Exanthematous Pustulosis Research Articles

Pustular and crusted lesions in systemic lupus erythematosus: A case report

ABSTRACT Systemic lupus erythematosus (SLE) is the prototype of an autoimmune disease with various manifestations in the skin and several other organs. Subacute cutaneous lupus erythematosus may present with annular and psoriasiform lesions. There have been case reports of pustular lesions in SLE. Herein, we describe a known case of SLE, who developed pustular and crusted lesions which complicated the course of the disease. We considered Acute Generalised Exanthematous Pustulosis, Subcorneal Pustular Dermatosis, Pustular Psoriasis, Pustular Vasculitis, and Pustular Folliculitis as our initial differential diagnosis. A potassium hydroxide mount from crusted lesions showed scabies mites. With these findings, a diagnosis of SLE complicated with crusted scabies was made. She was managed with multiple systemic immunosuppressant and antiscabetic measures following which clinical improvement was seen with remission of her lesions.

Macrolide Antibiotic-Induced Acute Generalized Exanthematous Pustulosis in an Epileptic Patient on Phenytoin Therapy

Macrolide Antibiotic-Induced Acute Generalized Exanthematous Pustulosis in an Epileptic Patient on Phenytoin Therapy

Acute generalised exanthematous pustulosis induced by betamethasone sodium phosphate: A case report

Acute generalised exanthematous pustulosis induced by betamethasone sodium phosphate: A case report

54640 Validation and Optimization of EuroSCAR Criteria in Diagnosis of Acute Generalized Exanthematous Pustulosis (AGEP)

54640 Validation and Optimization of EuroSCAR Criteria in Diagnosis of Acute Generalized Exanthematous Pustulosis (AGEP)

Diclofenac-induced Acute Generalized Exanthematous Pustulosis: A Rare Case Scenario

ABSTRACT Diclofenac, categorized as a nonsteroidal anti-inflammatory drug, is commonly employed in medical settings due to its effectiveness in alleviating pain and reducing inflammation. Despite its widespread usage, diclofenac is associated with a range of adverse drug reactions (ADRs). Acute generalized exanthematous pustulosis is an infrequent and serious skin condition marked by the abrupt appearance of multiple sterile pustules on an inflamed, red base. This case report seeks to illuminate a unique and severe adverse drug reaction directly linked to diclofenac use, underscoring the importance of increased awareness among healthcare practitioners regarding the varied and occasionally serious nature of ADRs associated with diclofenac.

Clinical characteristics, treatment and outcome of clindamycin induced acute generalized exanthematous pustulosis.

Acute generalized exanthematous pustulosis (AGEP) is a serious and rare adverse reaction to clindamycin. This study investigated the clinical features of clindamycin-induced AGEP and provided reference for the prevention and treatment of AGEP. Case reports, case series and clinical studies of clindamycin-induced AGEP were collected by retrieving English and Chinese database from inception until May 31, 2024. Of the 35 patients included, 25 (71.4%) were female, and the median age was 57 years (1.6-88 years). The duration of AGEP onset is 2 days (range 0.04,13) after initial administration. The main clinical morphology of AGEP is a non-follicular pustular on an erythematous base, which may be accompanied by fever (54.3%) and pruritus (40.0%). These lesions mainly involved extremities and trunk. The median elevated neutrophil count was 13.3 × 109/L (range 10.3, 31.4). Histologic features of AGEP are characterized by intracorneal, subcorneal, and/or intraepidermal pustules with papillary dermal edema containing neutrophilic, lymphocytic, andeosinophilic infiltrates. Patients gradually recovered after the withdrawal of clindamycin and supportive therapy with a median time of 9 days (range 2, 30). Clinicians should be aware of AGEP as a rare adverse effect of clindamycin. When clindamycin is prescribed, it should be stopped in time when AGEP occurs, and active systemic treatment should be given. AGEP is a self-limiting disease with a good prognosis.

Acute generalised exanthematous pustulosis associated with upadacitinib treatment

Acute generalised exanthematous pustulosis associated with upadacitinib treatment

Acute generalized exanthematous pustulosis: European expert consensus for diagnosis and management.

Acute generalized exanthematous pustulosis (AGEP) is a rare, usually drug-induced, acute pustular rash. Despite the lack of strong data supporting the effectiveness of topical or systemic corticosteroids in this drug reaction, they are widely used. More generally, there is no consensus on the diagnostic modalities and the management of patients with AGEP. We aimed to provide European expert recommendations for the diagnosis and management or patients with AGEP. Members of the ToxiTEN group of the European Reference Network (ERN)-skin, all dermatologists and/or allergologists with expertise in drug reactions, elaborated these recommendations based on their own experience and on a review of the literature. Recommendations were separated into the following categories: professionals involved, assessment of the diagnosis of AGEP, management of the patient and allergological work-up after the acute phase. Consensus was obtained among experts for the list of professionals involved for the diagnosis and management of AGEP, including the minimum diagnostic work-up, the setting of management, the treatments, the modalities and the timing of allergological work-up and follow-up. European experts in drug allergies propose herein consensus on the diagnosis and management of patients with AGEP. A multidisciplinary approach is warranted, including dermatologists, allergologists and pharmacovigilance services.

Antibiotic-induced severe cutaneous adverse reactions: a single-center retrospective study over ten years.

Severe cutaneous adverse reactions (SCARs) are rare but life-threatening, with antibiotics being the main cause. This retrospective study from a single center was designed to analyze the culprit drugs, clinical features and treatment outcomes of antibiotic-induced SCARs. We analyzed cases of antibiotic-induced SCARs in a tertiary hospital in China between January 2013 and January 2024, including Steven-Johnson syndrome (SJS) or Stevens-Johnson syndrome-toxic epidermal necrolysis (SJS-TEN) overlap, toxic epidermal necrolysis (TEN), drug reaction with eosinophilia and systemic symptoms (DRESS) and acute generalized exanthematous pustulosis (AGEP). Descriptive analysis of the demographic characteristics, clinical manifestations, treatment and prognosis were carried out. Among 354 cases of SCARs, 63 validated antibiotic-related cases were included. Cephalosporins (31.7%), penicillins (25.4%), and quinolones (19.0%) were the most common triggers for SCARs. Overall, liver (50.8%), lungs (31.7%), and kidneys (23.8%) were the most frequently affected organ in SCARs cases. Eight patients (28.6%) in the SJS/SJS-TEN overlap group and 8 patients (80.0%) in the TEN group received combination therapy of corticosteroids and IVIG. Patients with SCARs caused by penicillins or cephalosporins could receive alternative treatments such as lincomamides, quinolones, and tetracyclines. The mortality rate in the TEN group was the highest at 20.0%, followed by the SJS/SJS-TEN overlap group (7.1%), and no deaths were observed in the DRESS and AGEP groups. The identification of the culprit antibiotics and the application of alternative antibiotic therapies are crucial for the management of antibiotic-induced SCARs. If complicated underlying conditions and complications like advanced age, cancer and pneumonia coexist with SCARs, patients might be more at risk for mortality.

Generalised pustular psoriasis triggered by monkeypox vaccination successfully treated with spesolimab

AbstractGeneralised pustular psoriasis (GPP) is characterised by a widespread rash with pustules and fever. Clinical and the histological overlap with the delayed hypersensitivity reaction acute generalised exanthematous pustulosis may delay the diagnostics and the initiation of correct treatment. In this case‐report, we describe an Human immunodeficiency virus positive patient with GPP possible triggered by monkeypox vaccination and eventually successfully treated with spesolimab, an interleukin‐36 inhibitor.

Evolution of the spectrum of drugs associated with acute generalized exanthematous pustulosis over time: A pharmacovigilance study

Evolution of the spectrum of drugs associated with acute generalized exanthematous pustulosis over time: A pharmacovigilance study

A Swollen Red Leg: Sweet’s Syndrome

Neutrophillic dermatoses comprise a range of conditions where the hallmark is histologic infiltrate of neutrophils. Ranging from skin disorders such as acute generalised exanthematous pustulosis or subcorneal pustulosis (Sneddon Wilkinson) which affect the epidermis, the neutrophilic infiltrate can affect the dermal portion such as in Neutrophillic urticaria, Sweet’s syndrome, or eccrine neutrophilic hidradenitis, or it can affect both epidermis and dermis such as in pyoderma gangrenosum. Many times the diagnosis of a neutrophilic dermatosis poses a challenge as the main differential are infectious diseases and a high degree of suspicion needed. Generally, the diagnosis is reached with clinical and dermatopathology correlation. We present the case of an 87-year-old woman with swollen red leg in whom the diagnosis posed a challenge

Recognizing rare rashes: A case of acute generalized exanthematous pustulosis.

Acute generalized exanthematous pustulosis (AGEP) is a rare, pustular rash that occurs most commonly after exposure to a medication (typically antibiotics or diltiazem). This case describes a patient who developed a widespread pustular eruption shortly after beginning empiric antibiotics for community-acquired pneumonia. Diagnosis of AGEP was difficult in this scenario due to the patient's pulmonary infection and atypical skin biopsy results. However, after AGEP was correctly identified, the offending agents were discontinued and the patient had subsequent resolution of her symptoms.

Severe cutaneous adverse reactions.

Severe cutaneous adverse reactions (SCARs), which include Stevens-Johnson syndrome and toxic epidermal necrolysis, drug reaction with eosinophilia and systemic symptoms (also known as drug-induced hypersensitivity syndrome), acute generalized exanthematous pustulosis, and generalized bullous fixed drug eruption, are life-threatening conditions. The pathogenesis of SCARs involves T cell receptors recognizing drug antigens presented by human leukocyte antigens, triggering the activation of distinct T cell subsets. These cells interact with keratinocytes and various immune cells, orchestrating cutaneous lesions and systemic manifestations. Genetic predisposition, impaired drug metabolism, viral reactivation or infections, and heterologous immunity influence SCAR development and clinical presentation. Specific genetic associations with distinct SCAR phenotypes have been identified, leading to the implementation of genetic screening before prescription in various countries to prevent SCARs. Whilst systemic corticosteroids and conventional immunomodulators have been the primary therapeutic agents, evolving strategies, including biologics and small molecules targeting tumour necrosis factor, different cytokines, or Janus kinase signalling pathways, signify a shift towards a precision management paradigm that considers individual clinical presentations.

Genetic analysis of different subtypes of aseptic pustulosis in the Chinese population.

Aseptic pustulosis involves inflammatory skin conditions with nonbacterial pustules on erythema, accompanied by neutrophil and eosinophil infiltration in the epidermis. Dysregulation of the interleukin (IL)-36 pathway leads to neutrophil aggregation and pustule formation. Variants in IL36RN, CARD14, AP1S3, MPO, SERPINA3 and BTN3A3 have been identified in generalized pustular psoriasis (GPP) in the past. Some patients with acrodermatitis continua of Hallopeau (ACH), palmoplantar pustulosis and acute generalized exanthematous pustulosis (AGEP) also exhibit mutations in IL36RN, CARD14 and AP1S3, albeit with regional and population-specific variations. This study aims to explore a shared genetic foundation among those with aseptic pustulosis. We performed Sanger sequencing on six genes in 126 patients with aseptic pustulosis. Genetic analysis identified IL36RN variants strongly associated with ACH, AGEP and subcorneal pustular dermatosis (SPD). Immunohistochemistry revealed elevated inflammatory cytokines in all subtypes. This study establishes a significant association between IL36RN variants and ACH, AGEP and SPD, emphasizing the IL-1/IL-36-chemokine-neutrophil axis as a common pathogenic mechanism. Targeting this axis holds promise for therapeutic interventions for aseptic pustulosis.

Trips Through the Skin: Reviewing Cutaneous Drug Reactions to Psychedelics and Hallucinogens.

Although psychedelic and hallucinogenic substances have gained popularity for therapeutic use, their dermatologic adverse effects are poorly characterized. This review characterizes the cutaneous reactions associated with psychedelic and hallucinogenic drugs. A review of PubMed and Scopus was conducted from the inception of databases to August 31, 2023. Search terms included drug names and classes (cannabis, MDMA, ecstasy, 3,4-methylenedioxymethamphetamine, psychedelics, hallucinogens, peyote, marijuana, lysergic acid diethylamide, LSD, ketamine, dimethyltryptamine, DMT, phencyclidine, PCP, dextromethorphan, psilocybin, and ayahuasca), and dermatosis terms (dermatitis, contact dermatitis, drug eruption, skin reaction, and urticaria). Studies were included if there was an association with a psychedelic or hallucinogenic and any cutaneous reaction; studies without both components were excluded. Twenty-two studies met inclusion criteria, describing reactions to cannabis (10 studies), MDMA (5 studies), ketamine (4 studies), and psilocybin (3 studies). Forty total patients were included. Among cannabis-related reactions, the most common reaction was type I hypersensitivity by topical exposure (n = 21). Three patients reported type IV hypersensitivity reactions to contact with cannabis or cannabis-derived oils, all of whom experienced vesicular contact dermatitis. Two additional patients presented with an erythema-multiforme-like reaction and acute generalized exanthematous pustulosis after systemic administration, respectively. MDMA was associated with acneiform eruptions (2 cases), an urticarial eruption, a guttate psoriasis-like reaction, a fixed drug eruption, and Stevens-Johnson syndrome (1 case). Four patients reported type I hypersensitivity reactions to ketamine. Four patients reported vesicular eruptions, cyanosis, or widespread jaundice to psilocybin. Of the cases, 8 patients had cutaneous reactions that resolved with drug cessation, 10 resolved with cessation plus treatment, and resolution in 7 cases was not reported. Zero studies were found describing other psychedelic or hallucinogenic compounds. Further research is required to characterize reactions and treatments linked to the variety of extant psychedelics and hallucinogens.

Generalised exanthematous pustular dermatophytids during Trichophyton violaceum tinea capitis

AbstractDermatophytids are defined as the acute occurrence of noninfectious secondary lesions at distance from a primary dermatophytic infection. They are well‐known after intertrigo of the toes. We report an 8‐year‐old boy, who developed generalised pustular lesions during the treatment of tinea capitis caused by Trichophyton violaceum. He had a rapid response to local corticosteroid ointment, while oral terbinafine was efficient on the scalp condition. Dermatophytids are the result of a type IV delayed hypersensitivity reaction to dermatophytic antigens. They may occur after initiation of an antifungal treatment. Generalised exanthematous pustular dermatophytids is a clinical variant of dermatophytids. They present as nonfollicular, nonconfluent pustules with an erythematous base, of abrupt onset. Main differential diagnoses are acute generalised exanthematous pustulosis and pustular psoriasis. Medical history, clinical presentation, laboratory findings and, if necessary, biopsy will help to distinguish between those conditions. Evolution is rapidly favourable under local or oral corticotherapy and antifungal treatments should not be withdrawn. Dermatophytids are a differential diagnostic of acute generalised pustulosis during tinea capitis.

COVID-19 and Autoimmunity in Dermatology: A Moroccan Case Series and Literature Review.

Since the beginning of the pandemic, many skin manifestations associated with COVID-19 have been reported. New reports show that COVID-19 can lead to autoimmune diseases (AIDs) and autoinflammatory diseases, especially dermatological. A prospective study was conducted by the dermatology department of the Centre Hospitalier Universitaire Ibn Rochd (CHU Ibn Rochd) of Casablanca in Moroccosince the beginning of the pandemic including 18 patients with COVID-19-related skin manifestations. Eighteen cases were collected with confirmed SARS-CoV-2 infection. The mean COVID score was 0.7. A percentage (94.44%) of the cases had general symptoms. Skin involvement was variable, mainly maculopapular rash (44.44%), purpura (27.77%), urticaria, varicelliform rash, necrotic lesions of the face, and pityriasisrosea Gibert (PRG)-like lesions. Mucosal involvement was found in 50%. Viral reactivation was found in 5.55%. Telogen effluvium was found in 22.22%. Moreover, AID was triggered by COVID-19: lupus (11.11%), associated with antiphospholipid syndrome (APL Sd) (5.55%), psoriasis (11.11%), alopecia, and pemphigus. Severe toxidermia was potentiated by SARS-CoV-2 infection (22.22%): Stevens-Johnson syndrome (Sd), acute generalized exanthematous pustulosis (APEG), and drug reaction with eosinophilia and systemic symptoms (DRESS). The interest of this work is to report our experience during the COVID-19 pandemicto understand some pathophysiological mechanisms of its dermatological manifestationsand to draw the attention of clinicians to the link of this infection with autoimmune and autoinflammatory diseases and toxidermia.

Determine and remove the drug trigger, usually antibiotics, that causes acute generalised exanthematous pustulosis

Determine and remove the drug trigger, usually antibiotics, that causes acute generalised exanthematous pustulosis

Real-World Efficacy of Spesolimab: Reports from Four Patients with Generalized Pustular Psoriasis

Introduction: Generalized pustular psoriasis (GPP) is a rare, chronic, autoinflammatory skin disease characterized by widespread pustular eruptions that may be accompanied by systemic symptoms. GPP is painful, distressing, and adversely affects patient quality of life (QoL). Spesolimab is a first-in-class anti-IL-36 receptor monoclonal antibody approved for the treatment of GPP flares. Methods: We describe 4 cases from our respective clinical practices, where individuals with GPP were treated successfully with spesolimab. Results: Four patient cases of GPP were included; an 18-year-old male, a 58-year-old female; a 36-year-old male, and a 40-year-old female. One patient had a history of GPP, and had experienced 3 prior flares in the preceding year. Two patients had a history of plaque psoriasis. Events preceding the current GPP flare included hydroxychloroquine treatment (n=2), and corticosteroid treatment (n=3). All patients presented with sudden onset of worsening pustular rash (affecting lower extremities [n=1]; extremities and trunk [n=1]; whole body [n=2]). Two patients had systemic symptoms at presentation (1 case: fever, chills, malaise, fatigue; 1 case: severe weakness), and required hospitalization. Two patients received an initial mis-diagnosis of acute generalized exanthematous pustulosis (AGEP). Significant impacts on QoL were reported; all 4 patients were unable to perform normal daily activities; the 18-year-old patient was targeted at school because the skin lesions were perceived to be contagious. The 4 patients received multiple therapies prior to, and concomitantly with, spesolimab treatment, including topical agents, antibiotics, corticosteroids, retinoids, immunosuppressants, and biologics. All 4 patients received two 900 mg intravenous infusions of spesolimab with treatment intervals between 7 and 20 days. Treatment was given at an outpatient infusion center (n=3*), or using a home infusion service (n=1); *1 patient received their first infusion as an inpatient. Significant clinician-assessed improvement in pustules occurred within 1 week of the first spesolimab infusion in 3 patients; the remaining patient reported improvement 1-2 weeks after the second infusion. One of the hospitalized patients experienced 75% improvement in itching, pain, and pustules within 24 hours of the first spesolimab infusion, and was discharged the next day. Spesolimab treatment also improved QoL, with patients experiencing rapid improvements in pain, and their ability to resume their normal daily activities. Conclusions: These cases demonstrate the efficacy of spesolimab in the real-world, highlighting the ability of spesolimab treatment to rapidly improve GPP symptoms and patient QoL. Administration of spesolimab in an inpatient setting allowed for rapid patient improvement and discharge the next day. These cases also highlight the association of certain drugs as triggers for GPP flares, as well as the importance of AGEP in the differential diagnosis of GPP