Granulomatosis with Polyangiitis Presenting with Pyoderma Gangrenosum–Like Ulceration and Negative Cytoplasmic Antineutrophilic Cytoplasmic Antibodies in a Child

Kass, Ana Isabel García; Fagan, Jake; Long, Paul

doi:10.1111/pde.13230

Cited by 8 publications

(7 citation statements)

References 9 publications

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“…Several pediatric cases of GPA have been reported with PG-like ulcers mainly presenting on the face as the initial presenting manifestation of the disease. This can even precede the classic systemic symptoms [23][24][25][26][27] [Figure 9]. Localized variants of GPA may present with long standing recalcitrant ulcerative skin lesions mainly localized to the face and upper respiratory tract mucosa [28,29] .…”

Section: Rare or Unusual Clinical Presentations Of Vasculitic Skin Le...mentioning

confidence: 99%

Cutaneous manifestations of anti-neutrophil cytoplasmic antibody associated vasculitis

Abdel‐Halim¹,

Mahmoud²,

Ragab³

2022

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Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a distinct group of systemic vasculitis with severe multi-organ involvement. It includes three types: granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA), and microscopic polyangiitis (MPA). Cutaneous manifestations are present at the time of presentation in around 35% of cases and are more frequently encountered in EGPA than in GPA or MPA. They can also occur during the course of the disease or mark a disease relapse. They can be classified into specific or non-specific according to the presence or absence of vasculitis, whether or not associated with granuloma, in skin biopsies. The most common presentation of vasculitic skin lesions in AAV is palpable purpura. Other manifestations include hemorrhagic blisters, tender subcutaneous nodules, livedo reticularis/racemosa, painful ulcers that may mimic pyoderma gangrenosum (PG), and digital gangrenes. A wide range of non-specific cutaneous manifestations can occur in association with AAV including urticarial rash, prurigo, sterile pustules, and oral lesions. The presence of cutaneous lesions is usually associated with severe organ involvement and systemic manifestations. Since skin lesions in AAV can occur in other types of vasculitis and in other disorders, attributing them to AAV requires meticulous clinical, laboratory, and serological correlation. Awareness of cutaneous lesions of AAV is important for all physicians working in the field of systemic vasculitis as a skin biopsy may provide an easy clue to diagnose AAV in such cases.

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Section: Rare or Unusual Clinical Presentations Of Vasculitic Skin Le...mentioning

confidence: 99%

Cutaneous manifestations of anti-neutrophil cytoplasmic antibody associated vasculitis

Abdel‐Halim¹,

Mahmoud²,

Ragab³

2022

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“…Characteristic PG‐related comorbidities are inflammatory bowel disease, haematological malignancies and arthritis, whereas AAV is commonly associated with pulmonary involvement, upper respiratory tract lesions and renal disease 3 . ANCA positivity supports a diagnosis of AAV, but a negative ANCA test should not exclude the diagnosis 5 . In a systematic review of 28 published cases of GPA with PG‐like ulcers, most patients had ANCA positivity of PR3 (21/28).…”

Section: Reportmentioning

confidence: 99%

“…1 Palpable purpura with leucocytoclastic vasculitis (LCV) are the most common manifestations of AAV; however, although less common, ulceration of the skin with features akin to pyoderma gangrenosum (PG) can also be seen, particularly in granulomatosis with polyangiitis (GPA). 2,3 Several case reports have described PG-like ulceration in association with AAV, 4,5 but its true incidence is unknown. In addition, PG is frequently misdiagnosed because diagnosis may rely on exclusion of other causes of ulcers.…”

Section: Antineutrophilmentioning

confidence: 99%

“…3 ANCA positivity supports a diagnosis of AAV, but a negative ANCA test should not exclude the diagnosis. 5 In a systematic review of 28 published cases of GPA with PG-like ulcers, most patients had ANCA positivity of PR3 (21/28). In fact, a considerable percentage of patients with AAV can have negative ANCA testing either initially or remain sero-negative.…”

Section: Reportmentioning

confidence: 99%

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Pyoderma gangrenosum‐like skin ulceration in antineutrophil cytoplasmic antibody‐associated vasculitis: a retrospective case series

Shakshouk¹,

Gibson

2022

Clin Experimental Derm

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Antineutrophil cytoplasmic antibody‐associated vasculitis (AAV) can be associated with various cutaneous manifestations. Several case reports have described skin ulceration resembling pyoderma gangrenosum (PG), particularly in granulomatosis with polyangiitis (GPA); however, the true incidence of this PG‐like ulceration in various diseases is unknown. In addition, PG is frequently misdiagnosed, and diagnosis may rely on exclusion of other causes of ulcers. We aimed to describe clinical and histopathological features of PG‐like ulcerations occurring in association with AAV and identify clues to differentiate these ulcers from PG. Retrospective search was conducted to include patients with AAV presenting with PG‐like ulcers treated at our institution. This large case series highlights presentation of PG‐like ulcers occurring in patients with AAV. Care should be taken to avoid delayed or missed diagnosis of AAV. Distinction between AAV and PG is challenging yet mandatory for proper treatment. Diagnosis relies on a constellation of detailed cutaneous clinical examination, systemic symptoms or illness, histopathological features and laboratory tests.

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“…Careful clinical examination, particularly in the head and neck and oral areas, can assist in diagnosis of GPA. At times, the PG‐like lesions of GPA begin as subtle papules which may resemble acneiform lesions or even a neutrophilic dermatosis prior to ulceration 15 . These acneiform lesions appear to be seen more often in childhood and adolescence.…”

Section: Granulomatosis With Polyangiitis (Gpa)mentioning

confidence: 99%

Cutaneous manifestations of antineutrophil cytoplasmic antibody‐associated vasculitis (AAV): a concise review with emphasis on clinical and histopathologic correlation

Gibson

2022

Int J Dermatology

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Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) comprises a group of small vessel vasculitides grouped by commonalities of clinical manifestations and ANCA testing. Skin findings are not uncommon, although there is considerable overlap and many times nonspecificity. In general, patients with skin findings tend to have more significant systemic illness, and skin lesions most often develop simultaneously or following onset of systemic symptoms. There are clinical and pathological clues of help in the differentiation of skin findings in these disorders. Purpura of various forms with leukocytoclastic vasculitis is common to all AAV. Granulomatosis with polyangiitis (GPA) comprises the largest number of patients with an AAV. Upper airway, oral, ear, and facial lesions, with or without granuloma, are more commonly seen in this AAV. Pyoderma gangrenosum-like lesions, including facial location, while not common are most closely associated with GPA.Eosinophilic granulomatosis with polyangiitis (EGPA) as its name implies is more closely associated with eosinophilic, allergic, or asthmatic conditions. Papular lesions of the extensor extremities showing extravascular granulomatous changes are characteristic but not specific for EGPA. Microscopic polyangiitis (MPA) is more closely associated with livedoid skin changes, and vascular inflammation tends to be deeper in the skin than with the other AAV. Extravascular granulomas are not expected. While the skin findings in AAV can be nonspecific and overlapping, combining careful full skin examination with histopathologic study of selected lesions is critical to making the correct diagnosis and in ruling out other similar diseases not ANCA related. The aim of this article is to encourage increased participation by dermatologists and dermatopathologists in the care of these patients.

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Granulomatosis with Polyangiitis Presenting with Pyoderma Gangrenosum–Like Ulceration and Negative Cytoplasmic Antineutrophilic Cytoplasmic Antibodies in a Child

Cited by 8 publications

References 9 publications

Cutaneous manifestations of anti-neutrophil cytoplasmic antibody associated vasculitis

Cutaneous manifestations of anti-neutrophil cytoplasmic antibody associated vasculitis

Pyoderma gangrenosum‐like skin ulceration in antineutrophil cytoplasmic antibody‐associated vasculitis: a retrospective case series

Cutaneous manifestations of antineutrophil cytoplasmic antibody‐associated vasculitis (AAV): a concise review with emphasis on clinical and histopathologic correlation

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