Abstract Introduction Generalized pustular psoriasis (GPP) is a rare immune-mediated skin disorder representing 1% of psoriasis cases. It is characterized by tender, yellow pustular eruption surrounded by erythema, which can present after chronic psoriasis or acutely with life-threatening complications and high mortality. Diagnosis is challenging because GPP can resemble other pustular dermatoses such as acute generalized exanthematous pustulosis (AGEP). We present a case of acute GPP in multifactorial shock. Case A 37-year-old female with history of celiac disease, Raynaud’s, and migraine, presented to the emergency department with presyncope and worsening rash. One month earlier, she was diagnosed with pustular eruption and treated with steroid injections, cyclosporine, triamcinolone, and hydroxychloroquine without improvement. On arrival, she was noted to have diffuse pustular lesions. She was transferred to a tertiary center for dermatologic evaluation. On arrival, she developed refractory hypotension requiring transfer to the intensive care unit. Dermatology, ENT, and Ophthalmology were consulted given the extensive involvement of her rash. Skin biopsies were obtained. The patient’s shock was treated with fluid resuscitation, empiric antibiotics, and vasopressors. At this time, GPP was the favored diagnosis given the temporal association of the rash with steroid exposure. Addition of steroids for septic shock was avoided. After infection was ruled out after an extensive workup, infliximab was initiated. The rash improved considerably, and she was discharged with a plan to start spesolimab as an outpatient. Discussion Generalized pustular psoriasis is characterized by rapid, generalized, painful erythrodermic eruption with leukocytosis, decreased albumin, and malaise. Diagnosis can be challenging as the rash may resemble AGEP, subcorneal pustular dermatosis, and IgM pemphigus. Differentiating etiology of pustular dermatitis directly impacts management strategy, especially when confounded by refractory shock. For example, early systemic corticosteroids are a mainstay for several diffuse pustular eruptions such as AGEP. Likewise, in typical septic shock, corticosteroids shorten the duration of vasopressor and ventilatory support. In contrast, systemic corticosteroids are generally contraindicated in GPP due to their association with disease exacerbation. In this patient, despite a presumed septic contribution to refractory shock, systemic steroids were intentionally withheld following early recognition of GPP. Biologic therapy, including IL-36, TNF-α, IL-17/17R, and IL-23 inhibitors, is considered first-line once infection is excluded. Infliximab was chosen due to its rapid onset and unavailability of spesolimab. Untreated, GPP may progress to multiorgan failure and death. Early recognition and prompt biologic initiation are essential to reduce morbidity in acute presentations complicated by shock. This abstract is funded by: None
Barnhart et al. (Fri,) studied this question.