RARE DISEASE
Acute generalized exanthematous pustulosis
Acute generalized exanthematous pustulosis
Acute generalized exanthematous pustulosis
Synonyms: AGEP, Pustular drug eruption, Toxic pustuloderma
Synonyms: AGEP, Pustular drug eruption, Toxic pustuloderma
Synonyms: AGEP, Pustular drug eruption, Toxic pustuloderma
Drug discovery
0
drugs
With orphan designations
Overview
Acute Generalized Exanthematous Pustulosis (AGEP) is a rare, severe cutaneous adverse reaction characterized by rapid onset of sterile, nonfollicular pustules on erythematous skin, fever, and neutrophilia. Over 90% of cases are drug-induced, commonly by antibiotics (e.g., penicillins, sulfonamides), antifungals, or hydroxychloroquine. AGEP typically resolves within 1–2 weeks after discontinuing the offending agent. Systemic involvement (e.g., liver, kidney) occurs in up to 20% of cases, but mortality remains low (<5%) [1][2][10][12].
Burden
Morbidity: Self-limiting but may require hospitalization for systemic complications (e.g., hepatitis, acute kidney injury) in 17–20% of patients [1][7][10].
Economic impact: Hospital costs and delayed resolution (e.g., hydroxychloroquine-associated AGEP may persist ≥1 month) [7][10].
Mortality: <5%, typically due to comorbidities or superinfections [5][10].
Therapies
First-line: Immediate discontinuation of the causative drug and supportive care (hydration, antipyretics) [1][5][10].
Topical/systemic steroids: Used for pruritus and inflammation; systemic steroids reserved for severe cases or organ involvement [3][12].
Biologics: Secukinumab (IL-17 inhibitor) reported effective in steroid-refractory cases [3][7].
Categories: rare skin diseases
Research Papers
154 drug discovery papers about Acute generalized exanthematous pustulosis, with 1 first-in-class emerging drug candidates forecasted to outperform the average preclinical success rate. Recent publications:
154 drug discovery papers about Acute generalized exanthematous pustulosis, with 1 first-in-class emerging drug candidates forecasted to outperform the average preclinical success rate. Recent publications:
categories:
Small molecules
small molecules
2026-06-24 | Biologics and Small Molecule Inhibitors: Novel Therapeutic Strategies for Cutaneous Adverse Drug Reactions.
Cutaneous adverse drug reactions (cADRs) are unintended and harmful skin responses to medications that impose significant clinical and economic burdens worldwide. The increasing use of novel agents, particularly immune checkpoint inhibitors, has further compounded this challenge. While most cADRs are mild and resolve upon drug discontinuation, approximately 2-6.7% progress to severe, potentially fatal conditions. The most common severe forms include acute generalized exanthematous pustulosis, Stevens-Johnson syndrome/toxic epidermal necrolysis, drug reaction with eosinophilia and systemic symptoms/drug-induced hypersensitivity syndrome, and drug-associated bullous pemphigoid. Current therapeutic options for refractory cADRs remain limited, primarily relying on systemic corticosteroids, conventional immunosuppressants, and intravenous immunoglobulin. Growing insights into disease pathogenesis and the subsequent repurposing of novel targeted therapies offer promising solutions to the persistent challenges of cADRs. Emerging agents, such as biologics targeting tumor necrosis factor-α, interleukins (IL-4/IL-13, IL-5, IL-6, IL-17, IL-36), immunoglobulin E, and CD20, along with small molecule inhibitors of Janus kinases and phosphodiesterase 4, hold the potential to revolutionize management paradigms, though their long-term efficacy and safety profiles await robust clinical validation.
2026-06-01 | A rare case of acute generalized exanthematous pustulosis-like Sweet syndrome.
Acute generalized exanthematous pustulosis (AGEP) is a pustular drug eruption characterized by superficial pustules, commonly caused by β-lactam antibiotics. Management of AGEP involves discontinuation of the offending medication and supportive therapy with topical corticosteroids and analgesia, given the self-limiting nature of this condition. In contrast, Sweet syndrome (SS), or acute febrile neutrophilic dermatosis, is characterized by the sudden onset of painful, inflamed skin lesions associated with fever. SS typically requires investigations for associated conditions and systemic corticosteroids, resulting in rapid improvement within days. We present a case of AGEP-like SS, initially diagnosed clinically as AGEP in the setting of intravenous antibiotic use, where the patient deteriorated, despite antibiotics being stopped. Skin biopsy revealed papillary dermal oedema with neutrophilic infiltrate, and scattered histiocytoid cells and eosinophils, resulting in a revised diagnosis of AGEP-like SS. The patient was started on oral corticosteroids and achieved complete resolution of disease within 2 days. This case highlights AGEP-like SS as an important differential diagnosis in patients presenting with AGEP-like eruptions, as the management of AGEP and SS is different.
2026-04-17 | Apalutamide-induced severe cutaneous adverse reactions in prostate cancer: a comprehensive review of reported cases and clinical strategies.
Prostate cancer (PCa) is the most common fatal malignancy among men and a major cause of cancer-related death. Apalutamide, a second-generation androgen receptor inhibitor, is approved for non-metastatic castration-resistant prostate cancer (nmCRPC) and metastatic castration-sensitive prostate cancer (mCSPC). However, an increasing number of potentially life-threatening severe cutaneous adverse reactions (SCARs) associated with apalutamide have raised clinical concerns. This review aims to characterize apalutamide-induced SCARs and summarize effective management strategies based on reported cases. We systematically searched PubMed, Europe PMC, and CNKI for case reports of SCARs associated with apalutamide. Keywords included "Apalutamide" and "Drug eruptions." A total of 18 cases were identified and analyzed. We reviewed the clinical characteristics and treatment of 3 cases of DRESS, 14 cases of SJS/TEN, and 1 case of AGEP, all highly suspected to be caused by apalutamide, reported globally. The most frequent SCARs associated with apalutamide are SJS/TEN. The median onset time of SCARs in these cases was 39.5 days, significantly shorter than the onset time of rash in phase III clinical trials of apalutamide. Geographically, the majority of reported cases originated from East Asia. By analyzing the treatment regimens and clinical outcomes of these patients, combined with a literature review, we proposed a set of definitive therapeutic strategies. Apalutamide-induced SCARs tend to occur earlier than common rashes observed in clinical trials, with a predominance of reported cases in East Asian populations. Immediate discontinuation of the suspected drug is the cornerstone of SCAR management. Supportive care, systemic corticosteroids, intravenous immunoglobulin (IVIG), cyclosporine, plasmapheresis, and TNF-α antagonists play important roles in treatment. Personalized dosing strategies based on body weight or body surface area, along with proactive rash management, may help mitigate risk and optimize therapeutic continuity.
2026-03-30 | Case Report: Secukinumab induced pustular eruption in a patient with ankylosing spondylitis.
We report a case of a 33-year-old male with ankylosing spondylitis (AS) who had been receiving secukinumab (150 mg monthly) for 2 years. Approximately 1 year after initiating treatment, the patient developed recurrent, multiple sterile pustules on the palms and soles. Various topical treatments, including corticosteroids and calcipotriol, yielded minimal improvement. 1 week prior to presentation, new pustular lesions emerged on the trunk and extremities, accompanied by pruritus followed by burning sensations and tenderness. Histopathological examination revealed subcorneal pustule formation within the epidermis and sparse lymphocytic infiltration in the dermis. A diagnosis of secukinumab-induced pustular reaction was made. Following the discontinuation of secukinumab and the initiation of upadacitinib (15 mg/day) for over 1 month, the pustules largely resolved, and symptoms were significantly alleviated. This case suggests that IL-17A inhibitors may trigger pustular cutaneous reactions in AS patients. Clinicians should remain vigilant regarding such adverse effects and adjust treatment regimens promptly. JAK inhibitors may provide an effective therapeutic option for managing these drug-induced reactions.
2026-03-03 | Acute Generalized Exanthematous Pustulosis in a Neonate Following Hepatitis B Vaccination: A Case Report.
Acute generalized exanthematous pustulosis (AGEP) is a rare severe cutaneous adverse reaction in neonates and is most commonly drug-induced. Vaccine-associated cases are uncommon in this age group. We report a term male neonate who developed clusters of non-follicular pustules on erythematous plaques approximately 6 hours after receiving a birth dose of hepatitis B vaccine. The infant remained clinically stable, without fever or systemic involvement, and had no exposure to concomitant medications. The rapid onset following a single exposure, characteristic morphology, and prompt resolution were highly suggestive of AGEP. Laboratory investigations and skin biopsy were not performed due to rapid clinical improvement. Infectious etiologies and transient neonatal pustular dermatoses were considered less likely based on the clinical course. Patch testing with the same vaccine lot was positive, supporting a type IV hypersensitivity mechanism. The eruption resolved almost completely within 48 hours with topical hydrocortisone, without complications or recurrence.
antibodies
2026-07-20 | Desquamating Skin Diseases Requiring Burn Team Involvement: Spectrum, Clinical Outcomes, and Management, A Scoping Review
This scoping review aims to map the existing literature on non-burn desquamating, exfoliative, blistering, bullous, and epidermal detachment skin diseases requiring burn team involvement. These conditions may include Stevens-Johnson syndrome/toxic epidermal necrolysis, staphylococcal scalded skin syndrome, pemphigus, pemphigoid, linear IgA bullous dermatosis, DRESS, AGEP, severe cutaneous adverse reactions, and other related dermatologic conditions associated with significant skin loss. The purpose of this review is to characterize the spectrum of diseases managed with burn team involvement, describe the inpatient care settings in which these patients are treated, and summarize the role of burn teams in management. Burn team involvement may include primary admission under a burn service, co-management with another specialty, or consultative input for wound care, dressing management, surgical procedures, or other clinical decision-making. This review will also chart reported outcomes and management variables, including diagnosis at referral or admission, confirmed diagnosis, physical care setting, burn team role, wound care approaches, surgical interventions, immunomodulatory therapies, ICU admission, length of stay, mortality, complications, and resource utilization where available. Findings will be synthesized descriptively to identify patterns in the literature, highlight gaps in current evidence, and inform future research on the role of burn teams in the care of patients with severe non-burn desquamating skin disease.
2026-06-08 | When Conventional Therapy Fails: IL-36 Inhibition for Severe Hydroxychloroquine-Induced Acute Generalized Exanthematous Pustulosis in Systemic Lupus Erythematosus: A Case Report
Introduction: Acute generalized exanthematous pustulosis (AGEP) is a rare but severe cutaneous adverse drug reaction characterized by the sudden onset of widespread sterile pustules on an erythematous and edematous base, often accompanied by fever and neutrophilia. Over 90% of cases are drug-induced. Its management can be particularly challenging in patients with autoimmune diseases such as systemic lupus erythematosus (SLE), where cutaneous manifestations and immune dysregulation may confound clinical assessment. Case Presentation: We report a case of severe hydroxychloroquine-induced AGEP in a patient with active SLE. The disease was refractory to systemic corticosteroids and further worsened after IVIG infusion. Given the emerging role of IL-36 pathway dysregulation in pustular dermatoses, the patient was treated with spesolimab, a monoclonal antibody targeting the IL-36 receptor, resulting in rapid defervescence and near-complete resolution of pustules within days. Conclusion: This case underscores the need for prompt recognition of drug-induced AGEP in patients receiving antimalarials or other immunomodulatory agents for connective tissue diseases. Crosstalk between IL-36 signaling and neutrophil extracellular traps (NETs) may amplify inflammation, linking AGEP with autoimmune pathology. IL-36 inhibition with spesolimab represents a potential rescue therapy for severe, treatment-refractory AGEP, particularly in patients with underlying autoimmune disorders where conventional therapies fail.
2026-05-01 | C50-29 From Rash to Shock: A Life Threatening Presentation of Acute Generalized Pustular Psoriasis
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
2026-04-06 | Refractory acute generalized exanthematous pustulosis—Successfully treated with bimekizumab
Acute generalized exanthematous pustulosis (AGEP) is a rare, usually drug-induced, severe cutaneous adverse effect. Standard therapy includes withdrawal of the causative agent and often systemic corticosteroids, yet refractory cases remain challenging.1
2026-04-01 | Which drugs have the potential to cause Acute Generalized Exanthematous Pustulosis (AGEP)?
Acute Generalized Exanthematous Pustulosis (AGEP) has been associated with a variety of drugs including beta-lactam antibiotics, immune-stimulatory anti-cancer drugs, and certain antiretrovirals, among others.
proteins
2023-07-29 | Pustular Eruption following COVID-19 Vaccination: A Narrative Case-Based Review
From the beginning of public vaccinations until the relaxation of COVID-19 measures, many case reports, case series and case–control studies have been published indicating cutaneous side effects of COVID-19 vaccination. Post-vaccination pustular eruption was reported as well, with a challenging differential diagnosis between pustular psoriasis, AGEP (acute generalized exanthematous pustulosis) and neutrophil pustular eruptions. We report a case of 56-year-old woman presented with acute generalized pustular flare up culminated 5 days after the second dose of BNT162b2(Pfizer) vaccination. She was diagnosed with pustular psoriasis flare and due to the regulating role of IL-1 in pustular psoriasis and in the cytokine storm observed in cases of COVID-19 postvaccination inflammation; we decided to treat the patient with an IL-1 antagonist, subcutaneous anakinra (100 mg daily) along with acitretin. One week later, after anakinra withdrawal, she presented a pustular psoriasis flare and a 7-day anakinra re-administration led to a satisfactory improvement in the skin lesions. We also reviewed the medical literature and found 28 case reports with pustular eruption after the COVID-19 vaccination. We compared the patients reported, regarding sex, age, number of doses, post-vaccination period and vaccine brand, and compared those results with our patient. Finally, as indicated by our case and other cases with similarly treated pustular eruptions. targeted therapy to this cytokine imbalance such as anakinra (IL-1) antagonist can improve the clinical course of the patient.
2022-04-29 | Skin toxicity after Filgrastim treatment for an Ewing's sarcoma patient.
Filgrastim is a granulocyte colony-stimulating factor (GSCF) used in some chemotherapy regimen to prevent febrile neutropenia. Most common reaction of filgrastim are aches and pain including headaches, nausea and skin rash. We report the case of a patient who developed unusual, non-commonly reported adverse toxidermy to filgrastim. At first the eruption was limited to the lower members and genetics organs. Then it slowly spread across the whole body presenting as a polymorphic exanthematous-pustulosis lesions. A cutaneous biopsy was done, identifying a toxidermy modified by systemic treatment. A pharmacological study linked the role of filgrastim to these lesions. After switching from filgrastim to lénograstim, his lesions are completely gone and haven't flared up again. Thus, clearly imputing the use of filgrastim. The cutaneous reaction that has reported with use of GSCF are sweet syndrome, erythema nodosum, pyoderma nodosum and pyoderma gangrenosum. As far as we know, no acute generalized exanthematous pustulosis due to GSCF has been reported.
small molecules
2026-06-24 | Biologics and Small Molecule Inhibitors: Novel Therapeutic Strategies for Cutaneous Adverse Drug Reactions.
Cutaneous adverse drug reactions (cADRs) are unintended and harmful skin responses to medications that impose significant clinical and economic burdens worldwide. The increasing use of novel agents, particularly immune checkpoint inhibitors, has further compounded this challenge. While most cADRs are mild and resolve upon drug discontinuation, approximately 2-6.7% progress to severe, potentially fatal conditions. The most common severe forms include acute generalized exanthematous pustulosis, Stevens-Johnson syndrome/toxic epidermal necrolysis, drug reaction with eosinophilia and systemic symptoms/drug-induced hypersensitivity syndrome, and drug-associated bullous pemphigoid. Current therapeutic options for refractory cADRs remain limited, primarily relying on systemic corticosteroids, conventional immunosuppressants, and intravenous immunoglobulin. Growing insights into disease pathogenesis and the subsequent repurposing of novel targeted therapies offer promising solutions to the persistent challenges of cADRs. Emerging agents, such as biologics targeting tumor necrosis factor-α, interleukins (IL-4/IL-13, IL-5, IL-6, IL-17, IL-36), immunoglobulin E, and CD20, along with small molecule inhibitors of Janus kinases and phosphodiesterase 4, hold the potential to revolutionize management paradigms, though their long-term efficacy and safety profiles await robust clinical validation.
2026-06-01 | A rare case of acute generalized exanthematous pustulosis-like Sweet syndrome.
Acute generalized exanthematous pustulosis (AGEP) is a pustular drug eruption characterized by superficial pustules, commonly caused by β-lactam antibiotics. Management of AGEP involves discontinuation of the offending medication and supportive therapy with topical corticosteroids and analgesia, given the self-limiting nature of this condition. In contrast, Sweet syndrome (SS), or acute febrile neutrophilic dermatosis, is characterized by the sudden onset of painful, inflamed skin lesions associated with fever. SS typically requires investigations for associated conditions and systemic corticosteroids, resulting in rapid improvement within days. We present a case of AGEP-like SS, initially diagnosed clinically as AGEP in the setting of intravenous antibiotic use, where the patient deteriorated, despite antibiotics being stopped. Skin biopsy revealed papillary dermal oedema with neutrophilic infiltrate, and scattered histiocytoid cells and eosinophils, resulting in a revised diagnosis of AGEP-like SS. The patient was started on oral corticosteroids and achieved complete resolution of disease within 2 days. This case highlights AGEP-like SS as an important differential diagnosis in patients presenting with AGEP-like eruptions, as the management of AGEP and SS is different.
2026-04-17 | Apalutamide-induced severe cutaneous adverse reactions in prostate cancer: a comprehensive review of reported cases and clinical strategies.
Prostate cancer (PCa) is the most common fatal malignancy among men and a major cause of cancer-related death. Apalutamide, a second-generation androgen receptor inhibitor, is approved for non-metastatic castration-resistant prostate cancer (nmCRPC) and metastatic castration-sensitive prostate cancer (mCSPC). However, an increasing number of potentially life-threatening severe cutaneous adverse reactions (SCARs) associated with apalutamide have raised clinical concerns. This review aims to characterize apalutamide-induced SCARs and summarize effective management strategies based on reported cases. We systematically searched PubMed, Europe PMC, and CNKI for case reports of SCARs associated with apalutamide. Keywords included "Apalutamide" and "Drug eruptions." A total of 18 cases were identified and analyzed. We reviewed the clinical characteristics and treatment of 3 cases of DRESS, 14 cases of SJS/TEN, and 1 case of AGEP, all highly suspected to be caused by apalutamide, reported globally. The most frequent SCARs associated with apalutamide are SJS/TEN. The median onset time of SCARs in these cases was 39.5 days, significantly shorter than the onset time of rash in phase III clinical trials of apalutamide. Geographically, the majority of reported cases originated from East Asia. By analyzing the treatment regimens and clinical outcomes of these patients, combined with a literature review, we proposed a set of definitive therapeutic strategies. Apalutamide-induced SCARs tend to occur earlier than common rashes observed in clinical trials, with a predominance of reported cases in East Asian populations. Immediate discontinuation of the suspected drug is the cornerstone of SCAR management. Supportive care, systemic corticosteroids, intravenous immunoglobulin (IVIG), cyclosporine, plasmapheresis, and TNF-α antagonists play important roles in treatment. Personalized dosing strategies based on body weight or body surface area, along with proactive rash management, may help mitigate risk and optimize therapeutic continuity.
2026-03-30 | Case Report: Secukinumab induced pustular eruption in a patient with ankylosing spondylitis.
We report a case of a 33-year-old male with ankylosing spondylitis (AS) who had been receiving secukinumab (150 mg monthly) for 2 years. Approximately 1 year after initiating treatment, the patient developed recurrent, multiple sterile pustules on the palms and soles. Various topical treatments, including corticosteroids and calcipotriol, yielded minimal improvement. 1 week prior to presentation, new pustular lesions emerged on the trunk and extremities, accompanied by pruritus followed by burning sensations and tenderness. Histopathological examination revealed subcorneal pustule formation within the epidermis and sparse lymphocytic infiltration in the dermis. A diagnosis of secukinumab-induced pustular reaction was made. Following the discontinuation of secukinumab and the initiation of upadacitinib (15 mg/day) for over 1 month, the pustules largely resolved, and symptoms were significantly alleviated. This case suggests that IL-17A inhibitors may trigger pustular cutaneous reactions in AS patients. Clinicians should remain vigilant regarding such adverse effects and adjust treatment regimens promptly. JAK inhibitors may provide an effective therapeutic option for managing these drug-induced reactions.
2026-03-03 | Acute Generalized Exanthematous Pustulosis in a Neonate Following Hepatitis B Vaccination: A Case Report.
Acute generalized exanthematous pustulosis (AGEP) is a rare severe cutaneous adverse reaction in neonates and is most commonly drug-induced. Vaccine-associated cases are uncommon in this age group. We report a term male neonate who developed clusters of non-follicular pustules on erythematous plaques approximately 6 hours after receiving a birth dose of hepatitis B vaccine. The infant remained clinically stable, without fever or systemic involvement, and had no exposure to concomitant medications. The rapid onset following a single exposure, characteristic morphology, and prompt resolution were highly suggestive of AGEP. Laboratory investigations and skin biopsy were not performed due to rapid clinical improvement. Infectious etiologies and transient neonatal pustular dermatoses were considered less likely based on the clinical course. Patch testing with the same vaccine lot was positive, supporting a type IV hypersensitivity mechanism. The eruption resolved almost completely within 48 hours with topical hydrocortisone, without complications or recurrence.
antibodies
2026-07-20 | Desquamating Skin Diseases Requiring Burn Team Involvement: Spectrum, Clinical Outcomes, and Management, A Scoping Review
This scoping review aims to map the existing literature on non-burn desquamating, exfoliative, blistering, bullous, and epidermal detachment skin diseases requiring burn team involvement. These conditions may include Stevens-Johnson syndrome/toxic epidermal necrolysis, staphylococcal scalded skin syndrome, pemphigus, pemphigoid, linear IgA bullous dermatosis, DRESS, AGEP, severe cutaneous adverse reactions, and other related dermatologic conditions associated with significant skin loss. The purpose of this review is to characterize the spectrum of diseases managed with burn team involvement, describe the inpatient care settings in which these patients are treated, and summarize the role of burn teams in management. Burn team involvement may include primary admission under a burn service, co-management with another specialty, or consultative input for wound care, dressing management, surgical procedures, or other clinical decision-making. This review will also chart reported outcomes and management variables, including diagnosis at referral or admission, confirmed diagnosis, physical care setting, burn team role, wound care approaches, surgical interventions, immunomodulatory therapies, ICU admission, length of stay, mortality, complications, and resource utilization where available. Findings will be synthesized descriptively to identify patterns in the literature, highlight gaps in current evidence, and inform future research on the role of burn teams in the care of patients with severe non-burn desquamating skin disease.
2026-06-08 | When Conventional Therapy Fails: IL-36 Inhibition for Severe Hydroxychloroquine-Induced Acute Generalized Exanthematous Pustulosis in Systemic Lupus Erythematosus: A Case Report
Introduction: Acute generalized exanthematous pustulosis (AGEP) is a rare but severe cutaneous adverse drug reaction characterized by the sudden onset of widespread sterile pustules on an erythematous and edematous base, often accompanied by fever and neutrophilia. Over 90% of cases are drug-induced. Its management can be particularly challenging in patients with autoimmune diseases such as systemic lupus erythematosus (SLE), where cutaneous manifestations and immune dysregulation may confound clinical assessment. Case Presentation: We report a case of severe hydroxychloroquine-induced AGEP in a patient with active SLE. The disease was refractory to systemic corticosteroids and further worsened after IVIG infusion. Given the emerging role of IL-36 pathway dysregulation in pustular dermatoses, the patient was treated with spesolimab, a monoclonal antibody targeting the IL-36 receptor, resulting in rapid defervescence and near-complete resolution of pustules within days. Conclusion: This case underscores the need for prompt recognition of drug-induced AGEP in patients receiving antimalarials or other immunomodulatory agents for connective tissue diseases. Crosstalk between IL-36 signaling and neutrophil extracellular traps (NETs) may amplify inflammation, linking AGEP with autoimmune pathology. IL-36 inhibition with spesolimab represents a potential rescue therapy for severe, treatment-refractory AGEP, particularly in patients with underlying autoimmune disorders where conventional therapies fail.
2026-05-01 | C50-29 From Rash to Shock: A Life Threatening Presentation of Acute Generalized Pustular Psoriasis
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
2026-04-06 | Refractory acute generalized exanthematous pustulosis—Successfully treated with bimekizumab
Acute generalized exanthematous pustulosis (AGEP) is a rare, usually drug-induced, severe cutaneous adverse effect. Standard therapy includes withdrawal of the causative agent and often systemic corticosteroids, yet refractory cases remain challenging.1
2026-04-01 | Which drugs have the potential to cause Acute Generalized Exanthematous Pustulosis (AGEP)?
Acute Generalized Exanthematous Pustulosis (AGEP) has been associated with a variety of drugs including beta-lactam antibiotics, immune-stimulatory anti-cancer drugs, and certain antiretrovirals, among others.
proteins
2023-07-29 | Pustular Eruption following COVID-19 Vaccination: A Narrative Case-Based Review
From the beginning of public vaccinations until the relaxation of COVID-19 measures, many case reports, case series and case–control studies have been published indicating cutaneous side effects of COVID-19 vaccination. Post-vaccination pustular eruption was reported as well, with a challenging differential diagnosis between pustular psoriasis, AGEP (acute generalized exanthematous pustulosis) and neutrophil pustular eruptions. We report a case of 56-year-old woman presented with acute generalized pustular flare up culminated 5 days after the second dose of BNT162b2(Pfizer) vaccination. She was diagnosed with pustular psoriasis flare and due to the regulating role of IL-1 in pustular psoriasis and in the cytokine storm observed in cases of COVID-19 postvaccination inflammation; we decided to treat the patient with an IL-1 antagonist, subcutaneous anakinra (100 mg daily) along with acitretin. One week later, after anakinra withdrawal, she presented a pustular psoriasis flare and a 7-day anakinra re-administration led to a satisfactory improvement in the skin lesions. We also reviewed the medical literature and found 28 case reports with pustular eruption after the COVID-19 vaccination. We compared the patients reported, regarding sex, age, number of doses, post-vaccination period and vaccine brand, and compared those results with our patient. Finally, as indicated by our case and other cases with similarly treated pustular eruptions. targeted therapy to this cytokine imbalance such as anakinra (IL-1) antagonist can improve the clinical course of the patient.
2022-04-29 | Skin toxicity after Filgrastim treatment for an Ewing's sarcoma patient.
Filgrastim is a granulocyte colony-stimulating factor (GSCF) used in some chemotherapy regimen to prevent febrile neutropenia. Most common reaction of filgrastim are aches and pain including headaches, nausea and skin rash. We report the case of a patient who developed unusual, non-commonly reported adverse toxidermy to filgrastim. At first the eruption was limited to the lower members and genetics organs. Then it slowly spread across the whole body presenting as a polymorphic exanthematous-pustulosis lesions. A cutaneous biopsy was done, identifying a toxidermy modified by systemic treatment. A pharmacological study linked the role of filgrastim to these lesions. After switching from filgrastim to lénograstim, his lesions are completely gone and haven't flared up again. Thus, clearly imputing the use of filgrastim. The cutaneous reaction that has reported with use of GSCF are sweet syndrome, erythema nodosum, pyoderma nodosum and pyoderma gangrenosum. As far as we know, no acute generalized exanthematous pustulosis due to GSCF has been reported.
Access all drug discovery papers and probability of success in trials forecasts:
Access all drug discovery papers and probability of success in trials forecasts:
Drug Discovery Landscape
0 orphan drug designations.
0 orphan drug designations.
Let's accelerate rare disease drug discovery
Let's accelerate drug discovery
Get access to Explority AI’s forecasts to outperform average preclinical success rates. Whether you’re expanding your R&D pipeline, evaluating a partnership, or simply have a question — we’d love to hear from you.