-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