Exanthematous Pustulosis Research Articles

Acute generalized exanthematous pustulosis: Epidemiology, clinical course, and treatment outcomes of patients treated in an Asian academic medical center

BackgroundAcute generalized exanthematous pustulosis (AGEP) is a rare severe cutaneous adverse drug reaction. Although acutely patients have significant morbidity and occasional systemic involvement, the clinical course is generally self-limited. To date, there has been no consensus on treatment.ObjectiveThe aim of our current study was to evaluate the clinical features, drug association, treatment, and outcomes in a cohort of patients treated in an academic medical center.MethodsA retrospective review of electronic medical records over a period of 10 years from 2009 to 2018 in a single tertiary academic medical center in Singapore was performed. Forty-three medical records with probable/definite diagnosis of AGEP were identified and analyzed for statistical significance.ResultsDrug association was identified in 93% of cases. The most frequent drug class was antibiotics, including penicillins, cephalosporins, and vancomycin. Systemic involvement was reported in 13.9% of patients. All cases of AGEP resolved with cessation of the offending drug. There was no mortality attributed to AGEP. Treatment with systemic steroid was associated with a decreased length of hospital stay (P = .035) in patients with AGEP.ConclusionAGEP was a self-limiting adverse drug reaction that was commonly caused by antibiotics. Although there was no difference in mortality, there was a significant reduction in the length of hospitalization with systemic corticosteroid treatment compared with that of topical corticosteroid treatment of AGEP.

Acute generalised exanthematous pustulosis secondary to prednisolone: an unlikely suspect.

Acute generalised exanthematous pustulosis secondary to prednisolone: an unlikely suspect.

Dermoscopic Aspects of Cutaneous Adverse Drug Reactions.

Little is known about the dermoscopic evaluation of cutaneous adverse drug reactions (CADRs). To evaluate the dermoscopic patterns of CADRs and identify those associated with severe cutaneous adverse reactions to drugs (SCARDs). Patients included in this study from May 2015 to April 2016 had presented with CADRs. CADR presentation and classification were based on standard criteria. SCARDs included Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), overlap SJS/TEN, drug reaction with eosinophilia and systemic symptoms (DRESS), and acute generalized exanthematous pustulosis (AGEP). The dermoscopic features of CADRs were described and compared according to the severity of the reactions. Sixty-nine patients were included. Sixteen patients (23.2%) presented SCARDs. The main dermoscopic findings in SJS, overlap SJS/TEN and TEN were black dots or necrotic areas (100%). Erosion [respectively, 4/6 (66.7%), 3/3 (100%) and 1/1 (100%)], necrotic borders [respectively, 4/6 (66.7%), 3/3 (100%) and 1/1, (100%)] and epidermal detachment [respectively, 5/6 (83.3%); 2/3 (66.7%) and 1/1 (100%)] were also common among these reactions. Erythema and purpuric dots were the main dermoscopic findings [respectively, 5/6 (83.3%) and 4/6 (66.7%)] in DRESS. In non-severe reactions, the most prevalent structures were erythema and purpura in exanthema [respectively, 31/33 (93.9%) and 24/33 (72.7%)] and erythema and vascular structures in urticarial reactions [respectively, 6/6 (100%) and 3/6 (50%)]. Black dots or necrotic areas, epidermal detachment, necrotic borders and erosion were highly associated with SCARDs (P < 0.001). Dermoscopy improves clinical recognition of SCARDs.

A retrospective, 5-year, clinicoepidemiological study of severe cutaneous adverse reactions (SCARs).

A severe cutaneous adverse reaction (SCAR) is a rare, clinically heterogeneous, life-threatening phenomenon that results in serious skin damage, systemic complications, and significant morbidity or mortality comprising of Stevens-Johnson syndrome (SJS), toxic epidermal necrolysis (TEN), or SJS-TEN overlap, acute generalized exanthematous pustulosis (AGEP), exfoliative dermatitis, and drug rash with eosinophilia and systemic symptoms (DRESS). A retrospective study was conducted on all cases of SCARs admitted in a tertiary care referral hospital from January 2015 to December 2019. Clinical and epidemiological details were retrieved from the records of patients as per predesigned proforma. The data collected were analyzed and statistically evaluated. A total of 142 patients (67 males, 75 females) with SCARs, constituting 0.08% of total hospital admission and 0.027% of total dermatology outpatient department (OPD), were studied. Age group ranged from 2 to 61years, with the mean age of 33.6years (SD=17.43). Most patients belonged to SJS-TEN complex (75 cases; SJS 48, SJS-TEN 18, TEN nine) followed by exfoliative dermatitis (27 cases/19%), AGEP (26 cases/18.3%), and DRESS (14 cases/9.8%). Anticonvulsants were most commonly implicated (22%) followed by antibiotics (20%), nonsteroidal anti-inflammatory drugs (NSAIDs) (17%), and ayurvedic (7%). There was only single mortality in a DRESS patient. SCAR has considerable disease burden with marginal female preponderance in SJS/TEN and exfoliative dermatitis and can involve even the pediatric population. Anticonvulsants followed by antibiotics, NSAIDs, and ayurvedic medicines are common groups known to cause SCARs. To date, there are no definitive recommendations regarding their optimal treatment regimen, hence early diagnosis, prompt withdrawal of culprit drug, high standard of nursing care, and interdisciplinary consultations are vital steps to avoid disease progression and restore health.

The Role of In Vivo and Ex Vivo Diagnostic Tools in Severe Delayed Immune-Mediated Adverse Antibiotic Drug Reactions

The use of invivo and exvivo diagnostic tools for delayed immune-mediated adverse drug reactions is currently ill defined. To determine whether the combination of skin testing and/or IFN-γ enzyme-linked immunoSpot assay (ELISpot) can aid diagnosis of these allergy phenotypes. Patients with antibiotic-associated severe delayed immune-mediated adverse drug reaction hypersensitivity, including Stevens-Johnson syndrome and toxic epidermal necrolysis, drug reaction with eosinophilia and systemic symptoms (DRESS), acute generalized exanthematous pustulosis, generalized bullous fixed drug eruption, and severe maculopapular exanthema, were prospectively recruited. Invivo testing was completed to the implicated drug(s), and exvivo testing was performed with the patient's PBMCs stimulated with the relevant antibiotic concentrations for IFN-γ release ELISpot measurement. Eighty-one patients met the inclusion criteria, with DRESS (42; 51.9%) accounting for most cases. Among the 63 (78%) who had an ELISpot assay performed, 34 (54%) were positive to at least 1 implicated antibiotic (median spot-forming units/million cells, 99.5; interquartile range, 68-187), with glycopeptide being a strong predictor of positivity (adjusted odds ratio, 6.11; 95% CI, 1.74-21.42). In combination (invivo and exvivo), 51 (63%) of those tested were positive to an implicated antibiotic. For DRESS and severe maculopapular exanthema associated with penicillins and cephalosporins, this combination confirmed the culprit agent in 11 of the 12 cases and in 6 of 7 for DRESS associated with glycopeptides. This study demonstrates that using invivo in combination with exvivo testing can enhance the diagnostic approach in these severe phenotypes by assisting with the identification of possible culprit antibiotics.

Acute Generalized Exanthematous Pustulosis (AGEP) in 12 Patients Treated for SARS-CoV-2 Positive Pneumonia

The novel coronavirus disease 2019 (COVID-19) caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is rapidly spreading throughout the world. The study describes 12 patients with SARS-CoV-2 pneumonia, who developed an acute erythematous rash with nonfollicular pinhead-sized pustules, without mucosal involvement. The clinical differential diagnosis was viral rash, acute generalized exanthematous pustulosis (AGEP), or multiform erythema. computed tomography with a diagnosis of interstitial pneumonia and a respiratory tract sample positive for SARS-CoV-2 in a reverse transcriptase polymerase chain reaction assay. Patients had signs of respiratory distress and were treated with hydroxychloroquine, darunavir, ritonavir, heparin, ceftriaxone, and azithromycin. Punch biopsies showed subcorneal pustules typical of AGEP. Dermal microvascular injury and thrombosis as described in skin damage by SARS-CoV-2 infection was not observed. The direct immunofluorescence for IgG, IgA, IgM, and C3 was negative in 8 patients investigated. A polymerase chain reaction for RNA SARS-CoV-2 performed on frozen skin was negative in 5 of 6 patients. Most of our patients were treated with systemic corticosteroids. After some days (4-10), the diffuse erythema and pustules had improved. AGEP is classified as a severe cutaneous adverse reaction, provoked by drugs and acute infections. Characteristically, removal of the offending agent leads to spontaneous resolution typically in less than 15 days. The recognition of AGEP is important, in order to avoid confusion with a systemic infection and consequently to avoid incorrect treatment. Cutaneous adverse reactions to drugs are common and are major health problems worldwide causing considerable costs for health care systems. We suggest that in the patients with AGEP during SARS-CoV-2 pneumonia, viral infection is a risk factor for developing drug reaction.

The Role of Frozen Section in the Rapid Diagnosis of Severe Cutaneous Adverse Drug Reactions.

Context:Early diagnosis is the mainstay in the management of severe cutaneous adverse reactions (SCARs) to drugs.Aims:To study the role of frozen section in the rapid diagnosis of SCARs and the impact on outcome of the affected patients.Settings and Design:A single-blind, hospital-based study was conducted from December 2014-July 2016.Methods and Material:We biopsied 32 adults with SCARs diagnosed by clinical features and standard criteria. The histopathological features seen on frozen sections were compared to that of paraffin blocks. The impact of rapid diagnosis on the clinical outcome was studied in toxic epidermal necrolysis (TEN), Stevens-Johnson syndrome (SJS), drug rash with eosinophilia and systemic symptoms (DRESS) and acute generalized exanthematous pustulosis (AGEP).Statistical Analysis:Z test was used to compare two proportions. Kappa statistic, sensitivity, specificity, positive predictive value, and negative predictive value of the frozen section diagnosis were calculated in TEN/SJS and DRESS using MedCalc software.Results:Frozen and paraffin sections were done in TEN/SJS spectrum (13), DRESS (17), and AGEP (2). The sensitivity, specificity and kappa values for frozen section diagnosis in SJS/TEN and DRESS were 91.7%, 95%, 0.867 and 94.4%, 100%, 0.937 respectively. The concordance between frozen and paraffin section diagnosis was 100% in TEN, SJS, DRESS and AGEP. All the 6 patients with TEN and 2 with AGEP survived. Taking the worst-case scenario, the mortality in SJS was 28.6%. The mortality among patients with DRESS was 11.8%.Conclusions:Frozen section helps in the rapid diagnosis and early treatment of SCARs and differentiates it from diseases that mimic it.

The Role of In Vitro Detection of Drug-Specific Mediator-Releasing Cells to Diagnose Different Phenotypes of Severe Cutaneous Adverse Reactions.

ProposeThe purpose of this study was to investigate panels of enzyme-linked immunospot assays (ELISpot) to detect drug-specific mediator releasing cells for confirming culprit drugs in severe cutaneous adverse reactions (SCARs).MethodsFrequencies of drug-induced interleukin-22 (IL-22)-, interferon-gamma (IFN-γ)-, and granzyme-B (GrB)-releasing cells were measured by incubating peripheral blood mononuclear cells (PBMCs) from SCAR patients with the culprit drugs. Potential immunoadjuvants were supplemented to enhance drug-induced mediator responses.ResultsTwenty-seven patients, including 9 acute generalized exanthematous pustulosis (AGEP), 10 drug reactions with eosinophilia and systemic symptoms, and 8 Stevens-Johnson syndrome and toxic epidermal necrolysis (SJS/TEN) were recruited. The average frequencies of drug-induced IL-22-, IFN-γ-, and GrB-releasing cells were 35.5±16.3, 33.0±7.1, and 164.8±43.1 cells/million PBMCs, respectively. The sensitivity of combined IFN-γ/IL-22/GrB ELISpot was higher than that of IFN-γ ELISpot alone for culprit drug detection in all SCAR subjects (77.8% vs 51.9%, P < 0.01). The measurement of drug-induced IL-22- and IFN-γ releasing cells confirmed the culprit drugs in 77.8% of AGEP. The measurement of drug-induced IFN-γ- and GrB-releasing cells confirmed the culprit drugs in 62.5% of SJS/TEN. Alpha-galactosylceramide supplementation significantly increased the frequencies of drug-induced IFN-γ releasing cells.ConclusionThe measurement of drug-induced IFN-γ-releasing cells is the key for identifying culprit drugs. The additional measurement of drug-induced IL-22-releasing cells enhances ELISpot sensitivity to identify drug-induced AGEP, while the measurement of drug-induced GrB-releasing cells could have a role in SJS/TEN. ELISpot sensitivity might be improved by supplementary alpha-galactosylceramide.Trial RegistrationClinicalTrials.gov Identifier: NCT02574988

Development of Acute Generalized Exanthematous Pustulosis due to Hydroxychloroquine Use for COVID-19 Treatment

Acute generalized exanthematous pustulosis (AGEP) is a cutaneous drug eruption, which is characterized by nonfollicular, sterile pustules, rapidly and severely progresses on an erythematous base. Although antibiotics have been considered to be responsible for most of the development of AGEP, many drugs, such as hydroxychloroquine (HCQ), may also cause it. Recently, HCQ is frequently used in the prophylaxis and treatment of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection along with antiviral drugs. In our study, we have presented an AGEP reaction case that has been developed in one patient who is being treated for coronavirus disease-19 (COVID-19) and using the HCQ.

An Atypical Case of Acute Generalized Exanthematous Pustulosis with Steven Johnson’s Syndrome-Like Features Triggered by a Bacterial Infection

An Atypical Case of Acute Generalized Exanthematous Pustulosis with Steven Johnson’s Syndrome-Like Features Triggered by a Bacterial Infection

Acute generalized exanthematous pustulosis caused by a probable spider bite: A case report and review of the literature

Acute generalized exanthematous pustulosis caused by a probable spider bite: A case report and review of the literature

Potentially life‑threatening severe cutaneous adverse reactions associated with tyrosine kinase inhibitors (Review).

Tyrosine kinase inhibitors (TKIs) have emerged as a new frontier of cancer therapy. These agents include inhibitors of epidermal growth factor receptor (EGFR), human epidermal growth factor receptor2 (HER2), BRAF, mitogen‑activated protein kinase kinase (also referred to as MEK), bcr‑abl, c‑KIT, platelet‑derived growth factor (PDGFR), fibroblast growth factor receptor(FGFR), anaplastic lymphoma kinase (ALK) and vascular endothelial growth factor (VEGF). Along with the evolving applications of TKIs, there has been an increased recognition of the breadth of potential cutaneous toxicities to these agents. In this review, we provide an overview of potentially life‑threatening severe cutaneous adverse reactions (SCARs) that may occur during therapy with TKIs. These toxicities include Stevens‑Johnson Syndrome (SJS), toxic epidermal necrolysis (TEN), drug reaction with eosinophilia and systemic symptoms (DRESS), and acute generalized exanthematous pustulosis (AGEP).

Cutaneous manifestations of COVID-19: experience from a tertiary care center for COVID-19 management in South India

&lt;p class="abstract"&gt;Cutaneous manifestations observed in COVID-19 include maculopapular rash, urticaria, pseudo chilblain, vesicles, livedo reticularis, petechiae, erythema multiforme-like, symmetric drug related intertriginous and flexural exanthem (SDRIFE)-like and pityriasis rosea-like lesions. Maculopapular rash involving the trunk and sparing palms and soles is the most common cutaneous manifestation reported in COVID-19 patients. We report cutaneous manifestations in six COVID-19 patients from the corona isolation ward and intensive care unit of a tertiary care center for COVID-19 management in Kerala, South India. The skin lesions observed were urticaria (two patients), atypical maculopapular rash with urticarial and erythema multiforme-like lesions, eruptive pseudoangiomatosis, exaggerated insect bite reaction and acute localized exanthematous pustulosis (ALEP)-like lesions. To the best of our knowledge atypical maculopapular rash with urticarial and erythema multiforme-like lesions, eruptive pseudoangiomatosis and acute localized exanthematous pustulosis-like lesions are not reported in COVID-19.&lt;/p&gt;

Acute generalized exanthematous pustulosis in children and adolescents in Singapore: A ten-year retrospective review.

Acute generalized exanthematous pustulosis (AGEP) is a rare, severe, cutaneous adverse reaction. Although most commonly caused by drugs, it can also be triggered by infections, especially in children. This is a retrospective study involving children and adolescents aged 16years or younger, diagnosed with AGEP between January 2010 and March 2020 in our tertiary pediatric hospital. Information pertaining to the patient's demographics, clinical presentation and progress, biochemical, microbiological, and histopathological investigations, treatment, and outcomes was analyzed. Eight patients were diagnosed with AGEP with mean age 8.2years (range: 1.7-16.0years). None of the patients had a personal or family history of psoriasis. Almost all patients had fever (n=7, 87.5%). Although all 8 patients had intercurrent illness, 5 cases were attributed to infection, while the other 3 were likely precipitated by drugs. Abnormal hematological and biochemical parameters included a raised absolute neutrophil count (mean: 11.5×109 /L, range: 5.0-30.9×109 /L), C-reactive protein (mean: 52.5mg/L, range: 5.0-143.7mg/L), and erythrocyte sedimentation rate (mean: 38.6mm/h, range: 6-64mm/h). All patients developed post-pustular desquamation and subsequently recovered. The mean duration from onset to cessation of acute pustulation was 5.6days (range: 3.0-10.0days). One patient developed a recurrent episode of AGEP. AGEP is rare and may be more commonly caused by infections in children. The condition is self-limiting with overall good outcomes in this age-group. In cases with concomitant infection and drug use, formal allergy testing should be arranged after resolution of the infection to confirm the underlying etiology.

DRESS syndrome following furosemide administration: An unusual association

IntroductionDrug reaction with eosinophilia and systemic symptoms (DRESS) is a severe and rare adverse drug reaction. Several drugs are known to induce DRESS. Furosemide, a sulfonamide loop diuretic drug, is known to induce hypersensitive reactions such as bullous eruptions, acute generalized exanthematous pustulosis and lichenoid eruptions, but rarely DRESS. We describe herein a case of furosemide-induced DRESS that recurred after bumetanide administration. Case reportA 67-year-old man was admitted to the nephrology department for hypertension, gout and chronic renal failure. He received a multidrug therapy including captopril, nifedipine, allopurinol and furosemide. Six weeks after starting this treatment, he developed a maculopapular itchy and edematous skin reaction, facial edemaand fever. The laboratory findings showed 2200/mm3 of eosinophils (20%). Creatinine clearance decreased from 18.9 to 14.4 mL/min. Lactate dehydrogenase was at 600 IU/L (normal range 190-390 IU/L). Chest X-ray showed an interstitial lung injury. Skin biopsy findings were in accordance with a hypersensitive reaction. Furosemide was withdrawn and symptoms resolved completely three weeks later. A patch test with furosemide performed six weeks later was negative. The patient was given bumetanide, another sulfonamide loop diuretic, with recurrence of symptoms two months later. Bumetanide was withdrawn with a complete resolution of both clinical and biological symptoms within three weeks. ConclusionWe add to the literature another case of furosemide-induced DRESS with the particularity of cross reactivity with bumetanide.

Acute generalized exanthematous pustulosis and Stevens-Johnson syndrome overlap due to hydroxychloroquine: a\xa0case report

BackgroundSince the World Health Organization declared a global pandemic due to the novel coronavirus disease2019, there have been targeted efforts to establish management modalities. Hydroxychloroquine has been suggested as a possible treatment; however, it is associated with multiple adverse reactions. We report a rare case of a patient with acute generalized exanthematous pustulosis with Stevens-Johnson syndrome due to hydroxychloroquine. Acute generalized exanthematous pustulosis is characterized by acute onset of a generalized rash that is pustular and erosive in nature, affecting limbs; trunk; face; and, less often, mucosal membranes. Although rare, it is important to be mindful of this side effect because the diagnosis is often delayed, and the disease has the potential to be life-threatening.Case presentationA 68-year-old American woman presented to our hospital with a painful, rapidly spreading rash. Its morphologic features included erythema multiforme–like lesions with extensive skin sloughing in various regions of the head, neck, and trunk and mucosal involvement. Her Nikolsky sign was negative, and she had no evidence of lesions on areas of skin trauma. Four weeks prior, she had been initiated on hydroxychloroquine for a presumed diagnosis of cutaneous sarcoidosis. Three punch biopsies of the head and neck area revealed subcorneal pustules consistent with acute generalized exanthematous pustulosis. Treatment began with high doses of methylprednisolone, leading to only minimal improvement of existing areas and ongoing spread to new areas. Treatment with intravenous immunoglobulin was initiated, at which point disease stability was achieved. The patient’s rash ultimately resolved, as did her cutaneous pain and pruritus.ConclusionsAmong many potential adverse reactions involving hydroxychloroquine, cutaneous side effects are varied and can lead to significant morbidity or even death. The drug is currently being investigated in a multitude of trials for coronavirus disease2019 treatment, prevention, and prophylaxis after exposure to severe acute respiratory syndrome coronavirus 2. Acute generalized exanthematous pustulosis is a rare side effect of hydroxychloroquine, and even fewer cases demonstrate histologic evidence of acute generalized exanthematous pustulosis while clinically presenting with Stevens-Johnson syndrome. Patients who develop Stevens-Johnson syndrome/toxic epidermal necrolysis require best supportive care with aggressive fluid and electrolyte replacement and prevention of further breakdown of the skin barrier. With the potential of widespread hydroxychloroquine use, it is important that providers be aware of its potential severe adverse drug reactions.

Acute generalized exanthematous pustulosis with a focus on hydroxychloroquine: A 10-year experience in a skin hospital

ObjectiveAcute generalized exanthematous pustulosis (AGEP) is a severe skin pustular drug reaction that can lead to life-threatening consequences. In this study, we have investigated the characteristics and outcomes of patients with AGEP in a tertiary skin hospital. MethodsFrom March 2007 to December 2019, medical records of all patients diagnosed with AGEP, were assessed. Demographic data, culprit drug, past medical history, laboratory tests, recurrence, and systemic organ involvement were all documented as well. ResultsSeventy-four patients, including 54 women (73%) and 20 men (27%), with a mean age of 44.3 ± 16.5 years were evaluated. The most common comorbidities among the patients were rheumatoid arthritis and diabetes. In addition, hydroxychloroquine, cephalosporin, and amoxicillin were found as the three most common medications associated with AGEP induction. Among the study group, seventeen (23%) patients had systemic organ involvement (nine (12.2%), six (8.1%), and five (6.8%) had hepatic, renal and pulmonary involvement, respectively). All patients responded to oral prednisolone within a median of five days (IQR = 4; ranged 2–14). The median duration of treatment was significantly longer in hydroxychloroquine group compared to other drugs (8 versus 5 days; HR 0.57,95%CI 0·35–0.91). Likewise, the median duration of treatment was significantly longer in febrile patients compared to the afebrile ones (7 versus 4 days; HR 0.46, 95%CI 0.25–0.85). Recurrence occurred in six patients after resuming treatment with the same medication. The mean Naranjo score was 7.6 ± 0.9 denoting a probable causal relationship. ConclusionIn this study, we found that using hydroxychloroquine and presence of fever are the risk factors potentially leading to a prolonged treatment duration of AGEP.

Efficacy of Cyclosporine in Hydroxycholorokine Induced Steroid Resistant Acute Generalized Exanthematous Pustulosis

Efficacy of Cyclosporine in Hydroxycholorokine Induced Steroid Resistant Acute Generalized Exanthematous Pustulosis

Acute Generalized Exanthematous Pustulosis Secondary to Oral Nystatin

Acute Generalized Exanthematous Pustulosis Secondary to Oral Nystatin

ACUTE GENERALIZED EXENTHEMATOUS PUSTULOSIS MIMICKING SEPTIC SHOCK: UNUSUAL PRESENTATION OF AN UNUSUAL DISEASE

SESSION TITLE: Medical Student/Resident Critical Care Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Acute generalized exanthematous pustulosis (AGEP) is a rare disorder, characterized by the development of acute sterile pustules. It is mostly caused by drugs, with antibiotics being the most likely culprits. AGEP is a self-limiting disease with favorable prognosis in the vast majority of patients. We present a case of a woman, admitted to the intensive care unit (ICU) with an unusually severe form of AGEP mimicking septic shock. CASE PRESENTATION: A 45-year-old female with systemic lupus erythematosus (SLE), underwent left hip replacement due to avascular necrosis. She received prophylactic cephazolin and discharged two days after surgery with an uncomplicated post-operative course. Two days later, she was admitted to the ICU with fever (103.6 F), tachycardia, and hypotension (54/36 mmHg). On physical exam, she had diffuse small pustules on an erythematous base (Fig 1). The incision site showed no signs of infection (Fig. 2). Labs were significant for leukocytosis (WBC 22,000), neutrophilia (18,500), left shift (Bands 13.9), eosinophilia (160), acute kidney injury (Cr 5.3, baseline 0.8), increased inflammatory markers (ESR 19, CRP 32.7), procalcitonin (2.49) and lactic acidosis. Random cortisol was normal and urine analysis was clear. Chest X-Ray showed no infiltrates and left hip CT scan showed no hematoma or abscess. She was initially managed as septic shock, started on broad spectrum antibiotics and required 2 vasopressors. Her hemodynamic status dramatically improved by the second day. Given her typical eruption and no source of infection identified, she was diagnosed with AGEP and antibiotics were discontinued on day 2. Patient continued to improve; vasopressors were weaned off by the third day. Cultures showed no growth. Kidney function and lactate levels normalized by the fifth day and she was discharged from the hospital on day 11. The skin pustules gradually coalesced into plaques (Fig. 3, 4) with subsequent sloughing. DISCUSSION: AGEP is a rare acute cutaneous reaction, with characteristic numerous, pinhead-sized pustules on an edematous erythematous background. It commonly presents with fever, neutrophilia, and skin eruption. Thus, often misinterpreted as an acute infectious process. AGEP is self-limiting, tends to resolve spontaneously with cessation of the offending agent, and no further measures are usually required. Multi-organ involvement is rare and highly atypical of AGEP. In contrast to most reported cases of AGEP, our case demonstrated an atypical presentation with severe systemic involvement and multi-organ failure mimicking septic shock. The patient had SLE and was on chronic corticosteroid treatment, which might explain her unusual life-threatening course. CONCLUSIONS: Prompt diagnosis of AGEP is crucial as the mainstay of treatment is the withdrawal of the offending drug. Every effort should be made to distinguish it from other causes of shock, mainly infection. Reference #1: idoroff A, Halevy S, Bavinck JN, Vaillant L, Roujeau JC. Acute generalized exanthematous pustulosis (AGEP)--a clinical reaction pattern. J Cutan Pathol. 2001;28(3):113-9 Reference #2: Roujeau JC, Bioulac-Sage P, Bourseau C, Guillaume JC, Bernard P, Lok C, et al. Acute generalized exanthematous pustulosis. Analysis of 63 cases. Arch Dermatol. 1991;127(9):1333-8 Reference #3: Krishna S, Ortega-Loayza A, Malakouti N, Brinster N. A rapidly progressive and fatal case of atypical acute generalized exanthematous pustulosis. J Am Acad Dermatol. 2014;71(3):e89-90 DISCLOSURES: No relevant relationships by Mohamed Elmassry, source=Web Response No relevant relationships by Amr Ismail, source=Web Response No relevant relationships by Haneen Mallah, source=Web Response No relevant relationships by Barbara Mantilla, source=Web Response No relevant relationships by Jasmine Sekhon, source=Web Response No relevant relationships by Victor Test, source=Web Response No relevant relationships by Myrian Vinan Vega, source=Web Response