Temporary tracheostomy may be necessary in case of extended mucosal damage. MRY, MGS, EN and GC designed the study, selected scientifically relevant information, wrote and revised the manuscript. 2010;88(1):608. J Clin Apher. Heat loss is another major concern that accompanies a defective skin barrier in patients with exfoliative dermatitis. 1991;97(4):697700. The exact source of FasL production has not been yet identified as different groups have postulated that the production might be sought in keratinocytes themselves [33] or in peripheral blood mononuclear cells [34]. AQUACEL Ag in the treatment of toxic epidermal necrolysis (TEN). Tumor necrosis factor : TNF- seems also to play an important role in TEN [41]. 543557. 2015;49(3):33542. J Am Acad Dermatol. Drug specific cytotoxic T-cells in the skin lesions of a patient with toxic epidermal necrolysis. In contrast with DRESS, eosinophilia and atypical lymphocytes are not described in patients with SJS or TEN. Ann Allergy Asthma Immunol. The incidence of erythema multiforme, StevensJohnson syndrome, and toxic epidermal necrolysis. 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). These levels could reflect the interaction between culprit drugs and aldehyde dehydrogenase that is the enzyme which metabolizes retinoid acid. Clin Exp Allergy. Exfoliative dermatitis (ED) is defined as diffuse erythema and scaling of the skin involving more than 90% of the total body skin surface area. Drugs that have been implicated in the causation of LPP include captopril, cinnarizine, ramipril, simvastatin, PUVA, and antituberculous medications. Recurrent erythema multiforme in association with recurrent Mycoplasma pneumoniae infections. J Popul Ther Clin Pharmacol. After 24 hours, split formation was evident in hematoxylin and eosin-stained sections of HOSCs treated . Br J Dermatol. The SCORTEN scale is based on a minimal set of parameters as described in the following table. Since cutaneous function as a multiprotective barrier is so disrupted in exfoliative dermatitis, the body loses heat, water, protein and electrolytes, and renders itself much more vulnerable to infection. ALDEN has shown a good accuracy to assess drug causality compared to data obtained by pharmacovigilance method and casecontrol results of the EuroSCAR casecontrol analysis for drugs associated with TEN. Medical genetics: a marker for StevensJohnson syndrome. PubMed Bastuji-Garin S, et al. The https:// ensures that you are connecting to the Clin Exp Dermatol. The dermis shows an inflammatory infiltrate characterized by a high-density lichenoid infiltrate rich in T cells (CD4+ more than CD8+) with macrophages, few neutrophils and occasional eosinophils; the latter especially seen in cases of DHR [5, 50]. J Invest Dermatol. Overall, incidence of SJS/TEN ranges from 2 to 7 cases per million person per year [9, 1820], with SJS the commonest [21]. Ramirez GA, Yacoub MR, Ripa M, Mannina D, Cariddi A, Saporiti N, Ciceri F, Castagna A, Colombo G, Dagna L. Biomed Res Int. Stern RS. J Am Acad Dermatol. The site is secure. 1997;19(2):12732. Bullous FDE. Disclaimer. 2018 Feb;54(1):147-176. doi: 10.1007/s12016-017-8654-z. This site needs JavaScript to work properly. Recurrent erythema multiforme: clinical characteristics, etiologic associations, and treatment in a series of 48 patients at Mayo Clinic, 2000 to 2007. Khalil I, et al. This content is owned by the AAFP. Topical treatment. 2010;85(2):1318. Chung W-H, et al. Diagnosis in a routine setting is based on patch test (PT) while skin test (prick and intradermal tests) with a delayed reading are contraindicated in these patients [72]. Fas-FasL interaction: Fas is a membrane-bound protein that after interaction with Fas-ligand (FasL) induces a programmed cell death, through the activation of intracellular caspases. Am J Clin Dermatol. In: Eisen AZ, Wolff K, editors. Tohyama M, et al. A multidisciplinary team is fundamental in the therapeutic management of patients affected by exfoliative DHR. One of the most common malignancies associated with exfoliative dermatitis is cutaneous T-cell lymphoma, which may not manifest for months or even years after the onset of the skin condition. The type of rash that happens depends on the medicine causing it and your response. A severity-of-Illness score for toxic epidermal necrolysis (SCORTEN) has been proposed and validated to predict the risk of death at admission [81]. The overall mortality rate is roughly 30%, ranging from 10% for SJS to more than 30% for TEN, with the survival rate worsening until 1year after disease onset [9, 1821]. Allergol Immunopathol (Madr). 2008;58(1):3340. Although the final result of this dual interaction is still under investigation, it seems that the combination of TNF-, IFN- (also present in TEN patients) and the activation of other death receptors such as TWEAK can lead to apoptosis of keratinocytes [44]. T and NK lymphocytes can produce FasL that eventually binds to target cells. In most severe cases the suggested dosage is iv 11.5mg/kg/day. Advise of potential risk to a fetus and use of effective contraception. Increased peripheral blood flow can result in high-output cardiac failure. f. This material may not otherwise be downloaded, copied, printed, stored, transmitted or reproduced in any medium, whether now known or later invented, except as authorized in writing by the AAFP. Abe R, et al. Severe adverse cutaneous reactions to drugs. oboda J, Dudzik A, Chomyszyn-Gajewska M. Ramirez GA, Ripa M, Burastero S, Benanti G, Bagnasco D, Nannipieri S, Monardo R, Ponta G, Asperti C, Cilona MB, Castagna A, Dagna L, Yacoub MR. Microorganisms. Expression of alpha-defensin 1-3 in T cells from severe cutaneous drug-induced hypersensitivity reactions. 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. Manage cookies/Do not sell my data we use in the preference centre. Br J Clin Pharmacol. Albumin is recommended only is albumin serum level is <2.5mg/dL. Stamp LK, Chapman PT. In any case all authors concluded that the blockage of FasL prevents keratinocyte apoptosis [35]. J Pharm Health Care Sci. Furosemide or ethacrynic acid may be required to maintain an adequate urinary output [90]. These studies have confirmed an association between carbamazepine-induced SJS/TEN with HLA-B*1502 allele among Han Chinese [27], carbamazepine and HLA-A*3101 and HLA-B*1511 [16], phenytoin and HLA-B*1502 [28], allopurinol and HLA-B*5801 [29]. Infliximab: chimeric IgG monoclonal anti-TNF- antibody. Check the full list of possible causes and conditions now! 2002;118(4):72833. When it precedes cutaneous T-cell lymphoma lesions, exfoliative dermatitis becomes the presenting sign of the underlying malignancy. Still, treatment indication, choice and dosage remain unclear, and efficacy yet unproven. As written before, Sassolas B. et al. Hypothermia can result in ventricular flutter, decreased heart rate and hypotension. Pregnancy . Malignancies are a major cause of exfoliative dermatitis. Usually, but not always, the palms of the hands, the soles of the feet and the mucous membranes are spared. Exfoliative dermatitis is a rare inflammatory skin condition that is characterized by desquamation and erythema involving more than 90% of the body surface area. 2004;59(8):80920. In this study, 965 patients were reviewed. Clinical, etiologic, and histopathologic features of StevensJohnson syndrome during an 8-year period at Mayo Clinic. The administration of a single dose of 5mg/kg was able to stop disease progression in 24h and to induce a complete remission in 614days. SCORTEN: a severity-of-illness score for toxic epidermal necrolysis. Pharmacogenomics J. The velocity of infusion should be regulated according to patients arterial pressure with the aim of 30mL/h urinary output (1mL/kg/h in case of a child). Antiepileptic medications, antihypertensive medications, antibiotics, calcium channel blockers and a variety of topical agents (Table 2)2,3,69 can cause exfoliative dermatitis, but theoretically, any drug may cause exfoliative dermatitis. Ardern-Jones MR, Friedmann PS. CAS Schwartz RA, McDonough PH, Lee BW. They usually have fever, are dyspneic and cannot physiologically feed. Pehr K. The EuroSCAR study: cannot agree with the conclusions. Takahashi R, et al. Drug rashes are the body's reaction to a certain medicine. Yamada H, Takamori K. Status of plasmapheresis for the treatment of toxic epidermal necrolysis in Japan. 2023 Jan 30;11(2):346. doi: 10.3390/microorganisms11020346. Skin testing and patch testing in non-IgE-mediated drug allergy. Download. Australas J Dermatol. Clin Exp Dermatol. Pathophysiology DIP. Drug-induced exfoliative dermatitis is usually short-lived once the inciting medication is withdrawn and appropriate therapy is administered. While nearly any medication can, in theory, cause a reaction if you're sensitive, medications linked to exfoliative dermatitis include: sulfa drugs; penicillin and certain other antibiotics . Genotyping is recommended in specific high-risk ethnic groups (e.g. Bookshelf Erythema multiforme (EM), StevensJohnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are the main clinical presentations of drug induced ED. Basal-cell carcinoma; Other names: Basal-cell skin cancer, basalioma: An ulcerated basal cell carcinoma near the ear of a 75-year-old male: Specialty Semin Dermatol. Careers. 1998;282(5388):4903. 2015;56(4):298302. The time interval between the appearance of exfoliative dermatitis and the appearance of cutaneous T-cell lymphoma lesions can vary from months to years or even decades. Nature. J Invest Dermatol. Erythema multiforme and toxic epidermal necrolysis: a comparative study. Toxic epidermal necrolysis: effector cells are drug-specific cytotoxic T cells. Erythema multiforme (EM), Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are the main clinical presentations of drug induced ED. In the acute phase, before determination of the etiology, treatment consists of measures to soothe the inflamed skin. . In: Eisen AZ, Wolff K, editors. It is a clinical manifestation and usually associated with various underlying cutaneous disorders, drug induced reactions and malignancies. EMM is a clinically severe, potentially life-threatening, extensive sloughing of epidermis, generally involving mucosal tissue. In EMM lesions typically begin on the extremities and sometimes spread to the trunk. 2004;428(6982):486. 2011;66(3):3607. Contact Dermatitis. Effects of treatments on the mortality of StevensJohnson syndrome and toxic epidermal necrolysis: a retrospective study on patients included in the prospective EuroSCAR Study. Systemic and potentially life-threatening complications include fluid and electrolyte imbalance, thermoregulatory disturbance, fever, tachycardia, high-output failure, hypoalbuminemia, and septicemia. (scFv) (directed against Dsg1/3) or AK23 (directed against Dsg3) with (as a control) or without exfoliative toxin A (ETA). Erythema multiforme (photo reproduced with permission of Gary White, MD): typical target lesions (white arrows) together with atypical two-zoned lesions (black arrows). Arch Dermatol. Wetter DA, Camilleri MJ. 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). (adult rickets), anticonvulsant-induced rickets and osteomalacia, osteoporosis, renal osteodystrophy . J Am Acad Dermatol. Apoptosis-inducing factors and lymphocyte-mediated cytotoxicity have been deeply investigated in ED. https://doi.org/10.1186/s12948-016-0045-0, DOI: https://doi.org/10.1186/s12948-016-0045-0. Options include use of PUVA light therapy, total-body electron beam irradiation, topical nitrogen mustard, systemic chemotherapy and extracorporeal photopheresis. Two Cases in Adult Patients. Loss of normal vasoconstrictive function in the dermis, decreased sensitivity to the shivering reflex and extra cooling that comes from evaporation of the fluids leaking out of the weeping skin lesions all result in thermoregulatory dysfunction that can cause hypothermia or hyperthermia.6 The basal metabolic rate also is increased in patients with exfoliative dermatitis. StevensJohnson syndrome and toxic epidermal necrolysis. A population-based study of StevensJohnson syndrome. 2010;37(10):9046. 2007;56(5 Suppl):S1189. Erythema multiforme (EM), Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are the main clinical presentations of drug induced ED. Descamps V, Ranger-Rogez S. DRESS syndrome. [Stevens-Johnson Syndrom and Toxic Epidermal Necrolysis--based on literature]. The strength of association with the development of SJS/TEN may vary among countries and historical periods, reflecting differences in ethnicities and prescription habits among the studied populations [6164]. 2010 Oct;35(7):723-8. doi: 10.1111/j.1365-2230.2009.03718.x. Granulysin is a key mediator for disseminated keratinocyte death in StevensJohnson syndrome and toxic epidermal necrolysis. The diagnosis of GVDH requires histological confirmation [87]. A population-based study with particular reference to reactions caused by drugs among outpatients. In case of a respiratory failure, oxygen should be administrated and a NIMV may be required. Antipyretic therapy. Exfoliative dermatitis may happen as a complication of other skin issues. Trautmann A, et al. All authors read and approved the final manuscript. Liver injury and exfoliative dermatitis caused by nifuratel[J]. Ko TM, et al. Chang CC, et al. PubMedGoogle Scholar. ), Phenolphthalein (Agoral, Alophen, Modane), Rifampin (Rifadin, Rimactane; also in Rifamate), Trimethoprim (Trimpex; also in Bactrim, Septra). Patients with carcinoma of the colon, lung, prostate and thyroid have presented with erythroderma. J Am Acad Dermatol. Mardani M, Mardani S, Asadi Kani Z, Hakamifard A. Dermatol Ther. A switch to oral therapy can be performed once the mucosal conditions improve. It can lead to pain, appear on large parts of the body and may require hospitalization. Retrospective review of StevensJohnson syndrome/toxic epidermal necrolysis treatment comparing intravenous immunoglobulin with cyclosporine. Gastrointestinal: pancreatitis, glossitis, dyspepsia. Cho YT, et al. [71] realized an algorhitm named ALDEN (algorithm of drug causality for epidermal necrolysis) which helps to establish a cause/effect relationship as probable or very probable in 70% of cases. Not responsive to therapy. Mild to severe alopecia and transient or permanent nail dystrophy also may be encountered. Szary syndrome, the leukemic variant of mycosis fungoides, is also associated with exfoliative dermatitis.
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