Recurrent pyoderma gangrenosum and agnogenic myeloid metaplasia

Abstract

Pyoderma gangrenosum has been associated with myelogenous leukemia and plasma cell dyscrasia. When associated with leukemia, pyoderma gangrenosum often has a distinctive clinical presentation with an advancing bullous margin. The pathogenesis of this disorder is unknown, although defective immune mechanisms may be operative. The occurrence of pyoderma gangrenosum and agnogenic myeloid metaplasia in the same patient has now been reported sufficiently to make it a recognized association.

Bullous pyoderma gangrenosum as the presenting sign of fatal acute myelogenous leukemia

“…Hematologic abnormalities reported in association with bullous pyoderma gangrenosum have included 'blast' transformation of chronic myelogenous leukemia in a patient with a history of polycythemia vera [9,10], acute myelogenous leukemia [9,15,19 -21], myelofibrosis [6,10], chronic myeloid leukemia [15,22], chronic lymphocytic leukemia [9], refractory anemia with excess blasts [15], IgG-producing multiple myeloma [8] and disseminated intravascular coagulation preceding the diagnosis of chronic myelomonocytic leukemia [16]. At least 2 patients with myeloid malignancies developed bullous pyoderma gangrenosum after treatment with granulocyte-colony-stimulating factor was initiated [23,24].…”

“…Pathergy, a phenomenon characterized by exacerbation of a disease after trauma, is commonly observed, especially after debridement of a lesion which, in an early stage, may be mistaken for a furuncle or abscess [3]. Because laboratory evaluations and histologic features of pyoderma gangrenosum are nonspecific, definitive diagnosis requires both clinical recognition and exclusion of infectious or neoplastic disorders in the differential diagnosis [6].…”

Bullous Pyoderma Gangrenosum in a Patient with Acute Myelogenous Leukemia as a Pathergic Reaction after Bone Marrow Biopsy

“…Definitive diagnosis requires both clinical recognition and exclusion of infectious or neoplastic disorders [ 3 ]. PG is usually associated with an underlying systemic disease [ 1 , 4 ].…”