21.3 Severe Integumentary & Soft-Tissue Emergencies: SJS/TEN, SSSS, Necrotizing Fasciitis & Complex Dermatoses

Severe Integumentary and Soft-Tissue Emergencies

Quick Answer: Assess the depth of skin injury, mucosal findings, speed of progression, pain and perfusion. Pain extending beyond a rapidly spreading lesion with shock requires immediate evaluation for necrotizing infection and operative source control. In suspected SJS/TEN, stop the culprit drug and arrange early specialist skin and eye care. Superficial sloughing with spared mucosa in a young child suggests staphylococcal scalded skin syndrome. Proptosis or painful, restricted eye movement with eyelid swelling raises concern for postseptal cellulitis.

Stevens–Johnson Syndrome and Toxic Epidermal Necrolysis

SJS/TEN causes keratinocyte death and full-thickness epidermal necrosis. Painful dusky macules or flat atypical targets, blistering, detachment and mucosal erosions are important clues. Examine the mouth, eyes and genital and perianal areas. Mucositis is common and can be severe. Consider the overall morphology and evolution even when fewer than two sites are involved. For the usual SJS/TEN classification, estimate detached and detachable epidermis as a percent of total body surface area (TBSA). Erythema alone is excluded. SJS involves less than 10%, overlap 10% through 30%, and TEN more than 30%. If the diagnosis is uncertain, involve dermatology for biopsy and differentiation from mimics. Pediatric BAD guideline

• Identify and stop suspected culprit drugs promptly. Review recent starts, dose changes and infections. Lamotrigine, carbamazepine, phenytoin, sulfonamide antibiotics and allopurinol are examples. The U.S. lamotrigine label advises discontinuation at the first rash unless the rash is clearly unrelated. Arrange alternative seizure coverage with neurology if indicated. Document the suspected reaction and communicate avoidance at discharge. Before starting carbamazepine, follow U.S. labeling for HLA-B*15:02 screening in patients with ancestry from populations where the allele may be present. Follow drug-specific guidance for other genotype results. Lamotrigine label · Tegretol label · CPIC carbamazepine and oxcarbazepine
• Support the skin barrier and organ function. Involve dermatology, pediatric critical care and a burn or specialist skin-failure service according to the extent of detachment, instability and local expertise. Significant eye or airway disease also changes transfer needs. Minimize shear and adhesive trauma. Use nonadherent dressings and discuss preservation of clean detached epidermis with the wound team. Provide analgesia, temperature support and early nutrition. Titrate fluids to perfusion, urine output and electrolytes. Thermal burn formulas do not determine SJS/TEN replacement. Reserve systemic antibiotics for clinical infection, with timely empiric treatment when systemic infection is suspected.
• Protect vision early. Arrange experienced ophthalmology assessment within 24 hours of diagnosis. Continue frequent examination during the acute phase. Lubrication and topical therapy depend on the exam. Extensive ocular surface epithelial loss warrants urgent discussion of amniotic membrane treatment. If the child has 35% detachment with hemorrhagic oral and ocular erosions after lamotrigine, TEN is the leading diagnosis and ocular care starts immediately, even if the skin is already dressed.

Mucositis After Infection and DRESS

Reactive infectious mucocutaneous eruption (RIME) often follows a respiratory infection and can produce severe oral and ocular mucositis with sparse skin lesions. MIRM describes the subset associated with Mycoplasma pneumoniae. Review the infection and medication timelines together. Give analgesia, mucosal care and fluid and nutrition support, and obtain ophthalmology input for eye involvement. Treat an identified or strongly suspected bacterial infection when indicated. Antibiotic treatment of the trigger has no established benefit for the mucositis itself. PeDRA update

Drug reaction with eosinophilia and systemic symptoms (DRESS) commonly develops weeks after medication exposure. Fever, facial edema, widespread morbilliform rash, eosinophilia or atypical lymphocytes, and hepatitis or other organ injury raise suspicion. Assess blood count, liver and renal function, and other organs as indicated. Stop the suspect drug and trend findings because organ involvement can evolve. The pediatric EAACI approach uses topical corticosteroids with close monitoring for grade 1 DRESS and systemic corticosteroids for grade 2 or higher. The taper follows organ response and specialist assessment. EAACI pediatric position paper

Staphylococcal Scalded Skin Syndrome

Circulating S. aureus exfoliative toxins cleave desmoglein-1 in the superficial epidermis. Infants and young children may have tender erythema, flexural accentuation, periorificial crusting and fragile bullae that peel with gentle pressure. True mucosal surfaces are generally spared. A positive Nikolsky sign can occur in both SSSS and SJS/TEN. Superficial splitting and the exam of the mucosa help distinguish them. Desmoglein-1 experiments

Hospitalized children usually receive an IV beta-lactam active against MSSA, such as cefazolin. Choose MRSA-active therapy when indicated by prior MRSA, severity and local microbiology. Routine adjunctive clindamycin is unnecessary in typical cases managed on the CHOP pathway. Look for the primary staphylococcal focus and treat hydration and skin-barrier losses. CHOP SSSS pathway · Pediatric comparative cohort

Necrotizing Soft-Tissue Infection

Severe pain beyond visible erythema, hard subcutaneous edema, rapid spread or shock after an apparently minor injury, varicella or an operation should prompt concern for deep infection. Hemorrhagic bullae, anesthesia and crepitus may appear later. A child with rapidly spreading thigh erythema, wooden induration and hypotension after varicella needs immediate surgical exploration. Obtain cultures when feasible and start shock resuscitation and broad IV antibiotics in parallel. Imaging, a laboratory score or a culture result must never delay exploration when suspicion is high.

Empiric treatment covers MRSA, streptococci, gram-negative organisms and anaerobes. Vancomycin plus piperacillin-tazobactam is one broad option. Add toxin-suppressing therapy such as clindamycin when GAS or toxin-mediated shock is suspected per the pediatric pathway. For proven GAS necrotizing fasciitis, use penicillin plus clindamycin. Debride devitalized tissue and reassess for repeat source control. IVIG can be considered in severe streptococcal toxic shock, with uncertain efficacy and no delay to surgery or antimicrobials. IDSA SSTI guideline · UCSF pediatric pathway · CDC GAS guidance

Orbital Cellulitis and Device-Related Pressure Injury

The orbital septum separates preseptal eyelid disease from postseptal infection. Sinus infection, particularly ethmoiditis, can extend into the orbit. Proptosis, painful or restricted eye movement, reduced visual acuity or a relative afferent pupillary defect prompt urgent evaluation. When swelling prevents an adequate eye exam, obtain urgent ophthalmology input. For clear orbital signs, the CHOP pathway calls for contrast CT of the orbits and sinuses, IV antibiotics and urgent ophthalmology and ENT assessment. Intracranial extension may need additional imaging and specialty treatment. Ampicillin-sulbactam is one pediatric empiric option for orbital infection without intracranial extension. Add MRSA coverage based on severity, exposure history and local guidance. Vision compromise, abscess extent and clinical course guide drainage. The Chandler categories run from preseptal edema through orbital cellulitis, subperiosteal abscess, orbital abscess and cavernous sinus thrombosis. CHOP pathway · UCSF antibiotic guidance · CPS pediatric practice point

In the PICU, check skin under masks, tube ties, cervical collars, oximetry probes and splints as safe. Examine the occiput and other pressure points. The Braden QD tool accounts for device exposure and immobility. In its eight-center validation cohort, 64 of 86 observed hospital-acquired pressure injuries were device-related, about 74% of injuries in that cohort. Fit devices properly, offload when safe and tailor repositioning and skin inspection to the device, skin findings and clinical stability. Consider protective dressings at pressure points if they preserve device fit and function. Braden QD validation · International device guidance · Repositioning guidance