Pdf Erythema multiform(1).pdf

Erythema multiforme  Definition :it is acute self limiting vesiculobullous inflammatory disease of skin and oral mucosa with multiple cutaneous lesion. Etiology : EM is immunologically mediated disease that may be initiated by : A- deposition of immune complexes in superficial microva...

Erythema multiforme  Definition :it is acute self limiting vesiculobullous inflammatory disease of skin and oral mucosa with multiple cutaneous lesion. Etiology : EM is immunologically mediated disease that may be initiated by : A- deposition of immune complexes in superficial microvasculature (mostly IgM and c3) of skin and oral mucosa. triggering factor of immune complexes: Herpes simplex virus Drug reaction such as antibiotics , barbiturates , carbamazepine sulphonamides B-cell mediated immunity (herpes associated EM)  TYPES: 1- Major form A- Steven Johnson’s syndrome B- Toxic epidermal necrolysis (TEN) 2- Minor form 3- Chronic form(in immunosuppressive patient) 4-Herpes associated erythema multiforme A- Steven Johnson’s syndrome It is one of muco-cutaneous-ocular syndromes in which their is oral ,skin ,eye and genital involvement and characterized by sub epithelial vesiculobullous lesion. 1-MAJOR FORM  Age :infants ,children and young adults Sex :both Onset :  sudden with fever, headache ,malaise and the patient may develop severe vesiculobullous lesion within 24-48 hs.  Fever may persist 4- 5 days and 1- 2 weeks the patient appear frustrated and dehydrated  vesicles appear in any site of oral mucosa (rare in gingiva) which rupture and produce –ulcers are large , shallow, irregular ,painful ,surrounded by erythema , hemorrhagic ulcer Positive Nikolsky’s sign is usually evident on the red oral mucosa. Lips : there is bloody crusted lesion Tongue :is enlarged with marginal indentation and e rosion Oral lesion:  The epithelium splits and the superficial layers slide on gentile pressure and lateral force using cotton applicator on clinically normal or intact mucosa Nikolsky’s sign:  Symptoms: pain ,inability to eat ,swallowing difficulty if the lesion extend to oropharynx and increased salivation Fate : healing with no scar formation within 2-3 weeks or more  1- Maculopapular lesions : red macules or papules on the back of hands ,forearms ,feet &knees and side of the neck 2- Iris Or Target Lesion : it is typical skin lesion have concentric erythematous rings called iris or target lesion according to number of color zones. SKIN LESION :  3-vesiculeo bullous lesions : first appear as red macule and soon develop a bullae then bullae rupture giving extensive skin sloughing which may lead to death due to secondary infection or \ and fluid and electrolyte imbalance Eye lesions: Diffuse conjunctivitis with corneal ulceration which may lead to scaring and blindness Urogenital : a-male :non specific urethritis and balanitis b- female : vaginal ulcer  Definition : it is rare syndrome representing the most severe form of erythema multiform resulting from drug reaction. Site : skin and mucous membrane of mouth , throat , nasal passage , trachea, conjunctiva ,genital and anus Note: mucous membrane lesion may precede skin lesion by 1 - 2 week B- Toxic Epidermal Necrolysis (TEN)  Character : 1) sever erythema followed by vesiculo bullous lesion and then detachment of epidermis occur of the skin (resembling burn with hot steam). 2) Positive Nikolsky’s sign with peeling of large areas of skin ,leaving painful exudative surface. 3) The patient is actually ill and has rising fever 4) Death may occur due to secondary infection ,loss of fluid and electrolyte/ fluid imbalance 5) healing may occur without scaring unless secondary infection occur  The skin and \ or the oral mucous membrane are involved without any systemic manifestation Clinical features are similar to but less sever as in major form 2- MINOR FORM  It is a rare type associated with immuno compromised patient 3- Chronic Erythema Multiforme  EM may be initiated by cell mediated immune reaction to recurrent HSV infection (herpes labialis or genital herpes) Some patients prone to develop EM 10 - 14 days after recurrent herpes simplex infection patient who prone to develop EM after recurrent herpes labialis or recurrent intra oral herpes should be placed on prophylactic maintenance dose of acyclovir Acyclovir is not effective in managing EM once the lesion of EM appear. 4- Herpes Associated Erythema Multiforme  Done by exclusion of other disease 1) History :  minor type :sudden onset of vesiculo bullous lesions develop rapidly (1-2 days) and no systemic feature  major : sudden onset of fever then vesiculo bullous lesion develop rapidly with systemic feature  recurrence  EM may develop 10-14 days after recurrent herpes infection DIAGNOSIS 2) Clinical examination: The character and distribution of oral lesion are fairly typical If the skin lesion are present the diagnosis may be facilitated thus the skin should be examined carefully if the oral lesion suggestive to EM 3) special investigation : No laboratory tests of diagnostic value for EM May be helpful only in excluding other disease  Minor form :lesion persist for 2-3 week Major form and chronic type :lesion persist up to 4 week It is self limiting disease healed with no scare formation unless secondary infection occur The prognosis is generally good ,although some fatality occur in major form. COURSE AND PROGNOSIS: 1) Mild Cases : treatment is supportive soft or liquid diet and directed toward elimination of Lesion :by topical steroid Pain : by topical anaesthesia Infection : by tetracycline Treatment Of EM : 2) Moderately Severe Cases: Systemic steroid 30-50mg (prednisone) daily or more according to severity of the case Systemic antibiotic if there is secondary infection An ophthalmological opinion should be obtained if the conjunctiva is involved 3) Severe Form : need hospital admission due to Feeding problem To correct Fluid imbalance For Skin graft which may be needed in TEN To manage frequent serious complication. 4) Prophylactic use of antiviral drugs is helpful in the management of patients with recurrent herpes associated EM. (400mg acyclovir \ day may be effective in preventing recurrence.