ACUTE BLISTERING AND EXFOLIATIVE SKIN

ACUTE BLISTERING AND EXFOLIATIVE SKIN Nyoman Suryawati

Bagian/SMF Ilmu Kesehatan Kulit dan Kelamin FK UNUD/ RSUP Sanglah DenpasarEmergency In Dermatology1DefinitionA serious situation or occurrence that happens unexpectedly and demands immediate actionA condition of urgent need for action or assistance : a state of emergeny.

2Some potential emergent dermatologic disease:

Stevens- Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN) Staphylococcal Scalded Skin Syndrome (SSSS)

3Erythema Multiforme (EM)An acute self limited-disease, usually mild, and often relapsing mucocutaneus syndrome Related to an acute infection, most often a recurrent Herpes Simplex Virus (HSV) infectionClinical characteristics : target-shaped plaques predominant on the face and extremitiesOccurs in patients all ages, but mostly in adolescences and young adultThere is a slight male preponderanceEM is recurrent in at least 30% of patients4Erythema 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 involvement5Erythema Multiforme (EM)..Etiology : Infection : viral (HSV), bacterial (M. pneumoniae) Immunization Drugs (rare)Clinical manifestation :Prodromal symptoms: upper respiratory infection (cough, rhinitis, low grade fever)skin rash occur in a symetric, acral distribution on the extensor surfaces of the extremities (hands and feet, elbows, and knees), face and neckFirst appear acrally and then spread in a centripetal manner

6Typical target lesion consist of at least 3 concentric components: a dusky central disk or blister more peripherally, an infiltrated pale ring an eryhtematous haloAn atypical target lesion consist of 2 rings Mucous membrane lesion often limited to oral cavity Eye involvement begins with pain and bilateral conjunctivitis

7Erythema Multiforme (EM)..

Erythema Multiforme.8

Erythema Multiforme.9

Typical target lesions on the palm

Multiple concentric vesicular rings (herpes iris of Bateman)Erythema Multiforme.10

The aim of treatment are to reduce the duration of fever, eruption, and hospitalizationM. pneumoniae infection : antibiotics (macrolides in children, macrolides or quinolone in adult)Liquid antacids, topical glucocorticoids, local anesthetics relief symptoms of painful mouth erosion

11Erythema Multiforme.Stevens- Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN)SJS and TEN are acute life threatening mucocutaneous reactions characterized by extensive necrosis and detachment of epidermisThese 2 conditions represent severity variant of identical process that differs only in the percentage of body surface involvementOccurs in patients all ages, with the risk increasing with age after the fourth decadeMore frequently affects womenThe overal mortality is 20-25 % : SJS (5-12%), TEN (>30%)

12Clinically begins within 8 weeks (4-30 days) after the onset of drug exposureNon specific symptoms : such as fever, headache, rhinitis, myalgias may precede the mucocutaneous lesion by 1-3 daysPain on swalloling and burning or stinging of the eyesCourse is much more prolonged and severe than erythema multiforme minorIn a review stomatitis (100%), ocular involvement (86%), genital mucosal or urethral involvement (41%)

13SJS and TENThe eruption is initially symetrically distributed on the face, the upper trunk, and the proximal extremitiesThe initial skin lesions : erythematous, dusky red, purpuric macules, irregularly shaped, which progressively coalesceConfluent of necrotic lesions leads to extensive and diffuse erythemaNikolsky sign (dislodgement of the epidermis by lateral pressure) : + on eryhtematous zoneThe lesion evolve to flacid blister which spread with pressure and break easily

14SJS and TENPatients 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% BSAMucous membrane involvement (at least 2 site): 90% casesIt begins with erythema followed by painful erosions of the bucal, ocular, and genital mucosa 85% with conjuctival lesion : hyperemia, erosions, chemosis, photophobia, lacrimation

15SJS and TENEtiopathogenesis: Cell mediated cytotoxic reaction

The 4 etiologic categories are; 1. infectious 2. drug-induced 3. malignancy-related 4. idiopathic. Infectious diseases that have been reported include herpes simplex virus (HSV), influenza, mumps, cat-scratch fever, mycoplasmal infection, lymphogranuloma venereum (LGV), histoplasmosis, and cholera. In children, Epstein-Barr virus and enteroviruses have been identified.

16SJS and TENMedication and the risk of epidermal necrolysisHigh risk : Sulfamethoxazole, allopurinol, sulfadiazine, sulfapyridine, sulfadoxine, sulfasalazine, carbamazepine, lamotrigine, phenobarbital, phenytoin, phenylbutazone, nevirapine, oxicam NSAID, thiacetazone

Lower risk:Acetic acid NSAID (diclofenac), aminopenicillins, cephalosporins, quinolones, cyclins, macrolides

SJS is idiopathic in 25-50% of cases.

17SJS and TEN

18

SJS and TENNikolsky sign19

SJS and TEN

Erythema multiforme: typical targetsB. Stevens-Johnson syndrome: Confluence of individual lesions remains limited, involving less than 10% of the body surface area. C. Overlap Stevens-Johnson syndrometoxic epidermal necrolysis detachment of the epidermis and erosions on 10% to 29% of the body surface area. D. Toxic epidermal necrolysis: widespread detachment of epidermis on more than 30% of the body surface area. 20Patients with SJS and TEN then should be treated with special attention to airway and hemodynamic stability, fluid status, wound/burn care, and pain control hospitalizationTreatment of SJS and TEN is primarily supportive and symptomatic Fluid Replacement Sterile technique Manage oral lesions with mouthwashes. Areas of denuded skin must be covered with compresses of saline solution. Consultation eye , ENT, internal department

21SJS and TENUnderlying diseases and secondary infections must be identified and treated. Offending drugs must be stopped. Treatment with systemic steroids (MethylPrednisolon, Cortison, Dexametasone) controversion life saving drug

22SJS and TEN

23SJS and TENThe performance of the score is at its best on day 3 of hospitalizationSyn- Ritters disease or Pemphigus neonatorumInduced epidermolytic exotoxins (exfoliatin) A and B, released by S. aureus and cause detachment within the epidermal layer throgh damage of desmosomeDesmosomes are the part of the skin cell responsible for adhering to the adjacent skin cell. The toxins bind to a molecule within the desmosome called Desmoglein 1 and break it up so the skin cells become gap intra epidermal.24Staphyloccal Scalded Skin Syndrome (SSSS).The syndrome begins by fever and diffuse erythema. Large flaccid bullae with clear fluid form and rupture almost immediately.Characterised by red blistering skin that looks like a burn or scaldSSSS has no initial target lesions, the erosions are more superficial and less weepy, oropharyngeal lesions are rareSkin biopsy is helpful intraepidermal separationThis condition associated with high mortality is seen commonly in infants and children.

25SSSS.26

Nikolsky signgentle pressure to the skin of the arm has sheared off the epidermis, which folds like tissue paper.

SSSS.Can be differentiated from TEN by a Tzanck testIn SSSS, the blister cleavage plane is intraepidermalIn TEN, separation is seen below the basement membrane (in the upper dermis)

27SSSS.Requires hospitalisationintravenous antibiotics are generally necessary to eradicate the staphylococcal infection. A penicillinase-resistant, anti-staphylococcal antibiotic such as flucloxacillin is used. Depending on response to treatment, oral antibiotics can be substituted within several days. 28SSSS.Other supportive treatments include: Paracetamol when necessary for fever and pain. Maintaining fluid and electrolyte intake. Skin care (the skin is often very fragile)

29SSSS.SJSTENSSS20-40 yrUncommon in young childrenCommon in infant and childrenEtiologyHypersensitivity reaction (drug)Hypersensitivity reaction (drug)Infection S. aureusClinical FeatureSkin, > 2 mocous membarane, target lesion10 % body surfaceConfluent morbiliform eruption, blistering skin exfoliation>30 % body surfaceRed blistering skin, scalded, look like burnBullaSubepidermalSubepidermalIntraepidermal PatogenesisCell mediated cytotoxic reactionCell mediated cytotoxic reactionExotoxin Staphyloco. aureusMortality10 %20-40 % (severe variant of SJS)< 3 %TherapyFluid n Electrolyte, CorticosteroidFluid n Electrolyte, CorticosteroidAntibiotic

30Thank You3131Learning TaskCase 1

A male, twenty years old, come to emergency room Sanglah hospital with itchy rash all over the body. There were history of fever, malaise, sore throat 5 days before, and took some medicine such as amoxicillin and paracetamol 2 days before the rash.

32General condition is weak, compos mentis, good nutritional status, blood pressure 120/80 mmHg, temperature 39C, heart rate 80x/minute, 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.33Learning 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 the patien to prevent the same reaction?34Self Assesment

Explain the patomechanism of the caseDiscuss about complication of the caseExplain the prognostic of the case

35Case IIA baby, 2 month old, come to dermatology polyclinic, Sanglah hospital with peel of skin on the neck since 2 days ago.There were history of fever and cough 4 days before and wound around the nose since 1 day before the skin problem. General condition is weak, good nutritional status, temperature 38,5C, heart rate 120x/minute and respiration rate 20x/minute. Skin examination from neck area, there were eryhematous macule with ill defined margine, some area with peel of skin. From nose area we find multiple erythematous macule covered with honey-colored crusted plaque.

36Learning 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 the patien to prevent the same reaction?

37Self Assesment

Explain the patomechanism of the caseDiscuss about complication of the case

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