Skin Ulcers in a Patient Afflicted With Microscopic Polyangiitis

6/1/2009
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Author(s):

Antonio G. Richetta, MD, PhD; Carlo Mattozzi, MD; Elisa Maiani, MD; Valentina Carboni, MD; Valentina Carlomagno, MD; Manuela Cimillo, MD; Simona Giancristoforo, MD; Sara D’Epiro, MD; Stefano Calvieri, MD

Patients usually present purpura and petechiae, livedo, and erythema especially on the hands and fingers.³

Only one case of cutaneous involvement without visceral diseases has been described in the literature.⁴

Therapy is often divided into two phases: an initial “remission-induction” phase is used to control active disease and a “maintenance” phase, which uses less intensive therapy and is used to maintain disease remission while lowering the risk of adverse medication related events. The first-line treatment is represented by cyclophosphamide plus corticosteroids or methotrexate plus corticosteroids. Treatments such as plasma exchange, intravenous immunoglobulin, anti-tumor necrosis factor (TNF)-α therapy, T-cell depletion therapy with antithymocyte globulin, and B-cell depletion therapy with rituximab have been used.^5,6 After the induction of clinical remission with an agent such as cyclophosphamide, patients are generally converted to azathioprine or methotrexate; additional agents that have been used in selected patients include mycophenolate mofetil (MMF), leflunomide, and cyclosporine.^5–7

The diagnosis of pyoderma gangrenosum would make sense in this case but was excluded due to the absence of systemic disease and the clinical course.

Conclusion

To our knowledge this is a rare case of microscopic polyangiitis characterized by a long time cutaneous involvement in absence of any internal organ lesions8 and it demonstrates that clinical manifestations may be limited to the skin. It is possible that a MPA variant limited to the skin may exist and it does not evolve or rarely relates to systemic disease. Further studies are necessary to characterize this possible variant. We hope this report contributes to the ongoing development of knowledge about the clinical-pathological spectrum of MPA and provides further indications about the wide range of its possible clinical manifestations.

References:

1. Bertoli AM, Alarcón GS. Classification of the vasculitides: are they clinically useful? Curr Rheumatol Rep. 2005;7(4):265–269.
2. Guillevin L, Durand-Gasselin B, Cevallos R, et al. Microscopic polyangiitis: clinical and laboratory findings in eighty-five patients. Arthritis Rheum. 1999;42(3):421–430.
3. Seishima M, Oyama Z, Oda M. Skin eruptions associated with microscopic polyangiitis. Eur J Dermatol. 2004;14(4):255–258.
4. Irvine AD, Bruce IN, Walsh MY, Bingham EA. Microscopic polyangiitis. Delineation of a cutaneous-limited variant associated with antimyeloperoxidase autoantibody. Arch Dermatol. 1997;133(4):474–477.
5. Jayne D. Evidence-based treatment of systemic vasculitis. Rheumatology (Oxford). 2000;39(6):585–595.
6. Keogh KA, Wylam ME, Stone JH, Specks U. Induction of remission by B lymphocyte depletion in eleven patients with refractory antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 2005;52(1):262–268.
7. Pagnoux C, Mahr A, Hamidou MA, et al. Azathioprine or methotrexate maintenance for ANCA-associated vasculitis. N Engl J Med. 2008;359(26):2790–2803.
8. Hautmann G, Campanile G, Lotti TM. The many faces of cutaneous vasculitis. Clin Dermatol. 1999;17(5):515–531.