Emergency In DermatologyACUTE BLISTERING AND EXFOLIATIVE SKIN Nyoman Suryawati Bagian/SMF Ilmu Kesehatan Kulit dan Kelamin FK UNUD/ RSUP Sanglah Denpasar 1 Definition 1. A serious situation or occurrence that happens unexpectedly and demands immediate action 2. A condition of urgent need for action or assistance : a state of emergeny. 2 Some potential emergent dermatologic disease: 1. 2. Stevens- Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) Staphylococcal Scalded Skin Syndrome (SSSS) 3 and often relapsing mucocutaneus syndrome Related to an acute infection. usually mild.Erythema Multiforme (EM) An acute self limited-disease. but mostly in adolescences and young adult There is a slight male preponderance 4 . most often a recurrent Herpes Simplex Virus (HSV) infection Clinical characteristics : target-shaped plaques predominant on the face and extremities Occurs in patients all ages. Erythema Multiforme (EM)……… Erythema multiforme subtype: erythema multiforme minor : skin lesions without involvement of mucous membranes erythema multiforme mayor : skin lesions with involvement of mucous membranes herpes-associated erythema multiforme mucosal erythema multiforme : mucous membrane lesion without cutaneous involvement 5 . Etiology : Infection : pneumoniae) Immunization Drugs (rare) Clinical viral (HSV). elbows. face and neck First appear acrally and then spread in a 6 centripetal manner . and knees). rhinitis. manifestation : Prodromal symptoms: upper respiratory infection (cough..Erythema Multiforme (EM)……. bacterial (M. low grade fever) skin rash occur in a symetric. acral distribution on the extensor surfaces of the extremities (hands and feet. Erythema Multiforme (EM)……. an infiltrated pale ring an eryhtematous halo An atypical target lesion consist of 2 rings Mucous membrane lesion often limited to oral cavity Eye involvement begins with pain and bilateral conjunctivitis 7 . Typical target lesion consist of at least 3 concentric components: a dusky central disk or blister more peripherally.. Erythema Multiforme………. 8 . Erythema Multiforme………. Typical target lesions on the palm Multiple concentric vesicular rings (herpes iris of Bateman) 9 . 10 .Erythema Multiforme………. topical glucocorticoids. The aim of treatment are to reduce the duration of fever. pneumoniae infection : antibiotics (macrolides in children.Erythema Multiforme………. and hospitalization M. local anesthetics relief symptoms of painful mouth erosion 11 . macrolides or quinolone in adult) Liquid antacids. eruption. Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) SJS and TEN are acute life threatening mucocutaneous reactions characterized by extensive necrosis and detachment of epidermis These 2 conditions represent severity variant of identical process that differs only in the percentage of body surface involvement Occurs in patients all ages. with the risk increasing with age after the fourth decade More The frequently affects women overal mortality is 20-25 % : SJS (5-12%). 12 TEN (>30%) .Stevens. headache. myalgias may precede the mucocutaneous lesion by 1-3 days Pain on swalloling and burning the eyes or stinging of Course is much more prolonged and severe than erythema multiforme minor a review stomatitis (100%).SJS and TEN……… Clinically begins within 8 weeks (4-30 days) after the onset of drug exposure Non specific symptoms : such as fever. ocular involvement (86%). rhinitis. genital mucosal or urethral involvement (41%) In 13 . dusky red. and the proximal extremities The initial skin lesions : erythematous. irregularly shaped. which progressively coalesce Confluent of necrotic lesions leads to extensive and diffuse erythema Nikolsky sign (dislodgement of the epidermis by lateral pressure) : + on eryhtematous zone The lesion evolve to flacid blister which spread with pressure and break easily 14 .SJS and TEN……… The eruption is initially symetrically distributed on the face. purpuric macules. the upper trunk. ocular. erosions. chemosis. photophobia.SJS and TEN……… Patients • • • are classified according to the total of area in which the epidermis is detached : SJS : 10% of BSA SJS/TEN overlap : 10-30% TEN : > 30% BSA Mucous membrane involvement (at least 2 site): 90% cases It begins with erythema followed by painful erosions of the bucal. lacrimation 15 . and genital mucosa ± 85% with conjuctival lesion : hyperemia. infectious 2. malignancy-related 4. influenza. lymphogranuloma venereum (LGV). and cholera. mycoplasmal infection. 1. Epstein-Barr virus and enteroviruses have been identified. 16 . cat-scratch fever. In children. Infectious diseases that have been reported include herpes simplex virus (HSV). idiopathic. histoplasmosis.SJS and TEN……… Etiopathogenesis: Cell –mediated cytotoxic reaction The 4 etiologic categories are. drug-induced 3. mumps. oxicam NSAID. quinolones.SJS and TEN……… Medication and the risk of epidermal necrolysis High risk : Sulfamethoxazole. phenytoin. thiacetazone Lower risk: Acetic acid NSAID (diclofenac). sulfapyridine. cyclins. sulfadiazine. lamotrigine. sulfasalazine. carbamazepine. allopurinol. phenobarbital. phenylbutazone. nevirapine. sulfadoxine. macrolides SJS is idiopathic in 25-50% of cases. aminopenicillins. 17 . cephalosporins. SJS and TEN……… Nikolsky sign 18 . SJS and TEN……… 19 . involving less than 10% of the body surface area. 20 . Overlap Stevens-Johnson syndrome–toxic epidermal necrolysis detachment of the epidermis and erosions on 10% to 29% of the body surface area. Toxic epidermal necrolysis: widespread detachment of epidermis on more than 30% of the body surface area. B. D. Stevens-Johnson syndrome: Confluence of individual lesions remains limited.A. Erythema multiforme: typical targets C. ☻ Consultation eye . ENT. fluid status. and pain control hospitalization ☻ Treatment of SJS and TEN is primarily supportive and symptomatic Fluid Replacement ☻ Sterile technique ☻ Manage oral lesions with mouthwashes.SJS and TEN……… ☻ Patients with SJS and TEN then should be treated with special attention to airway and hemodynamic stability. wound/burn care. internal 21 . ☻ Areas of denuded skin must be covered with compresses of saline solution. ☻ Treatment with systemic steroids (MethylPrednisolon.SJS and TEN……… ☻Underlying diseases and secondary infections must be identified and treated. Cortison. Dexametasone) controversion life saving drug 22 . ☻ Offending drugs must be stopped. SJS and TEN……… The performance of the score is at its best on day 3 of hospitalization 23 . Staphyloccal Scalded Skin Syndrome (SSSS)…………. aureus and cause detachment within the epidermal layer throgh damage of desmosome Desmosomes are the part of the skin cell responsible for adhering to the adjacent skin cell. Syn.Ritter’s disease or Pemphigus neonatorum Induced epidermolytic exotoxins (exfoliatin) A and B. released by S. The toxins bind to a molecule within the desmosome called Desmoglein 1 and break it up so the skin cells become gap intra24 . the erosions are more superficial and less weepy. oropharyngeal lesions are rare Skin biopsy is helpful intraepidermal separation This condition associated with high mortality is seen commonly in infants and25 .SSSS…………. Large flaccid bullae with clear fluid form and rupture almost immediately. Characterised by red blistering skin that looks like a burn or scald SSSS has no initial target lesions. The syndrome begins by fever and diffuse erythema. Nikolsky sign gentle pressure to the skin of the arm has sheared off the epidermis. 26 . which folds like tissue paper.SSSS…………. SSSS…………. Can be differentiated from TEN by a Tzanck test In SSSS. separation is seen below the basement membrane (in the upper dermis) 27 . the blister cleavage plane is intraepidermal In TEN. 28 . Depending on response to treatment. oral antibiotics can be substituted within several days. Requires hospitalisation intravenous antibiotics are generally necessary to eradicate the staphylococcal infection. antistaphylococcal antibiotic such as flucloxacillin is used.SSSS…………. A penicillinase-resistant. Maintaining Skin fluid and electrolyte intake. Other supportive treatments include: Paracetamol when necessary for fever and pain. care (the skin is often very fragile) 29 .SSSS…………. aureus Infection S. > 2 mocous membarane. Corticosteroid Exotoxin Staphyloco. Corticosteroid TEN SSS Uncommon in young children Hypersensitivity reaction (drug) Confluent morbiliform eruption. target lesion 10 % body surface Bulla Subepidermal Patogenesis Cell –mediated cytotoxic reaction 10 % Mortality Therapy Fluid n Electrolyte. aureus Red blistering skin. scalded. look like burn Intraepidermal <3% Antibiotic 30 .SJS 20-40 yr Etiology Hypersensitivity reaction (drug) Clinical Feature Skin. blistering skin exfoliation >30 % body surface Subepidermal Common in infant and children Cell –mediated cytotoxic reaction 20-40 % (severe variant of SJS) Fluid n Electrolyte. Thank You 31 . and took some medicine such as amoxicillin and paracetamol 2 days before the rash. malaise.Learning Task Case 1 A male. come to emergency room Sanglah hospital with itchy rash all over the body. twenty years old. 32 . There were history of fever. sore throat 5 days before. temperature 39°C. blood pressure 120/80 mmHg. 33 .General condition is weak. heart rate 80x/minute. compos mentis. good nutritional status. respiration rate 20x/minute. From skin examination we find purpuric lesion and multiple bullae on the erythematous skin with more than 30 % body surface area. Learning Task What other information should we ask from anamnesis ? What other physical examination we should do? What is the differential diagnosis for the case? Please explain what kind of laboratory examination should we do to perform the diagnosis? What is the diagnosis of the case? How should we manage the case? What information should we give to the34 . Self Assesment Explain the patomechanism of the case Discuss about complication of the case Explain the prognostic of the case 35 . good nutritional status.5°C. heart rate 120x/minute and respiration rate 20x/minute. General condition is weak. temperature 38.There were history of fever and cough 4 days before and wound around the nose since 1 day before the skin problem. come to dermatology polyclinic. some area with peel of skin.Case II A baby.36 . 2 month old. there were eryhematous macule with ill defined margine. Skin examination from neck area. Sanglah hospital with peel of skin on the neck since 2 days ago. Learning Task What other information should we ask from anamnesis ? What other physical examination we should do? What is the differential diagnosis for the case? Please explain what kind of laboratory examination should we do to perform the diagnosis? What is the diagnosis of the case? How should we manage the case? What information should we give to the37 . Self Assesment Explain the patomechanism of the case Discuss about complication of the case 38 .