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Toxic epidermal necrolysis (TEN) is an acute life-threatening dermatologic emergency. However, many dermatoses can present with a TEN-like eruption. Those “TEN-mimics” are a true diagnostic challenge and an alarming differential diagnosis to such a serious condition. Herein, we will expose and classify the landscape of TEN-mimics. Also, the key differentiating clinical and/or laboratory points will be highlighted to help an accurate diagnosis of either a TEN or a TEN-like presentation.
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Background: Acute generalised exanthematous pustulosis(AGEP) is a rare, cutaneous reaction characterised bysudden onset of numerous, non-follicular, sterile pustuleson oedematous erythematous skin, accompanied by feverand neutrophilia. AGEP is predominantly drug-induced. Skinlesions appear rapidly within 1-3 days of drug exposure andupon drug withdrawal, resolve rapidly within 15 days.Objective: To determine the clinical characteristics, culpritdrugs and outcome of patients with AGEP.Methods: A retrospective note review of all AGEP patientsseen from 2001-2015.Results: Among 21 AGEP patients, 76% were Malays, 9.5%Chinese, 9.5% Indians, and 5% Iban. Sixteen were femalesand 5 were males. Median age of patients was 40 years (IQR:26). The main culprit drug was amoxicillin (10 cases),followed by cloxacillin (three cases), phenytoin (two cases)and one case each of carbamazepine, sulphasalazine,allopurinol, cephalexin, ceftriaxone, celecoxib and herbalproduct. The median time from drug initiation to onset ofAGEP was 3 days (IQR: 5.5). Fever was documented in 52.4%, mucosal involvement 9.5%, purpura 4.7% and blisters4.7%. Neutrophilia was observed in 63.6% of patients andeosinophilia in 28.5%. While most patients requiredadmission (67%), all achieved complete recovery within 15days without any sequela.
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Introduction: Severe cutaneous adverse drug reactions(SCARs) are not uncommon and potentially lifethreatening.Our objective is to study the patientcharacteristics, the pattern of implicated drugs andtreatment outcome among patients with SCARs.Methods: A 10-year retrospective analysis of SCARscases in Penang General Hospital was carried out fromJanuary 2006 to December 2015. Data collection is basedon the Malaysian Adverse Drug Reactions AdvisoryCommittee registry and dermatology clinic records.Results: A total of 189 cases of SCARs were encountered(F:M ratio; 1.2:1.0; mean age of 45 year). The commonestmanifestation was Stevens-Johnson Syndrome [SJS](55.0%), followed by toxic epidermal necrolysis [TEN](23.8%), drug rash with eosinophilia and systemicsymptoms [DRESS] (12.7%), acute generalisedexanthematous pustulosis [AGEP] (4.8%), SJS/TENoverlap syndrome (2.6%) and generalised bullous fixeddrug eruptions [GBFDE] (1.1%). Mean time to onset forTEN/SJS/Overlap syndrome was 10.5±13 days; AGEP,three days; GBFDE, 2.5±0.7 days, and DRESS, 29.4±5.7days. The most common drugs implicated wereantibiotics (33.3%), followed by allopurinol (18.9%) andanticonvulsant (18.4%). Out of 154 cases ofSJS/TEN/overlap syndrome, allopurinol was thecommonest causative agents (20.1%). In DRESS,allopurinol accounts for 45.8% of the cases. The mortalityrate in SJS, TEN and DRESS were 1.9%, 13.3% and 12.5%respectively. No mortality was observed in AGEP andGBFDE.Conclusion: The commonest manifestations of SCARs inour setting were SJS, TEN and DRESS. Allopurinol wasthe most common culprit. Thus, judicious allopurinol useis advocated and pre-emptive genetic screening for HLAB*5801 should be consider
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Background Literature on acute generalized exanthematous pustulosis (AGEP) is restricted to case reports, with only one prior series study. More importantly, a detailed histologic comparison to pustular psoriasis has not been done. Objective To identify discriminatory characteristics, we compared the histologic features of 45 cases of AGEP and 19 cases of pustular psoriasis. Methods Demographic, historical, clinical, and histologic features of AGEP and pustular psoriasis were compared using specimens from 5 tertiary medical centers. Results The age of patients with AGEP ranged from 12 to 91 years (mean, 53 years) with a nearly equal M:F ratio. All 45 patients presented with a generalized erythematous, pustular eruption (mean duration of pustules, 12 days) and fever (present in 25/31 patients). Recent drug ingestion was documented in 36/38 (95%) patients. Of the 5 pediatric cases, two had prior upper respiratory tract infection, but were without a history of recent drug ingestion. No patient with AGEP had a history of psoriasis. AGEP was distinguished from pustular psoriasis based upon the following histologic features: necrotic keratinocytes, papillary dermal edema, presence of eosinophils within the dermis, and absence of parakeratosis with neutrophils (p <0.05). Conclusion While the precise etiology of AGEP remains unknown, our findings confirm that most AGEP cases in adults are drug-related. Certain histologic features appear to reliably discriminate AGEP from pustular psoriasis, and awareness of them may increase diagnostic accuracy.
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Acute generalized exanthematous pustulosis(AGEP) is characterized by the abrupt onset of widespread pustules on an erythematous base and rapid spontaneous healing. Most cases appear to be related to drug reactions, mainly antibiotics, viral infections, and hypersensitivity to mercury. We report two cases of acute generalized exanthematous pustulosis which presented with widespread tiny pustules on the whole body. Histopathologic examinations of both cases showed subcorneal neutrophilic pustules with perivascular polymorphous cellular infiltration. We suspected that the possible cause of two cases was inhalant mercury and carbamazepine.
Subject(s)
Acute Generalized Exanthematous Pustulosis , Anti-Bacterial Agents , Carbamazepine , Hypersensitivity , NeutrophilsABSTRACT
BACKGROUND: Generalized sterile pustular eruption with fever which occurs in generalized pustular psoriasis (GPP) and acute generalized exanthematous pustulosis(AGEP) present a diagnostic and therapeutic problems. In Korea, there are a few studies of clinical and histopathologic reviews of these diseases, but long term follow-up and comparative clinicopathologic studies of these two diseases are not available. OBJECTIVE: This study attempts to identify the differences of these two diseases in the aspects of clinical, laboratory, and histopathologic findings. METHODS: We evaluated the clinical features, laboratory and histopathologic findings in 41 patients with generalized pustular eruption who had visited Pusan National University hospital during the past 20 years and reviewed the literature. RESULTS: 1. The ratio of patients with GPP(n=32) to ones with AGEP(n=9) was 3.6:1. 2. The mean age at diagnosis was 32.9(male) and 28.9(female) years in GPP, and 10.3 years(male) and 62.8 years(female) in AGEP. 3. The number of patients of GPP with previous personal history of psoriasis vulgaris were 15/32(46.9%) and the number of ones with previous family history of psoriasis vulgaris were 2/32(6.3%). 4. There was no patient of AGEP with personal or familial history of psoriasis. 5. The number of patients with recent drug intake history were 4/32(12.9%) in GPP and 9/9(100%) in AGEP. And common drugs suspected to cause AGEP were antibiotics(4 cases) and analgesics(3 cases). 6. Associated systemic symptoms were fever(37.5%), arthralgia(18.8%), and itching(62.5%) in GPP, whereas 66.7%, 33.3%, and 55.6%, respectively in AGEP. 7. The mean duration of pustules was 32.9 days in GPP and 7.2 days in AGEP. 8. Reccurences of generalized pustular eruption were 46.9% in GPP and 0% in AGEP. 9. Laboratory findings revealed leukocytosis(34.4%), elevated erythrocyte sedimentation rate(28.1%), hypoalbuminamia(25.0%), and eosinophilia(6.3%) in GPP, whereas 77.8%, 55.6%, 33.3%, and 71.4%, respectively in AGEP. 10. GPP and AGEP are diseases sharing similar clinical features, but these two diseases show distinctive clinical, laboratory, and histologic features. We suggest that it is important to be aware of these distinctions for avoidance of unnecessary aggressive therapy indicated for GPP.