عنوان

Pyoderma gangrenosum: a presenting sign of myelodysplastic syndrome in undiagnosed Fanconi anemia

Number

LA9xj8b544

.Language of Text, Soundtrack etc

انگلیسی

Title Proper

Pyoderma gangrenosum: a presenting sign of myelodysplastic syndrome in undiagnosed Fanconi anemia

General Material Designation

[Article]

First Statement of Responsibility

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

Text of Note

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.

Date of Publication

2019

Title

Dermatology Online Journal

Volume Number

25/1

Entry Element

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

Electronic name

[Article]

275578

a

Y