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surface may be involved, including the eyes, lips ( Fig. 68.11 ), tongue, buccal
mucosa, nose, and genitalia. In severe cases, pulmonary and renal involvement
has also been reported. Ocular manifestations include conjunctivitis with
photophobia, keratitis, uveitis, or corneal ulcerations. Close ophthalmologic care
is essential to prevent long-term complications, such as corneal
neovascularization, symblepharon, and blindness. Bullae may not be obvious on
mucous membranes, and often appear as a gray–white film that leaves erosions,
ulcerations, and hemorrhagic crust. Mucosal involvement may lead to difficulty
eating, drinking, dysuria, and pain with defecation. Ultimately, if scarring occurs,
it can result in functional impairment from esophageal stenosis, urethral stenosis,
vaginal stenosis, and anal strictures.

FIGURE 68.10 Confluent epidermal necrosis on the face results in diffuse desquamation and
erosions. Note the involvement of the lips with hemorrhagic crust and erosions. (Reprinted with
permission from Garg SJ. Color Atlas and Synopsis of Clinical Ophthalmology—Wills Eye
Institute—Uveitis . Philadelphia, PA: Lippincott Williams & Wilkins; 2011.)


FIGURE 68.11 Hemorrhagic crust and erosions on the lip after desquamation. (Reprinted with
permission from Onofrey BE, Skorin L, Holdeman NR. Ocular Therapeutics Handbook .
Philadelphia, PA: Lippincott Williams & Wilkins; 2011.)

Drugs are the most common trigger for SJS/TEN. Antibiotics (trimethoprimsulfamethoxazole, minocycline), antiepileptics (carbamazepine, phenytoin,
phenobarbital, lamotrigine), NSAIDs, and nevirapine are frequent triggers for
SJS/TEN. The first signs of SJS/TEN can present approximately 7 to 21 days
after starting the medication. Similar to DHR, a genetic predisposition for
SJS/TEN development may be present. Since the identification of the association
between HLA-B*1502 and carbamazepine-induced SJS/TEN in patients of East
Asian descent, the Food and Drug Administration recommends HLA-B*1502
testing prior to carbamazepine initiation. Similarly, HLA-B*5801 has been
associated with allopurinol hypersensitivity and HLA-B*5701 has been

associated with abacavir hypersensitivity.
A mucosal predominant form of SJS associated with mycoplasma infection
was recently renamed Mycoplasma pneumoniae– induced rash and mucositis
(MIRM). Mucositis is a prominent feature of MRIM, though the cutaneous
involvement is absent or minimal. As a result, the clinical course of MRIM is
milder than SJS/TEN. MIRM-like reactions have also been reported with
influenza and Chlamydia pneumoniae infection. Early MRIM may be difficult to


distinguish from SJS/TEN. In those cases, initiation of treatment for SJS/TEN is
prudent while awaiting results from an infectious workup.
The differential diagnosis for SJS/TEN includes EM, staphylococcal scalded
skin syndrome (SSSS), and Kawasaki disease. As noted above, although
SJS/TEN can have targetoid lesions, these are not the classic target lesions seen in
EM. Additionally, SJS/TEN usually begins on the face and trunk, rather than the
extremities as in EM. Likewise, although EM can have mucous membrane
involvement, it does not involve two or more mucous membranes. SSSS affects
the superficial epidermis, resulting in superficial desquamation rather than the
full-thickness epidermal necrosis seen with SJS/TEN. SSSS also spares the oral
mucosa, while SJS/TEN affects the oral mucosa. In contrast, Kawasaki disease
often has a mucosal involvement, with conjunctivitis, strawberry tongue, and dry
and cracked lips. However, the degree of involvement is not as severe as that seen
in SJS/TEN, which may frequently consist of widespread erosions within the
mouth and thick hemorrhagic crust on the lips.
Similar to other drug reactions, the most important step in managing SJS/TEN
is stopping the causative medication. Wound care is critical to decrease the risk of
complications, including infection and scarring. Petrolatum gauze or plain
petrolatum should be liberally used to prevent scarring in all affected areas,
including the lips, genitals, and anus. Ophthalmology and urology should be
consulted if there is suspected ocular or urethral involvement. The skin should be

examined daily, and signs of infection should prompt aggressive treatment
because the primary cause of mortality is infection. Mortality from SJS/TEN can
be as high as 30%, with mortality increasing proportionally to the amount of body
surface area involved. Pain management and nutritional support may need to be
provided parenterally since oral involvement may limit oral intake.
Regarding medical treatment, there is no consensus as to whether systemic
steroids or intravenous immunoglobulins (IVIG) have benefit in this condition.
Systemic steroids have generally fallen out of favor because of the increased risk
of infection and delayed wound healing. IVIG is often used because it is thought
to block apoptosis signaling pathways. When used, IVIG is given at 0.5 to 1
g/kg/day for 2 to 4 days to reach a total dose of 2 to 4 g/kg. Recently, there is
evidence that tumor necrosis factor alpha (TNFa) inhibitors may be effective for
the treatment of SJS/TEN, though there have only been a few case reports of its
use in pediatrics.
Suggested Readings and Key References


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Morbilliform
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reactivation. Pediatr Dermatol 2013;30:608–613.
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Stevens–Johnson Syndrome/Toxic Epidermal Necrolysis
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