Wegener's granulomatosis: Cutaneous and oral mucosal disease

doi:10.1016/0190-9622(93)70098-E

Journal of the American Academy of Dermatology

Volume 28, Issue 5, Part 1, May 1993, Pages 710-718

https://doi.org/10.1016/0190-9622(93)70098-E Get rights and content

Background: Wegener's granulomatosis is a complex disease that can be difficult to diagnose, especially if the classic triad of necrotizing granulomas of the respiratory tract, generalized vasculitis, and glomerulitis are not present. A delay in diagnosis may result in irreversible kidney damage and ultimately death.

Objective: This study was done to identify clinical and histologic findings of the skin and oral mucosa that may aid in the diagnosis of this disease.

Methods: The records of 40 patients with Wegener's granulomatosis were reviewed.

Results: Of these patients 12 had cutaneous lesions, 2 had oral mucosal lesions, and 2 had both cutaneous and oral mucosal lesions. Cutaneous and oral mucosal lesions were a presenting sign in 10% and 5% of patients, respectively. Overall, 25% demonstrated at least one of the specific cutaneous histopathologic findings described for Wegener's granulomatosis, that is, necrotizing vasculitis, granulomatous vasculitis, and palisading granuloma. One patient had gingival hyperplasia with petechiae, considered to be pathognomonic for Wegener's granulomatosis.

Conclusion: This study demonstrates that cutaneous or oral mucosal findings may serve as valuable diagnostic aids when evaluating a patient suspected of having Wegener's granulomatosis.

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Case of strawberry gum associated with polyangiitis granulomatosa in relation to cytoplasmic antineutrophil cytoplasmic antibody
2024, Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology
Granulomatosis with polyangiitis (GPA), formerly known as Wegener’s granulomatosis, presents histopathologically as necrotizing granulomatous vasculitis of the entire body, necrotizing granulomatosis (mainly of the upper respiratory tract and lungs), and semilunar-forming nephritis. Gingival swelling due to GPA is a rare condition, described as “strawberry gum” because of the fragile, reddish-purple, granulomatous swollen appearance of the gingiva with petechial hemorrhagic plaques, that resembles an overripe strawberry. Herein, we report the case of a 75-year-old man who presented with chief complaints of gingival swelling and pain with generalized gingival bleeding. A 15 × 10-mm granular hemorrhagic mass was observed on the labial gingiva of the left maxillary anterior tooth and multiple other gingivae. After histopathological examination, gingival lesions associated with GPA were suspected. The patient was diagnosed with GPA approximately 9 years ago and had repeated remissions and relapses with prednisolone treatment. Because the cytoplasmic antineutrophil cytoplasmic antibody (cANCA) levels were elevated at the first medical examination, the patient was referred to an immunologist who increased the prednisolone dose, resulting in a decrease in the cANCA levels and resolution of the patient's strawberry gum. The patient's cANCA levels remained low the following year, and strawberry gum did not recur. The cANCA levels have continued to fluctuate after the GAP diagnosis; however, strawberry gum did not develop. Thus, cANCA levels do not predict the development of strawberry gum but must be low to prevent the recurrence of strawberry gum. This case report demonstrated a previously unexplained relationship between strawberry gum and cANCA.
A 44 years old patient with lip ulcer and cervical lymphadenopaty
2021, Medicina Clinica Practica
Atypical purpuric oedema of the nose during granulomatosis with polyangiitis
2020, Annales de Dermatologie et de Venereologie
La granulomatose avec polyangéite (GPA), anciennement maladie de Wegener, est une vascularite nécrosante atteignant les vaisseaux de petit et moyen calibre associée aux anticorps anti-cytoplasme des polynucléaires neutrophiles (ANCA). Elle se manifeste habituellement par une atteinte pulmonaire, rénale et des voies aériennes supérieures mais également des signes dermatologiques.
Nous rapportons le cas d’une patiente de 47 ans hospitalisée pour le bilan de deux nodules pulmonaires qui présentait des plaques œdémateuses purpuriques avec décollement bulleux du nez. On notait dans ses antécédents plusieurs épisodes d’otites séromuqueuses et un accident vasculaire cérébral récent. Le diagnostic de GPA, évoqué devant l’atteinte systémique et les manifestations cutanées, était confirmé par les images histologiques et la présence d’ANCA anti-Pr3. L’évolution était favorable avec un traitement associant corticothérapie générale et rituximab.
Le diagnostic de GPA est basé sur les critères établis par l’American College of Rheumatology. Comme dans notre cas, les manifestations dermatologiques sont souvent polymorphes. Elles sont rarement isolées mais permettent parfois d’évoquer le diagnostic précocement et d’améliorer ainsi le pronostic qui reste sombre en l’absence de traitement.
Granulomatosis with polyangeitis or Wegener's disease is a necrotizing vasculitis of small and medium vessels associated with antineutrophil cytoplasmic autoantibodies (ANCA). The most frequent sites are lung, ear, nose and throat and kidney.
We report the case of a 47-year-old woman presenting purpuric oedematous plaque with bullous detachment of the nose and hospitalised for the assessment of two suspicious neoplastic lung lesions discovered as a result of a recent stroke and repeated seromucosal otitis. Granulomatosis with polyangeitis was suspected because of multiple systemic lesions. The histopathology of skin lesions and laboratory investigation results were consistent with this diagnosis. A favourable outcome was achieved with corticosteroids and rituximab.
The diagnosis of GPA is based on criteria established by the American College of Rheumatology. The cutaneous clinical aspect described in our case confirms the polymorphism of the cutaneous lesions possibly associated with this disease. They are rarely isolated but, in some cases, allow early diagnosis with improved prognosis, which remains severe in the absence of treatment.
Three Patterns of Cutaneous Involvement in Granulomatosis with Polyangiitis
2020, Journal of Advanced Research
Skin involvement in granulomatosis with polyangiitis (GPA) is common and can appear as an initial presentation of the disease or more commonly through its course.
We report a case of a 24-year-old male patient, previously diagnosed as having GPA, admitted with fever, hemoptysis, generalized hemorrhagic blisters associated with arthralgia, fatigue, myalgia, nasal crusting, and vertigo. Three weeks prior to admission, he developed erythematous papules on both elbows, and purpuric papules on both lower limbs. Histopathological examination revealed: interstitial granulomatous dermatitis (elbows) and foci of dermal hemorrhage, foci of interstitial histiocytes and zones of altered necrobiotic collagen (lower limbs) consistent with cutaneous lesions of GPA. Two weeks later, his rash progressed to widespread purpura associated with hemorrhagic blisters. Another biopsy revealed leukocytoclastic vasculitis with fibrinoid necrosis of the vessel walls associated with perivascular infiltrate of neutrophils, nuclear dust and extravasated erythrocytes without an associated granulomatous inflammation or necrobiosis. The constellation of the results of the three biopsies together with clinical correlation pointed to a flare of GPA.
Skin involvement in GPA is quite common, and it can manifest in different forms in the same patient. Our patient developed three different skin pathologies within a short period of time.
Strawberry gingivitis: Challenges in the diagnosis of granulomatosis with polyangiitis on gingival specimens
2019, Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology
Citation Excerpt :
For severe organ-threatening ANCA-associated vasculitis, a single rituximab-based regimen, in combination with glucocorticoids, is considered to be as effective as 18 months of a conventional cyclophosphamide-based regimen with glucocorticoids followed by maintenance with azathioprine.31 In about 5% to 6% of cases, the main presenting oral manifestation of GPA is gingivitis or oral ulcers.10,32 An initial oral manifestation of strawberry gingivitis in the absence of other symptoms is very rare.17
Strawberry gingivitis is a rare oral manifestation of granulomatosis with polyangiitis (GPA, formerly known as Wegener's granulomatosis). It manifests as a red-purple hyperplastic gingivitis that frequently goes unrecognized as a disease-specific symptom, especially if it is the primary and only manifestation of the disease. GPA is a systemic necrotizing granulomatous vasculitis that takes a fatal course without treatment. Oral health care providers, who are among the first to examine the oral cavity, should be familiar with its typical appearance, clinical course, diagnostic parameters, and management.
This article highlights the challenges to early-stage diagnosis of initial multiple gingival enlargements because histologic biopsies are often nonspecific and histology alone may not be sufficient to make a correct diagnosis of GPA from gingival specimens. Because strawberry gingivitis may be the first manifestation of GPA, awareness of it should be increased so that it can be diagnosed by its unique clinical appearance and additional related diagnostic parameters even if the histologic gingival findings are nonspecific.
Multiple oral ulcerations: A very rare case of Churg-Strauss syndrome with renal disease
2018, Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology
Oral ulcerations are an extremely rare manifestation of Churg-Strauss Syndrome (CSS), which have not yet been documented as a potential prodromal sign of this multisystemic autoimmune vasculitis which may also involve the kidneys. The authors present a very rare case report of widespread oral ulcerations which preceded the onset of CSS with crescentic glomerulonephritis and persisted throughout the course of the disease. Oral manifestations included atypical clusters of oral aphthae and other lingual and mucosal ulcerations. The case demonstrates the disease spectrum and review of current understanding of this disease. The dentist may be the first health care professional to see patients with symptoms and findings of this condition. Definitive diagnosis is challenging owing to the subtle onset of the disease and variable clinical and laboratory findings. Clinicians should be informed about the possibility of oral manifestations of CSS to facilitate prompt disease recognition and to provide continued oral health care to these medically complex patients. Early diagnosis and treatment is the most important factor in the management of this potentially fatal disease as well as to promote quicker remission.

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¹: From the Department of Dermatology, Cleveland Clinic Foundation.

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Wegener's granulomatosis: Cutaneous and oral mucosal disease

Am J Med

Am J Med

Am J Med

Hum Pathol

Oral Surg

J Am Acad Dermatol

Oral Surg

Oral Surg

J Am Acad Dermatol

Oral Surg Oral Med Oral Pathol

Am J Med

Grenzformen der periarteritis nodosa

Z Path

Uber generalasierte septishe gefasserkrankungen

Verh Dtsch Pathol Ges

Wegener's granulomatosis: pathology and review of the literature

Arch Pathol

Limited Wegener's granulomatosis: unusual cutaneous, radiographic and pathologic manifestations

Cutis

Indolent Wegener's granulomatosis

Ann Rheum Dis

Cutaneous manifestations of Wegener's granulomatosis

Arch Dermatol

Wegener's granulomatosis: studies in 18 patients and review of the literature

Medicine