali AlMatrooshi; Chief Editor: Dirk M Elston, MD  more...
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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 papules Several necrotic, purpuric, and blistering papules and plaques on the hands.
Necrotic, purpuric, and blistering plaque on the w Necrotic, purpuric, and blistering plaque on the wrist.
Large ulceration of the pharynx covered with a den Large 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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