Table of Contents  
CASE REPORT
Year : 2013  |  Volume : 6  |  Issue : 4  |  Page : 475-477  

Cutaneous polyarteritis nodosa: A case report with a brief review of literature


Department of Dermatology, Padmashree Dr. D. Y. Patil Medical College, Hospital and Research Centre, Dr. D. Y. Patil Vidyapeeth, Pune, India

Date of Web Publication17-Sep-2013

Correspondence Address:
Neha C Virmani
Department of Dermatology, Dr. D.Y. Patil Medical College and Hospital, Santtukaram Nagar, Pimpri, Pune - 411 018, Maharashtra
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0975-2870.118298

Rights and Permissions
  Abstract 

Polyarteritis nodosa (PAN) is a rare vasculitis with multisystem involvement. The definition, diagnostic criteria and validity of cutaneous polyarteritis nodosa (c-PAN) as a variant of classic PAN limited to skin have continued to remain disputed. We revisit the definition of c-PAN, comment on advances in its treatment and describe one such case, a 26-year-old male, whose affliction consisting of two episodes over a period of 2 years remains restricted - without any constitutional feature and systemic involvement - to skin over legs as multiple, discoloured, tender, subcutaneous-a few ulcerated-nodules healing promptly without scarring in response to oral corticosteroids. The aim of presentation is to emphasize to the non-dermatologists physicians regarding its usual optimistic prognosis.

Keywords: Cutaneous polyarteritis nodosa, fibrinoid necrosis, livedo reticularis , systemic polyarteritis nodosa


How to cite this article:
Sharma YK, Dash K, Goyal N, Virmani NC. Cutaneous polyarteritis nodosa: A case report with a brief review of literature. Med J DY Patil Univ 2013;6:475-7

How to cite this URL:
Sharma YK, Dash K, Goyal N, Virmani NC. Cutaneous polyarteritis nodosa: A case report with a brief review of literature. Med J DY Patil Univ [serial online] 2013 [cited 2024 Mar 29];6:475-7. Available from: https://journals.lww.com/mjdy/pages/default.aspx/text.asp?2013/6/4/475/118298


  Introduction Top


Polyarteritis nodosa (PAN) is a vasculitis of medium-sized vessels (i.e., small or medium sized muscular arteries) affecting the skin and major organs. Variants of the disease limited to either muscle and nerve, single internal organ or skin, the last referred to as cutaneous polyarteritis nodosa (c-PAN)-have been described. [1] However, there is an ongoing debate, whether to restrict c-PAN to just the vasculitis involving arteries of the subcutis or also to include non-organ extracutaneous involvement such as that of peripheral nerves, muscles, etc., and even an exceptional transformation to classical PAN.


  Case Report Top


A 26-year-old-male presented with multiple, erythematous, tender subcutaneous nodules that appeared in crops over both legs since 2 weeks. Some of the nodules had ulcerated within a week of onset. Patient reported no constitutional symptoms or preceding infections. Past history revealed similar lesions 2 years back, that healed without scarring. There was no history of vaccination for hepatitis B. General physical examination revealed an averagely built, well nourished, normotensive individual without any obvious abnormalities except dental caries. Dermatological examination showed asymmetrical, irregular, lacy, broken fish net like purplish mottling "starburst livedo," and ulcerated subcutaneous nodules over both legs [Figure 1]. Routine investigations, including liver and renal function tests, erythrocyte sedimentation rate (ESR), anti-streptolysin titre, urine microscopy, echocardiogram and X-ray chest were normal. Serology for hepatitis B and C virus (HBV,HCV) and cytoplasmic-antineutrophil cytoplasmic antibody (c-ANCA) was negative. Histopathological examination of a nodule on deep incisional biopsy revealed fibrinoid necrosis of a medium-sized artery in the subcutis along with perivascular neutrophilic infiltrate [Figure 2], confirming our diagnosis of c-PAN. Patient was prescribed non-steroidal anti inflammatory drugs (NSAIDs)and 30 mg/day of prednisolone, later slowly tapered over a period of 1 month. The lesions showed regression with no recurrences on follow-up [Figure 3] over 2 years.
Figure 1: Erythematous nodules with ulceration over both legs

Click here to view
Figure 2: Histopathology view of fibrinoid necrosis of a medium sized artery in the subcutis with perivascular infiltrate (H and E, ×400)

Click here to view
Figure 3: Healed lesions following treatment

Click here to view



  Discussion Top


Kussmaul and Maier originally described PAN in 1866. [1] However, the concept of c-PAN or limited PAN as a lucid separate entity described over a century later [2],[3] continues to remain disputed.

Cutaneous PAN is usually said to be PAN affecting the skin with no major organ involvement but in which mild fever, muscle, joint and peripheral involvement may also occur. [4],[5],[6]

Nakamura et al. proposed further restriction of this definition, in which extracutaneous involvement such as myalgias and peripheral neuropathy may be limited to the same area as the skin lesion. [7] According to the diagnostic criteria by American College of Rheumatology, a disease with both cutaneous and at least one extra-cutaneous symptom with appropriate histopathological findings can be diagnosed as PAN. [5]

Exact cause of PAN is not known; an autoimmune hypothesis is further strengthened by the presence of immune complex deposits of Immunoglobulin M (IgM) and C3 over vessel walls as mentioned in some reports. [6],[8] Presence of high titres of IgM antiphosphatidylserine-prothrombin complex and lupus anticoagulant among patients with c-PAN have recently been shown to have pathogenetic significance by activating the classical complement pathway. [9] Associations with various viral (particularly HBV, also HCV, human immunodeficiency virus and parvovirus B19) and group A streptococcal infections have been reported. [6] Four of the nine patients of benign c-PAN studied by Minkowitz et al. revealed anti-HBV sereology and one had polyclonal hypergammaglobulinemia associated with acquired immunodeficiency syndrome. [10] With the use of vaccination against the virus, its association with hepatitis B has declined markedly. [4] Though no such cases have been reported, dental caries in our patient could have triggered the disease.

Whereas tender subcutaneous erythematous nodules, purple or black patches of infarcts and livedo reticularis as the characteristic lesions are present in all case of c-PAN, [4],[6] cutaneous involvement is seen only in about 25-30% patients with systemic PAN. These subcutaneous nodules (of 5-15 mm diameter) over legs (95%) may also involve arms, trunk, head, neck and buttocks in decreasing order of frequency. [6] Absence of life-threatening organ involvement in c-PAN (renal ischemia, testicular pain/epididymitis, ischemic myalgia and hypertension) distinguishes it from systemic PAN. [5] As mentioned already even the uncommon occurrence of constitutional features or neuromuscular involvement is not universally accepted.

Histopathological evidence of necrotising inflammation of the medium-sized arteries, definitive for the diagnosis, unifies both c-PAN and systemic PAN. Presence of elevated ESR and leucocytosis, however, is non-specific. [5],[8]

C-PAN waxes and wanes for months to years. Mild cases are treated with NSAID's, with severe forms needing systemic steroids. Immunosuppressives may also be used as steroid - sparing drugs. [6] Intravenous immunoglobulins and biologics (etarnercept, infliximab and rituximab) have been tried with good results. [5],[11] Penicillin prophylaxis has been useful in children with streptococcal infections as an underlying cause, however, recurrences have been common. [5],[6] No criteria for treatment of c-PAN have been laid down.

In conclusion, as per the current knowledge c-PAN is a benign, though chronic, affliction restricted usually to the medium-sized arteries of the subcutis, usually lacks neuromuscular and constitutional accompaniments and unlikely to progress to systemic PAN. Still it is prudent to keep the patient under long term follow-up without causing him undue panic to promptly manage an exceptional case that may progress to systemic PAN.


  Acknowledgment Top


We are grateful to Dr. Narayan K Panicker of Department of Pathology, Dr. D.Y. Patil Medical College and Hospital for the histopathology pictures.

 
  References Top

1.Kussmaul A, Maier R. Ueber eine bisher nicht beschriebene eigenthumliche Arteriener krankung, die mit Morbus Brightii und rapid fortschreitender allgemeiner Muskellahmung einhergeht. Dtsch Arch Klin Med 1866;1:484-518.  Back to cited text no. 1
    
2.Borrie P. Cutaneous polyarteritis nodosa. Br J Dermatol 1972;87:87-95.  Back to cited text no. 2
[PUBMED]    
3.Diaz-Perez JL, Winkelmann RK. Cutaneous periarteritis nodosa. Arch Dermatol 1974;110:407-14.  Back to cited text no. 3
[PUBMED]    
4.Merkel PA, Monach PA. Systemic necrotizing arteritis. In: Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ, Wolff K. editor. Fitzpatrick's Dermatology in General Medicine. 8 th ed. U.S.A: McGraw-Hill Publishers; 2012. p. 2025.  Back to cited text no. 4
    
5.Bansal NK, Houghton KM. Cutaneous polyarteritis nodosa in childhood: A case report and review of the literature. Arthritis 2010;2010:687547.  Back to cited text no. 5
[PUBMED]    
6.Khoo BP, Ng SK. Cutaneous polyarteritis nodosa: A case report and literature review. Ann Acad Med Singapore 1998;27:868-72.  Back to cited text no. 6
[PUBMED]    
7.Nakamura T, Kanazawa N, Ikeda T, Yamamoto Y, Nakabayashi K, Ozaki S, et al. Cutaneous polyarteritis nodosa: Revisiting its definition and diagnostic criteria. Arch Dermatol Res 2009;301:117-21.  Back to cited text no. 7
[PUBMED]    
8.Kim LH. Polyarteritis nodosa. Dermatol Online J 2001;7:17.  Back to cited text no. 8
[PUBMED]    
9.Kawakami T, Yamazaki M, Mizoguchi M, Soma Y. High titer of anti-phosphatidylserine-prothrombin complex antibodies in patients with cutaneous polyarteritis nodosa. Arthritis Rheum 2007;57:1507-13.  Back to cited text no. 9
[PUBMED]    
10.Minkowitz G, Smoller BR, McNutt NS. Benign cutaneous polyarteritis nodosa. Relationship to systemic polyarteritis nodosa and to hepatitis B infection. Arch Dermatol 1991;127:1520-3.  Back to cited text no. 10
[PUBMED]    
11.Eleftheriou D, Melo M, Marks SD, Tullus K, Sills J, Cleary G, et al. Biologic therapy in primary systemic vasculitis of the young. Rheumatology (Oxford) 2009;48:978-86.  Back to cited text no. 11
[PUBMED]    


    Figures

  [Figure 1], [Figure 2], [Figure 3]


This article has been cited by
1 Polyarteritis nodosa (PAN) associated with familial Mediterranean fever (FMF): An unusual case presentation and a review of the literature
Samaher Almousa
The Egyptian Rheumatologist. 2019;
[Pubmed] | [DOI]



 

Top
   
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this article
Abstract
Introduction
Case Report
Discussion
Acknowledgment
References
Article Figures

 Article Access Statistics
    Viewed5618    
    Printed159    
    Emailed0    
    PDF Downloaded314    
    Comments [Add]    
    Cited by others 1    

Recommend this journal