OBJECTIVE: TO PRESENT AND DISCUSS THE CASE OF A 9-YEAR OLD FEMALE DIAGNOSED WITH CUTANEOUS POLYARTERITIS NODOSA (CPAN) WITH ERYTHEMA NODSUM (EN), DIGITAL GANGRENE AND AUTO-AMPUTATION.
CASE: THE PATIENT IS A 9-YEAR-OLD FEMALE WHO INITIALLY PRESENTED WITH PAIN ON BOTH HANDS AND FEET AND BIPEDAL EDEMA. 2 MONTHS LATER, TENDER, BLUISH TO PURPLISH NODULES APPEARED ON HER LOWER EXTREMITIES UP TO THE THIGHS, ASSOCIATED WITH HIGH FEVER AND DIFFICULTY IN AMBULATION. SHE WAS CONFINED SEVERAL TIMES DURING WHICH SHE RECEIVED ANTIBIOTIC TREATMENT FOR PNEUMONIA AND STARTED ON QUADRUPLE ANTI-TUBERCULOSIS THERAPY. DESPITE COMPLIANCE TO TREATMENT AND INFECTION CONTROL, NODULES PERSISTED. THE DISCOLORED DIGITS BECAME GANGRENOUS AND EVENTUALLY AUTO-AMPUTATED. LABORATORY TESTS SHOWED PERSISTENT ANEMIA, LEUKOCYTOSIS, AND INCREASED ACUTE PHASE REACTANTS, AS WELL. PEDIATRIC RHEUMATOLOGY CONSULT WAS SOUGHT AND FULL THICKNESS SKIN BIOPSY OF AN EN WAS DONE. HISTOPATHOLOGY SHOWED NON-NECROTIZING VASCULITIS. SHE WAS DIAGNOSED WITH CPAN AND RECEIVED HIGH DOSE CORTICOSTEROIDS WHICH RESULTED IN RESOLUTION OF NODULES AND RECOVERY OF FULL RANGE OF MOTION OF ALL JOINTS. DUE TO THE PRESENCE OF MULTIPLE DIGITAL GANGRENE, SHE WAS STARTED ON METHOTREXATE. FIVE YEARS AFTER, SHE CAME BACK WITH HIGH FEVER, MULTIPLE EN, SEVERE PAIN ON BOTH HANDS AND FEET, AND GANGRENE INVOLVING MORE DIGITS. METHYPREDNISOLONE PULSE THERAPY WAS AGAIN ADMINISTERED RESULTED IN CLINICAL IMPROVEMENT. METHOTREXATE WAS RE-INITIATED. CURRENTLY, SHE IS MAINTAINED ON METHOTREXATE AND IS IN CLINICAL REMISSION.
CONCLUSION: CPAN FOLLOWS A CHRONIC, RELAPSING COURSE WHICH MAY LEAD TO AUTO-AMPUTATION OF DIGITS. IT MAY PRESENT SIMILARLY OR CONCOMITANTLY WITH INFECTIONS, PARTICULARLY TUBERCULOSIS. THE CLINICAL COURSE OF THIS 9-YEAR OLD GIRL HAD A RELATIVELY AGGRESSIVE PRESENTATION BUT WITH REMARKABLE RESPONSE TO CORTICOSTEROIDS AND A DISEASE-MODIFYING ANTI-RHEUMATIC DRUG.