A 63-year-old Japanese man with a 25-year history of paroxysmal nocturnal hemoglobinuria (PNH) treated with intermittent low-dose corticosteroid therapy presented with recurrent episodes of hemoglobinuria, progressive cutaneous purpura with hemorrhagic bullae, and high fever. Dermatologic examination found extensive cutaneous purpura with multiple hemorrhagic bullae on the abdomen, back, and all extremities (Figure 1A and B). There were no signs of cellulitis. Blood culture findings were negative. One week later, he developed extensive ulcerations and necrosis (Figure 1C).
Nagase K, Okawa T, Otsu M, Miura Y, Misago N, Narisawa Y. Extensive Cutaneous Ulcerations and Necrosis Associated With Paroxysmal Nocturnal Hemoglobinuria. Arch Dermatol. 2012;148(5):660–662. doi:10.1001/archdermatol.2011.3536
Customize your JAMA Network experience by selecting one or more topics from the list below.