HEPATITIS B

• Hepatitis B is a viral infection that attacks the liver and can cause both acute and chronic disease.
• The virus is transmitted through contact with the blood or other body fluids of an infected person - not through casual contact.
• About 2 billion people worldwide have been infected with the virus and about 350 million live with chronic infection. An estimated 600 000 persons die each year due to the acute or chronic consequences of hepatitis B.
• About 25% of adults who become chronically infected during childhood later die from liver cancer or cirrhosis (scarring of the liver) caused by the chronic infection.
• The hepatitis B virus is 50 to 100 times more infectious than HIV.
• Hepatitis B virus is an important occupational hazard for health workers.
• Hepatitis B is preventable with a safe and effective vaccine.


Hepatitis B is a potentially life-threatening liver infection caused by the hepatitis B virus. It is a major global health problem and the most serious type of viral hepatitis. It can cause chronic liver disease and puts people at high risk of death from cirrhosis of the liver and liver cancer.

Worldwide, an estimated two billion people have been infected with the hepatitis B virus (HBV), and more than 350 million have chronic (long-term) liver infections.

A vaccine against hepatitis B has been available since 1982. Hepatitis B vaccine is 95% effective in preventing HBV infection and its chronic consequences, and is the first vaccine against a major human cancer.

Symptoms
Hepatitis B virus can cause an acute illness with symptoms that last several weeks, including yellowing of the skin and eyes (jaundice), dark urine, extreme fatigue, nausea, vomiting and abdominal pain. People can take several months to a year to recover from the symptoms. HBV can also cause a chronic liver infection that can later develop into cirrhosis of the liver or liver cancer.

Who is most at risk for chronic disease?
The likelihood that an HBV infection will become chronic depends upon the age at which a person becomes infected, with young children who become infected with HBV being the most likely to develop chronic infections. About 90% of infants infected during the first year of life develop chronic infections; 30% to 50% of children infected between one to four years of age develop chronic infections. About 25% of adults who become chronically infected during childhood die from HBV-related liver cancer or cirrhosis.

About 90% of healthy adults who are infected with HBV will recover and be completely rid of the virus within six months.

Where is hepatitis B most common?
Hepatitis B is endemic in China and other parts of Asia. Most people in the region become infected with HBV during childhood. In these regions, 8% to 10% of the adult population are chronically infected. Liver cancer caused by HBV is among the first three causes of death from cancer in men, and a major cause of cancer in women. High rates of chronic infections are also found in the Amazon and the southern parts of eastern and central Europe. In the Middle East and Indian sub-continent, an estimated 2% to 5% of the general population is chronically infected. Less than 1% of the population in western Europe and North American is chronically infected.

Transmission
Hepatitis B virus is transmitted between people by contact with the blood or other body fluids (i.e. semen and vaginal fluid) of an infected person. Modes of transmission are the same for the human immunodeficiency virus (HIV), but HBV is 50 to 100 times more infectious Unlike HIV, HBV can survive outside the body for at least 7 days. During that time, the virus can still cause infection if it enters the body of a person who is not infected.

Common modes of transmission in developing countries are:

• perinatal (from mother to baby at birth)
• early childhood infections (inapparent infection through close interpersonal contact with infected household contacts)
• unsafe injections practices
• blood transfusions
• sexual contact
In many developed countries (e.g. those in western Europe and North America), patterns of transmission are different than those mentioned above. Today, the majority of infections in these countries are transmitted during young adulthood by sexual activity and injecting drug use. HBV is a major infectious occupational hazard of health workers.

HBV is not spread by contaminated food or water, and cannot be spread casually in the workplace.

The virus incubation period is 90 days on average, but can vary from about 30 to 180 days. HBV may be detected 30 to 60 days after infection and persist for widely variable periods of time.

Treatment
There is no specific treatment for acute hepatitis B. Care is aimed at maintaining comfort and adequate nutritional balance, including replacement of fluids that are lost from vomiting and diarrhoea.

Chronic hepatitis B can be treated with drugs, including interferon and anti-viral agents, which can help some patients. Treatment can cost thousands of dollars per year and is not available to most patients in developing countries.

Liver cancer is almost always fatal, and often develops in people at an age when they are most productive and have family responsibilities. In developing countries, most people with liver cancer die within months of diagnosis. In higher income countries, surgery and chemotherapy can prolong life for up to a few years in some patients.

Patients with cirrhosis are sometimes given liver transplants, with varying success.

Prevention
All infants should receive the hepatitis B vaccine: this is the mainstay of hepatitis B prevention.

The vaccine can be given as either three or four separate doses, as part of existing routine immunization schedules. In areas where mother-to-infant spread of HBV is common, the first dose of vaccine should be given as soon as possible after birth (i.e. within 24 hours).

The complete vaccine series induces protective antibody levels in more than 95% of infants, children and young adults. After age 40, protection following the primary vaccination series drops below 90%. At 60 years old, protective antibody levels are achieved in only 65 to 75% of those vaccinated. Protection lasts at least 20 years and should be lifelong.

All children and adolescents younger than 18 years old and not previously vaccinated should receive the vaccine. People in high risk groups should also be vaccinated, including:

• persons with high-risk sexual behaviour;
• partners and household contacts of HBV infected persons;
• injecting drug users;
• persons who frequently require blood or blood products;
• recipients of solid organ transplantation;
• those at occupational risk of HBV infection, including health care workers; and
• international travellers to countries with high rates of HBV.
The vaccine has an outstanding record of safety and effectiveness. Since 1982, over one billion doses of hepatitis B vaccine have been used worldwide. In many countries where 8% to 15% of children used to become chronically infected with HBV, vaccination has reduced the rate of chronic infection to less than 1% among immunized children.

As of December 2006, 164 countries vaccinate infants against hepatitis B during national immunization programmes - a major increase compared with 31 countries in 1992, the year that the World Health Assembly passed a resolution to recommend global vaccination against hepatitis B.

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SYSTEMIC LUPUS ERITEMATOSUS

DEFINISI Lupus Eritematosus Sistemik (Lupus Eritematosus Disseminata, Lupus) adalah suatu penyakit autoimun menahun yang menimbulkan peradangan dan bisa menyerang berbagai organ tubuh, termasuk kulit, persendian dan organ dalam. Pada setiap penderita, peradangan akan mengenai jaringan dan organ yang berbeda. Beratnya penyakit bervariasi mulai dari penyakit yang ringan sampai penyakit yang menimbulkan kecacatan, tergantung dari jumlah dan jenis antibodi yang muncul dan organ yang terkena.

PENYEBAB Dalam keadaan normal, sistem kekebalan berfungsi mengendalikan pertahanan tubuh dalam melawan infeksi. Pada lupus dan penyakit autoimun lainnya, sistem pertahanan tubuh ini berbalik melawan tubuh, dimana antibodi yang dihasilkan menyerang sel tubuhnya sendiri. Antibodi ini menyerang sel darah, organ dan jaringan tubuh, sehingga terjadi penyakit menahun. Mekanisme maupun penyebab dari penyakit autoimun ini belum sepenuhnya dimengerti. Penyebab dari lupus tidak diketahui, tetapi diduga melibatkan faktor lingkungan dan keturunan. Beberapa faktor lingkungan yang dapat memicu timbulnya lupus: Infeksi Antibiotik (terutama golongan sulfa dan penisilin) Sinar ultraviolet Stres yang berlebihan Obat-obatan tertentu Hormon. Meskipun lupus diketahui merupakan penyakit keturunan, tetapi gen penyebabnya tidak diketahui. Penemuan terakhir menyebutkan tentang gen dari kromosom 1. Hanya 10% dari penderita yang memiliki kerabat (orang tua maupun saudara kandung) yang telah maupun akan menderita lupus. Statistik menunjukkan bahwa hanya sekitar 5% anak dari penderita lupus yang akan menderita penyakit ini. Lupus seringkali disebut sebagai penyakit wanita walaupun juga bisa diderita oleh pria. Lupus bisa menyerang usia berapapun, baik pada pria maupun wanita, meskipun 10-15 kali lebih sering ditemukan pada wanita. Faktor hormonal mungkin bisa menjelaskan mengapa lupus lebih sering menyerang wanita. Meningkatnya gejala penyakit ini pada masa sebelum menstruasi dan/atau selama kehamilan mendukung keyakinan bahwa hormon (terutama estrogen) mungkin berperan dalam timbulnya penyakit ini. Meskipun demikian, penyebab yang pasti dari lebih tingginya angka kejadian pada wanita dan pada masa pra-menstruasi, masih belum diketahui. Kadang-kadang obat jantung tertentu (hidralazin, prokainamid dan beta-bloker) dapat menyebabkan sindroma mirip lupus, yang akan menghilang bila pemakaian obat dihentikan.

GEJALA Jumlah dan jenis antibodi pada lupus, lebih besar dibandingkan dengan pada penyakit lain, dan antibodi ini (bersama dengan faktor lainnya yang tidak diketahui) menentukan gejala mana yang akan berkembang. Karena itu, gejala dan beratnya penyakit, bervariasi pada setiap penderita. Perjalanan penyakit ini bervariasi, mulai dari penyakit yang ringan sampai penyakit yang berat. Gejala pada setiap penderita berlainan, serta ditandai oleh masa bebas gejala (remisi) dan masa kekambuhan (eksaserbasi). Pada awal penyakit, lupus hanya menyerang satu organ, tetapi di kemudian hari akan melibatkan organ lainnya. Otot dan kerangka tubuh Hampir semua penderita lupus mengalami nyeri persendian dan kebanyakan menderita artritis. Persendian yang sering terkena adalah persendian pada jari tangan, tangan, pergelangan tangan dan lutut. Kematian jaringan pada tulang panggul dan bahu sering merupakan penyebab dari nyeri di daerah tersebut. Kulit Pada 50% penderita ditemukan ruam kupu-kupu pada tulang pipi dan pangkal hidung. Ruam ini biasanya akan semakin memburuk jika terkena sinar matahari. Ruam yang lebih tersebar bisa timbul di bagian tubuh lain yang terpapar oleh sinar matahari. Ginjal Sebagian besar penderita menunjukkan adanya penimbunan protein di dalam sel-sel ginjal, tetapi hanya 50% yang menderita nefritis lupus (peradangan ginjal yang menetap). Pada akhirnya bisa terjadi gagal ginjal sehingga penderita perlu menjalani dialisa atau pencangkokkan ginjal. Sistem saraf Kelainan saraf ditemukan pada 25% penderita lupus. Yang paling sering ditemukan adalah disfungsi mental yang sifatnya ringan, tetapi kelainan bisa terjadi pada bagian manapun dari otak, korda spinalis maupun sistem saraf. Kejang, psikosa, sindroma otak organik dan sakit kepala merupakan beberapa kelainan sistem saraf yang bisa terjadi. Darah Kelainan darah bisa ditemukan pada 85% penderita lupus. Bisa terbentuk bekuan darah di dalam vena maupun arteri, yang bisa menyebabkan stroke dan emboli paru. Jumlah trombosit berkurang dan tubuh membentuk antibodi yang melawan faktor pembekuan darah, yang bisa menyebabkan perdarahan yang berarti. Seringkali terjadi anemia akibat penyakit menahun. Jantung Peradangan berbagai bagian jantung bisa terjadi, seperti perikarditis, endokarditis maupun miokarditis. Nyeri dada dan aritmia bisa terjadi sebagai akibat dari keadaan tersebut. Paru-paru Pada lupus bisa terjadi pleuritis (peradangan selaput paru) dan efusi pleura (penimbunan cairan antara paru dan pembungkusnya). Akibat dari keadaan tersebut sering timbul nyeri dada dan sesak nafas. Gejala dari penyakit lupus: - demam - lelah - merasa tidak enak badan - penurunan berat badan - ruam kulit - ruam kupu-kupu - ruam kulit yang diperburuk oleh sinar matahari - sensitif terhadap sinar matahari - pembengkakan dan nyeri persendian - pembengkakan kelenjar - nyeri otot - mual dan muntah - nyeri dada pleuritik - kejang - psikosa. Gejala lainnya yang mungkin ditemukan: - hematuria (air kemih mengandung darah) - batuk darah - mimisan - gangguan menelan - bercak kulit - bintik merah di kulit - perubahan warna jari tangan bila ditekan - mati rasa dan kesemutan - luka di mulut - kerontokan rambut - nyeri perut - gangguan penglihatan.

DIAGNOSA Diagnosis lupus ditegakkan berdasarkan ditemukannya 4 dari 11 gejala lupus yang khas, yaitu: Ruam kupu-kupu pada wajah (pipi dan pangkal hidung) Ruam pada kulit Luka pada mulut (biasanya tidak menimbulkan nyeri) Cairan di sekitar paru-paru, jantung, dan organ lainnya Artritis (artritis non-erosif yang melibatkan 2 atau beberapa sendi perifer, dimana tulang di sekitar persendian tidak mengalami kerusakan) Kelainan fungsi ginjal - kadar protein dalam air kemih >0,5 mg/hari atau +++ - adanya elemen abnormal dalam air kemih yang berasal dari sel darah merah/putih maupuan sel tubulus ginjal Fotosensitivitas (peka terhadap sinar matahari, menyebabkan pembentukan atau semakin memburuknya ruam kulit) Kelainan fungsi saraf atau otak (kejang atau psikosa) Hasil pemeriksaan darah positif untuk antibodi antinuklear Kelainan imunologis (hasil positif pada tes anti-DNA rantai ganda, tes anti-Sm, tes antibodi antifosfolipid; hasil positif palsu untuk tes sifilis) Kelainan darah - Anemia hemolitik atau - Leukopenia (jumlah leukosit <4000> Pemeriksaan untuk menentukan adanya penyakit ini bervariasi, diantaranya: Pemeriksaan darah Pemeriksaan darah bisa menunjukkan adanya antibodi antinuklear, yang terdapat pada hampir semua penderita lupus. Tetapi antibodi ini juga juga bisa ditemukan pada penyakit lain. Karena itu jika menemukan antibodi antinuklear, harus dilakukan juga pemeriksaan untuk antibodi terhadap DNA rantai ganda. Kadar yang tinggi dari kedua antibodi ini hampir spesifik untuk lupus, tapi tidak semua penderita lupus memiliki antibodi ini. Pemeriksaan darah untuk mengukur kadar komplemen (protein yang berperan dalam sistem kekebalan) dan untuk menemukan antibodi lainnya, mungkin perlu dilakukan untuk memperkirakan aktivitas dan lamanya penyakit. Ruam kulit atau lesi yang khas Rontgen dada menunjukkan pleuritis atau perikarditis Pemeriksaan dada dengan bantuan stetoskop menunjukkan adanya gesekan pleura atau jantung Analisa air kemih menunjukkan adanya darah atau protein Hitung jenis darah menunjukkan adanya penurunan beberapa jenis sel darah Biopsi ginjal Pemeriksaan saraf.

PENGOBATAN Jika gejala lupus disebabkan karena obat, maka menghentikan penggunaan obat bisa menyembuhkannya, walaupun diperlukan waktu berbulan-bulan. Penyakit yang ringan (ruam, sakit kepala, demam, artritis, pleuritis, perikarditis) hanya memerlukan sedikit pengobatan. Untuk mengatasi artritis dan pleurisi diberikan obat anti peradangan non-steroid. Untuk mengatasi ruam kulit digunakan krim kortikosteroid. Untuk gejala kulit dan artritis kadang digunakan obat anti malaria (hydroxycloroquine). Jika penderita sangat sensitif terhadap sinar matahari, sebaiknya pada saat bepergian menggunakan tabir surya, pakaian panjang ataupun kacamata. Penyakit yang berat atau membahayakan jiwa penderitanya (anemia hemolitik, penyakit jantung atau paru yang meluas, penyakit ginjal, penyakit sistem saraf pusat) seringkali perlu ditangani oleh ahlinya. Untuk mengendalikan berbagai manifestasi dari penyakit yang berat mungkin bisa diberikan kortikosteroid atau obat penekan sistem kekebalan. Beberapa ahli memberikan obat sitotoksik (obat yang menghambat pertumbuhan sel) pada penderita yang tidak memberikan respon yang baik terhadap kortikosteroid atau yang tergantung kepada kortikosteroid dosis tinggi. PROGNOSIS Beberapa tahun terakhir ini prognosis penderita lupus semakin membaik, banyak penderita yang menunjukkan penyakit yang ringan. Wanita penderita lupus yang hamil dapat bertahan dengan aman sampai melahirkan bayi yang normal, tidak ditemukan penyakit ginjal ataupun jantung yang berat dan penyakitnya dapat dikendalikan. Angka harapan hidup 10 tahun meningkat sampai 85%. Prognosis yang paling buruk ditemukan pada penderita yang mengalami kelainan otak, paru-paru, jantung dan ginjal yang berat.

Stevens-Johnson sindrom

Stevens–Johnson syndrome (SJS) is a form of erythema multiforme which is a life-threatening condition affecting the skin in which cell death causes the epidermis to separate from the dermis. The syndrome is thought to be a hypersensitivity complex affecting the skin and the mucous membranes. Although the majority of cases are idiopathic, the main class of known causes is medications, followed by infections and (rarely) cancers

Classification

There is agreement in the medical literature that Stevens–Johnson syndrome (SJS) can be considered a milder form of toxic epidermal necrolysis (TEN). These conditions were first recognised in 1922. Both diseases can be mistaken for erythema multiforme. Erythema multiforme is sometimes caused by a reaction to a medication but is more often a type IV hypersensitivity reaction to an infection (caused most often by Herpes simplex) and is relatively benign. Although both SJS and TEN can also be caused by infections, they are most often adverse effects of medications. Their consequences are potentially more dangerous than those of erythema multiforme.

Signs and symptoms

SJS usually begins with fever, sore throat, and fatigue, which is misdiagnosed and usually treated with antibiotics. Ulcers and other lesions begin to appear in the mucous membranes, almost always in the mouth and lips but also in the genital and anal regions. Those in the mouth are usually extremely painful and reduce the patient's ability to eat or drink. Conjunctivitis of the eyes occurs in about 30% of children who develop SJS. A rash of round lesions about an inch across arises on the face, trunk, arms and legs, and soles of the feet, but usually not the scalp.

Causes

SJS is thought to arise due to a disorder of the immune system.

Infectious

It can be caused by infections (usually following infections such as herpes simplex virus, influenza, mumps, cat-scratch fever, histoplasmosis, Epstein-Barr virus, mycoplasma pneumoniae or similar).

Medication/drugs

It can be due to adverse effects of drugs (allopurinol, diclofenac, etravirine, Isotretinoin, aka Accutane, fluconazole, valdecoxib, sitagliptin, oseltamivir, penicillins, barbiturates, sulfonamides, phenytoin, azithromycin, modafinil, lamotrigine, nevirapine, pyrimethamine, ibuprofen, ethosuximide, carbamazepine and gout medications)

Although Stevens–Johnson Syndrome can be caused by viral infections, malignancies or severe allergic reactions to medication, the leading cause appears to be the use of antibiotics and sulfa drugs.

Medications that have traditionally been known to lead to SJS, erythema multiforme and toxic epidermal necrolysis include sulfonamides (antibiotics), penicillins (antibiotics), barbiturates (sedatives), lamotrigine and phenytoin (e.g. Dilantin) (anticonvulsants). Combining lamotrigine with sodium valproate increases the risk of SJS.

Non-steroidal anti-inflammatory drugs are a rare cause of SJS in adults; the risk is higher for older patients, women and those initiating treatment. Typically, the symptoms of drug-induced SJS arise within a week of starting the medication. People with systemic lupus erythematosus or HIV infections are more susceptible to drug-induced SJS.

SJS has also been consistently reported as an uncommon side effect of herbal supplements containing ginseng. SJS may also be caused by cocaine usage.

Genetics

In some East Asian populations studied (Han Chinese and Thai), carbamazepine- and phenytoin-induced SJS is strongly associated with HLA-B*1502 (HLA-B75), an HLA-B serotype of the broader serotype HLA-B15.^[10][11][12] A study in Europe suggested that the gene marker is only relevant for East Asians.^[13][14] Based on the Asian findings, similar studies were performed in Europe which showed 61% of allopurinol-induced SJS/TEN patients carried the HLA-B58 (B*5801 allele - phenotype frequency in Europeans is typically 3%). One study concluded "even when HLA-B alleles behave as strong risk factors, as for allopurinol, they are neither sufficient nor necessary to explain the disease."

Treatment

SJS constitutes a dermatological emergency. All medications should be discontinued, particularly those known to cause SJS reactions. Patients with documented mycoplasma infections can be treated with oral macrolide or oral doxycycline.

Initially, treatment is similar to that for patients with thermal burns, and continued care can only be supportive (e.g. intravenous fluids and nasogastric or parenteral feeding) and symptomatic (e.g. analgesic mouth rinse for mouth ulcer). Dermatologists and surgeons tend to disagree about whether the skin should be debrided.

Beyond this kind of supportive care, there is no accepted treatment for SJS. Treatment with corticosteroids is controversial. Early retrospective studies suggested that corticosteroids increased hospital stays and complication rates. There are no randomized trials of corticosteroids for SJS, and it can be managed successfully without them.

Other agents have been used, including cyclophosphamide and cyclosporine, but none have exhibited much therapeutic success. Intravenous immunoglobulin (IVIG) treatment has shown some promise in reducing the length of the reaction and improving symptoms. Other common supportive measures include the use of topical pain anesthetics and antiseptics, maintaining a warm environment, and intravenous analgesics. An ophthalmologist should be consulted immediately, as SJS frequently causes the formation of scar tissue inside the eyelids, leading to corneal vascularization, impaired vision and a host of other ocular problems. Also, an extensive physical therapy program ensues after the patient is discharged from the hospital.

Prognosis

SJS proper (with less than 10% of body surface area involved) has a mortality rate of around 5%. The risk for death can be estimated using the SCORTEN scale, which takes a number of prognostic indicators into account. Other outcomes include organ damage/failure, cornea scratching and blindness.

Epidemiology

Stevens-Johnson syndrome is a rare condition, with a reported incidence of around 2.6 to 6.1 cases per million people per year. In the United States, there are about 300 new diagnoses per year. The condition is more common in adults than in children. Women are affected more often than men, with cases occurring at a three to two sex ratio.

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• Typically, the disease process begins with a nonspecific upper respiratory tract infection.
  • This usually is part of a 1- to 14-day prodrome during which fever, sore throat, chills, headache, and malaise may be present.
  • Vomiting and diarrhea are occasionally noted as part of the prodrome.
• Mucocutaneous lesions develop abruptly. Clusters of outbreaks last from 2-4 weeks. The lesions are typically nonpruritic.
• A history of fever or localized worsening should suggest a superimposed infection; however, fever has been reported to occur in up to 85% of cases.
• Involvement of oral and/or mucous membranes may be severe enough that patients may not be able to eat or drink.
• Patients with genitourinary involvement may complain of dysuria or an inability to void.
• A history of a previous outbreak of Stevens-Johnson syndrome (SJS) or of erythema multiforme may be elicited. Recurrences may occur if the responsible agent is not eliminated or if the patient is reexposed.
• Typical symptoms are as follows:
  • Cough productive of a thick purulent sputum
  • Headache
  • Malaise
  • Arthralgia
• Physical
  • • The center of these lesions may be vesicular, purpuric, or necrotic.
    • The typical lesion has the appearance of a target. The target is considered pathognomonic. However, in contrast to the typical erythema multiforme lesions, these lesions have only two zones of color. The core may be vesicular, purpuric, or necrotic; that zone is surrounded by macular erythema. Some have called these targetoid lesions.
    • Lesions may become bullous and later rupture, leaving denuded skin. The skin becomes susceptible to secondary infection. Extensive sloughing is shown in the image below.
    • 
      

      

      Note extensive sloughing of epidermis from Stevens-Johnson syndrome. Courtesy of David F. Butler, MD.

    • Urticarial lesions typically are not pruritic.
    • Infection may be responsible for the scarring associated with morbidity.
    • Although lesions may occur anywhere, the palms, soles, dorsum of the hands, and extensor surfaces are most commonly affected. Desquamation on the foot is shown in the image below.
    • 
      

      

      Sheetlike desquamation on the foot in a patient with toxic epidermal necrolysis. Courtesy of Robert Schwartz, MD.

    • The rash may be confined to any one area of the body, most often the trunk.
    • Mucosal involvement may include erythema, edema, sloughing, blistering, ulceration, and necrosis. An example of this type of involvement is shown in the image below.
    • 
      

      

      Hemorrhagic crusting of the mucous membranes in toxic epidermal necrolysis. Similar lesions are seen in Stevens-Johnson syndrome. Courtesy of Robert Schwartz, MD.

    • Although some have suggested the possibility of Stevens-Johnson syndrome (SJS) without skin lesions, most believe that mucosal lesions alone are not enough to establish the diagnosis. Some are now calling cases without skin lesions "atypical" or "incomplete." This group of authors suggested that the combination of urethritis, conjunctivitis, and stomatitis made the diagnosis of SJS in a patient with Mycoplasma pneumoniae -induced signs and symptoms.
  • The following signs may be noted on examination:
    • Fever
    • Orthostasis
    • Tachycardia
    • Hypotension
    • Altered level of consciousness
    • Epistaxis
    • Conjunctivitis
    • Corneal ulcerations
    • Erosive vulvovaginitis or balanitis
    • Seizures, coma
  • The rash can begin as macules that develop into papules, vesicles, bullae, urticarial plaques, or confluent erythema.
  
  

  Causes

• Drugs and malignancies are most often implicated as the etiology in adults and elderly persons.
• Pediatric cases are related more often to infections than to malignancy or a reaction to a drug.
• Oxicam NSAIDs and sulfonamides are most often implicated in western nations. In Southeast Asia, allopurinol is most common.
• A medication such as sulfa, phenytoin, or penicillin had previously been prescribed to more than two thirds of all patients with Stevens-Johnson syndrome (SJS). The anticonvulsant oxcarbazepine (Trileptal) has also been implicated. Hallgren et al reported ciprofloxacin-induced Stevens-Johnson syndrome in young patients in Sweden and commented on several others. Metry et al reported Stevens-Johnson syndrome in 2 HIV patients treated with nevirapine and mentioned one other in the literature. The authors speculated that the problem may extend to other non-nucleoside reverse transcriptase inhibitors. Indinavir has been mentioned.
• More than half of the patients with Stevens-Johnson syndrome report a recent upper respiratory tract infection.
• The 4 etiologic categories are (1) infectious, (2) drug-induced, (3) malignancy-related, and (4) idiopathic.
  • Viral diseases that have been reported include herpes simplex virus (HSV), AIDS, coxsackie viral infections, influenza, hepatitis, mumps, lymphogranuloma venereum (LGV), rickettsial infections, and variola.
  • Bacterial etiologies include group A beta streptococci, diphtheria, Brucellosis, mycobacteria, Mycoplasma pneumoniae, tularemia, and typhoid. An "incomplete" case was recently reported after Mycoplasma pneumoniae infection.
  • Coccidioidomycosis, dermatophytosis, and histoplasmosis are the fungal possibilities.
  • Malaria and trichomoniasis have been reported as protozoal causes.
  • In children, Epstein-Barr virus and enteroviruses have been identified.
  • Antibiotic etiologies include penicillins and sulfa antibiotics. Anticonvulsants including phenytoin, carbamazepine, valproic acid, lamotrigine, and barbiturates have been implicated. Mockenhapupt et al stressed that most anticonvulsant-induced SJS occurs in the first 60 days of use. In late 2002, the US Food and Drug Administration (FDA) and the manufacturer Pharmacia noted that Stevens-Johnson syndrome (SJS) had been reported in patients taking the cyclooxygenase-2 (COX-2) inhibitor valdecoxib. In 2007, the US FDA reported SJS/TEN in patients taking modafinil (Provigil). Allopurinol has recently been implicated as the most common cause in Europe and Israel.
  • The most recent additions to possible drug-induced cases include the antidepressant mirtazapine and the TNF-alpha antagonists infliximab, etanercept, and adalimumab.
  • Various carcinomas and lymphomas have been associated.
  • Stevens-Johnson syndrome (SJS) is idiopathic in 25-50% of cases.

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