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Acute Generalized Exanthematous Pustulosis

AGEP


Presentation

  • These include systemic presentations similar to toxic epidermal necrolysis (TEN) and drug-induced hypersensitivity syndrome (DIHS) and localized presentations mimicking other medication reactions.[ncbi.nlm.nih.gov]
  • This is a very rare and diagnostically challenging clinical presentation of acute generalized exanthematous pustulosis, which has been reported, to the best of our knowledge, only once previously.[ncbi.nlm.nih.gov]
  • Fever and rash are common presenting complaints to the Emergency Department. This report documents an uncommon diagnosis involving this presentation. The patient presented with signs and symptoms consistent with severe sepsis.[ncbi.nlm.nih.gov]
  • We present a severe case of AGEP, caused by flucloxacillin, clinically presenting with TEN-like features and pronounced systemic symptoms with haemodynamic and respiratory instability.[ncbi.nlm.nih.gov]
  • In conclusion, it is critical to realize that AGEP cannot be ruled out with a septic shock presentation.[ncbi.nlm.nih.gov]
Fever
  • She reported that two days previously she had developed fever and vomiting four hours after taking her first dose of fenofibrate (145 mg).[ncbi.nlm.nih.gov]
  • On day three postpartum she developed a 40.4 degrees C fever. Although ceftriaxone was administered, her fever persisted ( 38 degreesC).[ncbi.nlm.nih.gov]
  • It is commonly accompanied by fever and leukocytosis and usually resolves with discontinuation of the offending agent.[ncbi.nlm.nih.gov]
  • Its clinical hallmark is the sudden onset of multiple, disseminated, non-follicular, sterile pustules on an erythematous background usually arising in intertriginous folds, associated with fever, massive neutrophilia and sometimes eosinophilia.[ncbi.nlm.nih.gov]
  • It is accompanied by fever and acute eruption of non-follicular pustules overlying erythrodermic skin. The causative agents are most frequently antibacterial drugs.[ncbi.nlm.nih.gov]
Lymphedema of the Lower Extremity
  • A 67-year-old woman with cellulitis of the left lower extremity was admitted with marked bilateral lymphedema of the lower extremities and diffuse erythema of the left lower extremity from foot to knee. She was given clindamycin and then vancomycin.[ncbi.nlm.nih.gov]
Plethora
  • Sampson, Olga Klinkova, Julie Vitko and Beata Casanas, A Plethora of Pustules: Acute Generalized Exanthematous Pustulosis, The American Journal of Medicine, (2018).[doi.org]
Sore Throat
  • The patient presented with an erythematous and pustular eruption after taking 1 tablet of paracetamol for a sore throat.[thedoctorsdoctor.com]
  • throat was treated with three doses of acetaminophen (10 mg/kg), and 12 hours later, she developed an erythematous rash over her gluteal region and legs.[fda.gov]
Colic
  • We describe a case of a woman with AGEP, who presented with generalized pustulosis lesions after the use of piroxicam for renal colic.[ncbi.nlm.nih.gov]
Periodontitis
  • We assume that the administration of the 5 different antibiotics resulted in type IV allergy as well as secondary infection with Enterococcus faecium and Staphylococcus aureus, while the underlying periodontitis also contributed to the severity of this[ncbi.nlm.nih.gov]
Erythema
  • On day 5, her condition worsened, with erythema involving the entire back. Although treatment with clindamycin and vancomycin was discontinued, acute generalized exanthematous pustulosis developed.[ncbi.nlm.nih.gov]
  • A 42-year-old female presented with pustular lesions on the skin surface with erythema, facial edema, and occasional atypical target-like lesions after 21 days of treatment with 200mg/day hydroxychloroquine for rheumatoid arthritis, diagnosed one month[ncbi.nlm.nih.gov]
  • Two weeks after drug initiation, he developed a widespread oedematous erythema with sterile pustules. The histological examination showed subcorneal pustulosis formation with eosinophils.[ncbi.nlm.nih.gov]
  • A woman in her 60s with PV presented with diffuse erythema, nonfollicular pustules, and fever.[ncbi.nlm.nih.gov]
  • Acute generalized exanthematous pustulosis (AGEP) is a rare, drug-related pustular eruption usually starting from folds with edema and erythema and with subsequent spreading.[ncbi.nlm.nih.gov]
Exanthema
  • (a) At first presentation, the patient showed suberythrodermic exanthema with typical non-follicular pustules. (b) Histology revealed slight acanthosis and subcorneal pustules filled with neutrophils.[medicaljournals.se]
  • […] leg. with eosinophilia, psoriasis-like exanthema and AGEP [5].[symbiosisonlinepublishing.com]
  • Drug-induced hypersensitivity syndrome (DIHS) and exanthema in drug reaction with eosinophilia and systemic symptoms (DRESS) each display a variety of histopathological findings, which may partly correlate with the clinical manifestations.[ncbi.nlm.nih.gov]
  • The disulfiram can induce multiple cutaneous reactions. [4], [5] The acneiform eruptions, dermatitis, exanthema, purpura, rashes, urticaria, and vasculitis are reported in literature. [6] Disulfiram induces eczema in patients sensitized to rubber.[ijdd.in]
  • Gonzalo-Garijo MA, Pérez-Calderón R, de ArgilaFernández-Durán D (2008) Severe generalized exanthema due to etanercept given for severe plaque psoriasis. Ann Allergy Asthma Immunol 100: 621-622.[omicsonline.org]
Blister
  • We report a case of severe AGEP with extensive blistering mimicking toxic epidermal necrolysis (TEN) in a 47-year-old woman. This was associated with a life-threatening primary mumps infection, complicated by perimyocarditis and encephalitis.[ncbi.nlm.nih.gov]
  • Other skin signs such as facial edema, purpura, target-like lesions and blisters have been described but are not typical for AGEP.[ncbi.nlm.nih.gov]
  • A 58-year-old woman with type 1 diabetes was hospitalized for treatment of an extensive blistering rash on the buttocks and trunk accompanied by fever, hypotension, tachycardia, neutrophilia, impaired renal function, and liver enzyme abnormalities.[ncbi.nlm.nih.gov]
  • Stevens Johnson and TEN are characteristically different from AGEP by their slower onset (and longer duration) of 1-3 weeks, blisters instead of pustules, mucosal involvement, and lack of leukocytosis [4].[oatext.com]
  • The signs and symptoms of Acute Generalized Exanthematous Pustulosis may include the following: Skin rashes on the face, armpits, and groin Small blisters with white/yellow fluid Fever and malaise (feeling of illness) Facial swelling The onset of the[dovemed.com]
Pruritus
  • Furthermore, the patient suffered from severe pruritus in the affected areas.[karger.com]
  • The patient complained of a burning sensation of the skin in addition to pruritus; and simultaneously had a fever of 38-39 C as the eruption expanded. Discussion: A spider bite may represent a possible causative factor of AGEP.[jstage.jst.go.jp]
  • Cutaneous adverse reactions (CAR) attributed to fenofibrate are rare and include acute hypersensitivity reactions such as severe skin rashes, urticaria, photosensitivity reactions, contact dermatitis, Stevens-Johnson syndrome, toxic epidermal necrolysis, pruritus[edoj.org.eg]
  • CASE REPORT A 69-year-old male was referred to our clinic because of generalized pruritus and erythema accompanied by high fever. He had a history of hypertension for 20 years and had been taking 16 mg candesartan cilexetil tablets.[kjim.org]
  • Symptomatic treatment involves moist dressings during the pustular phase to relieve pruritus and prevent superinfection. Emollients may support skin barrier function restoration during the desquamation phase.[basicmedicalkey.com]
Erythema of the Chest
  • A 38-year-old primigravida whose labor had been induced developed erythema over her chest and abdomen. She was transferred to our department after a failed vacuum extraction, and delivered a mature infant by forceps.[ncbi.nlm.nih.gov]
Headache
  • The 2 groups were compared in terms of age, gender, systemic symptoms (such as fever, myalgias, or headaches), duration of disease, history of drug allergy, laboratory data, and pathologic findings.[doi.org]
  • The skin eruptions are often pruritic and accompanied by fever, headache, a high number of neutrophils and eosinophils in the blood, and elevated blood levels of markers for inflammation (i.e. erythrocyte sedimentation rate and C reactive protein).[en.wikipedia.org]
  • He gave history of taking a tablet of ASA for headache, few hours before development of the rash and fever. He had taken the same drug earlier innumerable times in last 15 years, without any side effects; however, it was a 325 mg tablet this time.[idoj.in]
  • After 24 hours, he was admitted with complaints of high fever (40 ), increased number of skin lesions, arthralgia, and headache. A dermatological examination revealed exanthema over 60% of the body accompanied by angioedema of the lips and eyelids.[kjim.org]
Neglect
  • While many of the most severe cutaneous reactions are likely to present initially to the emergency department, it is important not to neglect the role of the ambulatory care provider in managing those cases which present to a clinic.[ijcasereportsandimages.com]
Hand Tremor
  • Fortunately, the patient’s hand tremor ceased without requiring further treatment with other medication. We recommended valproic acid as an alternative drug in the event of recurring hand tremor.[zdoc.site]

Workup

  • We were able to obtain a rapid workup and quick referral to dermatology. Also unique is our suspicion that the inciting agent was a topical lotion, however, no etiology was objectively confirmed.[ijcasereportsandimages.com]
Liver Enzymes Abnormal
  • A 58-year-old woman with type 1 diabetes was hospitalized for treatment of an extensive blistering rash on the buttocks and trunk accompanied by fever, hypotension, tachycardia, neutrophilia, impaired renal function, and liver enzyme abnormalities.[ncbi.nlm.nih.gov]

Treatment

  • Although treatment with clindamycin and vancomycin was discontinued, acute generalized exanthematous pustulosis developed. After successful treatment of other complications, the skin condition improved.[ncbi.nlm.nih.gov]
  • With immediate discontinuation of terbinafine and various treatment protocols, both patients demonstrated recovery followed by skin desquamation during the subsequent weeks.[ncbi.nlm.nih.gov]
  • To report a rare case of drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome caused by antituberculosis (TB) drugs, which progressed to acute generalized exanthematous pustulosis (AGEP) after moxifloxacin treatment.[ncbi.nlm.nih.gov]
  • Hickson, Treatment of severe drug reactions by hemodialysis, International Journal of Dermatology, 57, 2, (177-182), (2017). Mindy M.[doi.org]
  • We found no differences between various treatment regimens (topical corticosteroid, systemic corticosteroid, and supportive treatment) regarding the time from drug cessation to pustules resolution (P 0.171). Conclusions.[ncbi.nlm.nih.gov]

Prognosis

  • The prognosis is generally good and the disease self limited, after withdrawal of the triggering drug. In this report the authors describe a case of AGEP, triggered by ceftriaxone in a patient with psoriasis vulgaris.[ncbi.nlm.nih.gov]
  • Although AGEP is a self-limiting disease with a favourable prognosis, secondary infections are a not infrequent complication in patients in poor general medical conditions. The reported mortality is about 5%.[ncbi.nlm.nih.gov]
  • Although AGEP and SJS/TEN are distinct entities with a different clinical picture, pathogenesis, prognosis and treatment, they may share some features, raising the hypothesis of overlap between both entities.[ncbi.nlm.nih.gov]
  • Hence, AGEP is known to have a good prognosis and to be life-threatening only in elderly patients or patients with chronic diseases.[ncbi.nlm.nih.gov]
  • The prognosis is generally good, as reported.[ncbi.nlm.nih.gov]

Etiology

  • The aim of this study is to characterize the etiology, clinical features, laboratory findings, management, and outcome of patients with AGEP in Asians. Patient/Methods.[ncbi.nlm.nih.gov]
  • We report the onset of disseminated non-follicular sterile pustules on an erythematous background in a 68-year-old man receiving a combination of isoniazid, pyrazinamide and rifampicin that may have been the etiologic agents.[ncbi.nlm.nih.gov]
  • Clinical Features: Acute-onset, generalized scarlitiniform eruption with sterile pustules, accompanied by fever Self-limited course of several days to a couple of weeks The etiology is a reaction to drugs, most commonly: Calcium channel blockers NSAIDs[dermpedia.org]
  • The drug etiology was excluded and spider bite was implicated as the etiological agent in these cases of AGEP which are the first such reported cases in India.[e-ijd.org]
  • Nevertheless, data suggest that a viral etiology is responsible for no more than 25% of the cases.[scielo.br]

Epidemiology

  • The purpose of the present review is to update knowledge on acute generalized exanthematous pustulosis (AGEP) in terms of epidemiology, pathogenesis, cause, clinical features, diagnosis, and treatment.[ncbi.nlm.nih.gov]
  • Abstract Purpose of review The purpose of the present review is to update knowledge on acute generalized exanthematous pustulosis (AGEP) in terms of epidemiology, pathogenesis, cause, clinical features, diagnosis, and treatment.[doi.org]
  • This will further enhance a systematized framework for translating epidemiological, clinical and immunopathogenetic advances into preventive efforts and improved outcomes for patients.[doi.org]
  • A clinical and epidemiological study of 104 cases. Br J Dermatol 1968;80:771–793. Beylot C, Bioulac P, Doutre MS: Acute generalized exanthematic pustulosis (four cases). Ann Dermatol Venereol 1980;107:37–48.[karger.com]
  • Generalized pustular psoriasis: A clinical and epidemiological study of 104 cases. Br J Dermatol. 1968; 80: 771- 793. PMID: 4236712 3. U.S. Food and Drug Administration. Drugs@FDA: Fenofibrate. Available at: . Accessed August 10, 2008. 4.[edoj.org.eg]
Sex distribution
Age distribution

Pathophysiology

  • The pathophysiological mechanism of AGEP remains unclear, although mutations in the IL36RN gene have been identified in a small subset of AGEP patients.[ncbi.nlm.nih.gov]
  • In this review, we highlight the main clinical, pathophysiological and diagnostic aspects of this peculiar form of drug allergy.[jle.com]
  • However, the pathophysiology and natural course are unknown.[ijdvl.com]
  • The pathophysiology for the development of these drug-independent cases of AGEP is unclear. Viral infections have also been observed to be associated with the development of SJS, SJS/TEN, and TEN in the absence of a causative drug.[en.wikipedia.org]
  • Pathophysiology Our understanding of the pathogenic mechanisms underlying AGEP is incomplete.[basicmedicalkey.com]

Prevention

  • Treatment is based on the removal of the causative drug, supportive care, infection prevention and use of potent topical or systemic steroids.[ncbi.nlm.nih.gov]
  • Lastly, other pustular cutaneous reactions may present similarly and therefore accurate identification of this disease can prevent unnecessary diagnostic testing.[ncbi.nlm.nih.gov]
  • Currently, there are no known measures to prevent Acute Generalized Exanthematous Pustulosis Avoiding medications similar to the one that caused the condition can help prevent its recurrence What is the Prognosis of Acute Generalized Exanthematous Pustulosis[dovemed.com]
  • Treatment focuses on removal of the causative drug, supportive care, infection prevention, and the often beneficial use of a potent topical steroid.[ncbi.nlm.nih.gov]
  • Definitive treatment of AGEP depends on removal of the offending agent, supportive care, and prevention of infection. The offending agent is a drug in 90% of cases [2]. More than 54 reported drugs have been implicated.[ijcasereportsandimages.com]

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