Pyoderma gangrenosum with renal and splenic impairment - Case report
A 22-year-old female patient was referred for evaluation of tender bullae and nodules on\nher lower limbs that rapidly progressed to ulcers with purulent and necrotic base and\nirregular and undermined borders ().\nLesions had been present for the previous four months and the patient also complained of\nfever (38º C) and abdominal pain. Her medical history revealed a persistent anemia since\nchildhood. On physical examination, splenomegaly and pallor of ocular mucosa were\ndetected.\nClinical diagnosis of PG was corroborated by the histopathologic finding of a dermal\nlymphocytic and histiocytic infiltrate with some perivascular neutrophils and\nplasmocytes. Subcutaneous fat tissue was densely infiltrated with lymphocytes and\nhistiocytes and numerous interlobular polymorphonuclear leukocytes (). Gram stain and KOH examination of the ulcer discharge\nyielded negative results.\nLaboratory workup showed a positive ANA (titer 1:160; nuclear, fine, speckled pattern)\nnormocytic and normochromic anemia, low serum iron and high ferritin levels. Bone marrow\naspiration displayed hypercellularity and 23% of sideroblasts, but no ringed\nsideroblast. Normal or negative exams included anti-SM, anti-Ro, anti-La, P-ANCA,\nC-ANCA, rheumatoid factor, antiphospholipid antibodies, cryoagglutinins, anti-HIV,\nhepatitis serology, blood and urine cultures, complement fractions C3 and C4.\nMultiple splenic nodular structures were detected on abdominal US. MRI showed\nintrasplenic and subphrenic collections (). Gram staining and bacterial culture of needle aspirated material from the\nspleen were negative (cold abscess).\nTreatment with an association of corticosteroid, cyclosporine and sulfasalazine resulted\nin significant improvement (). A control\nUS detected reduction of the dimensions of both the spleen and intrasplenic collections.\nLesions recurred upon tapering of corticosteroid and withdrawal of cyclosporine. A\nsecond MRI detected intrasplenic and subdiaphragmatic collections and an altered sign on\nthe right kidney upper pole (). Gram\nstaining and bacterial cultrure from material obtained by renal biopsy was negative.\nHistopathologic findings were consistent with PG adjacent to the renal parenchyma with a\ndense granular and mononuclear infiltrate, xanthomatous macrophages and a large area of\nnecrosis ().