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Stevens-Johnson Syndrome

Steven-Johnson Syndrome

Steven-Johnson Syndrome (SJS) is a severe allergic cutaneous disorder. This skin disorder is often brought about by the intake of common drugs like sulfa drugs, anti-convulsants, and antibiotics. The syndrome is characterized by generalized macular lesions that rapidly spread and consolidate leading to epidermal necrosis, blistering and epithelial sloughing.


Presentation

Constitutional signs of fever, fatigue, coughing, and sore throat may be observed several days before cutaneous signs develop among patients with SJS. The following signs and symptoms are seen among patients suffering from SJS:

Fever
  • High fever occurs in these conditions, frequently before the skin and/or mucosa is affected.[ncbi.nlm.nih.gov]
  • We present the case of a 40 years old lady who developed an overlap of the two condition after taking several doses of acetaminophen for fever.[ncbi.nlm.nih.gov]
  • Prior to this, she complained of fever and discomfort upon swallowing. Skin biopsy had shown epidermal necrosis compatible with Stevens Johnson Syndrome and Toxic Epidermal Necrolysis.[ncbi.nlm.nih.gov]
  • Johnson: A new eruptive fever associated with stomatitis and ophthalmia; report of two cases in children. American Journal of Diseases of Children, Chicago, 1922, 24: 526-533. E. Baader: Dermatostomatitis.[web.archive.org]
  • KEYWORDS: SJS-TEN; chikungunya; fever; necrosis; polyarthralgia; vesicles[ncbi.nlm.nih.gov]
Malaise
  • SJS/TEN manifest with an "influenza-like" prodromal phase (malaise, fever), followed by painful cutaneous and mucous membrane (ocular, oral, and genital) lesions, and other systemic symptoms.[ncbi.nlm.nih.gov]
  • According to a 2011 paper in the "Expert Review of Clinical Immunology," the earliest signs of Stevens-Johnson Syndrome, or SJS, are often fever and malaise, a vague feeling of body discomfort, such as achy joints and generalized soreness.[livestrong.com]
  • Patients may present with fever, malaise, myalgia and ocular manifestations. Mortality has been estimated at 5% of cases (3, 4). From 1973, to 2005, Health Canada received 4 reports of SJS suspected of being associated with ibuprofen.[apps.who.int]
  • Stevens-Johnson syndrome [ ste venz jon son ] a severe and sometimes fatal form of erythema multiforme in which the lesions may involve the oral and anogenital mucosa, eyes, and viscera, associated with such constitutional symptoms as malaise, headache[medical-dictionary.thefreedictionary.com]
  • Discontinue Isentress and other suspect agents immediately if signs or symptoms of severe skin reactions or hypersensitivity reactions develop (including, but not limited to, severe rash or rash accompanied by fever, general malaise, fatigue, muscle or[mnn.com]
High Fever
  • High fever occurs in these conditions, frequently before the skin and/or mucosa is affected.[ncbi.nlm.nih.gov]
  • On day 10, a high fever of 39.3 C and a diffuse, erythematous, pruritic full-body rash involving the palms of her hands and the soles of her feet developed, and she was diagnosed with Stevens-Johnson syndrome (SJS).[ncbi.nlm.nih.gov]
  • However, their clinical courses, including high fever, purulent eye and mouth discharge, and seasonal presentation, raised suspicion of an infectious cause.[jamanetwork.com]
  • . – In many cases preceded with flu like symptoms and high fever. – As it evolves the skin literally sloughs off. – Ocular involvement includes severe conjunctivis, iritis, palpebral edema, conjunctival and corneal blisters and erosions, and corneal perforation[sjsupport.org]
Weight Loss
  • Signs and Symptoms Approximate number of patients (when available) Abnormal blistering of the skin 90% Abnormality of temperature regulation 90% Acantholysis 90% Hypermelanotic macule 90% Malabsorption 90% Nausea and vomiting 90% Weight loss 90% Abnormality[web.archive.org]
Congestive Heart Failure
  • heart failure; coronary blockage; arthritis and cutaneous lesions, especially exanthemas (macular, maculopapular, morbilliform, papulovesicular or petechial); Stevens-Johnson syndrome; erythema nodosum; and wheals.[archbronconeumol.org]
Cough
  • The cough is typically a dry, nonproductive cough arising from increasingly irritated membranes in the airway and lungs.[livestrong.com]
  • Sometimes the mucous membranes of the digestive and respiratory tracts are involved, resulting in diarrhea and cough, pneumonia, and difficulty breathing.[merckmanuals.com]
  • Both Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis often start with symptoms such as cough, aching, headaches, a burning sensation in the eyes or fever.[aaaai.org]
Tachypnea
  • A prodrome of chills is followed by pharyngitis, headache, tachypnea, and tachycardia. In several days, bullous mucosal lesions develop, especially in the oropharynx.[aao.org]
Rhinorrhea
  • For five days before admission, the patient s family had been experiencing a cold epidemic with rhinorrhea, dry cough and mild wheezing and she said her health deteriorated progressively, with headache, earache, pain on swallowing, nausea without vomiting[archbronconeumol.org]
Sore Mouth
  • The initial signs of SJS are bloody crusts on the lips, a sore mouth that has a foul smell, and purple-red tender spots on the skin. Drinking and eating are difficult, and light is painful to the eyes.[childrensclinicofraceland.com]
  • mouth and throat Fatigue Cough Burning eyes When to see a doctor Stevens-Johnson syndrome requires immediate medical attention.[mayoclinic.org]
Lip Blisters
Arthralgia
  • Description A syndrome characterized by severe erythema multiforme-like eruption of the skin and lesions of the oral, genital and anal mucosa, and haemorrhagic crusting on the lips, associated with fever, headache and arthralgia.[web.archive.org]
  • Complications can include hepatitis, nephritis, gastrointestinal bleeding, pneumonia , arthritis , arthralgia, fever , and myalgia.[rxlist.com]
  • Complications can include hepatitis, nephritis, gastrointestinal bleeding, pneumonia, arthritis, arthralgia, fever, and myalgia.[medicinenet.com]
  • Heilwart Joseph Victor Klauder Noël Fiessinger A syndrome characterized by severe erythema multiforme-like eruption of the skin and lesions of the oral, genital and anal mucosa, and haemorrhagic crusting on the lips, associated with fever, headache and arthralgia[whonamedit.com]
Red Eye
  • •Rash, blisters or red splotches on the skin •Persistent fever •Blisters in mouth, eyes, ears, nose, genital area •Swelling of eyelids, red eyes •Conjunctivitis •Flu-like symptoms SOURCE: Stevens-Johnson Syndrome Foundation[dallasnews.com]
  • Ocular symptoms: painful red eye, purulent conjunctivitis, photophobia, blepharitis. Signs General examination: fever, tachycardia, hypotension; altered level of consciousness, seizures, coma.[patient.info]
Exanthema
  • […] the initial eruption of both patients presented well-demarcated, infiltrating firm papules mainly on the cheeks and the extensor aspects of the arms (case 1), and multiple vesicles on the soles and oral aphthosis (case 2), which closely mimicked viral exanthema[ncbi.nlm.nih.gov]
  • After 10 days in the burns unit under treatment with silver sulfadiazine cream, the exanthema had improved considerably and the patient was transferred back to the respiratory medicine ward.[archbronconeumol.org]
  • HLA-B*1502 was associated with severe cutaneous reactions (SCR) induced by AEDs, which included carbamazepine, phenytoin, and lamotrigine (p 0.001, odds ratio 17.6), but was not associated with maculopapular exanthema (MPE) (p 0.32).[ncbi.nlm.nih.gov]
  • The clinical manifestations of drug eruptions can range from mild maculopapular exanthema to severe cutaneous adverse drug reactions (SCAR), including drug-induced hypersensitivity syndrome/drug reaction with eosinophilia and systemic symptoms, Stevens-Johnson[ncbi.nlm.nih.gov]
  • Prevention of nevirapine-associated exanthema using slow dose escalation and/or corticosteroids. AIDS 2000, 14: 2153–2157. 15. Garcia-Doval I, LeCleach L, Bocquet H, Otero XL, Roujeau JC.[web.archive.org]
Epistaxis
  • In post-marketing experience, as with other azole antifungals, bleeding events (bruising, epistaxis, gastrointestinal bleeding, haematuria and melena) have been reported, in association with increases in prothrombin time in patients receiving fluconazole[web.archive.org]
Hematuria
  • Twenty patients (61%) complained of dysuria and one child had gross hematuria in the setting of meatal lesion. Petroleum jelly was used in the majority of patients.[ncbi.nlm.nih.gov]
  • Complications include: Blindness Pneumonitis (inflammation of the lungs) Myocarditis (inflammation of the muscles in the heart) Hepatitis Hematuria (blood in the urine) Kidney failure Sepsis (blood infection) Death Please consult your physician before[brownandcrouppen.com]
Headache
  • The case presented in this report is that of a 17-year-old female who presented to her primary care provider with a chief complaint of headache; she was initially diagnosed with a urinary tract infection and prescribed nitrofurantoin (Macrobid).[ncbi.nlm.nih.gov]
  • Description A syndrome characterized by severe erythema multiforme-like eruption of the skin and lesions of the oral, genital and anal mucosa, and haemorrhagic crusting on the lips, associated with fever, headache and arthralgia.[web.archive.org]
  • A 7-year-old boy was referred to a neurosurgery clinic with headache. Further imaging workup revealed hypothalamic PA. Partial resection of the lesions was performed with right-side pterional approach.[ncbi.nlm.nih.gov]
  • Both Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis often start with symptoms such as cough, aching, headaches, a burning sensation in the eyes or fever.[aaaai.org]
Seizure
  • Here we show that there is a strong association in Han Chinese between a genetic marker, the human leukocyte antigen HLA-B*1502, and Stevens-Johnson syndrome induced by carbamazepine, a drug commonly prescribed for the treatment of seizures.[ncbi.nlm.nih.gov]
  • Long Term use of Dilantin to treat seizure disorders and epilepsy. Dilantin can accumulate in the cerebellar cortex over long periods of time which can cause atrophy of the cerebellum.[stevens-johnsonsyndrome.org]
  • […] or mental illness The medicines most likely to cause problems in kids are sulfa antibiotics , Tylenol, and drugs that treat seizures, especially carbamazepine ( Carbatrol , Tegretol ).[webmd.com]
  • […] or mental illness The medicines most likely to cause problems in kids are sulfa antibiotics, Tylenol, and drugs that treat seizures, especially carbamazepine ( Carbatrol, Tegretol ).[webmd.com]
  • […] like sulfa and combination drugs including sulfa like Bactrim Tetracycline and Accutane used to treat acne NSAIDs (non-steroidal anti-inflammatory drugs) like ibuprofen, also marketed as Advil and Motrin Tegretol, Dilantin and Depakote, used to treat seizures[brownandcrouppen.com]
Neglect
  • Early manifestation of mucosal change without typical skin lesions should not be neglected in the diagnosis of incomplete SJS.[ncbi.nlm.nih.gov]

Workup

Patients are systematically examined physically for the eminent cutaneous lesion and associated signs of SJS. A detailed clinical history on the onset of the symptomatology will easily grasp the diagnosis for SJS. A skin biopsy is frequently done to definitively diagnose SJS in suspected patients.

Treponema Pallidum
  • Serological test results were negative for Mycoplasma; herpes simplex virus; Epstein-Barr virus; Cytomegalovirus; HIV; hepatitis A, B, and C viruses; and Treponema pallidum. Blood culture was negative.[web.archive.org]
HLA-B27
  • Mycoplasma infections and HLA-B27/-B51 were common. The Author 2017. Published by Oxford University Press on behalf of The Journal of the Pediatric Infectious Diseases Society. All rights reserved.[ncbi.nlm.nih.gov]

Treatment

The most important approach in the treatment of SJS is the sudden discontinuation of the inciting factor implicated in the initiation of the immune response. In cases where the inciting drug is not identified, physicians will usually ask their patients to discontinue all non-essential drugs to eliminate the possible causative agent. SJS is a medical emergency, thus all patients are managed promptly in the hospital to stabilize fluid imbalance and correct nutritional deficiencies. Adequate wound care and eye care may be in place where another specialist may be brought in for comanagement [9].

Antiinflammatory agents to control inflammation, itching, and pain may be used to allay these symptoms. Corticosteroid may also be given as an immune modulator agent for SJS [10]. Patients are given antibiotic therapy to control concurrent infections associated with the cutaneous disease.

Prognosis

Cutaneous lesion of mild SJS typically heals within 1 to 2 weeks from onset without any serious sequelae. However, this may be complicated by secondary skin infections that increases the morbidity rates among afflicted patients. The mortality rate in SJS varies directly with the body surface area (BSA) of the cutaneous sloughing. Mortality rates may climb beyond 50% for BSA percentage of 25% to 35% of skin affected [8]. Patients who survive SJS may develop a number of sequelae in some cases, these include photophobia, watery eyes, inverted eyelashes, and burning sensation of the eyes.

Etiology

In more than half the cases of SJS, drugs are commonly considered as the precipitating agent. The following are the most common drugs in the development of the severe cutaneous reaction in SJS:

  • Sulfa drugs (sulfasalazine and cotrimoxazole) [2]
  • Antibiotics (amoxicillin, ampicillin and fluoroquinolones)
  • Anticonvulsant agents (phenytoim, carbamazepine, and phenobarbital)
  • Other drugs (piroxicam and allopurinol)

There are other factors that may incite the hypersensitivity reaction in SJS, these are infections (most commonly by Mycoplasma pneumonia), Graft rejection disease, and vaccination reactions. Studies have demonstrated that patients with systemic lupus erythematosus (SLE) have an increased risk in developing SJS [3]. In some instances, the true etiology of SJS remains unknown or idiopathic in nature.

Epidemiology

In the United States, medic aid data has determined that the incidence of SJS has reached to 6.8 cases per million population per annum [4]. The majority of cases of SJS have a predilection to the winter and early spring seasons. Sulfonamides and piroxicam are the most common inciting factors implicated in the US, while allopurinol is more common in Southeast Asian nations like Malaysia and Hong Kong. SJS occurs in all races but is more commonly seen in the white races compared to their dark skinned counterparts. Large cohort studies have determined that the mean age of onset in SJS is 25 years old and this may occur in children as young as 3 years old.

Sex distribution
Age distribution

Pathophysiology

The pathophysiology in SJS is described as an idiosyncratic and delayed hypersensitivity reaction to an inciting factor. There are certain sectors of the population who are more prone to the disease inclusive are those in immunocompromised states (i.e. AIDS) and those under radiotherapy for cancer [5]. The antigen presentation and the production of acute phase reactants (like tumor necrosis factor) by the tissue dendrocytes will advertently result in the recruitment and the proliferation of the T-lymphocytes which enhances cytotoxicity [6].

There is a subsequent death in the keratocytes that leads to the separation of the dermis and the epidermis that clinically present as sloughing. The perpetuation of the inflammation by the chemokines leads to extensive epidermal necrolysis of the surface epithelium [7]. Inciting factors may be discontinued to stop and reduce the inflammatory process or may be titrated in smaller divided doses if drug is absolutely necessary and essential.

Prevention

Patients who have Asian lineage are perpetually advised to undergo genetic testing for SJS before starting on carbamazepine indicated for the control epilepsy. Patients who have had SJS in the past must avoid by all means to take in the identified inciting agent to avoid its recurrence. In the same way, family members are also discouraged to ingest the inciting drug because of the genetic risk factors involved.

Summary

Steven-Johnson syndrome (SJS) is a rare and life-threatening disorder of the skin and the mucous membrane [1]. This syndrome is usually a reaction to an infection or an intake of a medication that starts out as a reddish rash that disseminates in the skin surface and forms blisters. SJS is considered as a medical emergency that requires prompt treatment focusing on the elimination of the causative agent, the control of the symptoms, and preventing further complications.

Patient Information

Definition

Steven-Johnson syndrome is a rare and life-threatening disorder of the skin and the mucous membrane described to exaggerated hypersensitivity response to an inciting factor.

Cause

More than half the cases of SJS are initiated by the intake of drugs like sulfa drugs, antibiotics, anticonvulsants, and analgesics. Less common causes includes infections, graft rejection reactions and vaccinations.

Symptoms

Patients will present with severe skin and mucous membrane lesions which are erythematous, itchy, and blistering.

Diagnosis

A detailed clinical history and a meticulous physical examination of the skin may secure the diagnosis of SJS. Skin biopsy is the definitive test used for this kind of disease.

Treatment and follow-up

Treatment focuses on the prompt removal of the inciting factor, the stabilization of the patient through fluid resuscitation, and the control of the signs and symptoms.

References

Article

  1. French LE. Toxic epidermal necrolysis and Stevens Johnson syndrome: our current understanding. Allergol Int. Mar 2006; 55(1):9-16.
  2. Fernando SL, Broadfoot AJ. Prevention of severe cutaneous adverse drug reactions: the emerging value of pharmacogenetic screening. CMAJ. Mar 23 2010; 182(5):476-80.
  3. Horne NS, Narayan AR, Young RM, Frieri M. Toxic epidermal necrolysis in systemic lupus erythematosus.Autoimmun Rev. Feb 2006;5(2):160-4.
  4. Strom BL, Carson JL, Halpern AC, et al. A population-based study of Stevens-Johnson syndrome. Incidence and antecedent drug exposures. Arch Dermatol. Jun 1991; 127(6):831-8.
  5. Rotunda A, Hirsch RJ, Scheinfeld N, Weinberg JM. Severe cutaneous reactions associated with the use of human immunodeficiency virus medications. Acta Derm Venereol. 2003; 83(1):1-9.
  6. De Rojas MV, Dart JK, Saw VP. The natural history of Stevens Johnson syndrome: patterns of chronic ocular disease and the role of systemic immunosuppressive therapy. Br J Ophthalmol. Aug 2007; 91(8):1048-53.
  7. French LE, Trent JT, Kerdel FA. Use of intravenous immunoglobulin in toxic epidermal necrolysis and Stevens-Johnson syndrome: our current understanding. Int Immunopharmacol. Apr 2006; 6(4):543-9.
  8. Bastuji-Garin S, Fouchard N, Bertocchi M, et al. SCORTEN: a severity-of-illness score for toxic epidermal necrolysis. J Invest Dermatol. Aug 2000; 115(2):149-53.
  9. Inachi S, Mizutani H, Shimizu M. Epidermal apoptotic cell death in erythema multiforme and Stevens-Johnson syndrome. Contribution of perforin-positive cell infiltration. Arch Dermatol. Jul 1997; 133(7):845-9.
  10. Paquet P, Paquet F, Al Saleh W, Reper P, Vanderkelen A, Piérard GE. Immunoregulatory effector cells in drug-induced toxic epidermal necrolysis. Am J Dermatopathol. Oct 2000; 22(5):413-7.

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Last updated: 2018-06-21 22:59