Letter from the Editor: Management of cutaneous polyarteritis nodosa

Abstract

In this issue of the JAAD, Stewart et al describe a retrospective case series of 16 patients with cutaneous polyarteritis nodosa (CPAN) at 1 geographic center. Delays in diagnosis were common, with an average time to diagnostic biopsy of 32 months. Six patients required multiple biopsies to establish the diagnosis, and most experienced a chronic relapsing course. Eight of the 11 patients treated with methotrexate had a partial response. While significant limitations include the single-center nature of the study, lack of data regarding dose, and difficulty in judging response in the face of relapsing disease, this report provides much-needed data about a rare but important disease and suggests that methotrexate deserves further investigation. The nomenclature and prognosis of CPAN remain controversial, with some authors suggesting a continuum of disease that includes macular lymphocytic arteritis, cutaneous periarteritis nodosa, and systemic polyarteritis nodosa. A retrospective study of 79 cases of CPAN found that 39 patients had ulcers during the course of their illness. Women were affected more than men in this study, and cutaneous manifestations included painful nodules and edema of the lower extremities. In that study, 22% of patients had at least some evidence of neuropathy, but most laboratory findings were nonspecific, and the disease course was prolonged but benign. Systemic polyarteritis nodosa did not develop in any patient; there was no evidence of hepatitis B infection, and hepatitis C infection was present in only 1 patient. Corticosteroids given systemically induced remission in most of the acute presentations in that series, but the ulcerative disease was more prolonged and frequently associated with neuropathy.1Daoud M.S. Hutton K.P. Gibson L.E. Cutaneous periarteritis nodosa: aclinicopathological study of 79 cases.Br J Dermatol. 1997; 136: 706-713Crossref PubMed Scopus (191) Google Scholar A study of 35 patients with macular arteritis concluded that the incidence of complete remission was not different between patients with and without neutrophils in the infiltrate. Patients experienced livedo, nodules, or both, but ulceration was rare. Histologically, fibrin and disruption of the internal elastic membrane were noted in about 20% of patient specimens. None had systemic involvement, and 57% experienced complete remission.2Buffiere-Morgado A. Battistella M. Vignon-Pennamen M.D. et al.Relationship between cutaneous polyarteritis nodosa (cPAN) and macular lymphocytic arteritis (MLA): blinded histologic assessment of 35 cPAN cases.J Am Acad Dermatol. 2015; 73: 1013-1320Abstract Full Text Full Text PDF PubMed Scopus (29) Google Scholar Polyarteritis nodosa has been noted following immunization and in association with other syndromes, including VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, and somatic) syndrome and deficiency of adenosine deaminase 2. Deficiency of adenosine deaminase 2 is an autosomal recessively inherited disease with fevers, recurrent strokes, livedo racemosa, and polyarteritis nodosa. A single-center study of 60 patients suggested significant overlap between phenotypes. Common manifestations included stroke, red cell aplasia, immune-mediated neutropenia, pancytopenia, hypogammaglobulinemia, and inadequate response to vaccination, but infections were rare. No strokes were observed in 2026 patients after months of antitumor necrosis factor α therapy, and hematopoietic cell transplant enabled some patients to discontinue antitumor necrosis factor therapy.3Barron K.S. Aksentijevich I. Deuitch N.T. et al.The spectrum of the deficiency of adenosine deaminase 2: an observational analysis of a 60 patient cohort.Front Immunol. 2021; 12811473https://doi.org/10.3389/fimmu.2021.811473Crossref Scopus (15) Google Scholar A systematic review of treatments for CPAN concluded that available evidence is limited at best and that ulceration is associated with an increased risk of relapse. Systemic corticosteroids are widely used as induction treatment and often combined with a wide array of immunosuppressive agents.4Papachristodoulou E. Kakoullis L. Tiniakou E. Parperis K. Therapeutic options for cutaneous polyarteritis nodosa: a systematic review.Rheumatology (Oxford). 2021; 60: 4039-4047https://doi.org/10.1093/rheumatology/keab402Crossref PubMed Scopus (4) Google Scholar Clearly, we have much to learn about the spectrum of CPAN, subgroups with distinct outcomes, and optimal management. None disclosed. Cutaneous polyarteritis nodosa diagnosis and treatment: A retrospective case seriesJournal of the American Academy of DermatologyVol. 87Issue 6PreviewTo the Editor: Cutaneous polyarteritis nodosa (CPAN) is a small- to medium-vessel vasculitis with manifestations including subcutaneous nodules, livedo reticularis, ulcers, and purpura.1,2 It is diagnosed via characteristic cutaneous lesions, absence of systemic vasculitic features, and supporting histopathologic findings.1-3 There are limited data to guide the management of CPAN. We reviewed CPAN diagnosis and treatment at our center. Full-Text PDF