Skip to main content
Top
Published in: Pediatric Rheumatology 1/2014

Open Access 01-12-2014 | Case Report

Cutaneous polyarteritis nodosa successfully treated with topical diflucortolone valerate – a case report & review of the literature

Authors: Ruby Haviv, Maya Capua, Jacob Amir, Liora Harel

Published in: Pediatric Rheumatology | Issue 1/2014

Login to get access

Abstract

Cutaneous Polyarteritis Nodosa (cPAN) was first described in 1931. cPAN is considered a rare disease, its true incidence is unknown. The age of onset is diverse. Most studies have shown no significant gender predominance. cPAN presents with distinct skin findings, such as a maculopapular rash, subcutaneous nodules, livedoid vasculitis, panniculitis, ischemic finger lesions, or erythematous patchy rash.
Etiology is unclear. It is still believed to be an immune complex-mediated disease, although a possible mechanism recently proposed relates a familial form of the disease to impaired activity of Adenosine Deaminase 2. cPAN may reflect an underlying disease, infection or medical treatment.
There is no consensus as to initial treatment, dosage and length of treatment. Patients with constitutional symptoms, visceral involvement, a more severe course of the disease, or high acute phase reactants, were treated mainly with systemic corticosteroids and/or cytotoxic agents for varying durations. However, persistence of cutaneous lesions has been documented.
We describe a 14 year old male suffering from persistent cPAN, with no constitutional symptoms or involvement of internal organs. The patient was treated with a local corticosteroid-based ointment during exacerbations, until complete remission. Although reported in only one study, treatment with topical corticosteroid compound may result in significant improvement or complete regression of skin lesions in cPAN patients.
Appendix
Available only for authorised users
Literature
1.
go back to reference Morgan AJ, Schwartz RA: Cutaneous polyarteritis nodosa: a comprehensive review. Int J Dermatol. 2010, 49 (7): 750-756.CrossRefPubMed Morgan AJ, Schwartz RA: Cutaneous polyarteritis nodosa: a comprehensive review. Int J Dermatol. 2010, 49 (7): 750-756.CrossRefPubMed
2.
go back to reference Cassidy JT, Petty RE, Laxer RM, Lindsley CB: Textbook of Pediatric Rheumatology. 2011, Philadelphia, PA: Saunders, 6 Cassidy JT, Petty RE, Laxer RM, Lindsley CB: Textbook of Pediatric Rheumatology. 2011, Philadelphia, PA: Saunders, 6
3.
go back to reference Ozen S, Anton J, Arisoy N, Bakkaloglu A, Besbas N, Brogan P, García-Consuegra J, Dolezalova P, Dressler F, Duzova A, Ferriani VP, Hilário MO, Ibáñez-Rubio M, Kasapcopur O, Kuis W, Lehman TJ, Nemcova D, Nielsen S, Oliveira SK, Schikler K, Sztajnbok F, Terreri MT, Zulian F, Woo P: Juvenile polyarteritis: results of a multicenter survey of 110 children. J Pediatr. 2004, 145 (4): 517-522. 10.1016/j.jpeds.2004.06.046.CrossRefPubMed Ozen S, Anton J, Arisoy N, Bakkaloglu A, Besbas N, Brogan P, García-Consuegra J, Dolezalova P, Dressler F, Duzova A, Ferriani VP, Hilário MO, Ibáñez-Rubio M, Kasapcopur O, Kuis W, Lehman TJ, Nemcova D, Nielsen S, Oliveira SK, Schikler K, Sztajnbok F, Terreri MT, Zulian F, Woo P: Juvenile polyarteritis: results of a multicenter survey of 110 children. J Pediatr. 2004, 145 (4): 517-522. 10.1016/j.jpeds.2004.06.046.CrossRefPubMed
4.
go back to reference Stone MS, Olson RR, Weismann DN, Giller RH, Goeken JA: Cutaneous vasculitis in a newborn of a mother with cutaneous polyarteritis nodosa. J Am Acad Dermatol. 1993, 28 (1): 101-105. 10.1016/0190-9622(93)70020-T.CrossRefPubMed Stone MS, Olson RR, Weismann DN, Giller RH, Goeken JA: Cutaneous vasculitis in a newborn of a mother with cutaneous polyarteritis nodosa. J Am Acad Dermatol. 1993, 28 (1): 101-105. 10.1016/0190-9622(93)70020-T.CrossRefPubMed
5.
go back to reference Navon Elkan P, Pierce SB, Segel R, Walsh T, Barash J, Padeh S, Zlotogorski A, Berkun Y, Press JJ, Mukamel M, Voth I, Hashkes PJ, Harel L, Hoffer V, Ling E, Yalcinkaya F, Kasapcopur O, Lee MK, Klevit RE, Renbaum P, Weinberg-Shukron A, Sener EF, Schormair B, Zeligson S, Marek-Yagel D, Strom TM, Shohat M, Singer A, Rubinow A, Pras E, Winkelmann J, Tekin M, Anikster Y, King MC, Levy-Lahad E: Mutant Adenosine Deaminase 2 in a Polyarteritis Nodosa Vasculopathy. N Engl J Med. 2014, 370 (10): 921-931. 10.1056/NEJMoa1307362.CrossRefPubMed Navon Elkan P, Pierce SB, Segel R, Walsh T, Barash J, Padeh S, Zlotogorski A, Berkun Y, Press JJ, Mukamel M, Voth I, Hashkes PJ, Harel L, Hoffer V, Ling E, Yalcinkaya F, Kasapcopur O, Lee MK, Klevit RE, Renbaum P, Weinberg-Shukron A, Sener EF, Schormair B, Zeligson S, Marek-Yagel D, Strom TM, Shohat M, Singer A, Rubinow A, Pras E, Winkelmann J, Tekin M, Anikster Y, King MC, Levy-Lahad E: Mutant Adenosine Deaminase 2 in a Polyarteritis Nodosa Vasculopathy. N Engl J Med. 2014, 370 (10): 921-931. 10.1056/NEJMoa1307362.CrossRefPubMed
6.
go back to reference Daoud MS, Hutton KP, Gibson LE: Cutaneous periarteritis nodosa: a clinicopathological study of 79 cases. Br J Dermatol. 1997, 136 (5): 706-713. 10.1111/j.1365-2133.1997.tb03656.x.CrossRefPubMed Daoud MS, Hutton KP, Gibson LE: Cutaneous periarteritis nodosa: a clinicopathological study of 79 cases. Br J Dermatol. 1997, 136 (5): 706-713. 10.1111/j.1365-2133.1997.tb03656.x.CrossRefPubMed
7.
go back to reference Kawakami T, Yamazaki M, Mizoguchi M, Soma Y: High titer of anti-phosphatidylserine-prothrombin complex antibodies in patients with cutaneous polyarteritis nodosa. Arthritis Care Res. 2007, 57 (8): 1507-1513. 10.1002/art.23081.CrossRef Kawakami T, Yamazaki M, Mizoguchi M, Soma Y: High titer of anti-phosphatidylserine-prothrombin complex antibodies in patients with cutaneous polyarteritis nodosa. Arthritis Care Res. 2007, 57 (8): 1507-1513. 10.1002/art.23081.CrossRef
9.
go back to reference Fink CW: The Role of the Streptococcus in Poststreptococcal Recative Arthritis and Childhood Polyarteritis Nodosa. J Rheumatol Suppl. 1991, 29: 14-20.PubMed Fink CW: The Role of the Streptococcus in Poststreptococcal Recative Arthritis and Childhood Polyarteritis Nodosa. J Rheumatol Suppl. 1991, 29: 14-20.PubMed
10.
go back to reference Marie I, Miranda S, Girszyn N, Soubrane JC, Vandhuick T, Levesque H: Intravenous immunoglobulins as treatment of severe cutaneous polyarteritis nodosa. Intern Med J. 2012, 42 (4): 459-462. 10.1111/j.1445-5994.2012.02739.x.CrossRefPubMed Marie I, Miranda S, Girszyn N, Soubrane JC, Vandhuick T, Levesque H: Intravenous immunoglobulins as treatment of severe cutaneous polyarteritis nodosa. Intern Med J. 2012, 42 (4): 459-462. 10.1111/j.1445-5994.2012.02739.x.CrossRefPubMed
11.
go back to reference Uziel Y, Silverman ED: Intravenous Immunoglobulin Therapy in a Child with Cutaneous Polyarteritis Nodosa. Clin Exp Rheumatol. 1998, 16 (2): 187-189.PubMed Uziel Y, Silverman ED: Intravenous Immunoglobulin Therapy in a Child with Cutaneous Polyarteritis Nodosa. Clin Exp Rheumatol. 1998, 16 (2): 187-189.PubMed
12.
go back to reference Rogaslki C, Sticherling M: Panarteritis cutanea benigna – an entity limited to the skin or cutaneous presentation of a systemic necrotizing vasculitis ? Report of seven cases and review of the literature. Int J Dermatol. 2007, 46 (8): 817-821. 10.1111/j.1365-4632.2007.02975.x.CrossRef Rogaslki C, Sticherling M: Panarteritis cutanea benigna – an entity limited to the skin or cutaneous presentation of a systemic necrotizing vasculitis ? Report of seven cases and review of the literature. Int J Dermatol. 2007, 46 (8): 817-821. 10.1111/j.1365-4632.2007.02975.x.CrossRef
Metadata
Title
Cutaneous polyarteritis nodosa successfully treated with topical diflucortolone valerate – a case report & review of the literature
Authors
Ruby Haviv
Maya Capua
Jacob Amir
Liora Harel
Publication date
01-12-2014
Publisher
BioMed Central
Published in
Pediatric Rheumatology / Issue 1/2014
Electronic ISSN: 1546-0096
DOI
https://doi.org/10.1186/1546-0096-12-46

Other articles of this Issue 1/2014

Pediatric Rheumatology 1/2014 Go to the issue