- Which of the following statements is true about Steven Johnson Syndrome (SJS)?
a. Caused by a type IV hypersensitivity reaction
b. SJS leads to detachment of > 30% of skin over the entire body surface area
c. Both a and b
d. None of the above
2. What are the possible ocular features of SJS?
a. Destruction of limbal stem cells
b. Lid margin keratinization
c. Corneal ulceration
d. All of the above
3. Which of the following does not cause SJS?
a. Mycoplasma Pneumoniae
b. Anticonvulsant therapy
c. Systemic antibiotics
d. All of the above can cause SJS
4. Which of the following is true about ocular involvement in SJS?
a. Incidence may be as high as 50%
b. Amniotic membrane grafting can be performed in early stages
c. Mucous membrane grafting is the preferred procedure for treating lid margin keratinization
d. In bilateral end stage disease, keratoprosthesis may be an effective alternative for visual rehabilitation
e. All of the above
5. What are the possible indications for mucous membrane grafting in Ophthalmology?
a. Lid margin keratinization in SJS
b. Refractory vernal keratoconjunctivitis
c. None of the above
d. Both a and b
- a. Caused by a type IV hypersensitivity reaction.
Stevens-Johnson Syndrome (SJS) and toxic epidermal necrolysis (TEN) are severe cutaneous adverse reactions (SCAR) that belong to type IV hypersensitivity, mediated by immunological effect.
SJS and TEN differ in the extent of detached or detachable skin. SJS affects <10% of the body surface area with, SJS/TEN overlap affects 10%-30% of the body surface and TEN affects >30%.
Liotti L, Caimmi S, Bottau P, et al. Clinical features, outcomes and treatment in children with drug induced Stevens-Johnson syndrome and toxic epidermal necrolysis. Acta Biomed. 2019;90(3-S):52‐60. DOI: 10.23750/abm.v90i3-S.8165
Types of Hypersensitivity reactions are as follows:
2. d. All of the above
Corneal complications include epithelial defect, infiltration, and ulceration and are reported in 18%– 20% of cases. The lid margin changes occurring include meibomian gland metaplasia, trichiasis, punctal scarring, and lid margin keratinization.
Destruction of limbal stem cells results in replacement of corneal epithelium by conjunctival epithelium. This destruction to limbal stem cells is ongoing in SJS because of persistent inflammation. The preexisting primary damage to the limbal stem cells determines the further course of the disease—either squamous metaplasia or conjunctivalization.
Iyer G, Pillai VS, Srinivasan B, et al. Mucous membrane grafting for lid margin keratinization in Stevens–Johnson syndrome: results. Cornea. 2010 Feb;29(2):146-51 DOI: 10.1097/ICO.0b013e3181ae2691
3. d. All of the above can cause SJS.
Kim et al in a study of 82 patients attributed the occurrence of SJS- TEN to the following agents (table). Several infectious etiologies have also been implicated in SJS and TEN. Mycoplasma pneumoniae and herpes simplex virus are the most commonly reported.
Kim HI, Kim SW, Park GY, et al. Causes and treatment outcomes of Stevens-Johnson syndrome and toxic epidermal necrolysis in 82 adult patients. Korean J Intern Med. 2012;27(2):203‐210 doi:10.3904/kjim.2012.27.2.203
4. e. All of the above
In 50% of the patients ocular complications occur, which can lead to blindness in the absence of immediate ophthalmological treatment.
Chronopoulos A, Pleyer U, Mockenhaupt M. Okuläre Beteiligung bei Stevens-Johnson-Syndrom und Toxisch epidermaler Nekrolyse [Ocular involvement in Stevens-Johnson syndrome and toxic epidermal necrolysis]. Klin Monbl Augenheilkd. 2012;229(5):534‐539. DOI: 10.1055/s-0031-1299530
In a randomised control trial, eyes that underwent amniotic membrane grafting along with medical therapy in the acute stage of SJS had better visual acuity, better Schirmer test values, along with no incidence of corneal haze, no limbal stem cell deficiency, symblepharon, ankyloblepharon, or lid-related complications. These eyes thus showed better outcomes than eyes that received medical therapy alone.
Sharma N, Thenarasun SA, Kaur M, et al. Adjuvant Role of Amniotic Membrane Transplantation in Acute Ocular Stevens-Johnson Syndrome: A Randomized Control Trial. Ophthalmology. 2016;123(3):484‐491.doi: 10.1016/j.ophtha.2015.10.027
Mucous membrane grafting for lid margin keratinization definitely has a role to play not only in improving patient comfort, the ocular surface status, and visual acuity but also in preventing a deterioration of the ocular surface. Keratoprosthesis, specifically the modified osteo-odonto-keratoprosthesis, forms the mainstay for visual rehabilitation in the end-stage disease.
Iyer G, Srinivasan B, Agarwal S, et al. Treatment Modalities and Clinical Outcomes in Ocular Sequelae of Stevens-Johnson Syndrome Over 25 Years—A Paradigm Shift. Cornea. 2016 Jan;35(1):46-50 DOI: 10.1097/ICO.0000000000000680
5. d. Both a and b
Surgical excision of refractory giant papillae in vernal keratoconjunctivitis followed by MMG does have its advantages in reducing their corneal complications such as recurrence of shield ulcers, and it should be considered early in the management of exuberant refractory giant papillae.
Iyer G, Agarwal S, Srinivasan B. Outcomes and Rationale of Excision and Mucous Membrane Grafting in Palpebral Vernal Keratoconjunctivitis. Cornea. 2018;37(2):172‐176. DOI: 10.1097/ICO.0000000000001421