-IBIS-1.5.0-
tx
immune system
pemphigus
diagnoses
definition and etiology
definition:
This uncommon skin condition is characterized by intraepidermal bullae on otherwise healthy skin and mucous membranes.
etiology:
This condition usually occurs in middle-aged or older people, and is uncommon in children. It is probably an autoimmune condition. IgG antibodies are usually present at the site of epidermal disruption.
signs and symptoms
rigid or soft bullae are the primary lesions; commonly occurring in the mouth first where they can remain as open lesions
itching usually absent
bullae appear on the skin
in another form of the disease, the bullae may present as tiny vesicles and only appear on the face
Nikolsky's sign: epidermis is easily detached from the underlying skin
lab findings:
Tzanck smear of bullae to rule out viral infection
eosinophilia
antibody tests: antiskin, intraepithelial (+) in > 90% of cases; antiskin, dermal-epidermal (+) in > 80% of cases
course and prognosis
Initially, bullae occur in the mouth, followed by their appearance on the skin. These eventually rupture, leaving raw areas which are susceptible to infection.
Conventional medical approach is hospitalization (the condition can be fatal) and administration of corticosteroids to halt the eruption of new bullae.
differential diagnosis
bullous pemphigoid
benign mucosal pemphigoid
drug eruptions
toxic epidermal necrolysis
erythema multiforme
dermatitis herpetiformis
bullous contact dermatitis
footnotes