Stevens-Johnson syndrome


00:00 / 00:00



Stevens-Johnson syndrome


Pigmented skin disorders



Acneiform skin disorders

Acne vulgaris



Hidradenitis suppurativa

Papulosquamous and inflammatory skin disorders

Contact dermatitis

Atopic dermatitis

Lichen planus

Pityriasis rosea


Seborrhoeic dermatitis


Keratotic skin disorders

Actinic keratosis

Vesiculobullous skin disorders

Epidermolysis bullosa

Bullous pemphigoid

Pemphigus vulgaris

Desquamating skin disorders

Erythema multiforme

Stevens-Johnson syndrome

Skin integrity disorders

Pressure ulcer




Skin infections




Necrotizing fasciitis

Human papillomavirus

Varicella zoster virus

Poxvirus (Smallpox and Molluscum contagiosum)


Herpes simplex virus


Malassezia (Tinea versicolor and Seborrhoeic dermatitis)

Pediculus humanus and Phthirus pubis (Lice)

Sarcoptes scabiei (Scabies)

Human herpesvirus 6 (Roseola)

Parvovirus B19

Varicella zoster virus

Measles virus

Rubella virus

Skin neoplasms

Vascular tumors

Human herpesvirus 8 (Kaposi sarcoma)


Skin cancer

Hair and nail disorders

Alopecia areata

Telogen effluvium


Integumentary system pathology review

Pigmentation skin disorders: Pathology review

Acneiform skin disorders: Pathology review

Papulosquamous and inflammatory skin disorders: Pathology review

Vesiculobullous and desquamating skin disorders: Pathology review

Bacterial and viral skin infections: Pathology review

Skin cancer: Pathology review

Viral exanthems of childhood: Pathology review


Stevens-Johnson syndrome


0 / 21 complete

USMLE® Step 1 questions

0 / 3 complete

High Yield Notes

5 pages


Stevens-Johnson syndrome

of complete


USMLE® Step 1 style questions USMLE

of complete

A 30-year-old woman, gravida 2 para 1, at 12 weeks gestation, presents to the emergency department because of sloughing off of the skin and pain in and around the oral cavity. For the past four days, these symptoms were preceded by fever, joint pain, and malaise. She reports mouth and throat pain during swallowing both solids and liquids. She has a history of generalized tonic-clonic seizures and was recently switched to a different medication. She works as a postal carrier and traveled to Massachusetts two months ago to visit her aunt. Temperature is 38.5°C (101.3°F), pulse is 100/min, respirations are 20/min, and blood pressure is 102/60 mmHg. Physical examination shows an ill-appearing woman with extensive erosions of the oral mucosa and sloughed skin of the face and chest that is tender to touch. Which of the following is most likely associated with this condition?  

External References

First Aid








Bullae p. 487

Stevens-Johnson syndrome p. 494

Stevens-Johnson syndrome p. 191, 494, 565

as drug reaction p. 251

sulfa drug allergies p. 253


Content Reviewers

Rishi Desai, MD, MPH


Kara Lukasiewicz, PhD, MScBMC

Marisa Pedron

Stevens-Johnson Syndrome is named for the physicians who first reported an acute, immune-mediated condition that affects the skin and mucous membranes such as the mouth and genitals.

Stevens-Johnson syndrome and toxic epidermal necrolysis are considered to be the same condition, but are along a spectrum where toxic epidermal necrolysis is the more severe form.

Both can be fatal if not treated, and the risk of fatality increases with severity.

The skin plays an important role in protecting underlying muscles, bones, ligaments, and internal organs by forming a barrier to infectious pathogens; and preventing water loss from the body.

The outermost layer of the skin is the epidermis and, itself, is made of several layers of cells.

The deepest layer is called the stratum basale, or the base layer.

And cells here are anchored to a basement membrane, a thin layer of delicate tissue containing proteins like collagen and laminins, which attach the epidermis to the underlying skin layer called the dermis.

Similar to how the skin lines the outside of the body, mucous membranes, or mucosa, line the inner body surfaces like the mouth, tongue, respiratory tract, conjunctiva of the eyes, genitals, and anus.

Mucosa is made up of one or more layers of epithelial cells, which, again, are attached to a basement membrane that sits on top of a layer called the lamina propria.

Now, most cells in the body have a protein called major histocompatibility complex or MHC class I molecule on the surface of their membrane.

This protein presents peptides from within the cell to immune cells called cytotoxic T cells, also called a CD8+ T cells.

If the cell is healthy, the cytotoxic T cell doesn’t recognize the peptides as foreign, and nothing happens.

But if a cell is infected, say with a virus, the cytotoxic T cell can recognize the peptides as foreign and trigger an immune response.

Once all infected cells have been destroyed, the immune response resolves.


Stevens �Johnson syndrome is a life-threatening skin condition, in which cell death causes the epidermis to separate from the dermis. It is a type of IV hypersensitivity reaction where cytotoxic T-cells inappropriately attack and kill epithelial cells in the mucosa and skin epidermis. The most well-known causes are medications, such as lamotrigine and other seizure drugs. Treatment includes immediately removing the trigger, followed by pain medication, hydration, and sterile wound care.


  1. "Robbins Basic Pathology" Elsevier (2017)
  2. "Harrison's Principles of Internal Medicine, Twentieth Edition (Vol.1 & Vol.2)" McGraw-Hill Education / Medical (2018)
  3. "Pathophysiology of Disease: An Introduction to Clinical Medicine 8E" McGraw-Hill Education / Medical (2018)
  4. "Toxic epidermal necrolysis" Journal of the American Academy of Dermatology (2013)
  5. "Current Perspectives on Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis" Clinical Reviews in Allergy & Immunology (2017)

Copyright © 2023 Elsevier, except certain content provided by third parties

Cookies are used by this site.

USMLE® is a joint program of the Federation of State Medical Boards (FSMB) and the National Board of Medical Examiners (NBME). COMLEX-USA® is a registered trademark of The National Board of Osteopathic Medical Examiners, Inc. NCLEX-RN® is a registered trademark of the National Council of State Boards of Nursing, Inc. Test names and other trademarks are the property of the respective trademark holders. None of the trademark holders are endorsed by nor affiliated with Osmosis or this website.