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Journal of the American Academy of Dermatology
Sous presse. Epreuves corrigées par l'auteur. Disponible en ligne depuis le vendredi 7 février 2020
Doi : 10.1016/j.jaad.2019.10.068
accepted : 25 October 2019
Lymphocytic thrombophilic arteritis and cutaneous polyarteritis nodosa: Clinicopathologic comparison with blinded histologic assessment
 

Robert I. Kelly, FACD a, Edmund Wee, MBBS (Hons) a, , Showan Balta, FRCPA b, Richard A. Williams, FRCPA b
a Department of Dermatology, St Vincent's Hospital Melbourne, Melbourne, Australia 
b Departments of Anatomical Pathology, St Vincent's Hospital Melbourne, Melbourne, Australia 

Reprint requests: Edmund Wee, MBBS (Hons), Department of Dermatology, St Vincent's Hospital Melbourne, 41 Victoria Parade, Fitzroy VIC 3065, Australia.Department of DermatologySt Vincent's Hospital Melbourne41 Victoria ParadeFitzroyVIC3065Australia
Abstract
Background

Lymphocytic thrombophilic arteritis (LTA), or macular lymphocytic arteritis, is defined by a primary lymphocytic vasculitis. However, the nosology of LTA has been controversial, with speculation that it may represent an indolent non–nodule-forming variant of cutaneous polyarteritis nodosa (cPAN).

Objective

This study compares the clinicopathologic features of patients with LTA or cPAN to assess if these conditions should be considered distinct entities.

Methods

This is a cross-sectional study of all LTA and cPAN cases at a single tertiary center using prospectively collected clinical data and blinded histologic assessment.

Results

The study included 17 patients with LTA and 13 patients with cPAN. Clinically, cases of LTA were distinguished by a more widespread pattern of livedo racemosa, which was noninfiltrated and asymptomatic. In contrast, cPAN was associated with localized starburst livedo, purpura, and episodic features including nodules, pain, and large inflammatory ulcers. When patients were separated according to the presence (>5%) or paucity (≤5%) of neutrophils on blinded histology review, they had distinct clinical features and differences in disease course.

Limitations

This was a single-center study.

Conclusion

Our data support the classification of LTA and cPAN as separate entities rather than a spectrum of the same disorder and highlight the importance of clinicopathologic correlation in distinguishing these conditions.

The full text of this article is available in PDF format.

Key words : cutaneous polyarteritis nodosa, livedo racemosa, lymphocytic thrombophilic arteritis, lymphocytic vasculitis



 Funding sources: None.
 Conflicts of interest: None disclosed.



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