Pyoderma gangrenosum associated with Wegener's granulomatosis: partial response to mycophenolate mofetil

Hello, C. Le; Bonté, I; Mora, Juan José Hidalgo; Verneuil, L.; Nôël, Laure-Hélène; Guillevin, L

doi:10.1093/rheumatology/41.2.236

Cited by 20 publications

(11 citation statements)

References 7 publications

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“…67 Successful treatment of skin lesions in hypocomplementaemic urticarial vasculitis, ChurgeStrauss syndrome, microscopic polyangitis, and Wegner granulomatosis are all reported with overall good tolerability and successful response rates. [68][69][70][71][72][73][74] In light of the latter three named vasculitides, it is not surprising that interest has developed regarding the use of MMF for antineutrophil cytoplasm antibodiese associated vasculitides with the proposal that MMF may actually aid in the removal of these pathogenic antibodies. 69 Although effective in several vasculitides, an attempt at treating Behçet disease has proven nonbeneficial.…”

Section: Connective Tissue Disordersmentioning

confidence: 99%

Mycophenolate mofetil in dermatology

Orvis

Wesson

Breza

et al. 2009

Journal of the American Academy of Dermatology

114

106

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Section: Connective Tissue Disordersmentioning

confidence: 99%

Mycophenolate mofetil in dermatology

Orvis

Wesson

Breza

et al. 2009

Journal of the American Academy of Dermatology

114

106

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“…Renal involvement is usually associated with the renal involvement (4). Skin rash was reported in 14-47% of the patients, either during or at the onset of disease and may develop on unusual sites such as the trunk, neck and face (5,6). Cutaneous lesions may be in the form of palpable purpura, subcutaneous nodules, ulcers, pustules and pyoderma gangrenosum (7).…”

Section: Discussionmentioning

confidence: 99%

Herpetic whitlow during immunosuppressive therapy for Wegener’s Granulomatosis

et al. 2014

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Skin involvement may occur in patients with Wegener' s granulomatosis (polyangiitis with granulomatosis; WG) and is more frequent in the generalised form. However, when a patient with vasculitis develops digital ulceration, in addition to disease activation, other pathologies should be considered. One of them may be the herpetic whitlow mimicking paronychia. Here, we present a patient who developed herpetic whitlow during the course of immunosuppressive therapy due to WG. Just before the third course of cyclophosphamide therapy, she was re-admitted to the outpatient clinic with the above-mentioned ulcerated lesions. On physical examination, there was erythema and a painful, crusted ulceration in the distal phalanx of the right index finger involving the proximal nail fold. Similar lesions were also present in her lower lip. Due to the absence of clinical and laboratory findings suggesting the activation of WG and the Tzanck smear result, which is compatible with herpes virus infection, we do not believe that WG was responsible for our patient' s complaints. All of the patient' s lesions completely disappeared following the interruption of immunosuppressive therapy.

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“…[13] Pyoderma-like ulcers or necrotizing ulcerations resembling pyoderma gangrenosum are rare manifestations. [8] None of the skin biopsy changes could be considered diagnostic of WG. LCV was by far the most common histopathologic pattern in our series and occurred in patients with active multi-organ disease.…”

Section: Discussionmentioning

confidence: 99%

Immune deposits in cutaneous lesions of Wegener′s granulomatosis: Predictor of an active disease

et al. 2011

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A retrospective analysis was conducted of eight cases of Wegener's granulomatosis (WG), who presented with cutaneous lesions. The clinical, immunopathologic and histopathologic features of the cutaneous lesions were reviewed. Antineutrophil cytoplasmic antibody (ANCA) status of the patients was established. When possible, a comparison of immunofluorescence findings of skin biopsies was made with those of renal biopsies taken at the same time. In all except one, systemic and cutaneous disease developed concurrently. On histopathology, leukocytoclastic vasculitis was noted in five patients and features of lupus erythematosus and pyoderma gangrenosum in one case each. Four patients showed immunoglobulin deposits in subepidermal blood vessel walls, while one patient showed granular immune deposits at dermo-epidermal junction only. Immunoglobulin G was the most common immunoreactant detected. C-ANCA/proteinase 3 (PR3)-ANCA was positive in six patients, P-ANCA/myeloperoxidase (MPO)-ANCA in one patient, while one patient did not show ANCA positivity on indirect immunofluorescence. All four renal biopsies showed pauci-immune glomerulonephritis, irrespective of the presence (n=3) or absence (n=1) of immune deposits in the skin biopsy. Skin manifestations are encountered in nearly half of the patients with WG, thus it is important to be familiar with cutaneous histopathologic as well as immunofluorescence findings in WG patients.

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Pyoderma gangrenosum associated with Wegener's granulomatosis: partial response to mycophenolate mofetil

Cited by 20 publications

References 7 publications

Mycophenolate mofetil in dermatology

Mycophenolate mofetil in dermatology

Herpetic whitlow during immunosuppressive therapy for Wegener’s Granulomatosis

Immune deposits in cutaneous lesions of Wegener′s granulomatosis: Predictor of an active disease

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