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stevens - johnson syndrome ( sjs ) is an acute hypersensitivity reaction involving the skin and mucous membranes , first described in 1922 by stevens and johnson1 in two children with purpuric skin macules , stomatitis associated with widespread necrosis of the oral and peri - oral mucosa and severe purulent conjunctivitis .
the extent of skin / mucosal involvement distinguishes sjs from erythema multiformis ( em ) , which has the same etiology , but the lesions ( small erythematous areas ) are confined to the skin only .
these particular manifestations are often observed after bacterial or viral infections , as well as after drug use .
sjs is more severe than em , having a protracted course that in the worst cases may lead to death ( 5% of cases ) .
mortality is considerably higher ( up to 70% of cases ) when more than 30% of the body surface area is involved .
another variant was first described in 1956 by lyell2 , who described four patients with skin lesions resembling burns , dubbed toxic epidermal necrolysis ( ten)3 . in the years after publication of lyell 's article , many cases of ten were reported and it became clear that ten could be induced by the same etiological agents as em , namely infection and/or drug use .
the drugs most commonly implicated are antibacterial agents ( macrolides , cephalosporin , tetracyclin , sulfamides ) , antiepileptics ( lamotrigine , carbamazepin ) , gout medication ( allopurinol ) and anti - retroviral agents ( nevirapin ) .
sjs and ten are considered variants of severe epidermolytic reactions that differ in the degree of involvement of the skin surface .
sjs is associated with epidermal detachment of < 10% of the skin surface , whereas ten is diagnosed when > 30% of the skin surface is involved .
forms with skin detachment of 10% to 30% of the body surface are regarded as overlapping forms4 .
the degree of skin involvement is a major prognostic factor ; only necrotic or already detached skin ( boils , erosions ) and skin positive for nikolsky sign should be counted when assessing the body area involved .
the initial symptoms of sjs are almost always aspecific : fever , myalgia , arthralgia and headache , then sore throat , odynophagia and burning eyes , followed several days later by early skin lesions in the form of erythematous rosettes .
these may become necrotic , forming erosions , ulcers and large areas of epidermal detachment ( severe skin manifestations ) . in the absence of epidermal detachment
this sign is not specific for ten / sjs as may also be positive in cases of bullous skin diseases .
the presternal region of the chest and the face as well as palmar and plantar regions are usually the first affected .
oral mucosal , ocular and/or genital involvement occurs in 90% of patients . in some cases
eye involvement is frequent and includes palpebral edema , conjunctivitis , lacrimation , corneal ulcers with pseudomembranes and crusts and purulent eye secretions , which in severe cases may lead to scarring ( synechia ) .
however , the severity of acute eye manifestations is not usually predictive of later eye complications5 .
herein we report a case of sjs in a healthy young adult who regularly abused anabolic steroids for competitive bodybuilding .
a 41-year - old male presented at the emergency department with a burning sensation of the mouth and eyes , pharyngodynia , weakness , fever , tachycardia , headache , cough , and generalized malaise .
the patient had participated in competitive body - building for approximately 20 years , trained daily and regularly took doses of anabolic androgenic steroids ( aas ) .
twelve hours after the last injection of steroids ( drostanolone propionate , stanazol , and metenolone enantate ) , he experienced rapid deterioration : fever and erythematous rosettes appeared initially around the injection site and almost simultaneously on the palmar and plantar regions , later spreading to the trunk and the base of the lower limbs .
painful erythematous maculopapular and vesicular lesions appeared all over the body , including the genitals and oral mucosa .
fibroscopic examination revealed extensive involvement of the nasal mucosa ( ulcerations and crusts ) and also of the oropharyngeal mucosa ( pseudomembranes ) , whereas the laryngeal area was spared.(fig . 1 , 2 ) chest radiography and microbiological analysis excluded mycoplasma pneumoniae infection . during hospitalization , the patient developed sudden cardiac arrhythmia with atrial fibrillation .
initial attempts to regularize heart rate with intravenous ( iv ) amiodarone ( 150 mg in 100 ml glucosate in 2 hours , followed by a further 900 mg in 24 hours ) failed .
the patient was also treated with systemic glucocorticoids ( iv 40 mg methylprednisolone in 100 ml 5% glucosate every 24 hours for 6 days , then 20 mg methylprednisolone for 5 days , and finally 10 mg methylprednisolone for 4 days ) , an antithrombotic ( subcutaneous enoxaparin 4,000 iu / day for 1 month ) and was administered enteral nutrition through a nasogastric tube . for oral hygiene , mouthwash containing chlorhexidine digluconate
his condition slowly improved and he was discharged on day 23 in good general health . only minor conjunctival ulcers , which were treated by specialists after discharge , persisted .
sjs may be triggered by numerous agents , particularly bacteria and viruses , immune conditions , non - infectious agents such as food additives or chemicals ( benzoates and nitrobenzene ) and drugs6 .
other pathogens associated with ssj include mycobacterium tuberculosis , group a streptococcus , hepatitis b virus , epstein - barr virus , enteroviruses and yesinia enterocolitica7 . in our case ,
all serological tests excluded an infectious cause.(table 1 ) drug ingestion tends to trigger sjs , including of antibacterial agents ( macrolides , cephalosporin , tetracycline , sulfamides ) , antiepileptics ( lamotrigine , carbamazepin ) , gout medication ( allopurinol ) , anti - retroviral agents ( nevirapin ) and , less frequently , nonsteroidal anti - inflammatory drugs ( ketoprofen , ibuprofen ) .
aas were created in 1930 for hormone replacement in a patient with hypogonadism , impotence , infertility , growth retardation , cryptorchidism and eunuchoidism .
nowadays , however , many athletes , especially body - builders , exploit aas to promote muscle development . the most widely used molecules are testosterone , nandrolone , metandrostenolone , and stanazol8910 .
the most common clinical patterns are sjs ( 32% ) , exanthematous drug eruptions ( 24.5% ) , and ten ( 11%)11 . the pathogenic mechanism of sjs is tissue damage caused by precipitation of soluble immune complexes , which are produced rapidly and overabundantly in the peripheral circulation .
the mechanism of excess production of antibodies to viral , bacterial and pharmacological antigens may be related to genetic factors , but is not yet clear .
an important association between human leucocyte antigen ( hla ) and carbamazepin was discovered by chung et al.12 for the genotype hla - b 1502 .
another association between the hla genotype and sjs / ten was reported by the same author for allopurinol : 100% of han chinese , an ethnic group native to east asia , experienced adverse reactions to allopurinol , and all were hla - b5801 positive13 . to minimize the risk of developing tolerance , athletes take a cocktail of aas14
. we could not determine the dose of aas because our patient was taking a cocktail of different molecules purchased on the internet .
the initial symptoms of sjs are almost always aspecific and the clinical presentation may mimic different oral inflammatory diseases .
sjs can be difficult to differentiate from vesciculobullous disorders such as bullous autoimmune diseases ( pemphigus / pemphigoid ) , viral stomatitides , behet syndrome , crohn 's disease or staphylococcal scalded skin syndrome15 .
features more suggestive of sjs are : 
 - acute onset and rapidly worsening;- blistering and ulceration of the lips , appearing as crusted lips;- pleomorphic skin and other lesions . 
  - acute onset and rapidly worsening ; - blistering and ulceration of the lips , appearing as crusted lips ; - pleomorphic skin and other lesions .
the acute and/or chronic effects of aas depend on androgen type , dose , duration of treatment , sex and individual genetic susceptibility .
abuse of aas has also been linked to arrhythmia and sudden cardiac death ; considering this , the atrial fibrillation observed in our patient may have been related to aas use18 . employing the naranjo adverse drug reaction ( adr ) probability scale ,
there are no specific diagnostic tests for sjs , but the diagnosis is supported by tissue biopsy and exclusion of other causes through microbial cultures from blood or serum ( for example herpes simplex virus and mycoplasma serology ) .
furthermore , histologic examination and immunostaining often show intraepithelial edema and spongiosis early on , with satellite cell necrosis and vacuolar degeneration of the basement membrane zone15 . in cases of sjs ,
sjs is a life - threatening condition very similar to burns : supportive care ( enteral nutrition via nasogastric tube and iv glucosate ) is an essential part of therapy because the patient can experience rapid dehydration .
systemic corticosteroids are the standard treatment for limiting the inflammatory response , but in patient with a history of infectious diseases , there are associated risks . there is insufficient evidence to recommend the routine use of high - dose iv immunoglobulins .
stevens - johnson syndrome ( sjs ) is characterized by mucocutaneous tenderness and typical hemorrhagic erosions , erythema and epidermal detachment presenting as blisters and areas of denuded skin . 
 sjs is often observed after drug use as well as after bacterial or viral infections . 
 several drugs are at high risk of inducing sjs , but there are no cases in the english literature regarding anabolic steroid use triggering sjs . in our paper , we describe a case in which use of anabolic androgenic steroids ( aas ) was associated with sjs . 
 the patient participated in competitive body - building and regularly took variable doses of aas . 
 initial symptoms ( headache , weakness , pharyngodynia , and fever ) were ignored . after a week he presented to the emergency department with a burning sensation on the mouth , lips , and eyes . 
 painful , erythematous , maculopapular , and vesicular lesions appeared all over the body , including on the genitals . during hospitalization 
 , he also developed a cardiac complication . 
 the patient had not taken any drugs except aas .