Seckel syndrome with polyarteritis nodosa

Seckel syndrome is a rare genetic disorder with a typical "bird-headed" appearance. It could affect many organ systems but renal involvement is uncommon. Polyarteritis nodosa is systemic vasculitic disorder which also involves kidneys. We report a case of Seckel syndrome in a 9 year-old boy with renal involvement due to polyarteritis nodosa. According to the literature, this is the first report of polyarteritis nodosa in Seckel syndrome.     

Childhood cutaneous polyarteritis nodosa

Cutaneous polyarteritis nodosa is a form of polyarteritis nodosa. It is a rare disease in children and is characterized by its benign and chronic course. There is no evidence of hypertension or organ dysfunction. The aim of this study was to present a case of cutaneous polyarteritis nodosa and to review the literature. We describe the case of a 3-year-old male child with multiple, red, painful, edematous nodules of the extremities and trunk. Dermatologic findings were accompanied by fever and arthritis. Laboratory findings showed an acute phase response associated with raised antistreptolysin titer. Diagnosis was based on biopsy of the affected skin lesion in which necrotizing arteritis of the lower dermis with neutrophilic and eosinophilic infiltrates was found. The patient responded well to corticosteroid therapy. The evaluation of children with cutaneous polyarteritis nodosa should include laboratory studies to detect streptococcal infection.     

Fatal polyarteritis nodosa with gastrointestinal involvement in a child

Polyarteritis nodosa (PAN) is a necrotizing angiitis that predominantly affects small- and medium-sized arteries. Polyarteritis nodosa occurs rarely during childhood. Boys and girls seem to be equally affected, with a peak at the age of 10 years. Rarely, severe and fatal gastrointestinal involvement is seen in PAN. Here, we report a 15-year-old boy with PAN, who had gastrointestinal involvement with multiple aneurysms of the hepatic and superior mesenteric arteries. This involvement could be demonstrated with conventional angiography and gastrointestinal bleeding scintigraphy. The progression of the symptoms and the decrease in the size of the aneurysms were noted after combination treatment with cyclophosphamide and prednisolone, but there was severe bleeding from small bowel, and it was taken under control by resection of jejunum. However, the patient died because of sepsis. In conclusion, severe gastrointestinal involvement in PAN is usually fatal despite aggressive therapy, as is the case in our patient.     

Cutaneous polyarteritis nodosa in a patient with ulcerative colitis

Skin disease is common in patients with inflammatory bowel disease. Described herein is a child with ulcerative colitis and cutaneous polyarteritis nodosa. Review of the literature suggests cutaneous polyarteritis must be considered as another skin lesion associated with inflammatory bowel disease. Cutaneous polyarteritis tends to run a chronic relapsing course independent of bowel disease, however.     

Childhood polyarteritis nodosa: a clinical diagnosis

A six-year old boy presented with dry gangrene of toes and fingers with hypertension with no other systemic abnormalities. He had persistently high inflammatory parameters, was diagnosed as childhood classic polyarteritis nodosa and showed improvement with immunosuppressants along with antihypertensives. Toe gangrene required amputation in view of superadded infection.     

Juvenile polyarteritis nodosa associated with toxoplasmosis presenting as Kawasaki disease

Polyarteritis nodosa (PAN) is a vasculitis characterized by inflammatory necrosis of medium‐sized arteries. Juvenile PAN and Kawasaki disease (KD) both cause vasculitis of the medium‐sized arteries, and share common features. They have overlapping clinical features. Treatment should be managed according to the severity of symptoms and persistence of clinical manifestations. Herein is described the case of a 14‐year‐old boy first diagnosed with KD, who then fulfilled the criteria for juvenile PAN due to the development of severe myalgia, persistent fever, polyneuropathy and coronary arterial dilatation. He also had acute toxoplasmosis at the onset of vasculitis symptoms. The final diagnosis was of juvenile PAN associated with toxoplasmosis infection. Toxoplasma infection can be considered as an etiological agent for PAN and other vasculitis syndromes. Awareness of toxoplasmosis‐related PAN facilitates early diagnosis, and instigation of appropriate treatment.     

Inferior suprarenal artery aneurysm in polyarteritis nodosa

A 16-year-old boy with the complaints of fever, abdominal pain, arthralgia and hypertension was found to have polyarteritis nodosa. Selective renal angiography disclosed a 5-mm microaneurysm of the right inferior suprarenal artery, which has not been previously reported in polyarteritis nodosa. Aside from the diagnostic viewpoint, some of the perirenal and retroperitoneal hemorrhages of unknown origin seen in polyarteritis nodosa might be due to the rupture of suprarenal artery aneurysms. Received: 6 December 1995 Accepted: 8 February 1996     

Cutaneous polyarteritis nodosa in a young child.

A 5 1/2-year-old girl presented with an acute febrile illness associated with limb and facial swelling, and a skin eruption. She was diagnosed as suffering from cutaneous polyarteritis nodosa. She was told to stay in bed and was given soluble aspirin. Now, more than 3 years later, she is well and symptom-free. The important diagnostic feature of this benign condition, which is distinct from the systemic disease, is the presence of skin nodules showing the histology of a necrotising arteritis at the junction of dermis and subcutaneous tissue.     

Infantile polyarteritis nodosa presenting as hyponatraemic hypertensive syndrome

The association of arterial hypertension with hyponatraemic dehydration, known as hyponatraemic hypertensive syndrome (HHS), is a rare and serious hypertensive complication. Here, we describe a 17-mo-old girl who presented with severe hyponatraemic dehydration, hypokalaemia, polyuria, and nephrotic-range proteinuria associated with malignant arterial hypertension and systemic inflammatory disease. Diagnosis of classic polyarteritis nodosa (c-PAN) was made on the basis of renal arteriography demonstrating small arterial aneurysms in association with non-aneurismal changes such as arterial cut-off, arterial tapering stenosis and nephrogram perfusion defect. A decrease of blood pressure by antihypertensive treatment resulted in the normalization of HHS abnormalities. However, c-PAN became well controlled only after 4 mo of immunosuppressive therapy. CONCLUSION: The main interest of this case was the uncommon presentation of systemic polyarteritis nodosa in a very young child. Renal ischaemia from intrarenal vessel disease may have been the trigger event for HHS in our case. Management of PAN-associated severe arterial hypertension is based on immunosuppressive and antihypertensive treatment.     

Isolated polyarteritis nodosa of the gallbladder in older children

A 10-year-old-boy with acute acalculous cholecystitis due to polyarteritis nodosa of the gallbladder with limited involvement of this organ is reported. It is suggested that a search for other lesions is indicated when polyarteritis nodosa of the gallbladder has been diagnosed, but that no treatment is indicated when additional lesions are not found.     

Paediatric enteritis as a presentation of infantile polyarteritis nodosa

Multisystem arteritis is a rare condition in paediatric practice. We describe a 4-month-old boy presenting with an acute abdomen. At laparotomy the small bowel had the appearance of necrotising enterocolitis. The child died shortly after surgery from unsuspected cardiac involvement. The clinical features, diagnosis, and management are reviewed. Correspondence to: D. Talbot     

Anti-neutrophil cytoplasmic antibody for proteinase 3 in a child with polyarteritis nodosa

We report a case of a 15 year old boy with polyarteritis nodosa associated with antineutrophil cytoplasmic antibody (ANCA) against proteinase 3 (PR3). After months of steroid and immunosuppressant therapy, the symptoms subsided and the polyaneurysms almost disappeared. The levels of anti-PR3 antibody and of cytokines also decreased. The results indicate that ANCA is a good indicator of this disease activity, and may play some pathogenic role in the disease.