Childhood systemic lupus erythematosus. Association with pancreatitis, subcutaneous fat necrosis, and calcinosis cutis

Abstract

Pancreatitis developed in a 12-year-old girl with a one-year history of systemic lupus erythematosus. The pancreatitis was first manifested by panniculitis of the lower extremities. Calcinosis cutis subsequently developed, both in areas of panniculitis and in areas free of panniculitis. The patient's medications at the time of onset of pancreatitis included prednisone, hydrochlorothiazide, and azathioprine, all of which have been reported to be causes of pancreatitis. We wish to alert physicians that pancreatitis associated with cutaneous panniculitis and calcinosis cutis may develop in children with systemic lupus erythematosus.

Lupus Erythematosus Panniculitis in Children: Report of Three Cases and Review of Previously Reported Cases

“…Low white blood cell counts were seen in reports by Koransky and Esterly (6) (3,500 cells/mm 3 ), Winkelmann and Padilha‐Goncalves (7) (2,100 cells/mm 3 ), and Taieb et al (8) (3,300 cells/mm 3 ). High liver function studies were seen in reports by Wimmershoff et al (9) (patient 1), Marks and Levene (10), and Simons‐Ling et al (11). One specific test to consider in patients with lupus panniculitis is C4, because there have been three reports (8,12,13) of LEP developing in patients with partial C4‐deficiency, including a pediatric case report by Taieb et al (8).…”

“…Based on our review, the major adverse outcome associated with the disease is subcutaneous atrophy once the inflammation resolves. Only one of the previously reported pediatric patients was reported to have SLE (11). In children diagnosed with LEP, the risk of progression to SLE is unknown.…”

Pancreatic panniculitis in active systemic lupus erythematosus

“…The first of these was a 60‐year‐old woman with SLE and a pancreatic acinar cell carcinoma . The second was 12‐year‐old girl with SLE and pancreatitis in the setting of prednisone, hydrochlorothiazide, and azathioprine, all of which have been reported as causes of pancreatitis …”

Generalized Lupus Panniculitis and Antiphospholipid Syndrome in a Patient Without Complement Deficiency

“…Generalized forms of LEP are exceedingly rare and have been reported in association with hereditary deficiencies of complement, particularly of C2 and C4. Excluding a case of LEP associated with neonatal lupus (3) and a case of pancreatic panniculitis in a patient with lupus erythematosus (4), we have found four reported cases of generalized LEP (i.e., affecting more than one anatomic site) in children under age 13 in the English literature. In the only case with extensive complement studies, the patient was found to have hereditary deficiencies of C2 and C4 (5).…”