PHYSIOLOGICAL SOLUTION (NaCl 0,9%) AS ONE OF THERAPIES IN PATIENTS WITH ORAL LESIONS ASSOCIATED WITH STEVEN-JOHNSON SYNDROME

Stevens-Johnson syndrome (SJS) and its more advanced form, Toxic Epidermal Necrolysis (TEN) are severe adverse cutaneous reactions that predominantly involve skin and mucous membranes. In view of the current dearth of documented knowledge, this study the first of its kind from India was designed to categorically distinguish and compare the different associated factors and clinical manifestations relevant to the development of SJS/TEN syndrome among the immunocompromised (Human Immunodeficiency Virus 1 seropositive) patients in Eastern India. 16 out of 29 patients (55.1%) were found to be suffering from drug induced SJS or TEN, while the rest (44.8%) had severe pathogenic involvements. Neviraprine use was found to be the major cause among drug involved SJS (53.8%) and TEN (66.66%) cases followed by allopurinol use. The frequency of incidence of kidney disease (67% in SJS and 54% in TEN) and neurological impairment (42% SJS and 37%TEN) was found to be significantly higher among the SJS patients whereas that of hepatitis (38% SJS and 47% TEN) , ocular dysfunction (49% SJS and 63% TEN) and pulmonary dysfunction (47% SJS and 53% TEN) were higher among the TEN patients. This study also had another prominent motive, which is to elucidate the possible role of human cytomegalovirus in triggering the exfoliative inflammatory disease conditions among these patients. The incidence rate of SGPT and SGOT were significantly higher in TEN patients than in SJS (p=0.001 and p=0.002) . Mean sodium level in blood was within normal range in both the groups whereas potassium and chloride levels were much higher than normal Out of the 29 HIV seropositive patients with SJS/TEN we studied, only 4 (13.7%) had an active HCMV infection. In detailed study of the associated laboratory and clinical parameters, cytokine analysis profile, Immuno-histologic findings and rigorous analysis of the investigation reports identified HCMV infection as the probable trigger behind SJS/TEN development in these patients.

Stevens-Johnson syndrome (SJS) or erythema multiforme (EM) major is a complex immunological syndrome characterized by acute blistering, affecting skin and at least two mucous membranes. Toxic epidermal necrolysis (TEN) is the most severe form of EM involving skin, which sloughs in sheets. Corneal damage is the most severe long-term complication for survivors of SJS and TEN. Conjunctival inflammation is commonly encountered in Ophthalmic practice. In children, allergy and infections form the most common etiology of conjunctivitis and antibiotics are often prescribed for all conjunctival inflammations presenting with red eye. The purpose of this report is to report the rare case of SJS with topical use of fluroquinolone.

Toxic epidermal necrolysis (TEN) and Stevens Johnson Syndrome (SJS) are severe adverse cutaneous drug reactions that predominantly involve the skin and mucous membranes. Both are rare, with TEN and SJS affecting approximately 1or 2/1,000,000 annually, and are considered medical emergencies as they are potentially fatal. They are characterized by mucocutaneous tenderness and typically hemorrhagic erosions, erythema and more or less severe epidermal detachment presenting as blisters and areas of denuded skin. Currently, TEN and SJS are considered to be two ends of a spectrum of severe epidermolytic adverse cutaneous drug reactions, differing only by their extent of skin detachment. Drugs are assumed or identified as the main cause of SJS/TEN in most cases, but Mycoplasma pneumoniae and Herpes simplex virus infections are well documented causes alongside rare cases in which the aetiology remains unknown. Several drugs are at "high" risk of inducing TEN/SJS including: Allopurinol, Trimethoprim-sulfamethoxazole and other sulfonamide-antibiotics, aminopenicillins, cephalosporins, quinolones, carbamazepine, phenytoin, phenobarbital and NSAID's of the oxicam-type. Genetic susceptibility to SJS and TEN is likely as exemplified by the strong association observed in Han Chinese between a genetic marker, the human leukocyte antigen HLA-B*1502, and SJS induced by carbamazepine. Diagnosis relies mainly on clinical signs together with the histological analysis of a skin biopsy showing typical full-thickness epidermal necrolysis due to extensive keratinocyte apoptosis. Differential diagnosis includes linear IgA dermatosis and paraneoplastic pemphigus, pemphigus vulgaris and bullous pemphigoid, acute generalized exanthematous pustulosis (AGEP), disseminated fixed bullous drug eruption and staphyloccocal scalded skin syndrome (SSSS). Due to the high risk of mortality, management of patients with SJS/TEN requires rapid diagnosis, evaluation of the prognosis using SCORTEN, identification and interruption of the culprit drug, specialized supportive care ideally in an intensive care unit, and consideration of immunomodulating agents such as high-dose intravenous immunoglobulin therapy. SJS and TEN are severe and life-threatening. The average reported mortality rate of SJS is 1-5%, and of TEN is 25-35%; it can be even higher in elderly patients and those with a large surface area of epidermal detachment. More than 50% of patients surviving TEN suffer from long-term sequelae of the disease.

Stevens-Johnson syndrome (SJS) is considered as serious mucocutaneous autoimmune disease which is potentially fatal. It may be initiated by medical treatment or infection and sometimes both may be triggering factors. Skin reactions involving loss of skin and mucus membrane and in severe cases, systemic symptoms may be appeared in SJS. In most of the cases medication is the causative factor. Toxic Epidermal Necrolysis (TEN) is a severe form of SJS, as it involves more than 30% of the skin surface with extensive damage to the mucous membranes. SJS and TEN are distinguished based on the extent of the detached skin surface area. It starts with flu-like symptoms, rashes with pain; the upper layer of affected skin dies, sheds and begins to heal after several days. In severe cases, it may leads to life threatening condition. Here we are presenting drug induced SJS in three different cases with different drugs. In first case, a 40-years male treated with Phenytoin 400mg for epilepsy, in second case, a 50-years male was given Amoxicillin + clavulanic acid-625 for some skin lesions and in third case, 50-years old female was given Methotrexate 7.5mg for Rheumatoid arthritis. In all the three cases, SJS symptoms were started within a period of 5-10days. All the symptoms and reactions were noticed and assessed for the confirmations of SJS.

THE USE OF PHOTOBIOMODULATION IN ORAL LESIONS IN A PATIENT WITH STEVENS-JOHNSON SYNDROME

ABSTRACTIntroduction: Stevens-Johnson Syndrome (SJS) is an acute hypersensitivity reaction that manifests on the skin, oral mucosa, ocular, gastrointestinal, genital and anal area. It is also potentially life-threatening in concern of dehydration and infection. Oral mucosal lesions due to SJS resulted in a significant decrease of patient’s quality of life. When the oral mucosa involved, the intake of nutrients and fluids is disrupted contributing to electrolyte imbalance that aggravates dehydration. Moreover, oral mucosal lesions have become an entry point for infection. Purpose: This case report describes the important role of oral medicine specialists in the management of oral mucosal lesions in SJS patient. Review: A 26-year-old female patient was referred from the Department of Dermatology and Venereology with a diagnosis of SJS et causa suspected paracetamol and/or amoxycillin. The complaints comprised of pain on the lips and oral cavity, difficulty in mouth opening, and pain when swallowing. The management for oral lesions included: history taking, external and intra oral examinations, dexamethasone mouthwash, nystatin oral suspension, and sodium chloride (NaCl) 0.9% solution. The patient showed improvement in oral mucosal lesions within 3 weeks of treatment that was provided by oral medicine specialist and medical team collaboration. Conclusion: Based on this case report, the role of oral medicine specialist is very important as part of the management team for SJS patient. Oral medicine specialist can reduce morbidity that results from oral mucosal involvement. Collaboration with oral medicine specialist since the beginning of treatment is the key to success in SJS management. Keywords: Oral medicine specialist, Oral mucosal lesion, Stevens-Johnson Syndrome.

Stevens Johnson Syndrome is a rare autoimmune disorder which includes skin and mucous membrane. In India, the incidence of Stevens Johnson Syndrome (SJS) is 1.2 to 6 million patients per year. SJS is a very serious and life-threatening hypersensitivity reaction that can occurs due to infections (mycoplasma pneumonia) or as side effects of drugs (Sulfa Drugs, Phenytoin, Carbamazepine, Lamotrigine, Phenobarbital, Allopurinol, Piroxicam, Nevirapine and Diclofenac). Antibiotics can cause SJS and their contribution is around 40%. Fluoroquinolones are prescribed globally (11%) to treat lower respiratory tract infections, gastrointestinal and genitourinary infections. Norfloxacin and Ciprofloxacin are rarely associated with drug induced SJS. Most of the informative data, available on drugs induced SJS are based on case reports or case series. Here, we present three case reports of Fluoroquinolones induced SJS. In the following cases, patients had developed symptoms of SJS within two days. Whereas in Antibiotic induced SJS, it is reported that symptoms of SJS can appear within few days or even after a single dose of taking antibiotics. Therefore, we alleged that Fluoroquinolone could be the possible causative agent in our cases. The causality assessment had done based on the WHO-UMC causality scale and it was probable in all three cases of Fluoroquinolone induced SJS. This assessment generates a strong evidence that Fluoroquinolone induced SJS in all three cases. These patients were treated symptomatically with corticosteroids, parenteral solution and other non-pharmacologic agents and discharged after complete recovery.

Erythema Multiforme (EM) is an acute inflammatory disease of the skin and mucous membranes that causes a variety of skin lesions—hence the name “multiforme.” The oral lesions, typically inflammatory, are accompanied by rapidly rupturing vesicles and bullae. EM has several clinical presentations: from milder self-limiting form to severe life-threatening form that may present as either Stevens-Johnson syndrome or Toxic Epidermal Necrolysis (TEN).1 The most common triggers for episodes of EM are herpes simplex virus and drug reactions.2 EM includes both generalized symptoms such as fever and malaise and oral lesions starting as bullae on an erythematous base which break rapidly into irregular ulcers.1 Analysis of the dental literature indicated limited evidence about the use of dental removable prosthesis in oral lesions manifested by EM.3,4 This case report describes a multi-disciplinary approach for the treatment of erythema multiforme with the use of the intra-oral dental prosthetic device.

Stevens-Johnson syndrome (SJS) is a severe type of pleomorphic erythema and a rare disorder of the skin and mucous membranes, which can lead to serious infections, pulmonary embolism, acute respiratory distress syndrome, multiple organ dysfunction syndrome, and other serious consequences. Patients with SJS are usually treated in burn centers. SJS complicated by severe burns is very rare, and this is associated with a high risk of infection and other more serious complications. With SJS, the availability of donor sites is compromised given the lack of healthy epidermis, and this makes it more difficult to treat. The patient was a 52-year-old man with 45% TBSA burns with 40% TBSA full-thickness burns on both lower limbs. During treatment, his condition was complicated by SJS, renal failure, and respiratory failure. After 31 days, he was transferred to our department. On the 22nd day, the patient recovered from SJS, and after undergoing four skin grafting procedures, the burn wounds healed, and the donor site had healed spontaneously. He was discharged after 86 days of treatment in our department. In conclusion, major burns complicated with SJS are rare clinical presentations. The skin affected by the drug eruptions can be used as a donor site for transplantation to the burn wounds, and this donor area can also heal.

Case Reports1 February 1947STEVENS-JOHNSON SYNDROME, A VARIATION OF ERYTHEMA MULTIFORME EXSUDATIVUM (HEBRA): A REPORT OF TWO CASESHENRY S. WENTZ, M.D., HARVEY H. SEIPLE, M.D., F.A.C.P.HENRY S. WENTZ, M.D.Search for more papers by this author, HARVEY H. SEIPLE, M.D., F.A.C.P.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-26-2-277 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptTwo cases are presented—the first a syndrome of ulceromembranous stomatitis, purulent conjunctivitis, urethritis, and signs of constitutional toxicity; the second an eruptive bullous type febrile disease associated with stomatitis and ophthalmia. Both cases conform to the picture described by Stevens and Johnson. Stevens and Johnson3 in 1922 felt that their syndrome of an eruptive febrile disease associated with stomatitis and purulent conjunctivitis was a distinct clinical entity. Controversy exists, however, as to whether the syndrome should be differentiated from erythema multiforme exsudativum.Fletcher and Harris1in 1945 and Keil2in 1940 have presented excellent historical reviews. Erythemas were first described...Bibliography1. FLETCHERHARRIS MWRC: Erythema exsudativum multiforme (Hebra)—bullous type, Jr. Pediat., 1945, xxvii, 465-478. CrossrefGoogle Scholar2. KEIL H: Erythema multiforme exsudativum (Hebra), a clinical entity associated with systemic features, Ann. Int. Med., 1940, xiv, 450. Google Scholar3. STEVENSJOHNSON AMFC: A new eruptive fever associated with stomatitis and ophthalmia: report of two cases in children, Am. Jr. Dis. Child., 1922, xxiv, 526-533. CrossrefGoogle Scholar4. KOKE MP: Conjunctivitis in erythema exsudativum multiforme, Arch. Ophth., 1941, xxv, 78-88. CrossrefGoogle Scholar5. WHEELER JM: Destructive purulent ophthalmia accompanying an eruptive fever with stomatitis, Am. Jr. Ophth., 1930, xiii, 508-514. CrossrefGoogle Scholar6. BAILEY JH: Lesions of the cornea and conjunctiva in erythema exsudativum multiforme (Hebra), Arch. Ophth., 1931, vi, 362-379. CrossrefGoogle Scholar7. RUTHERFORD CW: Membranous conjunctivitis with loss of eyeballs, paper read before Sec. on Ophth., Am. Med. Assoc., July 8-12, 1929. Google Scholar8. GINANDES GJ: Eruptive fever with stomatitis and ophthalmia; atypical erythema exsudativum multiforme (Stevens-Johnson), Am. Jr. Dis. Child., 1935, xlix, 1148-1160. CrossrefGoogle Scholar9. EDGARSYVERTONSYVERTON KJJTE: Erythema multiforme with ophthalmia and stomatitis, Jr. Pediat., 1938, xii, 151-159. CrossrefGoogle Scholar10. CHICKWITZBERGER FECM: Erythema multiforme exsudativum accompanying oral Vincent's infection, Am. Jr. Dis. Child., 1938, lv, 573-578. Google Scholar11. LEVER WF: Severe erythema multiforme, Arch. Dermat. and Syph., 1944, xlix, 47-56. CrossrefGoogle Scholar12. ROSENBERGROSENBERG LJ: Erythema exsudativum multiforme (Hebra) with conjunctivitis and stomatitis, Arch. Dermat. and Syph., 1940, xli, 1066-1072. CrossrefGoogle Scholar13. GIVNERAGELOFF IH: Stevens Johnson disease with complete visual recovery, New York State Jr. Med., 1941, xli, 1762-1765. Google Scholar14. AGELOFF H: Erythema multiforme bullosum with involvement of the mucous membranes of the eyes and mouth (Stevens Johnson disease), New England Jr. Med., 1940, ccxxiii, 217-219. CrossrefGoogle Scholar15. MURPHY RC: An eruptive fever involving the mouth and eyes (Stevens Johnson disease), New England Jr. Med., 1942, ccxxx, 69-71. Google Scholar16. LEVY AT: Erythema multiforme bullosum with involvement of the mucous membranes of the mouth (Stevens Johnson disease), Jr. Am. Dent. Assoc., 1945, xxx. Google Scholar17. KOVE S: Stevens Johnson syndrome (eruptive fever with stomatitis and conjunctivitis), Am. Jr. Med. Sci., 1945, ccx, 611-623. CrossrefGoogle Scholar18. ERGER BD: Erythema multiforme pluriorificialis (Stevens Johnson disease), Mil. Surg., 1944, xcv, 308-312. Google Scholar19. MCCARTHY L: Histopathology of skin diseases, 1931, C. V. Mosby Co., St. Louis, p. 137. Google Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: Lancaster, Pennsylvania*Received for publication March 28, 1946. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byThe Nail in Dermatological DiseaseDiseases of the Nails in ChildrenBlindness, Anonychia, and Oral Mucosal Scarring as Sequelae of the Stevens-Johnson SyndromeDrugs as etiologic factors in the Stevens-Johnson syndromePain in the Extremities, Dysesthesia, PolyalgiaDas Stevens-Johnson-Syndrom.ERYTHEMA MULTIFORME EXUDATIVUM WITH MUCOUS MEMBRANE INVOLVEMENT: WITH A REPORT OF THREE CASESSTEVENS-JOHNSON SYNDROME TREATED WITH A.C.T.HThe Ocular Complications of Erythema Exudativum Multiforme*Further Studies of Stevens-Johnson's Disease*ERYTHEMA MULTIFORME EXUDATIVUM (THE STEVENS-JOHNSON SYNDROME)The diagnosis of pemphigus by its oral signsErythema multiforme exudativum:Stevens-Johnson syndromePneumonia and erythema multiforme exudativumAn unusual eruptive fever involving the skin and mucous membranesAn Eruptive Fever Involving the Skin and Mucous Membranes (Stevens–Johnson Disease) 1 February 1947Volume 26, Issue 2Page: 277-283KeywordsConjunctivitisErythemaToxicityUlcers ePublished: 1 December 2008 Issue Published: 1 February 1947 PDF downloadLoading ...

Fever, Rash, and Systemic Symptoms: Understanding the Role of Virus and HLA in Severe Cutaneous Drug Allergy

Stevens-Johnson syndrome (SJS) is a rare disease of the skin and mucous membrane. It develops as a result of a sudden skin reaction, which is most often triggered by the use of certain drugs. It is characterized by acute epidermal necrosis. The syndrome can lead to many complications and death. Currently, there are no guidelines and management schematics for this disease. Treatment has changed over the years due to the discovery of the possible pathomechanism of SJS. Nevertheless, because of the rarity of the disease, there is a lack of adequate prospective randomized studies that could provide valuable information on treatment. Objective: The purpose of this study was to present Stevens-Johnson syndrome, including clinical features, etiology, pathomechanism, complications and treatment. Methods: A literature search was performed in the PubMed medical publication database using the following keywords: Stevens-Johnson syndrome, acute drug-induced skin reaction, cutaneous adverse drug reactions. Results: Stevens-Johnson syndrome is an example of an acute cutaneous reaction to certain medicinal substances. The pathomechanism is not fully understood. There is an association of genetics with the risk of developing symptoms of the syndrome in selected populations. Treatment formerly was based on glucocorticosteroids, but now IVIG (intravenous immune globulin) is used. Dehydration, pneumonia or sepsis may develop as a complication of the syndrome. Conclusions: The discovery of a genetic predisposition to develop Stevens-Johnson syndrome offers the possibility of future effective disease prevention. It is necessary to create medical procedure schemes for the diagnosis and treatment of this disorder. The discovery of the possible pathomechanism has allowed the use of IVIG in the treatment of the syndrome. Effective and prompt diagnosis and treatment can prevent life-threatening complications and death from SJS.

Systemic lupus erythematosus (SLE) is a systemic disease that affects many organs. A few patients with SLE develop Stevens–Johnson syndrome (SJS), a life-threatening disease characterized by the appearance of a partial-thickness burn in the skin and mucous membranes. This report aims to increase awareness among clinicians about the relationship between SLE and SJS. An 18-year-old man was admitted to the rheumatology department of Omdurman Military Hospital with a skin rash that was preceded by symptoms of a short febrile illness. He had a maculopapular rash on his palms, soles, trunk, and mucous membranes. The patient had been diagnosed with SLE at 10 years of age and had had SJS three times since the diagnosis of SLE. Investigations to exclude other diagnoses were conducted, and a skin biopsy showed features consistent with early SJS. The patient received intravenous hydrocortisone, oral prednisolone, and oral acyclovir. The lesions resolved 3 weeks after treatment with acyclovir and he was discharged in good condition. A young patient with SLE and recurrent SJS with no immunodeficiency responded very well to the conventional SJS therapy after 3 weeks of treatment.

HLA Typing in Patients with Ocular Manifestations of Stevens-Johnson Syndrome

Stevens-Johnson syndrome (SJS) is an infrequent, multisystemic, fatal, immune-mediated hypersensitivity reaction. SJS may be due to adverse effects of drug intake often characterized by mucocutaneous rash, bullae, and blisters spread over the skin and mucous membranes, hyperpigmentation, puffiness, erosive lesions on lips and face. The most common cause of drug-induced SJS is antimicrobials, followed by NSAIDs, allopurinol, antipsychotics, and antiepileptic drugs. Two cases of atypical SJS presentation associated with the use of theophylline and meloxicam are reported here. Early identification and appropriate corticosteroid therapy might improve the condition. The reason for publishing these case reports is to raise an alarm among our health care fraternity and common man regarding medication-induced SJS, which may be dreadful especially due to theophylline used in bronchial asthma and meloxicam used for osteoarthritis and thereby preventing the expected serious sequelae in SJS.