Necrotizing Fasciitis in Paroxysmal Nocturnal Hemoglobinuria

Abstract

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, progressive, and life-threatening hematopoietic stem cell disorder characterized by complement-mediated intravascular hemolysis and a prothrombotic state. Patients with PNH might have slightly increased risk of infections due to complement-associated defects subsequent to CD59 deficiency. Here, we report a rare case of a 65-year-old male patient with necrotic ulcers on both legs, where the recognition of pancytopenia and microthrombi led to the diagnosis of PNH based on FLAER (FLuorescent AERolysin) flow cytometric analysis. He was subsequently started on eculizumab therapy, with starting and maintenance doses set as per drug labelling. Progression of the patient's leg ulcers during follow-up, with fulminant tissue destruction, purulent discharge, and necrotic patches, led to a later diagnosis of necrotizing fasciitis due to Pseudomonas aeruginosa and Klebsiella pneumonia infection. Courses of broad-spectrum antibiotics, surgical debridement, and superficial skin grafting were applied with successful effect during ongoing eculizumab therapy. This case highlights the point that it is important to maintain treatment of underlying disorders such as PNH in the presence of life-threatening infections like NF.

Figure 1

Lesions (a) at first arrival and (b) after debridement.

Figure 2

Lesions after infection was controlled with appropriate antibiotherapy and treatment of PNH. (a) Anteromedial part of upper right leg with newly developing scar tissue in the border of wound; (b) interior site of upper left leg showing successful debridement.

Figure 3

Lesions after skin grafting showing clean grafting site and successful surgery. (a) After skin grafting applied to anteromedial part of right upper leg, showing successful attachment; (b) successful healing of lesion located at posterolateral part of left upper leg.

Figure 4

Lesions at time of last control showing almost full healing. Almost totally resolved lesion in (a) right and (b) left upper leg.