Stevens-Johnson syndrome history and symptoms

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Anila Hussain, MD [2]

Overview

Patients with Stevens-Johnson Syndrome usually have a history of recent drug use within the last 2 months or some other inciting trigger like infection or vaccination. It starts with a flu-like illness followed by skin eruptions that involve trunk and extremities mainly. Skin involvement in SJS is always <10 percent of the body surface. Blisters, erosions, vesicles and bullae are seen along with confluent redness and sloughing of skin. Mucosal lesions can be seen in 2 or more surfaces and can involve eyes, oral mucosa, nasopharynx or genitals commonly[1].

History and Symptoms

History

Patients with SJS may have a positive history of:

  • Recent drug use
  • Recent viral or bacterial infection
  • Active malignancy

Symptoms

Symptoms usually start within 2 months of starting a drug or other inciting trigger.

  • Prodome of flu like symptoms 1-3 days before skin eruptions. May include
  • Skin lesions
    • Involve less than 10 percent of body surface
    • Start as red or reddish blue macules/ papules on trunk and extremities and later increase in size and coalesce
    • Blisters/erosions/bullae and vesicles are seen
    • Confluent redness with skin sloughing
    • Nikolsky Sign is Positive- which is the detachment of superficial epidermis on gentle pressure or rubbing of skin
    • Target lesions are not typically seen (as opposed to Erythema Multiforme)
  • Mucosal Lesions
    • Can be seen before or after the skin eruptions
    • Can involve 2 or more mucosal surfaces
    • Seen in 90 percent of cases of SJS/TEN[2]
    • Involved mucosal surfaces may include[3]
      • Buccal mucosa: Painful erosions leading to Stomatitis and Mucositis
      • Ocular: Seen frequently and range from acute conjuctivitis, eyelid edema and erythema, crusting, ocular discharge, Corneal erosion to corneal ulceration[4]
      • Urogenital : Can cause Urethritis, Vulvovaginitis
      • Nasopharynx
      • Tracheo-Bronchial and intestinal involvement is uncommon[5]

References

  1. Harr T, French LE (2010). "Toxic epidermal necrolysis and Stevens-Johnson syndrome". Orphanet J Rare Dis. 5: 39. doi:10.1186/1750-1172-5-39. PMC 3018455. PMID 21162721.
  2. Letko E, Papaliodis DN, Papaliodis GN, Daoud YJ, Ahmed AR, Foster CS (2005). "Stevens-Johnson syndrome and toxic epidermal necrolysis: a review of the literature". Ann Allergy Asthma Immunol. 94 (4): 419–36, quiz 436-8, 456. doi:10.1016/S1081-1206(10)61112-X. PMID 15875523.
  3. Harr T, French LE (2010). "Toxic epidermal necrolysis and Stevens-Johnson syndrome". Orphanet J Rare Dis. 5: 39. doi:10.1186/1750-1172-5-39. PMC 3018455. PMID 21162721.
  4. Sotozono C, Ueta M, Koizumi N, Inatomi T, Shirakata Y, Ikezawa Z; et al. (2009). "Diagnosis and treatment of Stevens-Johnson syndrome and toxic epidermal necrolysis with ocular complications". Ophthalmology. 116 (4): 685–90. doi:10.1016/j.ophtha.2008.12.048. PMID 19243825.
  5. Lebargy F, Wolkenstein P, Gisselbrecht M, Lange F, Fleury-Feith J, Delclaux C; et al. (1997). "Pulmonary complications in toxic epidermal necrolysis: a prospective clinical study". Intensive Care Med. 23 (12): 1237–44. PMID 9470079.

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