Relationship between cutaneous polyarteritis nodosa (cPAN) and macular lymphocytic arteritis (MLA): Blinded histologic assessment of 35 cPAN cases - 12/11/15
Abstract |
Background |
Cutaneous polyarteritis nodosa (cPAN) is a skin medium vessel neutrophilic arteritis with livedo, nodules, and ulcerations. Macular lymphocytic arteritis (MLA) is a small arteritis with erythematous or pigmented macules and typical histologic features (a lymphocytic infiltrate, concentric fibrin ring, no disruption of the internal elastic lamina).
Objective |
We sought to assess the frequency of clinical and histologic features of MLA in patients with cPAN.
Methods |
This was a monocentric retrospective analysis of patients given the diagnosis of cPAN with blinded assessment of skin biopsy specimens.
Results |
All 35 patients included had an infiltrated livedo, nodules, or both. Ulceration was rare. Erythematous or pigmented lesions were present in 54% of patients. Predominantly lymphocytic arteritis, a paucity of neutrophils, concentric fibrin ring, and absence of internal lamina elastic disruption were present in 60%, 20%, 18%, and 23% of patients, respectively. Median follow-up was 11 years. None of the patients had systemic involvement, and 57% had a complete remission. The incidence of complete remission was not different between patients having a predominant lymphocyte infiltrate or few neutrophils.
Limitations |
This was a retrospective, monocentric study without a control group of patients with MLA.
Conclusions |
Our data do not favor the classification of cPAN and MLA as distinct entities.
Le texte complet de cet article est disponible en PDF.Key words : cutaneous periarteritis nodosa, lymphocytic thrombophilic arteritis, macular arteritis, macular lymphocytic arteritis, periarteritis nodosa, vasculitis
Abbreviations used : ANCA, cPAN, CR, MLA, PAN
Plan
Funding sources: None. |
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Drs Buffiere-Morgado and Battistella have equally contributed to this work and share first authorship. |
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Conflicts of interest: None declared. |
Vol 73 - N° 6
P. 1013-1020 - décembre 2015 Retour au numéroBienvenue sur EM-consulte, la référence des professionnels de santé.
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