Pyoderma gangrenosum: A presenting sign of myelodysplastic syndrome in undiagnosed fanconi anemia

Prasanthi Kandula, Kishan M. Shah, Jay E. Wolverton, Cuong Le, Stephen Wolverton

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Abstract

A 26-year-old man with a history of congenital bilateral microtia, unilateral renal agenesis, left aural atresia, and right external auditory canal occlusion admitted for right rib cartilage graft harvest and left ear re-construction. Following surgery, an ulceration with violaceous borders and a yellow fibrinous base unresponsive to broad-spectrum antibiotics developed at the harvest site. The wound was expanding and not responsive to systemic broad-spectrum antibiotics. Biopsy revealed a dense dermal infiltrate of neutrophils with negative tissue cultures consistent with pyoderma gangrenosum (PG). He was treated with systemic, intralesional, and topical steroids, as well as doxycycline. Three weeks after the diagnosis of PG, he was found to have persistent anemia and leukopenia. Bone marrow aspiration analysis was consistent with hypocellular myelodysplastic syndrome and genetic testing was consistent with Fanconi anemia. There is a well-known association of PG with hematological disorders. Fanconi anemia is a rare genetic hematologic disorder with congenital defects leading to bone marrow failure and malignancy in long-standing disease. In our patient, we consider his development of PG a paraneoplastic sign associated with the onset of his hypocellular myelodysplastic syndrome.

Original languageEnglish (US)
JournalDermatology Online Journal
Volume25
Issue number1
StatePublished - Jan 1 2019

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Keywords

  • Fanconi anemia
  • Myelodysplastic syndrome
  • Pyoderma gangrenosum

ASJC Scopus subject areas

  • Dermatology

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