ABSTRACT
Pustular psoriasis is a rare and distinct variant of psoriasis characterized by sudden eruption of sterile pustules on erythematous skin surface accompanied with constitutional symptoms. It can be localized or generalized depending on the area involved. Though the exact cause of pustular psoriasis is widely unknown, multiple factors known to precipitate it include overtreatment with topical tar, use of anthralin, potent topical or oral corticosteroids, systemic agents like progesterone, infections, hypocalcemia, and associated pregnancy. Genetic factors, such as mutation in IL-1 and IL-36 receptor antagonist genes, leading to unopposed proinflammatory pathway, are another proposed trigger. The generalized variety of pustular psoriasis is a subtype that can be fulminating and life threatening if not managed adequately, as it can result in sepsis, acute renal failure, congestive cardiac failure, and acute respiratory distress syndrome. Adequate investigation and a prompt triage of a patient of generalized pustular psoriasis are recommended as it is associated with high mortality and morbidity if management is delayed. Supportive measures, identifying the trigger, and searching for its extracutaneous complications are as important as initiating specific therapy and forms an important aspect of managing pustular psoriasis. Specific therapy for management of pustular psoriasis depends on its subtype, age, comorbidities associated, and clinical presentation. In this chapter we focus on some challenging aspects with regard to pustular psoriasis.
