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Stevens-Johnson Syndrome-Toxic Epidermal Necrolysis: The aftermath

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By Warren Heymann, MD
May 26, 2021
Vol. 3, No. 21

Dr. Warren Heymann photo
The American Academy of Dermatology’s SkinSerious® campaign highlights how dermatologists treat serious diseases. Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) top the list. (1) Justifiably, all attention focuses on the immediate issues in an attempt to diminish the complications of a disorder with an approximate 30% mortality rate (although it has been suggested that averaging the mortality rates of TEN and SJS is not advised as SJS is mainly a mucocutaneous disorder with good prognosis versus TEN associated with systemic toxicity of multiple organs). (2)

The complications of SJS/TEN are legion — multidisciplinary management of fluid and electrolyte abnormalities, pain, thermodysregulation, ocular, pulmonary, gastrointestinal, and renal dysregulation is mandatory. Survivors may contend with life-altering cicatricial sequelae. This commentary focuses on the cutaneous complications of patients who have survived the acute ravages of SJS-TEN. It must be emphasized that SJS-TEN is a systemic disease, and ongoing management is multidisciplinary. I encourage you to read the outstanding review of the clinical presentations, prevention, and treatment of the long-term complications of SJS/TEN by Lee et al. The authors thoroughly discuss the cutaneous, ocular, oral, dental, pulmonary, urogenital, gynecological, gastrointestinal, hepatic, renal, psychiatric, and psychosocial sequelae. (3)

Cabañas Weisz et al A performed a 19-year (1998-2016) retrospective review of all patients with TEN admitted to Cruces University Hospital´s Burns Unit. Demographic and admission data were collected. Survivors were contacted for a follow-up multidisciplinary assessment involving dermatological, ocular, ENT, urological, gynecological, and psychological examination. Of the total of 22 patients — 7 died during hospitalization, 8 expired after discharge (due to age and oncological reasons), and 7 survived, with 6 survivors agreeing to be interviewed. The patients presented with both physical and psychological sequelae, including dermatological (100%), oropharyngeal (50%), and ophthalmologic complications (50%), with corneal damage and severe dry eye as the most frequent. The only male patient underwent phimosis surgery due to mucosal adhesions. Post-traumatic stress disorder was noted in 33.33%, and quality of life was affected in 66.67% of the patients by skin sequelae. The authors concluded that quality of life can be severely impaired by multiple long-term complications, suggesting that long-term follow-up might prevent or limit the progression of the chronic sequelae. (4)

Long-term cutaneous sequelae (3, 5) include:

  1. Postinflammatory dyspigmentation. Hyperpigmentation, hypopigmentation, or a combination of the two, occurs commonly. It may take years to resolve, or it may be permanent. This may be more frequent in the pediatric population.

  2. Scars. These may be hypertrophic or keloidal.

  3. Nail changes. Onycholysis and onychomadesis may accompany the acute episode due to complete nail matrix arrest. Beau lines may be noted. Other reported features include onychorrhexis, onychoschizia, koilonychia, erythronychia, and an oil-drop sign. (6) Nail loss may be permanent in up to 20% of cases; those nails that regrow may be dystrophic and abnormally pigmented. Scarring of the matrix could result in pterygium formation and anonychia. (7)

  4. Hair changes. Telogen effluvium is common. There has only been a solitary report of an alopecia areata-like presentation with TEN; the authors attributed this to resident memory T-cell activation. (8)

  5. Eruptive nevi and atypical nevi. Eruptive nevi have been reported in up to 20% of survivors, appearing 3 weeks to 3 years following the acute event, with most cases observed in children and young adults. Histologically, these are usually benign junctional or compound nevi. Gelfer and Rivers detailed the case of a man whose eruptive nevi were stable for 38 years. The authors note that eruptive nevi may be observed after bullous disorders or with immunosuppression (by disease or iatrogenically). They propose that those due to bullous disorders will remain benign, compared to those seen in the immunosuppressed population, who may be at a greater risk for melanoma. (9) Atypical nevi may complicate SJS/TEN. Because the melanoma risk is unknown it is recommended that these patients be followed carefully for any worrisome changes in the nevi. (10)

  6. Other cutaneous manifestations. Pruritus, hyperhidrosis, photosensitivity, heterotopic ossification, and disseminated ectopic sebaceous glands have all been reported. (3)

Clearly, dermatologists need to follow SJS/TEN survivors for the long haul, encouraging self-examination, sun protection, and treating specific issues, if amenable to standard therapy.

Although I found psychiatry academically fascinating as a medical student, I chose not to be a psychiatrist. Little did I realize that as a dermatologist, I would need to be a psychiatrist regardless. I can only imagine the fear and anxiety that accompanies acute SJS/TEN and the post-traumatic stress and depression in some survivors. It is incumbent that dermatologists address the psychological issues confronting SJS/TEN survivors as we compassionately manage the cutaneous manifestations of this devastating disorder.

For “Our Expert’s Viewpoint,” I invited one of my patients who survived TEN to share her story. Requesting anonymity, she graciously provided her touching, moving perspective that will stay with you whenever you encounter SJS/TEN.

Point to Remember: Long-term sequelae of Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis may adversely affect quality of life. A multidisciplinary approach dealing with medical and psychiatric complications is essential in providing optimal care for those who have survived severe disease.

Our expert’s viewpoint

By an anonymous TEN survivor

Initial symptoms

I was taking a combination of prescription medications to help regulate my mood when I started having several eye issues. They became red, itchy, and felt as if they were burning. I went to an eye doctor who prescribed eye drops. My eyes didn’t get any better, and I started to develop flu-like symptoms. I also noticed numerous red spots that covered my arms. Painful blisters started to appear on my lips and inside my mouth, which made it unbearable to eat.

In the burn unit

After a few days in the ER, I was transported to a burn ICU, where I stayed for three weeks. I was essentially unconscious for the first two weeks, and when I awoke the third week, I saw my body wrapped in bandages from head to toe, completely covered in second- and third-degree burns. I was told I had survived Toxic Epidermal Necrolysis and that over 85% of my epidermis and all of my mucous membranes were affected.


Upon discharge from the burn unit, I was assured that everything should be fine moving forward. But within the following days, weeks, and months, it was very clear that things were not fine. My entire epidermis continued to shed like the skin of a snake. My lips and other mucous membranes were patches of raw, bloodied scabs. Chunks of skin fell from my eyelids, ears, mouth, scalp, hair, and every single fingernail and toenail sloughed from my body. I remember just sitting and crying on the bathroom floor staring at a chunk of skin in the shape of part of my foot that had just sloughed off.

Life after TEN

After five years, I am still constantly reminded of my TEN sequelae, which includes complications with my nails, skin, eyes, esophageal tract, vulva, psychological well-being, and general activities of daily living.

Illustration for DWII SJS-TEN
Illustration for DWII SJS-TEN

Most of my fingernails and toenails are still missing, as the nail matrices were destroyed. The nailbeds are extremely sensitive, and the little slivers of nails that have grown back can be very painful, especially when they get caught on things. Raised scarring and new moles from the burns developed on my back, and the scarring on my face required special laser treatment.

Image for DWII SJS-TEN
Image for DWII SJS-TEN

While I am grateful to have my vision intact, there are several other ocular complications, including scarring on and underneath the eyelids, eyelashes growing in the wrong place and in the wrong direction, extreme dryness and redness, pain, and severe meibomian gland dropout, all of which require ongoing procedures and treatments. I have found some relief through wearing custom Prose lenses and by having the lashes either pulled manually or electro-epilated. Just managing my eyes adds about two hours to my daily routine.

Another impact of TEN was salivary gland damage, which has caused increased dryness in my mouth, and requires me to frequently clear thick mucus from my throat.

TEN also caused vulvar sequelae, which requires ongoing pelvic physical therapy and the use of various topical creams. These complications also impact relations with my husband. During the first four years after TEN, the vulva was so sensitive that I was unable to ride a bike or wear certain pants due to the large seams in the pelvic region. I am still unable to use tampons during my menstrual cycle.

Support for survivors

Ultimately, the psychological and emotional impact of this trauma has proved to be the most challenging aspect. For those coping with the effects of SJS or TEN, be sure to find a support group! I attended an international SJS/TEN conference, where I networked with medical professionals, met other survivors, and found support through SJS Canada and the Stevens Johnson Syndrome Foundation, which is making an incredible difference in my journey of healing.

  1. Heymann WR. SkinSerious: Disseminated Intravascular Coagulation Complicating Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis. J Am Acad Dermatol. 2021 Jan 27:S0190-9622(21)00229-2. doi: 10.1016/j.jaad.2021.01.072. Epub ahead of print. PMID: 33515629.

  2. El-Azhary RA, Nowsheen S, Gibson LE, DiCaudo DJ. Disseminated intravascular coagulopathy: a complication of Stevens-Johnson syndrome/toxic epidermal necrolysis. Int J Dermatol.2021; 60: 185-189. doi: 10.1111/ijd.15370. PMID: 33332598.

  3. Lee HY, Walsh SA, Creamer D. Long-term complications of Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN): the spectrum of chronic problems in patients who survive an episode of SJS/TEN necessitates multidisciplinary follow-up. Br J Dermatol. 2017 Oct;177(4):924-935. doi: 10.1111/bjd.15360. Epub 2017 Sep 22. PMID: 28144971.

  4. Cabañas Weisz LM, Miguel Escuredo I, Ayestarán Soto JB, García Gutiérrez JJ. Toxic epidermal necrolysis (TEN): Acute complications and long-term sequelae management in a multidisciplinary follow-up. J Plast Reconstr Aesthet Surg. 2020 Feb;73(2):319-327. doi: 10.1016/j.bjps.2019.07.015. Epub 2019 Aug 8. PMID: 31481319.

  5. Schwartz RA, McDonough PH, Lee BW. Toxic epidermal necrolysis: Part II. Prognosis, sequelae, diagnosis, differential diagnosis, prevention, and treatment. J Am Acad Dermatol. 2013 Aug;69(2):187.e1-16; quiz 203-4. doi: 10.1016/j.jaad.2013.05.002. PMID: 23866879.

  6. Lian SB, Oh CC, Yeo YW, Lee HY. Spectrum of Nail Sequelae in Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis. JAMA Dermatol. 2021 Jan 1;157(1):117-119. doi: 10.1001/jamadermatol.2020.4664. PMID: 33295942; PMCID: PMC7726697.

  7. Wanscher B, Thormann J. Permanent anonychia after Stevens-Johnson Syndrome. Arch Dermatol. 1977 Jul;113(7):970. PMID: 879819.

  8. Mashima E, Sawada Y, Inoue A, Saito-Sasaki N, Yamaguchi T, Yoshioka H, Ohmori S, Haruyama S, Yoshioka M, Okada E, Nakamura M. Alopecia Areata-like Hair Loss Accompanying Toxic Epidermal Necrolysis. Acta Derm Venereol. 2018 Oct 10;98(9):906-907. doi: 10.2340/00015555-2982. PMID: 29856464.

  9. Gelfer A, Rivers JK. Long-term follow-up of a patient with eruptive melanocytic nevi after Stevens-Johnson syndrome. Arch Dermatol. 2007 Dec;143(12):1555-7. doi: 10.1001/archderm.143.12.1555. PMID: 18087007.

  10. Balić A, Pavičić B, Marinović B, Jurakić Tončić R. Atypical Nevi in a Patient After Toxic Epidermal Necrolysis. Acta Dermatovenerol Croat. 2018 Jun;26(2):183-185. PMID: 29989878.

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