Year : 2007 | Volume
: 52 | Issue : 3 | Page : 153--154
Classic polyarteritis nodosa in childhood
Alok Kumar Roy, Mousomi Nandi, Sanat Kumar Ghosh, Arunasis Maiti
Department of Dermatology, NRS Medical College, Kolkatta - 700 014, WB, India
Alok Kumar Roy
Department of Dermatology, NRS Medical College, Kolkata - 700 014, WB
Polyarteritis nodosa (PAN) is a medium-sized vessel vasculitis that usually occurs in 40-60 years of age. It is a rare multisystemic disease that is frequently associated with cutaneous manifestation. It rarely occurs in childhood. We herewith report a case of classic PAN in a 6-years-old boy with digital gangrene.
|How to cite this article:|
Roy AK, Nandi M, Ghosh SK, Maiti A. Classic polyarteritis nodosa in childhood.Indian J Dermatol 2007;52:153-154
|How to cite this URL:|
Roy AK, Nandi M, Ghosh SK, Maiti A. Classic polyarteritis nodosa in childhood. Indian J Dermatol [serial online] 2007 [cited 2020 Jan 18 ];52:153-154
Available from: http://www.e-ijd.org/text.asp?2007/52/3/153/35096
Although originally described by Kussmaul and Maier in 1866 as periarteritis nodosa (PAN), the term PAN was coined by Ferrari in 1903 after he recognized it as transmural inflammation. PAN is a necrotizing vasculitis affecting the medium-sized arteries. Vasculitis can involve the arteries in almost any organ of the body producing varied systemic symptoms and signs that may manifest simultaneously from beginning or appear sequentially over a period of months to years. Approximately 20-50% of patient with PAN exhibit cutaneous signs.  It can occur at any age, but more commonly in fourth to sixth decade of life and males are found to be more commonly affected. The occurrence of PAN is extremely rare in childhood.  The causes of PAN are unknown, but the occurrence of PAN after group-A streptococcal infection and hepatitis-B infection suggests that it may be a postinfection autoimmune response to these agents in a susceptible person. Pathology in PAN is necrotizing vasculitis of varying grade from mild to serve fibrinoid necrosis, thrombosis and aneurysm formation. Clinical manifestation is variable depending on the system involved and usually presented with fever, malaise, weight loss, myalgia, arthralgia, limb pain, abdominal pain, hypertension, orchitis and different types of skin lesions. Depending on the predominant size of vessel and body parts involved, it can present as classic PAN, cutaneous PAN or microscopic polyangiitis .
A 6-year-old boy admitted in our hospital with high-grade intermittent and irregular fever for last one year was associated with fatigue, weight loss, anorexia, occasional abdominal pains and recurrent cough and cold. There was history of intermittent ankle, knee and elbow joint swelling and tenderness gradually progressing to mild restriction and pain on movement. The patient also had gangrenous changes in the toes of his left foot and localized skin necrosis over the medial aspect of left ankle, knee and left hand [Figure 1]. Four months back, the patient lost his left little finger due to gangrene [Figure 2]. There was no significant family history, perinatal history and immunization was up to date.
On examination, the child was conscious, oriented, but appeared to be very ill. There was mild pallor without icterus, edema, cyanosis, and conjunctival or mucosal congestion. The body temperature was high, pulse was 100/min, the respiratory rate was high and the BP was 160/110 mmHg. On systemic examination, only the liver was mildly palpable, but the central nervous system (CNS) and respiratory system were clinically normal. The examination of the skin revealed a blackish necrotic skin in a linear fashion at the left ankle and multiple small (approximately 0.5 cm) nodules at the anteromedial aspect of left leg that was distributed linearly upwards. Reticular erythematous, telangiectatic fine vascular lesions were also found at several places.
Significant investigation reports were are follows: Hb - 10 gm/dl, WBC - 41,500/cmm (N 85%), platelet count - 7,50,000/dl, CRP - 26/dl, HBsAg (- ve), Anti ds DNA (-ve). High ASO titer (1670 iu/ml) was observed and ELISA of HIV 1 and HIV 2 was negative; both cANCA and pANCA were negative. Serum urea, creatinin and urine (RE/ME) examinations were normal. Skin biopsy showed arterial wall inflammation with necrosis and perivascular infiltrate. Doppler study of renal artery shows increased restrictive index and the ACE time increased suggestive of the branched renal artery disease.
PAN is a rare syndrome with multifocal segmental necrotizing vasculitis of small and medium sized arteries. Although PAN is uncommon in infants, children and the adolescent group, there are a number of reports on childhood cases. Infantile PAN usually occurs before the age of 2 years and its presentation is slightly different from adult and childhood PAN, which most commonly affect the coronary arteries with aneurysm formation and congestive heart failure. However, the clinical manifestation of childhood PAN is more or less similar to adult PAN. The most common variety of PAN reported in children is cutaneous PAN. Classic PAN is a rare disease in adult and extremely rare in children. We report this case of classic PAN in a 6-year-old child who was presented with intermittent, medium- to high-grade fever for last one year with one episode of skin necrosis and digital gangrene, which occurred 4 months back. Gradually, the child lost weight, developed joint and muscle pains and mild intermittent abdominal pain; however, the cause of fever was undiagnosed till he attended our hospital with the second episode of cutaneous manifestation. The child presented with most of the classical skin signs of PAN such as palpable purpura, skin necrosis, skin nodule, livedo reticularis and digital gangrene with the loss of one finger in the left hand. The associated systemic signs were hypertension, fever, musculoskeletal pain, weakness and occasional abdominal pains.
Although CNS pathology is thought to occur in 15-66% of cases of childhood PAN and peripheral neuropathy occurs in 30-70% of patients;  however, our patient was well oriented with intact memory and cognition and there were no signs of peripheral neuropathy. There was no cardiological abnormality except sinus tachycardia.
The clinical criteria for diagnosis of PAN by American College of Rheumatology and Ozen's diagnostic criteria for PAN in childhood were met by our patient.
Although the association of hepatitis-B with classic PAN has been reported, our patient was HbsAg negative. Childhood PAN has also been reported to occur after streptococcal sore throat infection. In our case, although throat swab culture was negative, ASO titer was highly positive before treatment and it subsided after the treatment was started with penicillin and steroids. We started treating the patient with systemic prednisolone 2mg/kg/day along with nifedipine and the local care of the gangrenous area and rapid improvement was observed. Within three weeks, the patient became afebrile, skin ulceration was healed, skin nodule disappeared and the gangrenous changes improved. The blood pressure, musculoskeletal symptoms and general well-being of the patient improved. Gradually, ASO titer decreased, and the WBC and platelet count became normal. After two months of follow-up, the child became almost normal. Prednisolone 1 mg/kg /day was continued for 6 months.
We are reporting this case because classic childhood PAN is very rare with few reported cases in our country. Second, the patient has thrombocytosis, high ASO titer and characteristic skin ulceration, nodule and digital gangrene with amputation of one finger.
To conclude, we can state that the clinical finding and progression of PAN in childhood is highly variable and may be missed when presented with few symptoms at the beginning without definite diagnostic signs, which may appears gradually as the disease progresses. Therefore, a high suspicion and thorough clinical examination for any systemic or cutaneous finding may assist in the early diagnosis of such cases.
|1||Fiorentino DF. Cutaneaous vasculitis. J Am Acad Dermatol 2003;48:311-40.|
|2||Pachman LM. Vasculitis syndrome. In : Behrman RE, Kliegman RM, Jenson HB, editors. Nelson text book of pediatrics, 17 th ed. Elsevier: Philadelphia; 2004. p. 826-30.|
|3||Engel DG, Gospe SM, Tracy KA, Ellis WG. Lie in Fatal Infantile polyartentis Nodosa with predominant central nervous system involvement. Stroke 1995;26:699-701.|