 Hello everyone, welcome back to another session on dentistry and more. So let's continue our syndrome. So last class, we had seen few syndromes. So today's class is about Stevens-Johnson syndrome and Papillon-Liefer syndrome. Stephen-Johnson syndrome, it is also known as Toxic Epidomolysis Necrosis or Lial syndrome. So these two should be studied together. So let's see what is Stevens-Johnson syndrome and Papillon-Liefer syndrome. So let's begin with Stevens-Johnson syndrome. So we should study Stevens-Johnson syndrome along with Lial syndrome. It is also known as Toxic Epidomolysis Necrosis because the both having same clinical presentation differs only with the severity of clinical presentation that is the skin reactions. So it is an immune complex mediated hypersensitivity reaction. So this is a hypersensitivity reaction to certain dregs or certain infections and it is a severe expression of erythema multiforme and it is also known as erythema multiforme major. So we should know what is erythema multiforme. So erythema multiforme is basically a skin immune reaction due to an infection or medication. Its name comes from erythema multi-anformae. Erythema means redness, multi is many and formae is shapes. So it describes the main symptoms which is a rash on the body. So the rash on the body where each mark resembles a bullseye form. So it is a severe form of erythema multiforme it is also known as erythema multiforme major. So the basic etiology is infection. It could be a herpes simplex virus infection, cytomegalus virus infection, it could be due to the AIDS or Epstein bar virus infection and it could be due to drug induced. That is the main reason that is the penicillin drug induced reaction is 2 third of total cases of Stephens-Johnson syndrome and also it could be due to finitoin, NSAIDs or allopurinol. So these drugs can result in this hypersensitivity reaction and also it could be an idiopathic reaction. So it is nothing but an immune complex mediated hypersensitivity reaction. So we know classifications of hypersensitivity 1, 2, 3 and 4. So this is immune complex mediated hypersensitivity. So there will be always a causal factor that is either infection or a drug or it could be an idiopathic in nature. So it is also known as erythema multiforme major because clinical presentation is all same because it is forming erythematous reaction on the skin surface. And the risk factors include the males having more predilection compared to the female almost double because it is 2 is to 1 ratio we can see. So in risk factors the second one is age. It is most commonly seen in 20 to 40 years that is the middle age people are more affected with this Stephens-Johnson syndrome. So what are the clinical features? It is most commonly affecting the surface that is skin and mucus membranes are involved. Most commonly the oral, nasal, eye, GI tract, respiratory tract, urethral tract. So all these surfaces are involved with this muco, skin and mucus membrane. And also we can see sore throat, chills, malaise and fever associated symptoms with Stephens-Johnson syndrome. So it is like muco-cutaneous lesions they develop abruptly and clusters of outbreaks which last from 2 to 4 weeks and the lesions are typically non-pruritic. And fever will be there in almost 85 percentage of the cases and involvement of oral and mucus membrane may be severe enough that patient may not be able to eat or drink. And also there is conjunctivitis and patients with genital urinary involvement may complain of dysuria or an inability to void. So these are the basic clinical features and the next thing is if the basal body surface area involvement is less than 10 percentage we can say that it is a minor form of toxic epidermolysis necrosis or you can say that Stephens-Johnson syndrome is now very much void and the basal surface area is 10 to 30 percentage it is a combination of both Stephens-Johnson syndrome and toxic epidermolysis necrosis and if the basal surface area is greater than 30 percentage it is toxic epidermolysis necrosis. So that is a severe form of Stephens-Johnson syndrome. So basically what happens in this is there is a death of keratinocytes which causes separation of dermis from epidermis that is why the skin changes are seen. The keratinocytes which connects the dermis and epidermis is dying of then there is a separation of dermis from epidermis and what are the complications associated with Stephens-Johnson syndrome. So the complication includes esophageal strictures, renal failure, respiratory failure and also there might be scarring and deformity of face. So the esophageal strictures, renal failure and respiratory failure on extensive cases and also scarring and deformity due to this particular skin lesion. And regarding the investigations there is no laboratory studies other than biopsy exist which can aid the doctor in establishing the diagnosis. Basically the skin biopsy is a definitive diagnosis because we can see that bulle are sub epidermal and epidermal cell necrosis may be noted. So these are the pathological features and while coming to the treatment and management basically only symptomatic treatment is possible. So it is mostly dealt just like how it is in the extensive burns. So it is almost like an extensive burn case but the cause is little different. So that's all about Stephens-Johnson syndrome. It is an immune complex mediator disease which is an extreme form of erythema multiforma. So etiology could be infections and drugs and idiopathic nature and it involves skin and mucus membranes of various organs like oral cavity, nasal cavity, eye gastrointestinal and respiratory tract and it is associated with toxic epidermalysis necrosis if it is greater than 30 percentage and there is a death of keratinocytes that is why this is separated that's dermis and epidermis and the complications and treatment. So now let's move on to the papillone-leve syndrome. It is also known as parmoplantar keratoderma with periodontitis. So as the name suggests it has involvement of keratinization on palms and plantar region and also it associated with severe bone destruction that is the alveolar bone. So we can say that it is a disease with periodontitis and keratinization in the palms and plantar region. So it is a autosomal recessive region or recessive disorder. So it is an immune complex hypersensitivity. So when you are studying syndromes always study in 2 or 3 syndromes together. So you never get confused if you are studying one syndrome at a time the high chances of you mixing up the clinical features and the course with another one. So always study the syndromes 2 or 3 at a time that's why I am keeping the syndromes in a single board with 2 or 3 syndromes. So you always keep comparing the diseases and studying. So it will be in your memory for a very long time. So always compare and study not just one syndrome at a time. So 2 or 3 syndromes take at a time and study and compare the course, the clinical features, the manifestations and the treatment. So it will be very easy and it will be remembering for a very long time. So this is a autosomal recessive disorder and it is a disorder of keratinization. So what happens is there is a thickening of soles and palms. So severe keratinization causing thickening of soles and palms and also severe destruction of periodontal bone. So severe periodontitis is there. So while it is happening it is due to the mutation in cathepsine C gene. So that is a particular gene which is involved with this syndrome. So there is a mutation and causing this syndrome. So what are the clinical features? From the name itself you know that there is keratinization in palmar and plantar region and also periodontitis. So gingiva, stomatitis, periodontitis and swollen gingiva, extreme resorption of bone and deep pockets. So these are the periodontal manifestations. So thickening of soles of palm and plantar region. So the patient has premature loss of deciduous teeth and permanent teeth. So deciduous teeth it is exfoliated completely by the age of 10 to 12 years. That is a molars, deciduous molars replaced by primolars around 10 to 12 years. But in this case what happens is by age of 4 to 5 years complete tooth is lost. That is deciduous tooth is completely lost by age of 4 to 5 years. And if it is permanent teeth it could be completely gone by the age of 14 or 15 years. That is supposed to be for a lifetime it is completely lost because of severe periodontitis that is the extreme resorption of bone and deep pockets. So the teeth will be completely lost and the gingiva will back to its normal shape. So it is a very weird condition the loss of deciduous teeth. And skin lesions will be there white, brown, red or scaly in nature. So what happens is these types of lesions undergo crustacean cracking and deep fissuring. So the hand and foot region will undergo the crustacean cracking and deep fissuring. And also we can see follicular keratosis, hyperhidrosis, calcification of foccerbrae and choroid plexus. So these are the other features which is seen with this syndrome that is follicular keratosis, hyperhidrosis, calcification of foccerbrae and choroid plexus. So what are the histopathological features is hyperkeratosis, hypergrandelosis and acanthosis. So basically we treat this disease mainly we treat the periodontitis that is a infectious in nature that is scaling and root planning we can perform with antibiotics and retinoids and good oral hygiene by providing him continuous chloraxidine mouthwash. In case of unsavable tooth we can go for extraction and provide him a rehabilitation with removal dentures or complete dentures or even with implants. So that is about papillol leafy syndrome it is also known as parmo plantar keratoderma with periodontitis. So it is a autosomal recessive disorder of keratinization which causing tooth loss and parma and plantar keratinization that is papillol leafy syndrome Stephens Johnson syndrome is a different one it is also known as toxic epidermalysis necrosis or loyal disease in its severe form. Stephens Johnson syndrome is itself a severe expression of erythema multifirma so it starts with erythema multifirma then Stephens Johnson syndrome and toxic epidermalysis necrosis the severity is increasing. So that's all about Stephens Johnson syndrome and papillol leafy syndrome so we have more syndromes coming up in the further sessions so so far we are covered Frey syndrome, Gorlin got syndrome, Plummer Winston syndrome and Downe syndrome Stephens Johnson and papillol leafy syndrome so few more syndromes are left so I'll come up with those syndromes in my next sessions. Thank you.