1. BASIS OF NATURAL AND pathogenesis
Pemphigus is an autoimmune disease manifestations in the skin and
mucous membranes, have autoantibodies in the blood against cell surface
antigens and autoantibodies keratinocyt in both skin lesions and skin
healthy.
The genetic
mutations in patients with pemphigus impact on the cellular
immune competence, these cells circulating antibodies and antibody
spot.
The antibodies associated with the antigen under the action of the
complement system disorders causing yeast, which activates plasminogen
to release substances plasmin. plasmin destroying chemicals and soluble intercellular adhesive causing skin lesions.
2. CLINICAL MANIFESTATIONS
a- Body malignant (pemphigus vulgaris)
Expression in the skin: An injury to or met with bullous essentially
large, shallow wrinkled, application forms, the skin appears normal on
the fast break to put the background skin slippery, wet and scabbed more
.
Originally hurt less, localized in a particular region and mucosal
interstitial region and then spread throughout the body, starting with
the mucosa some time after the new skin
Crystal positive Nikolsky sign (hand or rubbing lightly clamp down on
the edge bullous skin or normal skin, the skin slipped off or bullous
form new)
Body: high fever, chills patients with intermittent fatigue dizzy
patients, neurological disorders, depression and rapid weight loss, body
smells like dead toads, patient declined rapidly lead to death within 6
months to 1 year. Nowadays, because of scientific advances, have better prognosis but problem is intractable disease mortality rate is very high.
b- The other:
- Pemphigus rough: (vegtant)
This rare and can be considered a period of pemphigus progressed normally.
Damage is fundamentally broken bullous skin quickly leaving skid
patches, then rough up the array papilloma pus, scaly weather, location
and selective mucosal folds large interstitial (armpits, under the
breasts, groin and buttocks). Patients may find itchy, smelly stinky, or fever and digestive disorders. After treatment or natural rough crowd and eventually collapse took leave bruises. Over the course progresses, patients with fever and loose bowel and exhaustion and died within three years.
- Pemphigus skin fat: (Seborrheique)
Damage is essentially vesicular, bullous skin on the fast break that
left erythematous, scaly thick yellow secretion, taking mucus glands in
the region: Face, chest, between the two shoulders, waist , at the very
hurt butterfly, tend to atrophy of the skin, mucosa unscathed. Full good little patient affected, this can usually be less severe
- Pemphigus leaf flakes (foliace)
Rare disease, bullous start small farm wrinkled, position in the face, back, abdomen.
lesions may appear on healthy skin patches or red skin, mucosa not
hurt, bullous disappear very quickly, was replaced by patches of
erythema, scaling drip, accompanied by itching and fever, signs Nikolsky
positive.
3. TREATMENT
Spot: potassium permanganate bath, apply the dye solution, severe cases to bed powder
Body:
- Use corticoits: loading dose of 1 -2 mg / 1 kg of body weight / day,
combined intravenous and oral, tapering and maintained for several
months or a lifetime, when to dose exacerbation frequency the back
- Immunosuppressive Drugs: Methotrexzt 20 mg / week intramuscularly. Cyclophtphamid 199 mg / day
- Symptomatic treatment: antibiotics, intravenous fluids, blood
transfusions, preventive monitoring and treatment of gastrointestinal
complications, hypertension, diabetes ... |
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