Extensive Cutaneous Ulcerations and Necrosis Associated With Paroxysmal Nocturnal Hemoglobinuria

A Middle‐Aged Woman With Necrotic Breast Lesions

“…Paroxysmal nocturnal hemoglobinuria. Similarly, paroxysmal nocturnal hemoglobinuria (PNH) is a disorder mediated by a deficiency of complement regulatory proteins such as CD55 and CD59 (11). PNH is associated with thrombotic microangiopathy and hemolytic anemia, causing fatigue, dyspnea, renal failure, hemoglobinuria, and cutaneous manifestations such as purpura and skin necrosis.…”

Extensive tissue injury in the setting of sepsis

“…The clinical syndrome is characterized by fever, myalgia and acute erythema followed by desquamation, and 5% of patients develop blistering 4,5 . The disorder is often complicated by circulatory shock, renal failure, disseminated intravascular coagulation and acute respiratory distress syndrome 1,6 …”

“…Sepsis‐related purpura fulminans is most commonly attributed to Neisseria meningitidis and Streptococcus pneumoniae ; however, it can occur in sepsis from group A and B streptococci, Haemophilus influenzae and Staphylococcus aureus 5 . The cutaneous microvascular occlusion in purpura fulminans is clinically recognized as retiform (or stellate) purpura, which rapidly progresses to necrosis and eschar formation 6 . An acquired protein C deficiency leads to a procoagulant state and thrombus formation in the vessels supplying the skin’s superficial vascular plexus 7 .…”

“…4,5 The disorder is often complicated by circulatory shock, renal failure, disseminated intravascular coagulation and acute respiratory distress syndrome. 1,6 The identification of a group A b-haemolytic streptococcus bacteraemia directs the clinical examination and investigations to uncover an infection source. This includes looking for both overt and occult soft tissue infections.…”

“…5 The cutaneous microvascular occlusion in purpura fulminans is clinically recognized as retiform (or stellate) purpura, which rapidly progresses to necrosis and eschar formation. 6 An acquired protein C deficiency leads to a procoagulant state and thrombus formation in the vessels supplying the skin's superficial vascular plexus. 7 In adults with sepsis-related purpura fulminans, use of activated protein C does not improve prognosis.…”