Dermatologic Manifestations of Wegener Granulomatosis 

  • Author: Christen M Mowad, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Aug 3, 2011
 

Types of Skin Involvement

Dermatologic manifestations occur in 35-50% of patients with Wegener granulomatosis (WG). Subcutaneous nodules, papules, vesicles, ulcers, petechiae, pyogenic gangrenosum, and Raynaud phenomenon have been reported.[1] Ulcers and vesicles are associated with more severe and extensive disease. Involvement of the oral mucosa may also occur.[2]

Cutaneous manifestations may be the presenting sign of disease in 13% of patients with WG.[3] Patients exhibiting cutaneous manifestations more commonly have renal and articular disease.[4] Skin involvement rarely dominates the clinical picture; instead, it parallels disease activity in other organs. The skin lesions generally respond to topical anti-inflammatory agents.[5]

Histopathologic findings may be as variable as cutaneous manifestations. More than half of all skin biopsy specimens return nonspecific findings. Necrotizing vasculitis, granulomatous vasculitis, extravascular palisading granulomas, and leukocytoclastic vasculitis are reported most commonly.[6, 7]

Necrotizing vasculitis is usually correlated with petechiae, purpura, and ecchymoses (especially on the lower extremities). Palisading granulomas are most commonly observed with indurated nodules on the upper extremities. Granulomatous vasculitis is associated with erythematous papules, nodules, and ulcerations.

For further information on this topic, see the Medscape Reference article Wegener Granulomatosis, as well as Neurologic Manifestations of Wegener Granulomatosis and Otolaryngologic Manifestations of Wegener Granulomatosis.

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Patient History and Physical Exam

Patients may report the following chronic, nonspecific constitutional symptoms:

  • Fever
  • General malaise
  • Fatigue
  • Arthralgias
  • Weight loss

Various cutaneous eruptions or ulcerations may be the only presenting symptoms.Cutaneous findings are variable and usually nonspecific. The lower extremities are most commonly affected.

Palpable purpura, papules, subcutaneous nodules, and ulcerations are the most common findings. Ulcerations may resemble pyoderma gangrenosum (see Pyoderma Gangrenosum).

Petechiae, vesicles, pustules, hemorrhagic bullae, livedo reticularis, digital necrosis, subungual splinter hemorrhages, and genital ulcers resembling squamous cell carcinoma have been reported. (See the images below.)

Necrotic, purpuric, and blistering plaque on the wNecrotic, purpuric, and blistering plaque on the wrist. Several necrotic, purpuric, and blistering papulesSeveral necrotic, purpuric, and blistering papules and plaques on the hands.
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Differential Diagnosis

Disorders to be considered in the differential diagnosis of cutaneous Wegener granulomatosis lesions include the following:

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Contributor Information and Disclosures
Author

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

Christen M Mowad, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Coauthor(s)

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, and Texas Medical Association

Disclosure: Nothing to disclose.

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.

Specialty Editor Board

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

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

Disclosure: Amgen Honoraria Consulting; Abbott Honoraria Consulting; Electrical Optical Sciences Consulting fee Consulting; Celgene Honoraria Safety Monitoring Committee; GSK - Glaxo Smith Kline Consulting fee Consulting; TenXBioPharma Consulting fee Safety Monitoring Committee

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

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

Disclosure: Nothing to disclose.

References

  1. 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. Jul 1994;130(7):861-7. [Medline].

  2. Patten SF, Tomecki KJ. Wegener's granulomatosis: cutaneous and oral mucosal disease. J Am Acad Dermatol. May 1993;28(5 Pt 1):710-8. [Medline].

  3. Hu CH, O'Loughlin S, Winkelmann RK. Cutaneous manifestations of Wegener granulomatosis. Arch Dermatol. Feb 1977;113(2):175-82. [Medline].

  4. Daoud MS, Gibson LE, DeRemee RA, Specks U, el-Azhary RA, Su WP. Cutaneous Wegener's granulomatosis: clinical, histopathologic, and immunopathologic features of thirty patients. J Am Acad Dermatol. Oct 1994;31(4):605-12. [Medline].

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

  6. Barksdale SK, Hallahan CW, Kerr GS, Fauci AS, Stern JB, Travis WD. Cutaneous pathology in Wegener's granulomatosis. A clinicopathologic study of 75 biopsies in 46 patients. Am J Surg Pathol. Feb 1995;19(2):161-72. [Medline].

  7. Mangold MC, Callen JP. Cutaneous leukocytoclastic vasculitis associated with active Wegener's granulomatosis. J Am Acad Dermatol. Apr 1992;26(4):579-84. [Medline].

 
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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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