Oral Manifestations of Skin Disease PDF

10 LAITH + TALAFHA Husam Alhurani … NOOR AL MAANI Page | 1 LSN ALL, WE WILL TALK ABOUT ORAL MANIFESTATIONS OF SKIN DISEASE Oral mucosa is considered a continuation of the skin, and you might be the first one to diagnose a potentially fatal skin condition by examinin...

10 LAITH + TALAFHA Husam Alhurani … NOOR AL MAANI Page | 1 LSN ALL, WE WILL TALK ABOUT ORAL MANIFESTATIONS OF SKIN DISEASE Oral mucosa is considered a continuation of the skin, and you might be the first one to diagnose a potentially fatal skin condition by examining the oral mucosa. ------------------------------------------------------------------------------------------------- What's the link (between skin and oral mucosa) ? -Shared embryological origin ; both arising from ectoderm ( ectoderm +/endoderm - ) -Shared function: mainly as a barrier against external stimuli. -Mucosa is considered a continuation of the skin. -It could reveal the first manifestation of dermatological condition. -Mucosa is an essential part of dermatological examination. ------------------------------------------------------------------------------------------------- The main difference is that oral mucosa is non-keratinized while the skin is; because of the repeated exposure to the external environment. *How is the oral mucosa different from the skin? -Color. -Moist surface. -Absence of adnexal skin structures such as hair follicles, sweat glands and sebaceous glands (exception in Fordyce's disease) -Fordyce's disease: sebaceous glands in oral cavity predominantly in upper lip , buccal mucosa and alveolar mucosa. -Presence of minor salivary glands in oral mucosa. Page | 2 -Texture of surface: oral mucosa is smoother than the skin ( few exceptions like dorsal tongue due to papillae ; hard palate – rugae ; gingiva – stippling ) -Firmness: Oral mucosa varies in firmness. For example, buccal mucosa and lips are loose and pliable whereas the gingiva and hard palate are firm so critical clinically while giving injections. ------------------------------------------------------------------------------------------------- -Manifestations of cutaneous diseases are divided into congenital and acquired. Also, can be divided into pigmented, white lesions, red lesions, swellings, ulcers, blisters and those with teeth anomalies. Importance of those manifestations? -Common -Serious -Could be the first manifestation -Associations (Cross referrals); it's a multidisciplinary approach, there is team work, dermatologists refer a lot to dentists and maxillofacial colleagues and vice versa. ------------------------------------------------------------------------------------------------- 1-Lichen Planus -A chronic disease that is itchy and characterized by the 5 p's: papules that are violaceous (darker than pink; purple ) , polygonal , planar ( flat-topped ) and pruritus. Notice that the papules have coalesced here to Page | 3 form like a plaque. -They occur on the external surfaces , mainly on the wrist. -You can see the lacy white surface and these are what we call Wickham’s striae. -Patients often come to the clinic with an itchy eruption, this can't only affect the skin but also nails, genital mucosa and the scalp leading to scarring alopecia where there is permanent loss of the hair. -The fifth place that we usually examine for lichen planus is the oral mucosa. -The importance of this is that if we diagnose the disease from oral mucosa: A- it may prompt you to refer the patient to dermatology clinic, and to ask the patient about other areas that are known to have involvement in the disease (for example: skin, genital and scalp) and by asking about the scalp you could be the first one to alert the patient that this could be a scarring disease and if left untreated, he could suffer permanent loss of all hair. B- if it's left untreated and the genital and oral mucosae were involved, particularly the severe cases, there could be dysplastic changes and involvement into squamous cell carcinoma. This is the white lacy appearance that we typically see on the skin surface (Wickham’s striae), these are very reminiscent of what we see in the oral mucosa (lips in left pic). Page | 4 ✓ It affects 0.2% of the population. ✓ 25% skin and oral LP-erosive, lichenoid (quarter of the patients will have oral involvement and this can be either lichenoid or erosive type with gingivostomatitis. ✓ If it's very severe in the mucosa not the skin, there could be some associations with HCV, amalgam, GvHD (graft versus host disease). ✓ Drugs can trigger or exacerbate it: B-blockers, calcium channel blockers, NSAID, ACE-inhibitors. ✓ Most affected sites: buccal mucosa and lateral tongue. ✓ Symmetrical ✓ Lasts longer than skin lesions (stubborn, persistent) (lichen planus of the skin lasts usually between 3-5 years while lichen planus affecting oral mucosa lasts longer). ✓ Can be asymptomatic, so if we find a patient with lichen planus of the skin, we always examine the oral mucosa and refer the patient to oral medicine department if any changes were detected. -Here we can see the clinical presentation of lichen planus in the oral cavity, notice the Wickham’s striae, erosive type (second& last pictures) -Third picture: typical appearance “snail-track appearance”. ‫ صفحة‬30 ‫ بلينك عشان ما يصير الشيت‬:P https://drive.google.com/drive/folders/1v7H-_Hejo_Wnqi3sHcP0W1Dc_NqsmCOB?usp=sharing Treatment The main form of treatment is with steroids, and this can be done as mouth rinses 2-3 times a day depending on the severity, if it's mild this might be enough > Chlorhexidine mouth wash, Betnesol mouth rinses 0.5 mg in 10 ml Page | 5 Sometimes we use Oral steroids + hydroxychloroquine and even immunosuppressive drugs such as Azathioprine. Importance ▪ Associated scarring (in the oral, genital mucosae and scalp) ▪ Risk of malignancy x50 ▪ Related to amalgam/HCV/drugs (can lead us to some associations) ▪ Associations: skin, vulva, eyes, ears (further assessment) ------------------------------------------------------------------------------------------------- 2- Pemphigus Vulgaris -Is a bullous disease, there is formation of blisters and it's immune- bullous disease; meaning there is an autoimmune phenomena, body forms Abs against skin antigens; this leads to fragility of the skin and formation of blisters. -We can see here the typical appearance of the disease. -This usually happens on the trunk, chest, back, face and perhaps on the scalp. - Characteristically, the blisters are soft and the proper term is flaccid; meaning that they can rupture easily. Page | 6 -That's because the Ab's are targeted towards superficial antigen within the epidermis not deep. -Most often, we see that the blisters have ruptured because they are flaccid, so it's uncommon to see intact blisters. There could be an involvement of the oral mucosa with erosions, also the gingiva, tongue mucosa of the eye and genital region. Importance -Associations: eye, skin, genital. -Paraneoplastic pemphigus: desmoglein 1 and 3 are the targeted antigens in this disease but sometimes, this pemphigus is paraneoplastic, so if we have an older patient who has this disease, we should always ask Page | 7 about the review of body systems and make sure that this patient does the screening tests that are appropriate for his/her age and gender like colonoscopy, mammograms, cervical smears…) because Ab's can target different antigens in this group, so we should always consider this paraneoplastic phenomena particularly in older patients with severe disease. Now, we are going to talk about abnormal teeth and the relationship to skin disease. Because we know that there is a shared ectodermal origin; whenever we have abnormal hair, we always ask about the skin, nails and teeth. Here, we can see conical teeth with abnormal sparse hair. He could have abnormal digits also. This should alert us about an ectodermal problem and in this case it's called ectodermal dysplasia (genetic skin disease that has various forms and genes), so whenever you see abnormal teeth, ask about hair, nails and skin. ‫نختم البارت األول بأبيات شعرية للشاعر اياد طنوس‬ ‫ ببقي علي طول حدو‬... ‫قلبي تعبان بدو‬ ‫ انت و مبسوووط‬.... ‫قلبك الي ردو‬ Page | 8 This is another genetic skin disorder called Epidermolysis Bullosa (EB), in this disease there is complete absence or abnormality of Collagen VII, Collagen VII is like the glue between epidermis & dermis, if it’s weak or absent then these two layers will easily come off each other causing a blister, this blister is usually deep in the skin unlike pemphigus, and usually seen as an intact blister, but this usually sloughs and the area becomes eroded. There is no cure for this disease, any disease where there is ulceration, healing then scarring there is always a risk of Squamous Cell Carcinoma, so in the most severe forms of EB (of the dystrophic type), patients usually develop SCC and die before the age of 50. There are many type of EB depending on the level of split in the skin, if it’s Superficial it’s called EB simplex, it may be Junctional (between the epidermis & dermis), or Dystrophic if it’s below the basement membrane, each type has many forms that can range between mild and severe. Page | 9 This is the Dystrophic type, where the problem is in Collagen VII, the blister is below the basement membrane, patients are born with skin fragility, the skin coming off in an area where maybe a hand has held the baby, a shearing force or rubbing will remove the skin. EB does not only affect the skin, it can also affect the mucosa, in the esophageal image there is narrowing of the esophagus, there is scarring of the eyes and there are erosions inside the mouth; the mucosa is fragile so the patient cannot eat hard foods (hard bread, chips etc.) in the most severe cases because it will shear the mucosa so they learn to eat softer foods and to chew more (to emphasize, this is in the most severe cases) Page | 10 The hospital where the Dr. trained had one of the biggest services for EB, she had a chance to work with many children with genetic skin diseases and they worked in close contact with patients with oral manifestations and even one of the doctors there had qualifications in both dermatology and oral medicine, so it was useful to have that input… A paper the dr. wrote about EB and its oral involvements, Oral Aphthae This is a very common condition, a lot of us have had it, and usually it resolves quickly, it’s short-lived and the ulcers are usually superficial, they last maybe a few days, usually they are of no consequence and they can come up at times of stress. We can see here that they are small-sized and superficial. Page | 11 We should always keep in our minds the diagnosis of Behcet disease, there are major & minor criteria, there may be oral, genital or neuro-ocular involvement, as well as dermatological (major criteria). This is pathergy at the site of needle penetration Page | 12 Oral ulceration that can occur with Behcet Importance Associations: (we have to look out for:) − Genital ulcers − Ocular manifestations − Aneurysms (vascular enlargement) − Coagulopathy − GI/CNS involvement This is something to be aware of called Orofacial Granulomatosis, in this condition the upper lip is more involved than the lower lip, there is episodic swelling of the upper lip and sometimes confused with Angioedema but here it feels firmer, and it doesn’t resolve quickly like angioedema, with time it stops being episodic and becomes fixed in this larger shape and on palpation it feels firm, Biopsy shows granulomatous changes. Page | 13 This is a milder orofacial granulomatosis patient. With orofacial granulomatosis we should look out for associations like Inflammatory Bowel Disease, Mycobacterium infections, or other granulomatous diseases that can be associated with changes of the lips, this is a case of Angular Cheilitis where there is dryness and inflammation at the angle of the mouth, sometimes can be seen in children perhaps due to lip-licking or in people who breathe through their mouth as they get a lot of dryness, but also we should be aware of bacterial or candidal infections, autoimmune diseases, Iron deficiency and also inflammatory bowel disease. Importance − Rule out Allergens (that can cause irritation, dryness and eczema of the skin) − Differentials: TB, Sarcoidosis (granulomatous diseases), Crohn’s disease, Inflammatory Bowel Disease − Can become permanent (in the case of Orofacial Granulomatosis). Page | 14 More common entities include Erythema Multiforme (can be minor or major) seen here is the typical Targetoid lesions, the top causes of which are infections and drugs, amongst others.. So there is involvement of the skin but also of the mouth, -Acrofacial predisposition on the acral surface of the face (016 sheet) On the Erythema multiforme spectrum we may have Minor EM, Major EM, Stevens-Johnson syndrome (SJS) or Toxic Epidermal Necrolysis (TEN) at the end of the spectrum. SJS (involvement of 10-30% of a body’s surface area as well as oral mucosa). TEN (involvement of more than 30% of the body, eyes and oral mucosa) Page | 15 we can see here there is ulceration, involvement of the eye and erythema At the end of the spectrum we have Toxic Epidermal Necrolysis, there is necrosis of the superficial layer of the skin and eventually the skin sloughs off, this results in loss of the skin barrier, disruption of electrolytes and loss of fluids, if the patient has co-morbidities like heart failure he can lose a lot of fluids and decompensate which could lead to consequences and be fatal, so these patients should be kept in an ICU unit where there is 1 to 1 care. Sloughing of the skin in Toxic Epidermal Necrolysis Page | 16 Involvement of the oral mucosa: SCORTEN A scoring system for the severity of TEN, again the causes are mainly drugs and then infections. Factors associated with worse prognosis (1 point is given for each): Age > 40 Presence of malignancy Heart rate > 120 (tachycardia) Initial percentage of epidermal detachment > 10% Serum Urea level > 10 mmol/L Serum Glucose level > 14 mmol/L Serum Bicarbonate level < 20mmol/L (the dr. said ‘high’ bicarbonate I think she meant the opposite) Prognosis of SJS/TEN depends on the scoring system on the 1st day of hospitalization when patients are firstly seen. Page | 17 Importance − EM- Common (cause is usually infections) − TEN- Fatal (in TEN/SJS cause is mostly drugs) − Need to stop offending drug − Sequelae ie inflammation, blistering then scarring of eyes, nails etc, so it’s important to recognize and treat early. Lupus Erythematosus Systemic Discoid (involves the skin only, can cause scarring in the skin, scarring alopecia and sub-acute lupus) Lupus is a photosensitive condition that usually affects photo-exposed and sun- exposed areas mainly on the face and can cause scarring of the skin and scarring alopecia: Page | 18 Involvement inside the oral mucosa which might not be examined regularly in patients with LE, but we know it’s one of the criteria where they get oral ulcerations, and it’s usually deeper than the regular Aphthae. We can see here how deep it is compared to the usual oral aphthous ulceration. Discoid lesions on the lips Importance Associations: − Scarring − Systemic involvement − Antiphospholipid syndrome (coagulation condition in which patients can get deep venous thrombosis, PE (pulmonary embolism), abortion. Page | 19