PubMed Central Effects of treatments on the mortality of StevensJohnson syndrome and toxic epidermal necrolysis: a retrospective study on patients included in the prospective EuroSCAR Study. Locharernkul C, et al. PubMed Central Drug induced exfoliative dermatitis (ED) are a group of rare and severe drug hypersensitivity reactions (DHR) involving skin and usually occurring from days to several weeks after drug exposure. CAS 22 Abacavir-induced hypersensitivity syndrome is strongly associated with HLA-B*5701 during treatment . 1996;135(2):3056. J Dermatol. Sassolas B, et al. Medicines have been linked to every type of rash, ranging from mild to life-threatening. Incidence of toxic epidermal necrolysis and StevensJohnson Syndrome in an HIV cohort: an observational, retrospective case series study. SJS and TEN are two overlapping syndromes resembling severe burn lesions and characterized by skin detachment. Erythema multiforme StevensJohnson syndrome and toxic epidermal necrolysis. Since the earliest descriptions of exfoliative dermatitis, medications have been known to be important causative agents. Google Scholar. Erythema multiforme (EM), StevensJohnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are the main clinical presentations of drug induced ED. Patients with carcinoma of the colon, lung, prostate and thyroid have presented with erythroderma. 2013;168(3):55562. Both DRESS and SJS may have increased liver enzymes and hepatitis, but they occur in only 10% of cases of SJS compared to 80% of DRESS. It recommended to used G-CSF in patients with febrile neutropenia [94, 95]. asiatic) before starting therapies with possible triggers (e.g. In particular, drug induced exfoliative dermatitis (ED) are a group of rare and more severe drug hypersensitivity reactions (DHR) involving skin and mucous membranes and usually occurring from days to several weeks after drug exposure [2]. Infectious agents are the major cause of EM, in around 90% of cases, especially for EM minor and in children. Clinical, etiologic, and histopathologic features of StevensJohnson syndrome during an 8-year period at Mayo Clinic. Cookies policy. Stamp LK, Chapman PT. . Utility of the lymphocyte transformation test in the diagnosis of drug sensitivity: dependence on its timing and the type of drug eruption. In some studies, the nose and paranasal area are spared. Erythema multiforme (EM), Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are the main clinical presentations of drug induced ED. Initial symptoms could be aspecific, as fever, stinging eyes and discomfort upon swallowing, occurring few days before the onset of mucocutaneous involvement. Anti-Allergic Agents Immunoglobulin E Allergens Cetirizine Histamine H1 Antagonists, Non-Sedating Histamine H1 Antagonists Loratadine Emollients Nasal Decongestants Dermatologic Agents Leukotriene Antagonists Antigens, Dermatophagoides Ointments Histamine Antagonists Eosinophil Cationic Protein Adrenal Cortex Hormones Terfenadine Antipruritics Antigens, Plant . New York: McGraw-Hill; 2003. pp. Granulysin as a marker for early diagnosis of the StevensJohnson syndrome. Kaffenberger BH, Rosenbach M. Toxic epidermal necrolysis and early transfer to a regional burn unit: is it time to reevaluate what we teach? Mawson AR, Eriator I, Karre S. StevensJohnson syndrome and toxic epidermal necrolysis (SJS/TEN): could retinoids play a causative role? The authors concluded that they couldnt demonstrate corticosteroids efficacy in monotherapy, but the use of steroid alone is not linked to an increased risk of mortality due to infective complications [108, 109]. 2000;22(5):4137. J Am Acad Dermatol. Etoricoxib-induced toxic epidermal necrolysis: successful treatment with infliximab. Next vol/issue Arch Dermatol. Antibiotic therapy. Blood counts and bone marrow studies may reveal an underlying leukemia. 2004;428(6982):486. Strom BL, et al. Nutr Clin Pract. Pharmacogenomics J. Unauthorized use of these marks is strictly prohibited. Curr Probl Dermatol. N.Z. Patients with underlying skin disorders may respond much more slowly to therapy, but clearing almost always occurs eventually. In: Eisen AZ, Wolff K, editors. HLA-B* 5801 allele as a genetic marker for severe cutaneous adverse reactions caused by allopurinol. These levels could reflect the interaction between culprit drugs and aldehyde dehydrogenase that is the enzyme which metabolizes retinoid acid. Kreft B, et al. Barbaud A. 2008;59(5):8989. CAS Summary: Drug induced interstitial nephritis, hepatitis and exfoliative dermatitis. EDs are serious and potentially fatal conditions. Drug induced exfoliative dermatitis: state of the art, https://doi.org/10.1186/s12948-016-0045-0, http://creativecommons.org/licenses/by/4.0/, http://creativecommons.org/publicdomain/zero/1.0/. Szary syndrome, the leukemic variant of mycosis fungoides, is also associated with exfoliative dermatitis. Gastric protection. Patients who have exfoliative dermatitis of unknown cause tend to have an unpredictable course, usually replete with multiple remissions and exacerbations.4. Tumor necrosis factor : TNF- seems also to play an important role in TEN [41]. Trautmann A, et al. Exfoliative dermatitis is also a risk factor for epidemic spread of methicillin-resistant Staphylococcus aureus.6,20. Recombinant granulocyte colony-stimulating factor in the management of toxic epidermal necrolysis. 2005;94(4):41923. Contact dermatitis from topical antihistamine . Burns. 2009;145(2):15762. Early sites of skin involvement include trunk, face, palms and soles and rapidly spread to cover a variable extension of the body. Etanercept therapy for toxic epidermal necrolysis. 2018 Jan 28;2018:9095275. doi: 10.1155/2018/9095275. 8600 Rockville Pike J Invest Dermatol. Kavitha Saravu. StevensJohnson syndrome and toxic epidermal necrolysis: assessment of medication risks with emphasis on recently marketed drugs. [Erythema multiforme vs. Stevens-Johnson syndrome and toxic epidermal necrolysis: an important diagnostic distinction]. Plasmapheresis may have a role in the treatment of ED because it removes Fas-L [96], other cytokines known to be implied in the pathogenesis (IL-6, IL-8, TNF-) [97, 98]. 2011;3(1):e2011004. The induction dosage in EMM is usually 1mg/kg/day that should be maintained until a complete control of the skin is obtained. Skin testing in delayed reactions to drugs. Int J Mol Sci. Paul C, et al. This hypermetabolic state is also furtherly increased by the inflammation present in affected areas. It could also be useful to use artificial tears and lubricating antiseptic gels. A marker for StevensJohnson syndrome: ethnicity matters. 2012;43:10115. 2012;167(2):42432. 2007;48(5):10158. Anyone you share the following link with will be able to read this content: Sorry, a shareable link is not currently available for this article. AQUACEL Ag in the treatment of toxic epidermal necrolysis (TEN). During the acute reaction, diagnosis of ED is mainly based on clinical parameters. Temporary tracheostomy may be necessary in case of extended mucosal damage. Mayes T, et al. Google Scholar. Mucosal involvement could achieve almost 65% of patients [17]. Bastuji-Garin S, et al. Medical genetics: a marker for StevensJohnson syndrome. DRUG- Induced- Dermatologic-RXNS lam University St. John's University Course Drug induced disease (CPP 6102) Academic year2023/2024 Helpful? 19 Key critical interactions are discussed below for each mpox antiviral. In more severe cases continuous iv therapy can be necessary. Among drug related cases, the main triggering factors are sulfonamides, nonsteroidal anti-inflammatories (NSAIDs), penicillins, and anticonvulsants (Table1) [59]. Granulysin is a key mediator for disseminated keratinocyte death in StevensJohnson syndrome and toxic epidermal necrolysis. 2010;2(3):18994. Prevalence is low, with mortality of roughly 512.5% for SJS and 50% for TEN [1, 2]. Blood gas analysis, glucose and creatinine levels together with electrolytes should be evaluated and therapy should be modified accordingly. Skin testing and patch testing in non-IgE-mediated drug allergy. StevensJohnson syndrome and toxic epidermal necrolysis: a review of the literature. 2008;53(1):28. The authors declare that they have no competing interests. Erythema multiforme (EM), StevensJohnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are the main clinical presentations of drug induced ED. Br J Dermatol. Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS): Focus on the Pathophysiological and Diagnostic Role of Viruses. N Engl J Med. Fitzpatricks dermatology in general medicine. Fernando SL. In any case all authors concluded that the blockage of FasL prevents keratinocyte apoptosis [35]. Chemicals and Drugs 61. If cutaneous pathology also mimics cutaneous T-cell lymphoma, it can be very difficult to differentiate a drug-induced skin condition from exfoliative dermatitis associated with a malignancy.2,9. A review of DRESS-associated myocarditis. 2005;62(4):63842. Moreover, transpiration and thermoregulation are greatly impaired with an elevated loss of fluids, proteins and electrolytes through the damaged skin and mucosae. Toxic epidermal necrolysis associated with severe cytomegalovirus infection in a patient on regular hemodialysis. Google Scholar. 2, and described below. Archivio Istituzionale della Ricerca Unimi, Nayak S, Acharjya B. Given the different histopathological features of the EM, SJS and TEN, we decided to discuss them separately. 2014;70(3):53948. J Dtsch Dermatol Ges. Paquet P, Pierard GE. doi: 10.1111/dth.15416. Beneficial effect of plasma exchange in the treatment of toxic epidermal necrolysis: a series of four cases. Some of these patients undergo spontaneous resolution. 2015;49(3):33542. Systemic and potentially life-threatening complications include fluid and electrolyte imbalance, thermoregulatory disturbance, fever, tachycardia, high-output failure, hypoalbuminemia, and septicemia. Generalized bullous fixed drug eruption is distinct from StevensJohnson syndrome/toxic epidermal necrolysis by immunohistopathological features. TEN is also known as Lyell syndrome, since it was first described by Alan Lyell in 1956 [2, 60]. 1995;5(4):2558. Epub 2018 Aug 22. Histopathological and epidemiological characteristics of patients with erythema exudativum multiforme major, StevensJohnson syndrome and toxic epidermal necrolysis. The management of toxic epidermal necrolysis. Read this article to find out all its symptoms, causes and treatments. J Am Acad Dermatol. The team should include not only physicians but also dedicated nurses, physiotherapists and psychologists and should be instituted during the first 24h after patient admission. PubMed Both hyperthermia and hypothermia are reported. The balance of fluids and electrolytes should be closely monitored, since dehydration or hypervolemia can be problems. ), Phenolphthalein (Agoral, Alophen, Modane), Rifampin (Rifadin, Rimactane; also in Rifamate), Trimethoprim (Trimpex; also in Bactrim, Septra). Morel E, et al. Half-life of the drug is approximately 54 h. Modification of nitisinone in liver and renal dysfunction is yet to be studied. Open Access This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. If after 4days there is not an improvement it is advised to consider the association of steroid or its replacement with one of the following drugs [49, 93]: Intravenous immunoglobulins (IVIG): play their role through the inhibition of FasFas ligand interaction that it is supposed to be the first step in keratinocytes apoptosis [33]. The most important actions to do are listed in Fig. Acute and chronic leukemia may also cause exfoliative dermatitis. Genome-scale investigation of drug-induced termination codon-readthrough in a model system of epidermolysis bullosa . Also a vesical catheter should be placed to avoid urethral synechiae and to have a precise fluid balance. Schwartz RA, McDonough PH, Lee BW. Drug induced exfoliative dermatitis: state of the art. 2016;2:14. When it precedes cutaneous T-cell lymphoma lesions, exfoliative dermatitis becomes the presenting sign of the underlying malignancy. Erythroderma (literally, "red skin"), also sometimes called exfoliative dermatitis, is a severe and potentially life-threatening condition that presents with diffuse erythema and scaling involving all or most of the skin surface area (90 percent, in the most common definition). 1993;129(1):926. Von Hebra first described erythroderma (exfoliative dermatitis) in 1868. EM is a self-limited skin condition mainly associated with infections and drugs [53, 54]. Moreover, the time necessary for cells to mature and travel through the epidermis is decreased. Mortality rate of patients with TEN has shown to be directly correlated to SCORTEN, as shown in Fig. Hospitalization and dermatologic consultation are indicated in most cases to ensure that all of the necessary cutaneous, laboratory and radiologic investigations and monitoring are performed. Before Google Scholar. . It should be considered only once the patient is stable and if the skin damage is still ongoing and doesnt respond to other conventional therapies (corticosteroids or IVIG). Inhibition of toxic epidermal necrolysis by blockade of CD95 with human intravenous immunoglobulin. Retrospective review of StevensJohnson syndrome/toxic epidermal necrolysis treatment comparing intravenous immunoglobulin with cyclosporine. 1990;126(1):437. Most common used drugs are: morphine, fentanyl, propofol and midazolam. 1996;134(4):7104. CAS Erythema multiforme (EM), Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are the main clinical presentations of drug induced ED. Infliximab was used in cases refractory to high-dosage steroid therapy and/or IVIG. . If it is exfoliative dermatitis that's drug induced, it's easy to treat . J Am Acad Dermatol. Here we provide a systematic review of frequency, risk factors, molecular and cellular mechanisms of reactions, clinical features, diagnostic work-up and therapy approaches to drug induced ED. -. Other clinical findings include lymphadenopathy, hepatomegaly, splenomegaly, edema of the foot or ankle4,6 and gynecomastia.19, The scaling that occurs in exfoliative dermatitis can have severe metabolic consequences, depending on the intensity and the duration of the scaling. 2023 Jan 30;11(2):346. doi: 10.3390/microorganisms11020346. The cutaneous T-cell lymphomas are the lymphomas most commonly associated with exfoliative dermatitis. Although the etiology is. J Allergy Clin Immunol. Google Scholar. In patients with SJS/TEN increased serum levels of retinoid acid have been found. The most common causes of exfoliative dermatitis are preexisting dermatoses, drug reactions, malignancies and other miscellaneous or idiopathic disorders. 2011;71(5):67283. Eur J Clin Microbiol Infect Dis. It is a reaction pattern and cutaneous manifestation of a myriad of underlying ailments, including psoriasis and eczema, or a reaction to the consumption of . Mortality rate of patients with TEN has shown to be directly correlated to SCORTEN. Ibuprofen Zentiva is a drug based on the active ingredient ibuprofen (DC.IT) (FU), belonging to the category of NSAID analgesics and specifically derivatives of propionic acid. Drug-induced Exfoliative Dermatitis & Eosinophils Increased Symptom Checker: Possible causes include Exfoliative Dermatitis. [117] described a cohort of ten patients affected by TEN treated with a single dose of etanercept 50mg sc with a rapid and complete resolution and without adverse events. Targeting keratinocyte apoptosis in the treatment of atopic dermatitis and allergic contact dermatitis. A catabolic state thus ensues, which is often responsible for significant weight loss. Drug induced exfoliative dermatitis (ED) are a group of rare and severe drug hypersensitivity reactions (DHR) involving skin and usually occurring from days to several weeks after drug exposure. Erythema multiforme and latent herpes simplex infection. Exfoliative dermatitis accounts for about 1 percent of all hospital admissions for dermatologic conditions.3, Although the disease affects both men and women, it is more common in men, with an average male-to-female ratio of 2.3:1. CAS J Am Acad Dermatol. 1995;14(6):5589. Etanercept: monoclonal antibody against the TNF- receptor. Students also viewed Nostra aetate - Summary Theology: the basics Principles of Risk Management and Insurance Chapters 1-4 Br J Dermatol. The syndrome has been described previously in association with phenindione administration, leptospirosis and heavy metal poisoning. Therefore, the clinician should always consider drugs as a possible cause. Even patients with clear histories of preexisting dermatoses tend to have biopsies that are not diagnostic when they present with erythroderma.2, Laboratory evaluation of patients with erythroderma is generally not very helpful in determining a specific diagnosis. PTs have to be performed at least 6months after the recovery of the reaction, and show a variable sensitivity considering the implied drug, being higher for beta-lactam, glycopeptide antibiotics, carbamazepine, lamotrigine, proton pump inhibitors, tetrazepam, trimethoprimsulfametoxazole, pseudoephedrine and ramipril [7376]. Patients can be extremely suffering because of the pain induced by skin and mucosal detachment. A population-based study with particular reference to reactions caused by drugs among outpatients. 2006;19(4):18891. Interleukin (IL)-1, IL-2, IL-8, intercellular adhesion molecule 1 (ICAM-1), tumor necrosis factor and interferon gamma are the cytokines that may have roles in the pathogenensis of exfoliative dermatitis.2. More recently, carcinomas of the fallopian tube,12 larynx13 and esophagus14 have been reported as causes of exfoliative dermatitis. Allergol Int. Karnes JH, Miller MA, White KD, Konvinse KC, Pavlos RK, Redwood AJ, Peter JG, Lehloenya R, Mallal SA, Phillips EJ. Paquet P, Pierard GE, Quatresooz P. Novel treatments for drug-induced toxic epidermal necrolysis (Lyells syndrome). Br J Dermatol. Journal of Pharmaceutical Research and health Care. Wetter DA, Camilleri MJ. Four cases are described, two of which were due to phenindione sensitivity. 2011;18:e12133. The clinical course of patients with malignancies depends on the type of malignancy and the response to appropriate therapy. Energy requirements of pediatric patients with StevensJohnson syndrome and toxic epidermal necrolysis. More than moderate, unresponsive to treatment, and which interferes with the Soldier's perfor-mance of duty. The authors wish to thank Dr. Gary White for the picture of EM showed in Fig. Schopf E, et al. [113] retrospectively compared mortality in 64 patients with ED treated either with iv or oral Cys A (35mg/kg) or IVIG (25g/Kg). Ethambutol Induced Exfoliative Dermatitis. 2007;56(5 Suppl):S1189. Erythema multiforme StevensJohnson syndrome and toxic epidermal necrolysis. Disclaimer. Pichler WJ, Tilch J. Patch testing in severe cutaneous adverse drug reactions, including StevensJohnson syndrome and toxic epidermal necrolysis. . Topical treatment. Hum Mol Genet. The PubMed wordmark and PubMed logo are registered trademarks of the U.S. Department of Health and Human Services (HHS). By using this website, you agree to our Rzany B, et al. Efficacy of plasmapheresis for the treatment of severe toxic epidermal necrolysis: is cytokine expression analysis useful in predicting its therapeutic efficacy? The lesions consist of pruritic, annular papules, vesicles, and bullae that are found in groups, clinically it is similar to dermatitis herpetiformis, without a gluten-sensitive enteropathy [85]. Immunoregulatory effector cells in drug-induced toxic epidermal necrolysis. 2010;125(3):70310. Toxic epidermal necrolysis: Part I Introduction, history, classification, clinical features, systemic manifestations, etiology, and immunopathogenesis. Pemphigus vulgaris, paraneoplastic pemphigus, bullous pemphigoid and linear IgA dermatosis have to be considered. Mittmann N, et al. Fritsch PO. Terms and Conditions, Clin Pharmacol Ther. In addition to all these mechanisms, alarmins, endogenous molecules released after cell damage, were found to be transiently increased in SJS/TEN patients, perhaps amplifying the immune response, including -defensin, S100A and HMGB1 [47]. Other dermatoses associated with erythroderma are listed in Table 1.2,3,68. Abe R, et al. Scientific evidences suggest a role for HLAs and drug-induced SJS/TEN, although some racial differences have been found that can be due to variation of frequencies of these alleles and to the presence of other susceptibility genes [26]. A significant number of these patients eventually progress to cutaneous T-cell lymphoma.8, Clinically, the first stage of exfoliative dermatitis is erythema, often beginning as single or multiple pruritic patches, involving especially the head, trunk and genital region. 2022 May;35(5):e15416. The https:// ensures that you are connecting to the 585600. All the linen must be sterile. In patients with this disorder, the mitotic rate and the absolute number of germinative skin cells are higher than normal. Herpes simplex virus (HSV) 1 and 2 are the main triggers in young adults (>80% of cases), followed by Epstein-Barr virus (EBV), and Mycoplasma pneumonia [5558]. Delayed reactions to drugs show levels of perforin, granzyme B, and Fas-L to be related to disease severity. 2013;69(2):187. CAS 2006;6(4):2658. Systemic derangements may occur with exfoliative. 2005;102(11):41349. Abe J, et al. Article Ann Pharmacother. Man CB, et al. Narita YM, et al. Some anti-seizure medicines have also been known to cause exfoliative dermatitis. Growth-factors (G-CSF). Interstitial nephritis is common in DRESS syndrome, occurring roughly in 40% of cases, whereas pre-renal azotemia may occur in SJS and TEN. For these reasons, patients should be admitted to intensive burn care units or in semi-intensive care units where they may have access to sterile rooms and to dedicated medical personnel [49, 88]. Recurrent erythema multiforme in association with recurrent Mycoplasma pneumoniae infections. Accessibility 2016 Nov 15;17(11):1890. doi: 10.3390/ijms17111890. Fluid balance is a main focus. In HIV patients, the risk of SJS and TEN have been reported to be thousand-fold higher, roughly 1 per 1000 per year [19]. Clinical, etiologic, and histopathologic features of StevensJohnson syndrome during an 8-year period at Mayo Clinic. The diagnosis of GVDH requires histological confirmation [87]. Drug-induced erythroderma invariably recovers completely with prompt initial management and removal of the offending drug. Albeit the lack of epidemiologic data regarding EM, its reported prevalence is less than 1% [710]. Autologous transplantation of mesenchymal umbilical cord cells seems also to be highly efficacious [102]. FDA Drug information Dupixent Read time: 6 mins Marketing start date: 04 Mar 2023 . statement and 2011;128(6):126676. HHS Vulnerability Disclosure, Help Gout and its comorbidities: implications for therapy. The taper of steroid therapy should be gradual [93]. 1998;37(7):5203. An epidemiologic study from West Germany. Manganaro AM. Drug-induced hypersensitivity syndrome (DiHS) or drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a severe adverse drug-induced reaction characterized by various symptoms: skin rash, fever, lymph node enlargement and internal organ involvement, which starts within 2 weeks to 3 months after drug initiation. Add 1 cup (about 236 milliliters) of vinegar to a bathtub filled with warm water. Do this 2 to 3 times a week. Talk to our Chatbot to narrow down your search. Chung WH, et al. 2002;118(4):72833. This compressed maturation process results in an overall greater loss of epidermal material, which is manifested clinically as severe scaling and shedding. f. 2012;13(1):4954. In patients who develop complications (i.e., infection, fluid and electrolyte abnormalities, cardiac failure), the rate of mortality is often high. An official website of the United States government. Tohyama M, et al. Chang CC, et al. McCormack M, et al. 2018 Feb;54(1):147-176. doi: 10.1007/s12016-017-8654-z. For the calculation, available values on vital and laboratory parameters within the first 3days after admission to the first hospital are considered when the reaction started outside the hospital (community patients) or at the date of hospitalization for in-hospital patients. Privacy 2008;52(3):1519. It should be used only in case of a documented positivity of cultural samples. 2003 Oct 25;147(43):2089-94. Drug induced exfoliative dermatitis (ED) are a group of rare and severe drug hypersensitivity reactions (DHR) involving skin and usually occurring from days to several weeks after drug exposure. Am J Clin Dermatol. Expression of alpha-defensin 1-3 in T cells from severe cutaneous drug-induced hypersensitivity reactions. Acute generalized exanthematous pustulosis (AGEP) is characterized by acute erythematous skin lesions, generally arising in the face and intertriginous areas, subsequently sterile pinhead-sized nonfollicular pustules arise and if they coalesce, may sometimes mimic a positive Nikolskys sign and in this case the condition may be misinterpreted as TEN [86]. J Allergy Clin Immunol. Exanthematous drug eruptions. SJS/TEN syndrome is associated with severe blistering, mucocutaneous peeling, and multi-organ damage and could be life threatening. Morel E, et al. 2010;62(1):4553. 2011;38(3):23645. Br J Dermatol. 1997;19(2):12732. Nature. Download Free PDF. Case Rep Dermatol. The efficacy of intravenous immunoglobulin for the treatment of toxic epidermal necrolysis: a systematic review and meta-analysis. Mockenhaupt M, et al. Among the anti-tubercular drugs exfoliative dermatitis is reported with rifampicin, isoniazid, ethambutol, pyrazinamide, streptomycin, PAS either singly or in combination of two drugs in some cases. Chung WH, Hung SI. If there is a high suspicion of infection without a documented source of infection, broad range empiric therapy should be started. Options include use of PUVA light therapy, total-body electron beam irradiation, topical nitrogen mustard, systemic chemotherapy and extracorporeal photopheresis. Atypical target lesions manifest as raised, edematous, palpable lesions with only two zones of color change and/or an extensive exanthema with a poorly defined border darker in the center(Fig. Khalaf D, et al. of Internal Medicine, University of Bari, Bari, Italy, Andrea Nico,Elisabetta Di Leo,Paola Fantini&Eustachio Nettis, You can also search for this author in Manage cookies/Do not sell my data we use in the preference centre. Clinical features; Delayed type hypersensitivity; Drug hypersensitivity; Erythema multiforme; Exfoliative dermatitis; Lyells syndrome; Pathogenesis; StevensJohnson syndrome; Therapy; Toxic epidermal necrolysis. Verma R, Vasudevan B, Pragasam V. Severe cutaneous adverse drug reactions. 1991;127(6):8318. 2012;166(2):32230. Chung W-H, et al. Valeyrie-Allanore L, et al. Recurrent erythema multiforme: clinical characteristics, etiologic associations, and treatment in a series of 48 patients at Mayo Clinic, 2000 to 2007. The authors concluded for a potential beneficial effect of Cys A and a possible improvement in survival compared to IVIG. Correction of hyperthermia or hypothermia Antibiotic administration when underlying infection is suspected or identified as cause of exfoliative dermatitis or when a secondary skin and soft. 2008;4(4):22431. Usually, but not always, the palms of the hands, the soles of the feet and the mucous membranes are spared. Epub 2022 Mar 9. Int J Dermatol. AB, CC, ET, GAR, AN, EDL, PF performed a critical revision on the current literature about the described topic, wrote and revised the manuscript. Paraneoplastic pemphigus is associated with neoplasms, most commonly of lymphoid tissue, but also Waldenstrms macroglobulinemia, sarcomas, thymomas and Castlemans disease. It is challenging to diagnose this syndrome due to the variety . Pharmacogenetics studies have found an association between susceptibility to recurrent EM in response to several stimuli and human leukocyte antigen (HLA) haplotypes of class II, in particular HLA DQB1*0301 [23]. The fluid of blisters from TEN patients was found to be rich in TNF-, produced by monocytes/macrophages present in the epidermis [42], especially the subpopulation expressing CD16, known to produce higher levels of inflammatory cytokines [43]. A multidisciplinary team is fundamental in the therapeutic management of patients affected by exfoliative DHR. Drug eruptions that initially present as morbilliform, lichenoid or urticarial rashes may progress to generalized exfoliative dermatitis.