Desquamation

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What kind of desquamation is this?

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Erythema multiforme

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Erythema multiforme

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Acute self-limited eruption characterized by a distinctive eruption (target lesion)

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EM minor

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Localized eruption of the skin with mild or no mucosal involvement

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EM major

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more sever mucosal and skin disease- potentially life threatening

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What causes erythema multiforme?

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Infections (HSV, mycobacterium, EBV), drugs (PCN, sulfa, anticonvulsants, salcylates, antituberculoids)

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Pathophysiology of erythema multiforme

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immune rxn with epidermal and dermal junction-- epidermal necrosis and blister formation.

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What kind of desquamation is this?

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Steven Johnson Syndrome

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What kind of desquamation is this?

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Toxic epidermal necrolysis

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What kind of desquamation is this?

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Staphylococcal scalded skin syndrome

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What kind of desquamation is this?

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Exanthematous drug reaction

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What kind of desquamation is this?

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Exanthematous drug reaction

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What kind of desquamation is this?

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Drug hypersensitivity syndrome

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Signs and symptoms of Erythema multiforme minor

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Prodromal symptoms mild/absent. Abrupt onset of rash usually occurs within 3 days, starts on extremities symmetrically with centripetal spreading

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Signs and symptoms of erythema multiforme major

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Most have non specific prodromes; usually 1-4 days before eruption

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Skin lesion appearance of erythema multiforme

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dull red macule or urticarial plaque over 24-48 hours. Small central papule, vesicle, or bulla develops followed by a raised, pale, edematous ring. Periphery eventually becomes cyanotic or violaceous.

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Distribution of erythema multiforme

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extensor surfaces of extremities with central spreading: palms, neck, and face are frequently involved- some mucosal involvement.

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Dx of erythema multiforme

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CBC, electrolytes, BUN, elevated ESR and LFTs, HSV Ag and cultures from blood, sputum, erosive areas

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Tx of Erythema multiforme

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Symptomatic- PO antihistamines, analgesics, local skin care, and soothing mouth washes; topical steroids
Low dose acyclovir for herpes related cases

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Complications of erythema multiforme

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secondary bacterial infx, dehydration, blindness, urinary retention, vaginal/urethral stenosis

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Prognosis of erythema multiforme minor

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lesions subside within 2-3 weeks without scarring; recurrence common and mostly preceded by apparent or subclinical HSV infx

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Prognosis of erythema multiforme major

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clearing may take 3-6 weeks; heal with hyperpigmentation/ hypopigmentation. Scarring usually absent

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Steven Johnson Syndrome

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Immune complex-mediated hypersensitivity complex that is a severe expression of erythema multiforme. Sononymous with TEN

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Etiology of SJS

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idopathic in 25-50% cases, infx (HSV, AIDS, mycobacterium), drugs (PCN, sulfa, anticonvulsants), malignancy mediated

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Pathophysiology of SJS

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immune complex mediated hypersensitivity disorder with no etiology in most cases.

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Signs and symptoms of SJS

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Nonspecific URTI, Clusters of mucocutaneous nonpruritic lesions develop abruptly-- MC = trunk. Dysphagia, dysuria, urinary retention, cough with thick sputum, orthostasis, altered consciousness

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Dx of SJS

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biopsy, CBC, UA, BUN, electrolytes, culture.

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Tx of SJS

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Supportive and symptomatic; ***TREAT AS BURN PATIENT without silver sufladiazine.

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Toxic Epidermal Necrolysis (TEN)

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Over 30% of BSA desquamated. Life threatening mucocutaneous skin disorder characterized by widespread erythema, necrosis, and bullous detatchment of the epidermis and mucous membranes, resulting in exfoliation sepsis and death

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Etiology of TEN

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medications (*sulfa)

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Pathophysiology of TEN

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Unknown. Immunologic mechanisms suspected. Epidermal apoptosis also suspected.

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Signs and symptoms of TEN

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Prodrome followed by poorly defined erythematous macular rash with purpuritic centers.--> forms into flaccid blisters and *sheet like epidermal detachment**TRUNK. Positive Nikolsky sign. In mucous membranes first

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Dx of TEN

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Per SJS

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Tx of TEN

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Discontinuation of offending drug and admission to burn unit. ***NO SILVER SULFADIAZINE. Antibx cream only.

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Complications of TEN

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Septicemia, hypoxemia, pulmonary edema, pneumonia, GI hemorrhage, hypovolemia, renal failure

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Prognosis of TEN

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Poor. High mortality rate. Lesions will scar

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Staphylococcal Scalded Skin Syndrome (Ritter's disease)

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Toxin mediated epidermolytic disease characterized by erythema and widespread detachment of the superficial layers of the dermis, most common in newborns.

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Localized form of SSSS

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bullous impetigo (least severe- mouth only)

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Generalized form of SSSS

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extensive epidermolysis and desquamation (body)

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Abortive form of SSSS

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Scarliatiniform variant. Initial rash with NO desquamation.

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Etiology of SSSS

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staph aureus- endotoxins cause disease

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Signs and symptoms of SSSS

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Fever, irritability. Local effects of bullous impetigo. Tender skin- scarlatiniform lesions with sandpaper appearance. Positive Nikolsky's sign. SPARES MUCOUSA! **CONTAGEOUS!

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Dx of SSSS

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gram stain, biopsy (intra-epidermal cleavage with splitting occurring in the stratum granulosum)

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Tx of SSSS

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hospitalization for IV fluids and electrolyte replacement. Use naf, ox, clox (DOC), diclox. Can use silver sulfadiazine.

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Prognosis of SSSS

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Good for children- adult mortality is high.

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Toxic Shock Syndrome

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Acute toxin-mediated illness caused by enterotoxin producing S. aureus characterized by rapid onset of fever, hypotension, generalized skin and mucosal erythema, organ hypoperfusion/MOSF, and desquamation.

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Dx of TSS

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Fever, sunburn rash, hypotension, organ system failure, dequamation (1-2 wks from onset) **MUST HAVE 3

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Tx of TSS

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Admit to ICU, REMOVE OFFENDING AGENT. Supportive treatment. IV antibx (naf, ox, clox, diclox or other PCN reistant)

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Prognosis of TSS

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Mortality rate 5-15%. recurrences reported at 30-40%. STSS- mortality 25-75%

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Exanthematous drug reactions

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MOST COMMON TYPE OF CUTANEOUS DRUG RXN! Adverse hypersentivity rxn to drug characterized by a cutaneous eruption that mimics a measles like viral exanthema (type IV rxn)

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Routes for producing an exanthemous drug eruption

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1. excessive therapeutic effect
2. pharm. side effect
3. immune hypersensitivity

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Signs and symptoms of an exanthemous drug eruption

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Pruritic but not painful. Bright red macules and/or papules that may become confluent; resolve in hues of tan and purple. Rash is generally SYMMETRIC- trunk and extremities

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Appearance of a drug eruption

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acne-form, bullous, eczematous, erythema multiforme, fixed drug eruption, hair loss, photosensitivity, pigmentation change, urticaria, vasculitis, possible mucosal involvement

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Most common appearance of toxic erythema

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Morbilliform rash or urticaria, trunk > extremities, accompanied by fever and peeling of the skin. Clears 1-2 wks after stopping drug.

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Dx of exanthematous drug reactions

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Clinical- sometimes by biopsy (perivasular lymphocytes and eosinophils elevated)

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Tx of exanthematous drug reactions

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DIFINITIVE STEP IS TO IDENTIFY THE OFFENDING DRUG AND DISCONTINUE USE. Also, antihistamine and topical steroids.

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Drug hypersensitivity syndromes

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Idiosyncratic drug reactions that begins acutely in the first 2 months after initiation of a drug

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Most common drugs which cause hypersensitivity

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antieptileptic, sulfonamides

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Pathophysiology behind drug hypersensitivity reactions

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Genetically determined inability to detoxify the toxic metabolic products of drugs; increased susceptibility of leukocytes to toxic metabolites.

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Signs and symptoms of drug hypersensitivity reactions

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Onset- 2-6 wks post admin, Rash is on FACE then trunk and extremities- SYMMETRIC. Constitutional symptoms.

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Dx of drug hypersensitivity reactions

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Elevated levels of: eosinophils, WBCs. Mononucleosis-like lymphocytes. Biopsy- mimics cutaneous lymphoma, eosinophils, dermal edema

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Diagnostic critera of hypersensitivity rxn

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1. Cutaneous drug eruption
2. Hematological abnormalities
3. Systemic involvement (heart, liver, kidneys, lungs, lymph)

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Tx of drug hypersensitivity rxn

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Identify and discontinue use of offending drug. Symptomatic treatment, antihistamines, topical and systemic steroids PRN.