Cutaneous Polyarteritis Nodosa – A Rare, Benign Vasculitis - medIND

Abstract
CASE REPORT
Cutaneous Polyarteritis Nodosa –
A Rare, Benign Vasculitis
Smita Gupta*, Lalit Duggal**, Neeraj Jain**
JIACM 2006; 7(2): 159-60
A 26-year-old lady presented with discolouration, subcutaneous nodules over limbs, and early gangrene of toe. She had incidental
urinary tract infection. Biopsy of the nodule revealed a picture of cutaneous polyarteritis nodosa. UTI was treated and she was
managed with steroids and methotrexate to which she responded in four months time.
Introduction
Cutaneous polyarteritis nodosa (PAN) is a rare form of
vasculitis which involves small and medium sized arteries
of dermis and subcutaneous tissue 1 . It is characterised by
tender subcutaneous nodules and livedo reticularis that
may ulcerate 2 . The pathogenesis of cutaneous polyarteritis
nodosa is not clearly understood. Systemic involvement is
uncommon except for peripheral neuropathy, and
progression to classical polyarteritis nodosa is an exception.
It is essentially a benign disorder and should be
distinguished from systemic PAN, as the clinical course and
management of the two conditions is different.
Case report
A 26-years-old lady presented with a history of reddish
discolouration over feet, lower limbs, and both forearms
alongwith painful nodules over lower limbs since last 6
months. She also had pain and bluish discolouration of
right second toe since 7 days. There was no associated
history of fever, joint pains, oral ulcerations, dysuria or
bloody/high coloured urine, abdominal pain, headache,
or paraesthesias of limbs. She was not a known
hypertensive. General physical and systemic examination
were normal, and her blood pressure was 120/80 mm Hg.
On local examination, she had hyperpigmented
maculopapular rashes, and tender subcutaneous nodules
over feet and lower legs alongwith bluish discolouration
of right second toe which was tender and cold (Fig. 1). On
investigations, her haemoglobin was 11.8 gm%, TLC-7,300/
cu mm, platelet count-3,08,000 and ESR-34 mm/1st hr.
Her kidney and liver function tests were normal. Urine
(routine examination) revealed WBC-60 - 80/hpf with no
casts. Urine culture showed E. coli (colony count > 10 5 ).
Her 24 hr urinary protein was normal. Hepatitis B and C
viral markers and CRP were negative. The ANA (by IF), p-
ANCA (ELISA), cryoglobulins, anticardiolipin antibody, and
lupus anticoagulants were negative. Arterial doppler of
lower limb, and nerve conduction velocity (NCV) were
normal. Skin biopsy taken from skin nodule was suggestive
of nodular arteritis with a polymorphonuclear infiltrate
involving medium sized arteries in deep reticular dermis
alongwith extensive fibrinoid necrosis and vascular
destruction (Fig. 2). A diagnosis of cutaneous polyarteritis
nodosa with early gangrene of toe with UTI was made.
She was started on prednisolone 0.5 mg/kg of body weight
alongwith methotrexate 10 mg/week and was also treated
for UTI. Steroids were tapered-off over 3 months. At followup
of 4 months she had residual erythema over feet, no
nodules, and the gangrenous toe was normal.
Fig. 1: Reddish discolouration of both lower limbs and feet, and bluish
discolouration of right second toe.
* Senior Resident, ** Consultant, Department of Medicine, Sir Ganga Ram Hospital, Rajinder Nagar, New Delhi - 110 060.

Fig. 2: Polymorphonuclear infiltrates in deep reticularis dermis
involving medium sized arteries with extensive fibrinoid necrosis.
Discussion
Cutaneous PAN is a rare form of cutaneous vasculitis. It
involves small, medium sized arteries of dermis and
subcutaenous tissue. It is also called periarteritis nodosa,
and should be differentiated from systemic PAN due to the
different clinical course and management of the two
conditions. The cause of cutaneous PAN is not known; it
could be a hypersensitivity reaction to certain infections 1 .
In a series published by Geralol M et al in 1991, 4/9 patients
had hepatitis B and 1 had a HIV infection 3 . Women were
affected slightly more than men 2 . Our patient had UTI, which
could have been a triggering factor, though there is paucity
of literature on this aspect.
Cutaneous PAN is characterised by tender subcutaneous
nodules, usually measuring 4 - 5 mm in diameter,
alongwith infarcts presenting as purple or black patches
and livedo reticularis that may ulcerate 2 . They are mostly
found over legs and feet. Systemic involvement is rare
in contrast to classical PAN where there is visceral
involvement.
These patients may have neuromuscular involvement in
the form of peripheral neuropathy that presents with
tingling, numbness, sensory disturbances, weakness, and
absent reflexes. In a study by Daud MS et al in 1997, 22%
of 79 cases had some evidence of neuropathy 2 . Millard H
et al in 1999 showed that 3/9 patients had neuropathy
without systemic involvement 4 . Patients may also have
some constitutional symptoms in the form of malaise,
fever, sore throat, joint/muscular pains. The above features
were absent in our patient. The definitive diagnosis is made
by doing a skin biopsy. No other lab parameter is
suggestive, except for high ESR which may show
inflammatory process. In cutaneous PAN, histopathological
examination shows features of nodular arteritis with
polymorphonuclear infiltrates involving medium sized
arteries in deep reticular dermis. There is extensive
fibrinoid necrosis. This is in contrast to classical PAN which
rarely shows nodular arteritis and the picture is of small
vessel leucocytoclastic.
Cutaneous PAN runs a chronic course lasting months to
years, and has a waxing and waning phenomenon. Patients
are generally treated with non-steroidal anti-inflammatory
drugs and oral steroids. Immunosuppressive drugs can also
be used in low doses in more severe kinds of cutaneous
PAN and as steroid-sparing drugs 5 . It is generally a benign
disease with progression to a classical disease being an
exception. A close watch however, should be kept for
progression to systemic involvement.
References
1. Khoo BP, Ng SK. Cutaneous polyarteritis nodosa: A case
report and literature review. Ann Acad Med Singapore 1998;
27: 868-72.
2. Daoud MS, Hutton KP, Gibson LE. Cutaneous polyarteritis
nodosa: a clinicopathological study of 79 cases. Br J
Dermatol 1997; 136 (5): 706-13.
3. Gerald M, Bruce RS, Scott McNutt N. Benign cutaneous
polyarteritis nodosa. Arch Dermatol 1991; 127: 1520-3.
4. Maillard H, Szczesniak S, Martin L et al. Cutaneous
polyarteritis nodosa: diagnostic and therapeutic aspects
of 9 cases. Ann Dermatol Venereol 1999; 126 (2): 125-9.
5. Kleeman D, Kempf W, Burg G, Hafner J. Cutaneous
polyarteritis nodosa. Vasa 1998; 27 (1): 54-7.
160 Journal, Indian Academy of Clinical Medicine Vol. 7, No. 2 April-June, 2006