Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) in Patients with Darker Skin

00:00 Welcome to our lecture on Stevens-Johnson syndrome and toxic epidermal necrolysis.

00:07 SJS/TEN. Stevens-Johnson syndrome and toxic epidermal necrolysis.

00:15 The rare, acute, serious, and potentially fatal skin reaction characterized by extensive necrosis and detachment of the epidermis.

00:26 They are classified based on percentage of the affected skin body area affected.

00:32 If less than 10% of surface body area is involved, then we call it Stevens-Johnson syndrome.

00:39 Stevens-Johnson syndrome.

00:40 Tn overlap includes all lesions that take up to 10 to 30% of surface body area.

00:47 If lesions cover more than 30% of surface body area, we call it toxic epidermal necrolysis or TEN .

00:57 It's a rare condition.

00:58 5 to 6 cases per million per year are seen.

01:01 The female to male ratio is 2:1 , meaning it's more common in females.

01:07 The risk factors include HIV infection, and in patients with HIV, there's a 12-fold increase in the risk of Stevens-Johnson syndrome or TEN.

01:17 The second risk factor is connective tissue diseases.

01:20 And here there's a twofold increase in risk.

01:24 Malignancy is another risk factor.

01:27 And it's 30 to 60 times higher than in the general population.

01:34 Age also is a risk factor with a high incidence in older patients.

01:41 Ethnicity is also another risk factor, with 2 to 3 times more common prevalence in people of African and Asian descent.

01:52 SJS/TEN is predominantly drug-specific T cell-mediated reaction, specifically type IV hypersensitivity.

02:02 The following are some of the drugs that are usually implicated in patients who develop Stevens-Johnson syndrome or toxic epidermal necrolysis.

02:14 Clinically, the onset occurs within a few days, up to two months, depending on the type of drug that is causing the problem.

02:24 One experience is a prodromal illness, which is a several days before the rash.

02:31 It's more like a flu-like illness with high fever, sore throat, runny nose, and cough.

02:40 The early lesions can mimic exanthematous morbilliform rash.

02:45 They are ill-defined .

02:47 They coalesce into erythematous macules, and of course, in dark skin, the erythema may be perceived as dusky or plum-like lesions.

03:02 The later lesions a patient may present with painful, extensive shit like detachment and erosions.

03:10 And we call this Nikolsky's sign if you put pressure on the skin and then it peels off.

03:17 The scalp is typically unaffected.

03:21 Late lesions also involve peeling of the palms and soles and painful, edematous erythema. Mucosal involvement is also important in these patients, and this may involve the lips, the tongue, the mouth.

03:40 Sometimes the involvement of the eyes.

03:44 In the eyes one sees conjunctivitis, corneal ulceration.

03:48 The pharynx, it may make it difficult to eat and cause dysphagia.

03:54 And the genital area and urinary tract one m ay see erosions, ulcers, and urinary tract retention.

04:02 The complications involve the following fluid imbalance, infections, acute respiratory distress syndrome, and GI side effects. This may also result in shock and multiple organ failure.

04:18 Thromboembolism and DIC has also been seen in these patients.

04:25 The diagnosis is based on the history and of course, the clinical findings.

04:31 The laboratory investigations that are usually undertaken are a full blood count, coagulation studies, protein, albumin, and of course, bacterial and fungal cultures, because these patients have an impaired skin barrier due to the denuded skin.

04:49 The skin biopsy shows full-thickness necrolysis, that is, separation of the epidermis at the dermoepidermal junction.

04:59 The differential diagnosis includes Exa-Morbilliform exanthematous rash, staphylococcal scalded skin syndrome and bullous pemphigoid.

05:15 We're now moving on to management of patients with Stevens-Johnson syndrome and TEN.

05:22 As this is a life-threatening condition, patients almost always require hospitalization. It is crucial that we discontinue the suspected drug or give replacement alternative drugs.

05:37 Nutritional and fluid replacement is mandatory.

05:41 Temperature maintenance is also crucial as these patients are can easily develop hypothermia. Pain relief is something that we sometimes forget, and we need to make sure that patients are comfortable and do not experience any pain.

05:58 The patient may need ICU care depending on the severity of the disease and associated comorbidities. Co-management with the internal physician is usually used for.

06:12 General skincare involves using topical antiseptics and gentle dressings, gauze with petrolatum.

06:20 This can also help to reduce pain, especially if systemic treatment is still not established. A variety of systemic immunosuppressive or immunomodulating agents have been utilized for the treatment, but the evidence is limited.

06:40 Always discuss these patients with your physicians and make sure that if there are crucial drugs that need to be used, an alternative drug is recommended for the patient. The overall mortality rate is approximately 25%, and the risk factors for poor prognosis are as listed at the age, the extent of skin detachment, presence of comorbidities, the severity of mucosal involvement, and the systemic complications.

07:12 The likelihood of recurrence is unknown in these patients.

07:18 There are long term sequelae if the patients are not treated early enough or the complications have not been aborted, and these are common, affecting more than 80% of patients.

07:30 Post-inflammatory hypo or hyperpigmentation is a problem.

07:36 I've actually had a patient who had Stevens-Johnson syndrome from the use of, um, allopurinol and the whole skin was depigmented.

07:46 It was really challenging to manage this patient.

07:48 It was very difficult to stimulate the hypopigmentation and stimulate the melanocytes. So this is something that can really affect the patients.

07:59 Scarring can also be a problem sometimes leading to keloids and at times things like ectropion a chronic pruritus can also be a challenge in these patients.