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CASE REPORT
Year : 2018  |  Volume : 19  |  Issue : 1  |  Page : 71-73

Cutaneous polyarteritis nodosa: Diagnosis lies skin deep


Department of Pediatrics, Seth G.S. Medical College, King Edward Memorial Hospital, Mumbai, Maharashtra, India

Date of Web Publication28-Dec-2017

Correspondence Address:
Rajwanti K Vaswani
Department of Pediatrics, King Edward Memorial Hospital, Old Hospital Building, Parel, Mumbai - 400 012, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijpd.IJPD_138_16

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  Abstract 

Cutaneous polyarteritis nodosa (CPAN) is an uncommon form of cutaneous vasculitis. Definitive diagnosis is based on skin biopsy. We present a rare case of CPAN in a 2-year-old male child who presented with a history of fever, cutaneous ulcers, and digital gangrene. He was treated with steroids and skin lesions had resolved completely. CPAN needs to be differentiated from systemic polyarteritis nodosa for specific prognosis and management. We report this case to highlight the need of early diagnosis for faster recovery with favorable outcome.

Keywords: Cutaneous polyarteritis nodosa, leukocytoclastic vasculitis, livedo reticularis


How to cite this article:
Amoghimath R, Vaswani RK, Deshmukh CT, Divecha C. Cutaneous polyarteritis nodosa: Diagnosis lies skin deep. Indian J Paediatr Dermatol 2018;19:71-3

How to cite this URL:
Amoghimath R, Vaswani RK, Deshmukh CT, Divecha C. Cutaneous polyarteritis nodosa: Diagnosis lies skin deep. Indian J Paediatr Dermatol [serial online] 2018 [cited 2019 Sep 19];19:71-3. Available from: http://www.ijpd.in/text.asp?2018/19/1/71/206067


  Introduction Top


Cutaneous polyarteritis nodosa (CPAN) is an extremely rare cutaneous vasculitis of lower dermis and subcutaneous tissues without systemic involvement. The etiology is not clear.[1] Common manifestations of CPAN include subcutaneous nodules, livedo racemosa, and skin ulcerations.[2] Constitutional symptoms such as arthralgia and peripheral neuropathy may occur, but visceral involvement is absent. Skin biopsy is essential for the diagnosis. CPAN has benign but chronic and relapsing course in the absence of systemic involvement; hence, it needs to be distinguished from systemic polyarteritis nodosa (PAN).[3],[4]


  Case Report Top


A 2-year-old male child presented with a history of high-grade continuous fever, rash, and pain in the extremities for 4 days. Rash was first noticed over both medial malleoli and was initially reticulated, erythematous, and nonpruritic [Figure 1]. Lesions ulcerated over the next 3–4 days. Similar ulceration was noted during illness on the extensor aspect of elbow and knees. Bluish discoloration of the distal phalanges of both lower limbs was noted during hospitalization. There was no history of rash, bleeding manifestation, photosensitivity, joint pain, drug ingestion, or similar complaints in the past.
Figure 1: (a) Reticulated, erythematous rash on both the medial malleoli, knee joint, and distal toes. (b) The same lesions healed with scarring

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The general examination revealed high-grade fever (above 101°F ), livedo racemosa, and ulcers predominantly over medial malleoli, extensor aspect of elbow and knee joints, and bluish discoloration of all the distal phalanges of lower limbs. There was no organomegaly.

Investigations on admission revealed leukocytosis with predominant polymorphs, elevated erythrocyte sedimentation rate, and C-reactive protein. Blood cultures were negative on three occasions. Abdominal ultrasonography, cardiac two-dimensional echography, arterial and venous Doppler of both upper and lower limbs, renal Doppler, and computed tomography brain were normal. Antinuclear antibody (ANA) was weak positive, anticardiolipin antibody (ACLA) IgM was 43.5 ng/ml (10–15), ds DNA and ANCA were negative. Other laboratory investigations were normal [Table 1]. Skin biopsy taken from rash revealed leukocytoclastic vasculitis.
Table 1: Laboratory test reports

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On suspicion of infection, the child was initially treated with antibiotics, but there was no improvement. Later, based on biopsy report, progressing skin lesions, and persistent fever, the child was started on oral prednisolone at a dose of 2 mg/kg/day for 8 weeks and then tapered over 2 weeks. Fever responded within a week of starting steroids, and the skin lesions completely healed over a month with some scarring [Figure 1]. The child had no relapse while following up for 6 months. Repeat ACLA and ANA values after 3 months were normal.


  Discussion Top


CPAN is a clinical variant of PAN with predominant skin involvement and no (or very limited) systemic involvement. It is uncommon in pediatric population with approximately 140 cases reported in literature. Etiology is unknown; an immune-mediated mechanism has been postulated. Both infectious and noninfectious conditions are implicated with both initiation and relapse of CPAN. Commonly associated infections are Streptococcus, parvovirus B19, mycobacterium, and hepatitis viruses B and C.[4],[5]

Isolated skin involvement and characteristic skin biopsy findings help in diagnosis. Segmentary leukocytoclastic vasculitis in the small- and medium-sized arterioles of the deep dermis, vascular occlusion, and lymphocytic or with a smaller neutrophilic infiltration are characteristic histopathological findings.[5],[6] Histopathological findings are similar in CPAN and systemic PAN. Although immunological testing does not confirm the diagnosis of CPAN, negative results help exclude other systemic vasculitis.[4]

Corticosteroids form the principal treatment for CPAN.[5] Cyclophosphamide, azathioprine, and methotrexate are used in cases resistant to steroids.[7] Severe refractory CPAN has also been treated with intravenous immunoglobulins at a dose of 1 g/kg/day for 2 days monthly till recovery.[8] Nonsteroidal anti-inflammatory drugs are tried only in the milder form of CPAN. The prognosis of CPAN is favorable and no known mortality from the disease itself.[9] Rarely, auto-amputation of tips of toes and fingers and sloughing of skin can occur among younger children.[10] Early recovery and faster improvement of fever, arthralgias, and skin lesions is noted with treatment.[9]

Our patient had fever and characteristic rash without any systemic involvement. All the etiologic workup was negative. The histopathological findings of our patient were consistent with that of CPAN. Our patient responded well to oral prednisolone with complete remission of the illness.


  Conclusion Top


CPAN is a rare cutaneous vasculitis. Delay in diagnosis leads to complications and prolonged morbidity. Relapses and exacerbation are common and in exceptional cases can progress to systemic PAN. Hence, early diagnosis and long-term follow-up is paramount and has a profound impact on outcome.

Acknowledgments

The authors wish to thank Dr. Shubhangi Parkar, Dean, Seth G. S. Medical College and King Edward Memorial Hospital, for allowing them to publish this article.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
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. 1
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2.
Kawakami T, Soma Y. Correlation of livedo racemosa, cutaneous inflammatory plaques, and antiphospholipid antibodies in patients with cutaneous polyarteritis nodosa. Medicine (Baltimore) 2011;90:119-24.  Back to cited text no. 2
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3.
Kumar L, Thapa BR, Sarkar B, Walia BN. Benign cutaneous polyarteritis nodosa in children below 10 years of age – A clinical experience. Ann Rheum Dis 1995;54:134-6.  Back to cited text no. 3
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4.
Subbanna PK, Singh NV, Swaminathan RP. Cutaneous polyarteritis nodosa: A rare isolated cutaneous vasculitis. Indian Dermatol Online J 2012;3:21-4.  Back to cited text no. 4
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5.
Bauzá A, España A, Idoate M. Cutaneous polyarteritis nodosa. Br J Dermatol 2002;146:694-9.  Back to cited text no. 5
    
6.
Vasculitic Disorders. Paller AS, Mancini AJ, ed. Hurwitz Clinical Pediatric Dermatology: A Textbook of skin disorders of childhood and adolescence. 4th ed. Philadelphia, USA: Elsevier Saunders; 2011. p. 483-96.  Back to cited text no. 6
    
7.
Schartz NE, Alaoui S, Vignon-Pennamen MD, Cordoliani F, Fermand JP, Morel P, et al. Successful treatment in two cases of steroid-dependent cutaneous polyarteritis nodosa with low-dose methotrexate. Dermatology 2001;203:336-8.  Back to cited text no. 7
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8.
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;42:459-62.  Back to cited text no. 8
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9.
Morgan AJ, Schwartz RA. Cutaneous polyarteritis nodosa: A comprehensive review. Int J Dermatol 2010;49:750-6.  Back to cited text no. 9
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Fathalla BM, Miller L, Brady S, Schaller JG. Cutaneous polyarteritis nodosa in children. J Am Acad Dermatol 2005;53:724-8.  Back to cited text no. 10
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