Guias 2016 Britanicas para el manejo del Sindrome de Steven Johnson/ Lyell.

Summary

Initial assessment on presentation	Take a detailed history from the patient and/or relatives
	Perform a full physical examination, including baseline body weight, and record the vital signs, including oxygen saturation
	Order a set of investigations: FBC, U&E, LFT, glucose, magnesium, phosphate, bicarbonate, mycoplasma serology, CXR, skin biopsy
	Initiate a primary management plan: (i) establish peripheral venous access; (ii) if patient cannot maintain adequate nutrition orally, insert a nasogastric tube and institute nasogastric feeding; (iii) insert a urinary catheter if urogenital involvement is causing significant dysuria/retention
	Strength of recommendation D (GPP)
Determination of drug causality	Identify causative agent and withdraw immediately
	Strength of recommendation D
Prognostic scoring	Calculate SCORTEN within the first 24 h
	Strength of recommendation C
Care setting	An MDT should be convened, coordinated by a specialist in skin failure, usually dermatology and/or plastic surgery, and including clinicians from intensive care, ophthalmology and skincare nursing
	Patients with > 10% BSA epidermal loss should be admitted without delay to a burn centre or ICU with experience of treating patients with SJS/TEN and facilities to manage the logistics of extensive skin loss wound care
	Patients must be barrier-nursed in a side room controlled for humidity, on a pressure-relieving mattress with the ambient temperature raised to between 25 °C and 28 °C
	Strength of recommendation D (GPP)
Skin management regimen 1 (applicable to all patients in all settings)	Employ strict barrier nursing to reduce nosocomial infections
	Take swabs for bacterial and candidal culture from three areas of lesional skin, particularly sloughy or crusted areas, on alternate days throughout the acute phase
	Administer systemic antibiotics only if there are clinical signs of infection
	Strength of recommendation D (GPP)
Skin management regimen 2 (this may involve a conservative and/or surgical approach based on the specialist MDT's daily review of the individual needs of the patient)	Institute a conservative approach in all patients as follows:
	Regularly cleanse wounds and intact skin by irrigating gently using warmed sterile water, saline or an antimicrobial such as chlorhexidine (1/5000)
	Apply a greasy emollient, such as 50% white soft paraffin with 50% liquid paraffin, over the whole epidermis, including denuded areas
	Apply a topical antimicrobial agent to sloughy areas only (choice should be guided by local microbiological advice). Consider silver-containing products/dressings
	The detached, lesional epidermis may be left in situ to act as a biological dressing. Blisters should be decompressed by piercing and expression or aspiration of tissue fluid
	Apply nonadherent dressings to denuded dermis [suitable dressings include Mepitel™ (Mölnlycke Health Care, Dunstable, U.K.) or Telfa™ (Covidien, Mansfield, MA, U.S.A.)]
	A secondary foam or burn dressing should be used to collect exudate [suitable dressings include Exu-Dry® (Smith & Nephew, London, U.K.)]
	Consider transfer to a Burn Centre in patients with TEN (> 30% BSA epidermal loss) and evidence of the following: clinical deterioration, extension of epidermal detachment, subepidermal pus, local sepsis, wound conversion and/or delayed healing. In a burn centre, conservative measures may be supplemented with a surgical approach:
	Remove necrotic/loose infected epidermis and clean wounds using a topical antimicrobial agent (e.g. betadine or chlorhexidine) under general anaesthetic
	Consider debridement with Versajet™ (Drytac, Bristol, U.K.)
	Physiological closure with Biobrane/allograft/xenograft skin in patients with early presentation involving noninfected and large confluent areas
	Strength of recommendation D (GPP)
Fluid replacement regimen	Site venous lines through nonlesional skin, whenever possible, and change peripheral venous cannulas every 48 h
	Monitor fluid balance carefully: catheterize if appropriate/necessary
	Establish adequate IV fluid replacement initially. Fluid replacement can be guided by urine output and other end-point measurements. Individualized fluid management should be adjusted on a daily basis
	With improvement of SJS/TEN mouth involvement, oral administration of fluids should be progressively increased
	Strength of recommendation D
Nutrition regimen	Provide continuous enteral nutrition throughout the acute phase
	Deliver up to 20–25 kcal kg−1 daily during the early, catabolic phase, and 25–30 kcal kg−1daily during the anabolic, recovery phase
	Strength of recommendation C
Analgesia	Use a patient-appropriate validated pain tool to assess pain in all conscious patients at least once daily
	Patients should receive adequate analgesia to ensure comfort at rest, with the addition of supplementary opiates, as required
	Additional analgesia may be needed to address increased pain associated with patient handling, repositioning and dressing changes
	Strength of recommendation D (GPP)
Supportive therapeutic measures	Immobile patients should receive low molecular weight heparin
	Patients in whom enteral nutrition cannot be established should receive a proton pump inhibitor to reduce the risk of stress-related gastrointestinal ulceration
	Neutropenic patients may benefit from recombinant human G-CSF
	Strength of recommendation C
Treatment of eye involvement	Daily ophthalmological review is necessary during the acute illness
	Apply an ocular lubricant (e.g. nonpreserved hyaluronate or carmellose eye drops) every 2 h through the acute illness
	Ocular hygiene must be carried out each day by an ophthalmologist or ophthalmically trained nurse
	Application of topical corticosteroid drops (e.g. nonpreserved dexamethasone 0.1% twice daily) may reduce ocular surface damage
	Administer a broad-spectrum topical antibiotic as prophylaxis (e.g. moxifloxacin drops four times daily) in the presence of corneal fluorescein staining or frank ulceration
	In the unconscious patient, prevention of corneal exposure is essential
	Strength of recommendation D (GPP)
Treatment of mouth involvement	Daily oral review is necessary during the acute illness
	Apply white soft paraffin ointment to the lips every 2 h through the acute illness
	Clean the mouth daily with warm saline mouthwashes or an oral sponge
	Use an anti-inflammatory oral rinse or spray containing benzydamine hydrochloride every 3 h, particularly before eating
	Use an antiseptic oral rinse containing chlorhexidine twice daily
	Use a potent topical corticosteroid mouthwash (e.g. betamethasone sodium phosphate) four times daily
	Strength of recommendation D (GPP)
Treatment of urogenital involvement	Daily urogenital review is necessary during the acute illness
	Apply white soft paraffin ointment to the urogenital skin and mucosae every 4 h through the acute illness
	Use a potent topical corticosteroid ointment once daily to the involved, but noneroded, surfaces
	Use a silicone dressing (e.g. Mepitel™) to eroded areas
	Strength of recommendation D (GPP)
Treatment of airway involvement	Respiratory symptoms and hypoxaemia on admission should prompt early discussion with an intensivist and rapid transfer to an ICU or burn centre, where fibreoptic bronchoscopy should be undertaken
	Strength of recommendation D (GPP)
Active therapy	If active therapy is instituted it should be given, ideally, under the supervision of a specialist skin failure MDT in the context of clinical research and/or case registry
	Strength of recommendation D
Discharge and follow-up	Give the patient written information about drug(s) to avoid
	Encourage the patient to wear a MedicAlert bracelet
	Drug allergy should be documented in the patient's notes; all doctors involved in the patient's care should be informed
	Report the episode to the national pharmacovigilance authorities
	Organize a dermatology outpatient clinic appointment, and, if required, a n ophthalmology outpatient appointment, within a few weeks of discharge
	Refer for review to unit with appropriate subspeciality interest
	Strength of recommendation D (GPP)
Diagnostic testing	Routine drug hypersensitivity testing is not recommended following an episode of SJS/TEN
	Seek specialist advice on hypersensitivity testing where (i) the culprit drug is not known or (ii) medication avoidance is detrimental to the individual or (iii) accidental exposure is possible
	Strength of recommendation D (GPP)

FBC, full blood count; U&E, urea and electrolytes; LFT, liver function tests; CXR, chest X-ray; GPP, good practice point; BSA, body surface area; ICU, intensive care unit; SJS/TEN, Stevens–Johnson syndrome/toxic epidermal necrolyis; MDT, multidisciplinary team; IV, intravenous; G-CSF, granulocyte colony-stimulating factor.

Benjamin Hidalgo-Matlock

Skin Care Physicians of Costa Rica

4000-1054

2208-8206

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