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Re: Stevens Johnson SynFrom: ainsron (ainsron@sbcglobal.net)Fri Sep 23 10:57:01 2005
Background: In 1922, Stevens and Johnson first described 2 patients, boys aged 7 and 8 years, with “an extraordinary, generalized eruption with continued fever, inflamed buccal mucosa, and severe purulent conjunctivitis.” Both cases were misdiagnosed by primary care physicians as hemorrhagic measles. Erythema multiforme (EM), originally described by von Hebra in 1866, was part of the differential diagnosis in both cases, but it was excluded because of the “character of skin lesions, the lack of subjective symptoms, the prolonged high fever, and the terminal heavy crusting.” In spite of leukopenia in both cases, Stevens and Johnson in their initial report suspected an infectious disease of unknown etiology as the cause. In 1950, Thomas divided EM into 2 categories, as follows: erythema multiforme minor (von Hebra) and erythema multiforme major (EMM; also known as Stevens-Johnson syndrome, or SJS). Since 1983, the eponym of Stevens-Johnson syndrome had been used as a synonym for EMM. In 1993 and 1994, Bastuji and Roujeau, respectively, proposed that EMM and SJS are 2 distinct disorders. They suggested that the denomination of EM should be restricted to patients with typical targets or raised edematous papules, with or without mucosal involvement. This clinical picture is in accordance with the original description by von Hebra. They further proposed that the denomination of SJS should be used for a syndrome characterized by mucous membrane erosions and widespread small blisters that arise on erythematous or purpuric maculae that are different from classic targets. According to this clinical classification, EMM and SJS could be 2 distinct disorders with similar mucosal erosions, but different patterns of cutaneous lesions. This hypothesis is supported further by a strong correlation between clinical classification and the probable cause. Conversely, several investigators propose that SJS and toxic epidermal necrolysis (TEN) are the same diseases of various severities. It has been suggested to use a unifying classification of “acute disseminated epidermal necrosis” or “exanthematic necrolysis.” A very strong argument against this unifying concept was that infection with herpes simplex virus (HSV) had been described as a frequent cause of SJS/EMM but not of TEN. However, recent reports showed that HSV infection has not been related to SJS and they do suggest that SJS and TEN, based on clinical manifestations and pathology results, are severity variants of the same disease, which differ from EM. SJS and TEN are characterized by identical clinical signs and symptoms, identical treatment approach, and identical prognosis. Patients with 90% skin detachment and diagnosed with TEN may have none or only mild ocular involvement with excellent prognosis quod visum, and patients with 10% skin detachment may have severe ocular involvement with blinding consequences, and vice versa. Pathophysiology: Delayed hypersensitivity reaction to drugs has been described in patients with SJS. The lack of lymphocyte response to native drug and positive patch test suggests that the immune response may be directed at drug-modified epidermal cells. Patients after drug hypersensitivity reactions can be distinguished from controls by exposure of their lymphocytes to oxidative drug metabolites generated by a murine hepatic microsomal system in vitro. It has been suggested that probably both immune hypersensitivity reactions and reactions mediated by toxic intermediates, generated as a result of impaired or altered drug metabolism, are involved in the pathogenesis of SJS. The defect in metabolism is highly specific for drugs, eg, for sulfonamides, the toxic-reactive metabolite has been identified as hydroxylamine, and for anticonvulsants and amineptin, the toxic-reactive metabolite seems to be an arene oxide. Deficiency of glutathione transferase, which is present in 50% of the population, and additional factors, particularly a qualitative deficiency of some other enzymes, may play a role in the pathogenesis of SJS. There is nothing in the description of the disease or pathophysiology that would make me worried about HRT being a major risk factor in these patients. Admittedly, like Efrain, I've not had any personal experience with it. Ronald E. Ainsworth, MD, FACOG -----Original Message----- From: ob-gyn-l@obgyn.net [mailto:ob-gyn-l@obgyn.net] On Behalf Of Karen Lee Sent: Thursday, September 22, 2005 4:05 PM To: Multiple recipients of list OB-GYN-L Subject: Stevens Johnson Syn Listers... I have a new 57 yo with severe Sx of menopause. She would like help...but, she has had Stevens Johnson Syndrome from Sulfa and two of her children have suffered the same, one due to NSAIDS, not sure about the other. What reading I have done has not helped me. (yes I feel dumb sometimes) I can't get a handle on whether she might be able to use estradiol/prometrium or perhaps Climara. Anyone have a thought about this? Karen Lee, ARNP OB/GYN Nurse Practitioner Certified Menopause Practitioner Mount Vernon, WA http://www.menopausematters.org "Courage is the opposite of the instinct to keep everything under control. It is against inertia and death, toward intensity and life. The little courages of life add up to big courage over time. Resist the offers of small safe quarters in your mind." *****HIPAA Confidentiality Notice***** The documents inside this electronic transmission contain confidential information belonging to the sender that is legally privileged. If you received this electronic transmission in error, please notify the sender immediately to arrange for return.
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