Review Status: Reviewed by Lawrence S.
Chan, M.D., Northwestern University
Figures:
Definition:
.
- A group of autoimmune blistering diseases in which there
is acantholysis (define?) and blister formation within
the epidermis
- Immunoglobulins and complements are found bound to the
intercellular substance in the perilesional epidermis
- ¡@
Etiology:
- World-wide distribution which effects both sexes almost
equally (under the age of 20 there is a predilection for
women)
- Disease of middle age ,very rarely affect children
- coexist with thymoma or myasthenia gravis are quite
common
- D-penicillamine is capable of producing an eruption
strikingly similar to pemphigus
- ¡@
Clinical
Features:
- Generalized nongrouped lesions
- Flaccid vesicles and bullae (Figure
1).
- Nikolsky's sign positive
-- The epidermis looks normal, but can be disloged by
sliding pressure from a finger
- Oral lesions: (Figure 2).
--very common (over 50% of patients can be seen), as the
initial lesions
--generalized bullous phase in most patients occurs some
5 or more months after the onset of oral oral lesions.
- Areas of predilection :face, trunk, pressure points,
groin , axillae
- Healing occurs without scarring but transient
hyperpigmentation is common.
Pathology:
- Suprabasal separation with acantholysis (Figure 3). (Figure
4).
- Immunoflurescence study (Figure 5).
--Direct: IgG & C3 at intercellular space
--Indirect:
-Intercellular antibody : 80-90%
-Titers often parallel disease activity
Treatment:
- Corticosteroid: high dose, 100-200 mg predinisolone/day
- Immunosuppressive therapy
-- Imuran 100 mg daily, diminish the need for
corticoseroids
Differential
Diagnosis:
- Thermal burns
- Bullous pemphigoid
- Toxic epidermal necrolysis
- Penicillamine-induced eruption
- Steven-Johnson syndrome
Editor: Hung-Bin Tsai
Supervisor: Yu-Chuan Li, M.D., Ph.D.
