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Clinical and histopathologic features of 8 patients with microscopic polyangiitis including two with a slowly
Tamihiro Kawakami1, Takeshi Kawanabe, Chihiro Saito
1Department of Dermatology, St Marianna University School of Medicine, Kanagawa, Japan. tami@marianna-u.ac.jp
Background:
Microscopic polyangiitis (MPA) is a systemic antineutrophil cytoplasmic autoantibody-associated vasculitis associated with necrotizing and crescentic glomerulonephritis and pulmonary capillaritis. MPA generally has a rapidly progressive clinical course, but there have been recent reports of slowly progressive cases.
Objective:
To evaluate the typical cutaneous findings of MPA, we recorded the clinical and histopathologic features of the cutaneous manifestations.
Methods:
Eight patients with MPA, who had presented with cutaneous manifestations between 2001 and 2005 in our department, were retrospectively reviewed. They had necrotizing vasculitis in their cutaneous lesions as confirmed by skin biopsy specimens. Patients with other known connective tissue diseases were not included in the study.
Results:
All 8 patients with MPA presented cutaneously with erythematous macules on their extremities. Livedo reticularis (5/8, 68%) was also observed. Six of the 8 patients with MPA were given the diagnosis within 3 months of their initial manifestation. In skin biopsy specimens, necrotizing vasculitis was noted in the reticular dermis to the subcutaneous fat. In contrast, the other two patients with MPA were given the diagnosis about 10 years after their initial manifestation. Histopathologic findings demonstrated necrotizing vasculitis with moderate neutrophilic infiltrations in the papillary to middle dermis in the latter two patients. Serum myeloperoxidase-antineutrophil cytoplasmic autoantibody levels were only moderlately elevated in the latter two patients and they were given the diagnosis of slowly progressive MPA. Histopathologically, palisading granulomas were present on the elbow of one of them.
Limitations:
The study was based on histopathological analysis in a limited number of patients due to the rareness of the investigated disease.
Conclusions:
There appears to be a correlation between a slowly progressive clinical course of MPA and the depth of dermal involvement and the severity of neutrophilic infiltration in biopsy specimens. Based on these results, we believe that these characteristic patterns may help clinicians establish an earlier diagnosis of possible MPA with positive antineutrophil cytoplasmic autoantibody titers.
Insights
Microscopic polyangiitis (MPA) skin findings, like erythematous macules and livedo reticularis, can indicate disease progression. Deeper dermal involvement and neutrophilic infiltration in biopsies may signal slowly progressive MPA, aiding earlier diagnosis.
Area of Science:
- Rheumatology
- Dermatology
- Pathology
Background:
- Microscopic polyangiitis (MPA) is a systemic autoimmune vasculitis.
- MPA is characterized by necrotizing glomerulonephritis and pulmonary capillaritis.
- While typically rapidly progressive, slowly progressive MPA cases have been reported.
Observation:
- This study retrospectively reviewed 8 patients with MPA and cutaneous manifestations.
- Typical findings included erythematous macules and livedo reticularis.
- Skin biopsies revealed necrotizing vasculitis in the reticular dermis to subcutaneous fat.
Findings:
- Two patients exhibited a slowly progressive course (diagnosis delayed by ~10 years).
- Their skin biopsies showed necrotizing vasculitis with moderate neutrophilic infiltration in the papillary to middle dermis.
- These patients had moderately elevated serum myeloperoxidase-antineutrophil cytoplasmic autoantibody levels.
Implications:
- Slowly progressive MPA may correlate with specific histopathologic features like dermal depth of involvement and neutrophilic infiltration.
- These findings may aid clinicians in earlier diagnosis of MPA, particularly in cases with positive antineutrophil cytoplasmic autoantibody titers.
- Further research is warranted due to the limited sample size and rarity of MPA.
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