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 Table of Contents  
CASE REPORT
Year : 2016  |  Volume : 2  |  Issue : 2  |  Page : 109-111

Cutaneous polyarteritis in adolescence with metabolic syndrome evolving into systemic PAN: A rare presentation


1 Department of Pharmacology, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India
2 Department of Pediatrics, Medical College, Kolkata, West Bengal, India

Date of Submission26-Jul-2016
Date of Acceptance16-Nov-2016
Date of Web Publication13-Jan-2017

Correspondence Address:
Sandeep Lahiry
Department of Pharmacology, Institute of Post Graduate Medical Education and Research, 244 B, A. J. C. Bose Road, Kolkata - 700 020, West Bengal
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2455-3069.198370

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  Abstract 

Cutaneous Polyarteritis nodosa (cPAN) is a rare vasculitis characterized by necrotizing arteritis of small to medium-sized arteries. It manifests as skin ulceration, hypertension, abdominal pain, digital gangrene, and subcutaneous nodules. There is no specific serological marker for diagnosis and confirmation is based on histopathology. Most common mode of pharmacological therapy includes corticosteroid or cyclophosphamide, along with antimicrobials for secondary infection prevention and plasmapheresis. Here, we present a case of a 15-year old boy with multiple skin ulceration, digital gangrene with few nonspecific signs and symptoms, diagnosed as a case of cPAN with features of metabolic syndrome initially, and, underwent regular therapy and follow-up.

Keywords: Childhood PAN, corticosteroids, mycophenolate mofetil


How to cite this article:
Lahiry S, Choudhury S, Mukherjee A, Chowdhury K, Sinha R. Cutaneous polyarteritis in adolescence with metabolic syndrome evolving into systemic PAN: A rare presentation. J Curr Res Sci Med 2016;2:109-11

How to cite this URL:
Lahiry S, Choudhury S, Mukherjee A, Chowdhury K, Sinha R. Cutaneous polyarteritis in adolescence with metabolic syndrome evolving into systemic PAN: A rare presentation. J Curr Res Sci Med [serial online] 2016 [cited 2020 Nov 30];2:109-11. Available from: https://www.jcrsmed.org/text.asp?2016/2/2/109/198370


  Introduction Top


Polyarteritis nodosa (PAN) is a multisystem, necrotizing vasculitis of small and medium-sized arteries. It was first described by Kussmaul and Maier in 1866.[1] Common clinical presentations are lower extremity ulcerations, livedo reticularis, subcutaneous nodules, digital gangrene, abdominal pain, hypertension or some nonspecific features as myalgia, malaise, weakness, etc.

cPAN is a rare disease, affecting the skin with no major skin involvement. The cutaneous symptoms may be similar to systemic PAN or Childhood PAN, and may present with mild fever and fibromyalgic symptoms.


  Case Report Top


A 15 year old obese male adolescent, presented with a history of painful skin ulceration on the back of trunk, shoulders and left gluteal region [Figure 1] for last 3 weeks. Skin ulceration in various stages of evolution, from fresh to old scarred ones was evident. Dry gangrene was present on the right index and middle fingers [Figure 2]. There was no history of fever, cough, weight loss, dysuria, dyspnea, sinusitis, testicular pain, seizure, or recent drug intake before an episode of skin lesions. No features of skin thickening or Raynaud's phenomenon were observed. No significant family history was present. There was no history of recent travel, TB exposure or animal contact. There was no evidence of genetic disorders in immediate family pedigree.
Figure 1: Photograph shows healing subcutaneous ulcer over thigh with scarring

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Figure 2: Healed gangrene of finger (i.e., autoamputated distal phalanx)

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He had been suffering from similar symptoms since 2 years old. Three months ago, he was admitted under Rheumatology Department with spontaneous onset erythematous skin rashes, gradually evolving into necrosis over inner side of both thighs inner side of the left arm and tip of right little finger for last 1 month.

He also had features of metabolic syndrome (obesity, insulin resistance, dyslipidemia) and delayed puberty during his last admission. Endocrine consultation was sought, and he was advised to take the lowest possible dose of steroid.

On examination, his pulse was 90/min with normal volume and regular rhythm and he looked well. Blood pressure was 110/70 mm of Hg. He had cushingoid-habitus and acanthosis nigricans over nape of the neck and axillary area. He had palmar erythema and erythematous papules on the dorsum of both feet. Systemic examination was unremarkable. Investigation revealed hemoglobin 8.8 g/dl, leukocyte count 19,300/cumm with neutrophil 70%, lymphocyte 26%, eosinophil 2%, monocyte 2%; platelet 4.26 × 109/L; serum bilirubin 0.6 mg/dl, albumin 3 g/dl, globulin 3 g/dl, serum urea 26 mg/dl, creatinine 0.6 mg/dl, alanine aminotransferase 104 IU/L, aspartate transaminase 45 IU/L, alkaline phosphatase 106 IU/L, serum sodium 141 mmol/L, potassium 3.5 mmol/L, calcium 10.1 mg/dl, total cholesterol 144 mg/dl, triglyceride 134 mg/dl, high-density lipoprotein 34 mg/dl, serum luteinizing hormone 0.5 mIU/ml, follicle-stimulating hormone 0.3 mIU/ml, serum iron 11 µg/dL, total iron binding capacity 274 µg/dL. All the serological markers including rheumatoid factor, antinucleotide antibody, antineutrophil cytoplasmic antibody, anti-cardiolipin antibody-IgM and IgG and lupus anticoagulant were negative. Hepatitis B surface antigen and anti-hepatitis C virus were nonreactive. Ultrasound of abdomen was unremarkable except for fatty change in liver. Chest X-ray and cervical X-Ray, echocardiography, ophthalmoscopy examinations were normal.

He first developed such features at 2 years of age, which had responded to steroid therapy for 1–3 months. There was polyarthralgia involving small joints of hands, proteinuria (2.4 g/day) without active sediments. Skin lesions used to recur at intervals of 6–9 months, after stopping steroid, for each episode.

Skin biopsy showed evidence of vasculitis of medium-sized vessels within dermis. Macroscopic and microscopic appearances of ulcers were like cutaneous PAN (cPAN). As the child had digital gangrene, polyarthritis, proteinuria, it fullfiled the criteria of Systemic PAN. Currently, he is on oral prednisolone, mycophenolate mofetil, hydroxychloroquine therapy along with oral amoxicillin-clavulanic acid for a secondary skin infection. Further approach is to include another immunosuppressant, oral cyclophosphamide after stopping mycophenolate. Calcium and calcitriol supplementation have also been added.


  Discussion Top


PAN is necrotizing arteritis of medium-sized vessels that has a predilection for involving the skin, peripheral nerves, mesenteric vessels (including renal arteries), heart, and brain, but PAN can virtually involve almost any organ.[2]

Cutaneous and systemic PAN share the same histopathologic features of necrotizing arteritis of small and medium sized vessels. For detecting a case of childhood-PAN, a systemic inflammatory disease with evidence of necrotizing vasculitis or angiographic abnormalities of medium or small-sized arteries (mandatory criterion) plus one of following five criteria: (1) skin involvement; (2) myalgia or muscle tenderness; (3) hypertension; (4) peripheral neuropathy; (5) renal involvement is to be fulfilled.[3] Pharmacotherapy involves corticosteroids, systemic immunosuppressant like cyclophosphamide, etc.

The involvement of kidney is usually limited, and glomerulonephritis is not seen. This distinguishes PAN from microscopic polyangiitis where small vessel vasculitis causes glomerulonephritis and alveolitis.[4]

Clinically, Childhood PAN often is part of the spectrum of Kawasaki disease (KD). Clinically, Childhood PAN with aneurysmal involvement of major coronary arteries and KD are clinically and pathologically indistinguishable. Indeed, the major distinction between KD and Childhood PAN is that the diagnosis of KD is based entirely on clinical criteria, while the diagnosis of Childhood PAN is based on histologic findings. Previously, it had been referred to as infantile polyarteritis nodosa. The use of the term infantile is too restrictive, as infancy connotes age 1 year or younger.

Our patient presented with multiple skin ulceration with digital gangrene since childhood. He satisfied the required 3/10 of the ACR criteria for the diagnosis of PAN and Ozen's criteria. His diagnosis was confirmed by skin biopsy showing vasculitis of medium-sized vessels. Although skin ulcers responded to oral steroid but several recurrences occurred. So as steroid-sparing agent mycophenolate mofetil was introduced and dose of prednisolone was kept as low as possible. He had some nonspecific complaints as weakness, malaise, headache, and myalgia; but did not present with hypertension or hemorrhage which are usually seen in PAN. This case was also not associated with hepatitis B infection.

Association of bacterial infections like streptococcal ones is described with recurrent gangrene in PAN.[5] Other connective tissue diseases such as childhood lupus and scleroderma may also cause gangrene. Despite being a rare disease, PAN is an important cause for gangrene and hypertension in children.[4]

Delayed or no treatment in PAN, may lead to significant morbidity and mortality.[6] In our case, he is continuously on treatment and follow-up since childhood. Corticosteroids are mainstay of treatment in PAN. However, to decrease the adverse effects of steroid in other systems, his dose of prednisolone was kept low and additional drugs are given. To conclude, systemic PAN and cPAN appear to be fairly distinct entities on a clinical continuum, and are challenging entity to diagnose and treat. Regular follow-up of the patient is required. There are only 5 reported cases of cPAN evolving into systemic PAN till date.[7],[8]

Acknowledgment

  • We are thankful to the faculty members of the Department of Medicine, Pediatrics, Pharmacology and Rheumatology of Institute of Post Graduate Medical Education and Research, Kolkata, India, for their invaluable support in letting us carry out the project work
  • We are thankful to the nursing staffs and other health personals of the Department of Medicine of Institute of Post Graduate Medical Education and Research, Kolkata, India for technical support.


Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Kussmaul A, Maier R. Ueber eine bisher nicht beschriebene eigenthümliche Arterienerkrankung (Periarteritis nodosa), die mit Morbus Brightii und rapid fortschreitender allgemeiner Muskellähmung einhergeht. Dtsch Arch Klin Med 1866;1:484-518.  Back to cited text no. 1
    
2.
Lhote F, Cohen P, Guillevin L. Polyarteritis nodosa, microscopic polyangiitis and Churg-Strauss syndrome. Lupus 1998;7:238-58.  Back to cited text no. 2
    
3.
Ozen S, Pistorio A, Iusan SM, Bakkaloglu A, Herlin T, Brik R, et al. EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: Final classification criteria. Ann Rheum Dis 2010;69:798-806.  Back to cited text no. 3
    
4.
Mogale KD, Shrivastava A, Report C. Childhood polyarteritis nodosa: A clinical diagnosis. Indian Pediatr 2006;43:911-3.  Back to cited text no. 4
    
5.
Albornoz MA, Benedetto AV, Korman M, McFall S, Tourtellotte CD, Myers AR. Relapsing cutaneous polyarteritis nodosa associated with streptococcal infections. Int J Dermatol 1998;37:664-6.  Back to cited text no. 5
    
6.
Watanabe K, Rajderkar DA, Modica RF. A case of polyarteritis nodosa associated with vertebral artery vasculitis treated successfully with tocilizumab and cyclophosphamide. Case Rep Pediatr 2016;2016:7987081.  Back to cited text no. 6
    
7.
Magilavy DB, Petty RE, Cassidy JT, Sullivan DB. A syndrome of childhood polyarteritis. The Journal of pediatrics 1977;91(1):25-30.  Back to cited text no. 7
    
8.
Chen KR. Cutaneous polyarteritis nodosa: a clinical and histopathological study of 20 cases. The Journal of dermatology 1989;16:429-42.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2]



 

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