dermatological emergencies

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DERMATOLOGICAL EMERGENCIES DR Y SRI HARSHA

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common conditions in dermatology which v encounter in a casualty

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DERMATOLOGICAL EMERGENCIES

DR Y SRI HARSHA

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Common dermatological conditions which we encounter in a casualty setting are:

1. STEVENS-JOHNSON SYNDROME2. TOXIC EPIDERMAL NECROLYSIS3. ERYTHEMA MULTIFORME4. ACUTE URTICARIA5. ACUTE ANGIOEDEMA6. ERYTHRODERMA7. DRUG ERUPTIONS8. LEPRA REACTIONS9. PEMPHIGUS10.STAPHYLOCOCCAL SCALDED SKIN SYNDROME

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STEVENS-JOHNSON SYNDROME & TOXIC EPIDERMAL NECROLYSIS

Synonyms: TEN – Lyell’s syndromeDefn: rare, acute, and life threatening mucocutaneous diseases that occur as a consequence of extensive keratinocyte cell death.

Differentiation of SJS from TEN 1. SJS – detachment < 10% of BSA, widespread erythematous or purpuric macules or flat atypical targets.2. Overlap SJS/TEN – detachment between 10% and 30% of BSA, widespread purpuric macules or flat atypical targets.3. TEN – detachment > 30% of BSA, widespread purpuric macules or flat atypical targets

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EtiologyThe factors implicated in the development of the rash are :

1. Drugs ( in more than 95% of the cases)2. Infections 3. Immunization

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PATHOGENESISThe current model for the pathogenesis of SJS/TEN is as follows:

1. upon exposure to certain types of drugs, an individual with particular predisposing factors mounts a specific immune reaction to the drug or one of its metabolites.

2. As a result of an interplay of cell types and cytokines that remains to be totally defined, there is a strong expression of the cytolytic molecule FasL on keratinocytes as well as granulysin secretion from CTLs, NK cells and NKT cells.

3. This leads to FasL- and granulysin-mediated apoptosis of keratinocytes and subsequent epidermal necrosis and detachment

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Stevens-Johnson syndrome-TENClinical features:- usually the rash appears 1- 3 weeks after the intake of the drug

1. Skin: preceded by prodromal symptoms( fever, headache, myalgia)

Starts as an erythematous macule dusky/ purpuric macule (irregularly shaped , discrete in the beginning & later coalesce with on another)

macules flaccid blisters( NIKOLSKY’S SIGN IS POSITIVE )

Necrotic epidermis comes off leaving large areas of red exudative dermis exposed

Sites: starts over the face, upper parts of the trunk and proximal parts of the extremities and rapidly spreads to the rest of the body

(ATYPICAL TARGET LESION)

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2. Mucous membranes: Buccal , ocular and genital mucosa are involved usually more than 1 site is effected in SJS/TEN

Can be seen as Erythema over the mucosa, small vesicles and painful erosions over the mucosa

Often more than 80 % of the patients have conjuctival involvement ( anterior uveitis , synechiae , corneal ulcerations )• Patients may complain of photophobia and burning micturition

3. Other systems :• Respiratory system: breathlessness, hypoxia, hemoptysis• Gastro intestinal tract : diarrhea, malena, esophageal necrosis• Renal system: proteinuria, hematuria, azotemia

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SJSEpidermal detachment in SJS

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Detachment of large sheets of epidermis in TEN

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Hemorrhagic crusts with oral mucosal involvement

Genital mucosal Involvement

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DIAGNOSISThe diagnosis of SJS/TEN is primarily based on the appearance of the skin lesions and their typical symmetrical distribution, especially if known risk factors are present.The following investigations can be done in all patients

• Complete blood count ( normochromic & normocytic anemia & lymphopenia)

• Serum electrolytes • Complete urine examination (hematuria & proteinuria

are indicative of renal involvement)• Blood sugars

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• Liver function tests (SGOT , SGPT are slightly elevated in half of the patients ; or due it frank hepatitis caused by drugs, sepsis or shock)

• Renal function tests ( elevated due to dehydration)• Chest x ray • HIV • Skin Biopsy ( to confirm the diagnosis)• Early lesion shows suprabasal layer apoptotic

keratinocytes.• Later lesion shows full-thickness epidermal necrosis

and separation of epidermis from dermis.

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Apoptotic keratinocytes are present individually and in clusters within the epidermis. Subtle vacuolar changes along the basal layer are accompanied by minimal inflammation, with scattered lymphocytes within the epidermis

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Erythema multiforme majorStaphylococcal scalded skin syndromeAcute generalised exanthematous pustulosisGeneralised bullous fixed drug eruptionBurnsGraft-versus-host diseasePemphigus

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Management 1. Promptly discontinue any and all possible offending drugs 2. Admit the patient in an ICU or burns ward 3. Assess the condition of the patient to determine the prognosis based on the

SCORTEN score

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Correct the fluid & electrolyte imbalance:• Fluid loss and electrolyte imbalance should be closely

monitored and corrected• Peripheral line is more recommendable than central,

which has a higher chance of infection, but good peripheral venous access is difficult to find

• The fluid requirement is calculated based on the parklands formula & 3/4ths of this total amount is given to a pt with TEN

Parkland’ formula = 4 ml/kg body wt × % BSA involved according to the rule of nine• All lines should be checked for signs of infection daily

and changed two times a week with tips of lines and catheters sent for culture

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Nutritional support:• Patient should be allowed to eat a soft, easily

chewable diet if oral feeding is feasible• If oral feeding is not possible, then nasogastric

tube feeding or parenteral feeding can be given • Early and continuous feeding decreases the risk

of stress ulcers, reduces bacterial dislocation, enterogenic infectionTemperature control:

• Should be maintained at 30-32 c to avoid heat loss

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Wound care : • Detached or detachable epidermis should be left in

position as a biological dressing & only the denuded skin be covered with a dressing

• Condy’s compresses or petrolatum impregnated gauzes can be used

• An air fluid bed / water bed may be used to make the patient comfortable

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Ophthalmic care : • 2 hourly instillation of eye drops ( saline or antibiotic

drops )• Apply ointments at night• Developing synechiae are disrupted by a blunt

instrument• ANTIBIOTICS:• Are indicated if there is widespread involvement

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Complications

1. Fluid and electrolyte imbalance

2. Infections ( due to loss of skin barrier)

3. Skin: scarring and dyspigmentation

4. Eye : adhesions blindness

5. Acute renal failure

Mortality rates:6. SJS : <5%7. TEN : 30%

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• SPECIFIC TREATMENTS:1. CORTICOSTEROIDS:• Role of corticosteroids is controversial• Systemic corticosteroids may delay wound healing,

increase the risk of infection, mask early signs of sepsis, and may precipitate gastrointestinal bleeding

• But if the involved area is more than 20% of BSA , then the advantages of treatment outweighs its drawbacks

• Give oral prednisolone (1-2 mg/kg/day) or parenteral steroids ( dexamethasone 8-16 mg daily or hydrocortisone) can be started within the first 72 hours in a patient with limited skin surface involvement to prevent wide spread diffusion, and continue for 3-5 days followed by rapid tapering

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2. CYCLOSPORINE:• It acts by inhibiting the activated T lymphocytes,

macrophages & also interferes with the metabolism of TNF-α and possesses anti apoptotic properties

• Cyclosporine interrupts the disease progression & decreases the time taken for complete reepithelization

• Dose is 3-5 mg/kg/day oral or IV upto 2 weeks followed by weaning over another 2 weeks

• Side effects are hypertension, renal toxicity ( but these effects are not seen with short duration of treatment)

• Watch out septic complications and severe leukopenia

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3. INTRAVENOUS IMMUNOGLOBULINS:• Can be considered if pt is seen within 48-72 hrs

of bulla onset & in cases with active progressing lesions even after 72 hrs

• Total dose is 2 gr/kg , which is given as 0.4 g/kg/day for 5 consecutive days

• Adverse effects are risk of thromboembolism, hemolysis, vasomotor symptoms & anaphylactic reactions

• But the major limiting factor is its high cost

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ERYTHEMA MULTIFORME

Definition: An acute, self limited skin disease characterised by abrupt onset of symmetrical fixed red papules, some of which evolve into typical/ atypical papular target lesions

Epidemiology: more commonly seen in young adults , with more male preponderance , is very uncommon in childhood

Etiopathogenesis: current understanding suggest that EM is most likely , & in most patients, a mucocutaneous manifestation of a distinct skin directed immune reaction that occurs in the setting of an infection in certain predisposed individuals HSV is the most common associated infectious agent

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Clinical features Skin lesions:

The characteristic lesion of EMF is the typical TARGET/IRS lesion• < 3cm in diameter, regular round shape and a well defined

border• Has 3 distinct zones 1. Central dusky zone2. Surrounding paler zone 3. Peripheral erythematous halo Only limited no of fully developed typical target lesions are seen in the patient Also ATYPICAL target lesions may also occur or may be the primary lesion4. Round , edematous , palpable & reminiscent of EMF5. Has only 2 zones & a poorly defined borderSimilar lesions can also be seen in SJS/TEN, but they are flat (macular) target lesions

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Typical TARGET lesions with 3 zones in EMF

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TARGET lesions Koebnerization in EMF

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Sites : commonly on the extremities ( dorsal aspects of hands, forearm, palms , neck, trunk), faceLess commonly over the legs Lesions tend to be grouped.Kobner’s phenomenon is seen

• Mucosal lesions: are limited/ absent in EMF minor, present in EMF major

Vesicobullous lesions painful erosions Seen over the buccal mucosa, lips, ocular & anogenital mucosa

• Systemic symptoms: are limited/ absent in EMF minor, present in EMF major

Fever , arthralgia, pulmonary involvement

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Natural history of the disease:Usually appears within 24 hours

Fully developed and pt complains of pruritus and burning senssation

Within 3 days

Heals without any sequelae

Ocular sequelae may occur if not properly taken care of May result in post inflammatory hypo/hyperpigmentation

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DIFFERENTIAL DIAGNOSIS

UrticariaFixed drug eruptionsSub acute lupus erythematoususErythema annulare centrifugumvasculitis

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HISTOPATHOLOGY• Early lesions:1. Interface dermatitis showing a lymphocytic infiltrate along the

dermoepidermal junction associated with hydropic & dyskeratosis of basal keratinocytes

2. Sparse to moderate lymphocytic infiltrate around the superficial plexuses

• Late lesions:1. Partial to full thickness epidermal necrosis, intraepidermal

vesiculation or sub epidermal blisters 2. Dermal inflammatory infiltrate is characterised by lymphocytes

& macrophages and occasional eosinophils

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Early lesion- focal sites of apoptosis of keratinocytes with an interface dermatitis andvacuolar degeneration of the basal layer (insert). A perivascular lymphocyticinfiltrate is also present

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TREATMENT• Treat the identified precipitating factors • EMF minor – symptomatic treatment is sufficient1. Topical antiseptics for eroded skin lesions2. Antiseptic/anti histamine rinses & local anesthetic lesions for

oral lesions 3. Topical ophthalmic preparations • In HSV associated EMF, supressive therapy can be given for a

period of 6 months in the form of acyclovir ( 10 mg/kg/day) or valacyclovir ( 500-1000 mg/day) or famciclovir ( 250 mg BD)to prevent recurrences

• In severe forms of EMF with functional impairment, systemic corticosteroids (prednisone: 0.5-1 mg/kg/day) can be given

• Severe cases of EMF are to be hospitalised and managed in an ICU or burns ward similar to SJS/TEN

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ACUTE URTICARIADefinition: Urticaria is characterized by recurrent, transient, cutaneous swelling & erythema due to fluid transfer from the vasculature to the dermisTypical lesion seen in urticaria is WHEALUrticaria can be :

1. Acute ( < 6 weeks duration)2. Chronic ( > 6 weeks duration )

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PATHOPHYSIOLOGYUrticaria results from the release of histamine, Bradykinin, leukotriene C4, PG D2, and other vasoactive substances from mast cells and basophils in the dermis These substances cause extravasation of fluid into the dermis leading to urticarial lesion

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ANGIOEDEMADefn: Localized, sudden, transient, and often recurrent swelling of the skin, subcutaneous tissues, or sub mucosa that usually resolves within 72 hours, occurring for <6 weeks, is referred to as acute angioedema It is associated with urticaria in 50% of the casesIt results from interstitial edema from mediators affecting capillary and venule permeability.

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Normal person After developing angioedema

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Angioedema of the eyelids Angioedema of the upeer lip

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Clinical features: acute pale or pink subcutaneous swelling , usually of the face( lips, eyelids, ears, nose) & less often of the extremities and GenitiliaSkin becomes warm, swollen & tenderFrequently a burning sensation is present, but pruritus is typically uncommonThe areas of involvement are frequently unilateral or asymmetricInvolvement of larynx, epiglottis and their surrounding tissue may impair swallowing and lead to upper airway obstruction( stridor )Edema of the intestinal wall leads to abdominal pain, nausea, vomiting & diarrhea( commonly in drug induced AE) Attacks of angioedema may last a few days and usually resolve spontaneously

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Complications of angioedema

Life-threatening

airway blockage

Anaphylaxis

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Approach to a pt with urticaria/angioedemaA comprehensive and detailed history(History should detail exposure to drugs, foods, physical triggers, infection (especially viral hepatitis), occupational exposures, and insect stings or bites.Laboratory investigations that may be done are:

1. A complete blood count with differential counts, ESR2. Urinalysis3. liver function tests4. antinuclear antibody test, anti thyroglobulin and antimicrosomal antibodies5. stool for ova and parasites (in patients with marked

eosinophilia),6. skin testing (for acute urticaria/angioedema with possible food

or venom triggers),7. skin biopsy with immunofluorescence

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Treatment of AngioedemaTreatment depends upon the severity of the condition In many cases the swelling is self-limiting and resolves spontaneously after a few hours or daysThe goals of emergency treatment of angioedema are to prevent spontaneous eruption, to maintain a patent airway if eruption does occur, and to stop progression of diseaseIn cases with laryngeal involvement, a definitive airway, such as an endotracheal tube or nasopharyngeal airway, should be established. If the airway cannot be effectively secured with an endotracheal tube, a surgical airway is indicated, usually in the form of an emergency cricothyrotomy

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H1 anti

histamines

• Diphenhydramine,cetirizine,loratadine,fexofenadine

H1 AND H2 anti

histamines

• Ranitidine, cimetidine

doxepin

• A tricyclic anti depressant• Has both H1 and H2 activity • Sedation limits its use

IV epinephrine (1:1000

)

• Used in pts who demonstrate upper airway obstruction, respiratory failure or shock

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Endotracheal intubation Cricothyrotomy

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Prevention

Avoid irritating the affected area.

Stay away from known allergens.

Never take medications that are not prescribed

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Erythroderma

Presence of erythema and scaling involving more than 90% of skin surface (can be caused by any inflammatory skin condition)

Primary: erythema (often initially on trunk) extends within few days to weeks to involve whole skin surface. Followed by scalingSecondary: generalisation of a preceding localized skin disease (e.g. psoriasis, atopic eczema)

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PathogenesisPathogenesis is still unclearit is believed that the condition is secondary to an intricate interaction of cytokines and cellular adhesion molecules, including interleukins-1, -2, and -8, intercellular adhesion molecule-1 (ICAM-1), and tumor necrosis factor (TNF).These interactions result in a dramatic increase in the epidermal turnover rate

1. Increased no of germinative cells & their absolute mitotic rate2. Shortened transit time of the cells through the epidermis

An increased loss of epidermalmaterial, together with a significant loss of protein and

folate.

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Clinical featuresErythroderma developing in primary eczema or associated with a lymphoma is often of sudden onsetInitially erythema is localised in the pruritic patches before spreading to involve the other body surface area

Within a few days Scaling occurs• Size: fine/large & lamellar ( larger scales in chronic

disease, smaller scales in acute disease)• Color: white- brownSkin becomes bright red, hot & dry (and the patient

may complain of irritation & pruritus and report a feeling of tightness which is characteristic )

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Hair loss ( in 25-30% cases due to scalp involvment, loss of body hair)

Nail changes are common dystrophic, ridged, or thickened or may be shed off

Palms, soles – fissuredVariable lymph node enlargement (dermatopathic

lymphadenopathy) Exudation may be seen (if skin is secondarily infected)The periorbital skin is inflamed and oedematous,

resulting in ectropion, with consequent epiphora.

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The general picture is modified according to the nature of any underlying disease, and the patient’s age and general physical condition.

1. ECZEMATOUS DERMATOSIS:occurs most frequently in the sixth and seventh decades. However, atopic erythroderma may occur at any ageExacerbation of existing lesions usually precedes the generalization, which follows the usual pattern.

2. PSORIASIS:In erythrodermic psoriasis, the clinical picture may be highly desquamative but when the erythroderma is fully developed ,the specific features of psoriasis are often lost.Emotional stress, intercurrent illness and phototherapy over dosage may also precipitate erythroderma.

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3. DRUGS:The eruption may start as a generalized eczema, or scarlatiniform or morbilliform erythema, often accompanied by some irritation, which increases steadily in severity.This group has the best prognosis of all the causes of erythroderma , often resolving in 2–6 weeksThe cutaneous manifestations of drug hypersensitivity may be accompanied by involvement of other organs (ex: DRESS)Multisystem involvement may include fever, eosinophilia, lymphadenopathy, hepatitis, renal dysfunction and pneumonitis.

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4. Malignancies ( lymphoma, leukemia )The clinical picture follows the pattern already described, but the accentuation of certain features should arouse suspicion that a lymphoma is associated, even if repeated investigations over a period of months fail to provide convincing confirmatory evidence.Pruritus is often very severeThe infiltration of the skin may be so severe that the patient’s features are deformedEnlargement of the lymph nodes may be considerable, even if they are histologically not involved by the lymphoma.

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5. Pemphigus Foliaceus:Moist adherent scaling preceded by crusting on the face and upper trunk & followed by erythema is typical • 6. Pityriasis Rubra Pilaris:Keratotic follicular plugs on the backs of the fingers, and on the knees and elbows are characteristic• 7. Erythroderma in neonates:Congenital icthyosiform erythroderma, colloidion baby and widespread candidiasis

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Systemic disturbances• increased blood flow to the skin hypothermia, high output cardiac failure

Compensated hyper metabolism , increased metabolic rate

• Loss of exfoliated scales may reach upto 9 gr/m2 of BSA

hypoalbuminemia

edema

• Altered immune response ( increase in gamma globulins)

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Diagnosis of erythrodermaA detailed history of the sequence of events leading to the development of erythroderma/exfoliative dermatitis is a prerequisite in all patients.A thorough clinical examination is required in order to diagnose the etiology of exfoliative dermatitis and to allow appropriate urgent symptomatic treatment.Histopathology is paramount and is rewarding in over 50% of cases Fine needle aspiration cytology (FNAC) may be vital to distinguish between dermatopathic lymphadenopathy and malignant lymphadenopathyOrder the necessary investigations depending on the suspected cause

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Management of erythroderma

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Complications

Secondary infectionDehydration Electrolyte imbalanceTemperature dysregulationhigh-output cardiac failurePost inflammatory hypopigmentation or hyper pigmentation

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Erythroderma in sezary’s syndrome A widespread drug rash going to erythroderma

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Erythroderma due to Pityriasis rubra pilaris Erythroderma due to Pemphigus foliaceus

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Erythrodermic psoriasis

Erythrodermic atopic dermatitis

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DRUG REACTIONS

Accounts for 5% of admissions to an input dermatology serviceRisk groups:

• More common in women• Increased chances with increasing age & no of

drugs taken by the patient• More in immunosuppressed individuals ( HIV )• Patients with history of atopy

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Pathogenesis• Immunological mechanisms:1. IgE dependent reactions: urticaria, angioedema2. Cytotoxic drug induced reactions: petechiae secondary to drug induced

thrombocytopenia3. Immune complex dependent: vasculitis, serum sickness4. Delayed type/ cell mediated drug reaction: exanthematous , fixed &

lichenoid drug eruptions, SJS, TEN• Non immunological mechanisms:1. Overdose2. Pharmacological side effects3. Cumulative toxicity4. Delayed toxicity5. Drug-drug interactions6. Altered metabolism

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EXANTHEMATOUS DRUG ERUPTIONSSynonyms: morbilliform drug eruptionsMost common type of drug eruptions effecting the skin Drugs advocated: aminopenicillins, sulfonamides, cephalosporins, anti convulsantsEntire pathophysiology is unclear & is probably thought to be immunogenicViral infections increase the incidence of exanthematous drug eruptionsThe eruption begins 7-14 days after taking the new medications & can even occur a few days after the drug has been discontinuedIt starts as an erythematous macule which is slightly palpable Sites: starts on the trunk & upper extremities and progressively becomes confluentIt is a polymorphic rash with urticarial lesions on limbs, confluent areas on thorax, purpuric lesions on ankles & feetMucous membranes are sparedPruritus, low grade fever are often present It usually resolves after 1-2 weeks without any complication or sequelaeDD : viral exanthems, TSS, Scarlet fever , Kawasaki's disease

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EXANTHEMATOUS DRUG RASH

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FIXED DRUG ERUPTION Drugs advocated are: sulfonamides, NSAIDS, barbiturates, tetracyclines, carbamazepineOnset of the eruption is within 1-2 weeks if exposed for the first time and within 24 hrs on later re exposure to same drugUpon readministering the causative drug lesions occur at exactly the same sites Lesions: one/few, round, sharply demarcated erythematous plaques are seen with dusky, violaceous hue/ central blister/detached epidermis Sites : lips, face, hands, feet, Genitilia but can occur anywhere on the bodyProgress of the lesions: fades over several days, often leaving a residual post inflammatory brown pigmentationHistopathology: superficial & deep interstitial & perivascular infiltrate within the dermis, composed of lymphocytes, eosinophils and sometimes neutrophilsDD: single lesion- insect bite reaction

multiple lesions – EMF, SJS

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FIXED DRUG ERUPTIONS

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ACUTE GENERALISED EXANTHEMATOUS PUSTULOSISSynonyms: pustular drug rash, toxic pustulodermaAn acute febrile drug eruption characterised by numerous small, primarily non follicular sterile pustules, arising within large areas of erythemaHLA –B5, HLA-DR11, HLA-DR3 have been found more frequently in patients with AGEPDrugs advocated: beta lactam antibiotics, macrolides, calcium channel blockers, anti malarials Onset of rash is within 2 days and lasts for 1-2 weeks followed by superficial desquamationLesions: numerous, small, non follicular, sterile pustules arise within large areas of edematous erythemaThe lesions are associated with pruritus/ burning / both sensationsSite: first appears over the face / intertriginous region and later disseminatesOther findings are : edema of hands & face, purpura, vesicles, bullae, EMF like lesions Laboratory findings: leukocytosis with increased neutrophil count, mild- moderate eosinophilia, hypocalcemiaDD:- acute pustular psoriasis of von-zumbusch type, exanthematous drug eruptions, DRESS

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ACUTE GENERALISED EXANTHEMATOUS PUSTULOSIS

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DRESS( drug rash with eosinophilia & systemic symptoms)

Synonyms: drug hypersensitivity syndromeAn adverse, idiosyncratic drug reaction characterised by a triad of fever, skin eruption, and internal organ involvementDrugs advocated are carbamazepine, phenytoin, sulfonamides, allopurinolThe rash starts 1-6 weeks after the intake of the drugsIt starts with a high fever accompanied by pharyngitis, cervical lymphadenopathyLater or simultaneously , skin eruption starts in the form of a macular erythema on the face , upper trunk, & upper extremities; to a dusky reddish & confluent papular rash that is pruritic and is associated with facial edemaIt can progress into exfoliative dermatitisMucous membrane involvement is rare Various internal organs can be involved like the liver, kidneys and hematological systemLaboratory findings: atypical lymphocytosis, neutrophilia, hemolytic anemia, elevated transamines, ALPDD: serum sickness like reaction, SJS, TEN

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A

DRESS

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MANAGEMENT OF DRUG ERUPTIONSStop the suspected offending drug Low to mid potency topical steroids and bland emollients can help relieve itchingOral histamines, oral corticosteroids may be needed in severe cases

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PEMPHIGUS

Defn: An autoimmune, intraepithelial, blistering disease affecting the skin and mucous membranes Epidemiology: commonly seen in middle aged people ( 3-89 years)

Winter exacerbations may be seen • Pathogenesis: It is associated with binding of IgG

autoantibodies to keratinocyte desmosomes and to desmosome free areas of the keratinocyte cell membrane which results in a loss of cell to cell adhesion (acantholysis) leading to an intraepidermal cleft giving rise to blisters

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Clinical Features

Types of pemphigus we commonly encounter are:1. Pemphigus vulgaris2. Pemphigus vegetans3. Pemphigus foliaceous 4. Pemphigus erythematosus

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PEMPHIGUS VULGARIS• Most commonly encountered variety• Skin: localised/ generalised vesicles & bullae on apparently normal/

erythematous skinInitial tense & clear bullae

2-3 days Flaccid & turbid bullae

Rupture easily to leave behind painful areas of oozing & denuded skin which shows little tendency to heal

Lesions often become crusted along with painful erosions

Heals with hypo/hyperpigmentation

NIKOLSKY’S SIGN – POSITIVEBULLA SPREAD SIGN - POSITIVE

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PEMPHIGUS VULGARIS WITH EROSIONS

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Oral involvement: seen in all pts with p.vulgaris• Buccal, palatine & gingival mucosa are commonly

effected• Few/many , small/large, superficial, painful, persistent,

irregularly shaped erosions, ulcers or collapsed bullae covered with greyish necrotic slough and having little/ no inflammation

• The lesions do not resolve spontaneously & continue to extend peripherally

• Due to extensive oral lesions, there is decreased food intake leading to weight loss , hypoproteinemia

• There may be super imposed candida infection

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PEMPHIGUS VULGARIS WITH ORAL EROSIONS

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Other mucosal surfaces may be involved including:

1. Conjuctival ( mucopurulent conjunctivitis & non specific conjuctival hyperemia)

2. Esophagus ( odynophagia / dysphagia)3. Labia , vagina, penis4. Urethral, anal , nasal mucosa• Nail changes seen are paronychia, nail plate

defects

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Diagnosis To confirm the diagnosis of the pemphigus :

1. Tzanck smear : shows acantholytic cells2. Histopathology from the edge of a blister: shows an intra

epidermal blister with a cleft at suprabasal level, multiple acantholytic cells in the blister cavity, basal keratinocytes gives a tombstone appearance

3. Direct immunofluorescence on normal appearing skin : shows the deposition of immunoglobulin G (IgG) & C3 at the surface of the keratinocytes

4. Indirect immunofluorescence using the patient’s serum if DIF is positive : demonstrates the presence of circulating IgG autoantibodies that bind to the epidermis

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ACANTHOLYTIC CELLS

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Suprabasilar acantholysis with a few acantholytic cells in the blister cavity.Attachment of the basal cells to the basement membrane via hemidesmosomes leadsto the “tombstones” appearance.

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Direct immunofluorescence showing intercellular immunoglobulin G throughout the epidermis of a patient with pemphigus vulgaris.

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DIFFERENTIAL DIAGNOSIS

Bullous pemphigoid Dermatitis herpetiformisErythema multiformeEpidermolysis bullosa acquisitaBullous impetigo

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PrognosisMost common causes of death in pemphigus are SEPTICEMIA & PULMONARY EMBOLISM

BAD PROGNOSIS• elderly patients • patients with P.vulgaris• Patients on prolonged steroid

therapy

GOOD PROGNOSIS• patients with minimal disease for

longer duration• Patients with cutaneous lesion only

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Treatment Assess the general condition of the patient and the extent & severity of the diseaseAdmit the patient Maintenance of fluid, electrolyte balanceProper nutrition in terms of high calorie and high protein diet which is well tolerated by the patientControl of secondary infections ( by means of antibiotics )Proper & regular dressing of raw areas till reepithelization occurs

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Topical therapy:• Patients with mild pemphigus can be managed

by topical therapy alone • Topical therapy is useful in treating localised

relapses after successful control of the disease• Agents used are topical clobetasol, tacrolimus• Intralesional corticosteroids:Triamcinolone ( 5-10 mg/ml for cutaneous lesions 10-20 mg/ml for mucosal lesions)0.05-0.1 ml/site every 1-2 wks till healing occurs

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Systemic corticosteroids: prednisolone• Main stay of treatment in pemphigus• Mild – moderate cases : 60-80 mg/day Severe cases: 80-120 mg/day • Dose may be increased by 50% once in every 4-

7 days till lesions heal • Tapering of the dose by 50% once every 2 weeks

is started only after 80-90% of lesions have healed

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• Oral corticosteroids alone may not be effective • So concomitant immunosuppressives / other

adjuvants are advocated if 1. If there are relative contraindications to use of

corticosteroids2. If serious effects due to corticosteroids develop3. If a reduction in the dose is not possible

because of repeated exacerbations of disease activity

4. If high dose of steroids are used to achieve remission

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Anti metabolites:Azathioprine ( 2-4 mg/kg/day) Cyclophosphamide ( 1-1.5mg/kg/day)Mycophenolate mofetil ( 2gr/day) • DEXAMETHASONE-CYCLOPHOSPHAMIDE PULSE THERAPY:• Involves the intravenous administration of 100 mg of

dexamethasone with 500 mg of cyclophosphamide in 500 ml of 5% dextrose over 1-2 hours on day 1, followed by daily administration of 100 mg of dexamethasone for the next 2 days.

• These pulses are repeated every 4 weeks • On the balance days, 50 mg of cyclophosphamide is

administered orally every day• Major advantages seen with this therapy are quick healing of

lesions, absence of side effects of corticosteroids

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Other agents used are :1. Tetracycline (2gr/day)2. Nicotinamide ( 1.5gr/day)3. Dapsone (100-300 mg /day)4. Cyclosporine (3-6 mg/kg/day)5. Rituximab (375 mg/m2 IV once weekly for 4

consecutive weeks)6. Intravenous immunoglobulin (2gr/kg/cycle

divided over 3-5 days, repeated every 3-4 weeks for a minimum of 3 cycles)

7. Plasmapheresis8. Adsorption of pathogenic antibodies

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Occur during the course of leprosy

They are sudden exacerbations in the clinical state of a patient with pauci/multi bacillary leprosy

They are due to immunological changes following effective treatment and decrease in the bacillary load

Other provoking factors are intercurrent infections

LEPRA REACTIONS

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Lepra reactions are of two types:1. Type 1 reactions:• A type IV hypersensitivity reaction• It is seen in the entire spectrum of leprosy except lepromatous type• It manifests as a local aggravation of an existing lesion which become

more erythematous and indurated with increased loss of sensation.• The lesions may be painful with paraesthesis.• The nerve may become extremely painful and tender• . It can be an UPGRADING reaction (occurs when the patient is

on treatment due to increased CMI) or DOWNGRADING reaction (occurs in patients who are not on treatment & is due to decreased CMI)

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Diagnosis of type 1 reaction:• Usually it is diagnosed clinically • Naaf’s & his team has proposed a criteria system for diagnosing

the condition1. Major: pre existing &/ new skin lesions become inflamed, red

and swollen2. Minor:• One or more nerves become tender and may be swollen• Crops of new ( painless ) lesions occur• Sudden edema of face and extremities• Recent loss of sensation in hands and feet or signs of recent

nerve damage in an area supplied by a particular nervePresence of one major criterion or at least two minor criterion (without signs of ENL) for the diagnosis of type 1 reaction

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Type 1 reaction

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2. Type 2 Reactions:• also known as erythema nodosum leprosum (ENL). • It is mediated by Humoral hypersensitivity• Usually occurs later during the course of treatment for

lepromatous and borderline lepromatous leprosy• manifests as sudden eruption of tender, erythematous,

evanescent nodules, appearing suddenly on any part of the body which may ulcerate

• Other features like joint pain, fever, malaise, tender enlarged nerves, lymphadenitis, epididymo-orchitis, iridocyclitis, edema of the extremities or face may be present

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Diagnosis of ENL:• Classical ENL with different morphological

patterns with or without constitutional features and associated manifestations are diagnostic

• Clinical tests:1. Ryrie’s test2. Elli’s test

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Type 2 reaction/ ENL

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LEPRA REACTIONS - TREATMENTType 1 Reaction:

1. start MB-MDT / continue using it if already started 2. Specific treatment has to be initiated depending on

the severity of the reaction• Mild reaction:1. Daily aspirin/ paracetamol for few weeks 2. Reassurance to the patient • Severe reaction & Acute neuritis:1. Oral corticosteroids (prednisolone) is the drug of

choice• Has to be administered in very high initial doses (1-

2mg/kg/day) , over a longer period of time with slow tapering to reduce the chance of recurrence of reactions

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DOSE DURATION40 mg 1st and 2nd week30 mg 3rd and 4th week20 mg 5th and 6th week15 mg 7th and 8th week10 mg 9th and 10th week5 mg 11th and 12th week

• Other immunosuppressives which are useful while tapering the dose of steroids are:

1. Methotrexate2. Cyclosporine3. Azathioprine

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Neuritis :• Keep the affected nerves in resting position with

appropriate splinting & padding• After the active phase is over, passive and active

exercises should be initiated• Oil massage, short wave diathermy (SWD),

ultrasonic therapy ( UST )• NSAID’S can be given to relieve the pain • Surgical decompression

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Treatment of Type 2 Reactions Assess the severity of the reaction ( mild, moderate, severe)

1. Mild- pt has few ENL lesions & no signs of involvement of organs other than the skin

2. Moderate- pt has mild fever (< 100 f) & mild to moderate crops of ENL. There is leukocytosis with involvement of some other organs except nerves, eyes or testes

3. Severe- pt has high fever, pustular/ ulcerative lesions of ENL, tender & enlarged lymph nodes, neuritis , orchitis, arthritis, iridocyclitis, persistent albuminuria with RBC’S in urine

• Treat the precipitating factors like sore throat, herpes simplex infection, intestinal parasites, filaria, malaria and psychological stress

• Continue the MBMDT/ start it if not started earlier along with specific treatment for type 2 reactions

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Specific management :1. Mild: • Analgesics and anti inflammatory drugs like aspirin ( 600 mg 6

hrly after food) , NSAID’S2. Moderate:• Chloroquine: an anti inflammatory drug , Dose : 250 mg TID for a week, then tapered to 250 mg BD for a week , then 250 mg OD daily, further tapering to 250mg A/D may be sufficient to maintain control • Sodium antimony gluconate ( stibophen ): inhibits complement

activationDose : available as a 10% aqueous solution , initial dose of 1.5 ml IM, if no adverse effects give 3ml IM after 2 days , followed by 3-5 ml IM every A/D• Colchicine: acts by inhibiting neutrophil chemotaxis Dose: 0.5 mg TID orally with due tapering

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Severe reaction:First attack of severe ENL

1. 1st option: prednisoloneA short 6-8 week course of oral prednisolone (0.5-1 mg/kg) till clinical improvement and then taper every week by 5-10 mg over 6-8 wks 2. 2nd option: prednisolone and clofazaminePrednisolone as dose mentioned above Clofazamine 300 mg od for 1 month, 200 mg od for 3-6 months, 100 mg od for as long as symptoms remain3. 3rd option : thalidomide100 mg 4 times a day for 3-7 days or till reaction in under control100 mg morning plus 200 mg evening for 4 weeks 200 mg evening od for 4 weeks100 mg evening od for 4 weeks 50 mg daily evening or 100 mg every A/D evening for 8-12 weeks

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Treatment of recurrent or chronic ENL1. 1st option: clofazimine plus prednisoloneClofazimine :300 mg daily for 3 months followed by 200 mg daily for 3 months followed by 100 mg daily as long as symptoms persistPrednisolone : 30 mg daily for 2 weeks followed by 25 mg daily for 2 weeks, 20 mg daily for 2 weeks, 15 mg daily for 2 weeks, 10 mg daily for 2 weeks, 5 mg daily for 2 weeks and then stop2 2nd option : thalidomide200 mg bd for 3-7 days followed by 100 mg morning plus 200 mg evening for 4 weeks 200 mg evening od for 4 weeks 100 mg evening od for 4 weeks 100 mg evening A/D for 8-12 weeks or more

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Staphylococcal scalded skin syndrome

STAPHYLOCOCCAL scalded skin syndrome (SSSS) is the term used to define a potentially life-threatening, blistering skin disease caused by exfoliative toxins (ETs) of certain strains of Staphylococcus aureus .Synonyms:- Ritter’s disease, Pemphigus neonatorumEpidemiology:-

Is primarily a disease of infancy and early childhood( less than 6 years), but occasionally seen in adults with chronic renal insufficiency and those who are immunocomprimisedA male-to-female predominance of SSSS has been documented (2:1 in sporadic cases; 4:1 in epidemics).

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Etiopathogenesis SSSS is caused by an exfoliative/epidermolytic toxin(ET) produced by staphylococcus aureus phage group 2 strains(types 3A,3C,55,71)ET produced by the bacteria has two antigenically different forms(ET-A,ET-B), both are capable of causing the diseaseET-A.B are serine proteases with a high specificity for human desmoglein 1(Dsg 1)They bind to Dsg 1 directly, causing a confirmational change in the toxin which cleaves the extracellular domain of Dsg1.They act at the granular layer of epidermis to cause a split and sterile bullae

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Clinical features The onset of SSSS may either be acute with fever and rash or be preceded by a prodrome of malaise, irritability, and cutaneous tenderness, often accompanied by purulent rhinorrhea, conjunctivitis, or otitis media.

• The typical rash presents as a faint, orange–red, macular exanthem, localized initially on the head (Figure 11.3A ) and spreading within a few hours to the remainder of the body, with peculiar periorificial and flexural accentuation

• Later on the skin develops a wrinkled appearance owing to the formation of flaccid bullae within the epidermis

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• NIKOLSKY’S SIGN is positive • In 1-2 days, the bullae are sloughed, leaving

behind moist skin and areas of varnish like crust • Patients may show “sad man” facies, perioral

crusting and radial fissuring with mild facial edema

• Scaling and desquamation continue for the next 3-5 days with re-epithelialization in 10-14 days

• Lesions heal without any scarring

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Extensive sloughing of the skin closely resembling that of vast scalding

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Diagnosis The diagnosis of SSSS is mainly clinical and should be taken into consideration in any child who develops a generalized, tender erythema, most prominent on periorificial (in particular perinostril and periocular) areas, associated with Nikolsky sign. Suspected SSSS is supported by the confirmation of staphylococcal infection in different sites (conjunctiva, nasopharynx, ear).

INVESTIGATIONS• Blood culture- negative• Culture from conjunctiva, nasopharynx,or pyogenic foci- positive• Leucocyte count may be elevated or normal• Slide latex agglutination, double immuno diffusion, ELISA are

used to confirm the diagnosis

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TEN• Common in adults, but

uncommon in children• Widespread skin

involvment with extensive mucosal involvment

• TEN shows subepidermal splitting, with full-thickness necrosis of the epidermis

• Tzanck smear taken from a fresh bulla shows necrotic keratinocytes with inflammatory cells in TEN

LEINER DISEASE the erythrodermicform of seborrhoeic dermatitis in newborns, lacksblisters or erosion, but yellowish scales are present all over the body.

KAWASAKI DISEASE Prolonged fever, heart involvement, and generalizedlymphadenopathy

DIFFERENTIAL DIAGNOSIS

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Histopathology

• A sharply demarcated zone of clevage at or below level of stratum granulosum

• No inflammatory cells in the bullae

• Upper dermis lacks an inflammatory infiltrate

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Management

Patients with SSSS require hospitalization because, besides the appropriate systemic antibiotic therapy, intensive general supportive measures are needed. The mainstay of treatment is to eradicate staphylococci from the focus of infection, which in most cases requires intravenous (IV) antistaphylococcal antibiotics (e.g., methicillin, flucloxacillin). Subsequently, parenteral therapy may be replaced with 1-week oral treatment with a -lactamase–resistant antibiotic (e.g., dicloxacillin, cloxacillin, cephalexin).

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Second-line therapies include IV macrolide (erythromycin or clarithromycin) or vancomycin. IV replacement of fluids and lacking electrolytes is recommended for prompt recovery oral fluid intake and careful monitoring of urinary Moist denuded areas should be lubricated with a bland emollient to decrease pruritus and tendernessIdentification and treatment of s. aureus carriers

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