BULLOUS DISEASES

Def: Skin diseases that characterized by bulla.

Clasification :
- Hereditary Epidermolysis Bullosa(EB) :
- EB Simplex
- Junctional EB
- Pemphigus Vulgaris
- Bullous Pemphigoid
- Dermatitis Herpetiformis
- Pemphigoid Gestationis

Epidermolysis Bullosa Simplex

(EBS)
trauma induced intraepidermal blistering
mutation of the gene for keratin 5&14
- Generalized EBS
dominantly inherited
onset : at birth-early infancy
clinically :
- generalized blistering following trauma
- predilection : traumatizied body sites
(feet, hands, elbows, knees)
- rapid healing & minimal scaling

Localized EBS
most common form EBS
onset : in childhood or later
clinically :
- theck-walled blisters on the
hands & feet
- occur ; after excessive exercise,
manual
work

Junctional EB
- Blister formation within lamina lucida
- Clinical feature depending on the type of
genetic lesion & environmental factors
JEB Gravis :
- often do not survive infancy 40%
mortality rate during first year-old
- generalized blistering at birth
- distinctive & severe periorificial
granulation
- loss of nail
- sometimes completely denuded oozing
painfull erosion

 JEB Mitis :
- moderat or severe JEB at birth clinically
improve with the age
DX : - clinical appearance & history
- histopathology : immunochemical mapping
Tx : syst : for complication
wound management
nutrional support
infection control

Pemphigus Vulgaris

Pemphigu

Def : serious, acute or chronic bullous,

autoimmune diseases of skin and
mucous membranes that is often fatal
unless treated with immunosupressive
agents.
Pathogenesis :
loss of normal cell-to-cell adhesion in
epidermis (acantholysis) result of
circulating antibodies of IgG bind to
cell surface glycoprotein

Skin

lesions :

- Round/oval vesicles & bullae with serous

content, flaccid, easily ruptured & weeping
arising on normal skin
- Extensive erosion bleed easily & crusts
- Nikolsky sign (+)
- Mucous membrane : erosion of mouth,
nose,
pharynx & vagina
Dx : biopsy direct immunoflorescence (IF):
Ig G
& C3 in lesional & paralesional of
epidermis

DD: another bullous diseases

Variants : - Pemphigus Vegetans
- Pemphigus Foliaceus
- Pemphigus Erythematosus
- Brazilian Pemphigus
Tx: Glucocorticoid :
- prednisone 2-3mg/kg BWnew blister (-)
/ Nikolsky sign (-) rapid reduction
(half
initial dose) almost clearvery
slow
tapering minimal effective
maintenance
dose
- cleansing bath, wet dressings, topical &
intralesional steroid & AB
- fluid imbalance

Def: an autoimmune disorder presenting

as chronic bullous eruption , mostly in
patients
over 60 years of age .
Pathogenesis : interaction of autoantibody
with bullous pemphigoid antigen
(BPAG1 &
BPAG2)in hemidesmosomes .

Skin lesions:
- localized/generalized
- Erythematous , papular or urticarialtype lesions may precede bullae
formations by months.
- Bullae : large, tense, firm-topped,oval &
round
arise in normal / erythematous skin &
contain serious or hemorrhagic fluid.
a group arciform/serpigenous pattern
ruptur less easily than in pemphigus

Sites of predilections :
- axillae, thighs, groins, abdomen,
medial
forearms, lower legs
- mucous membranes mouth
Dx : clinical appearance & histopathology
; subepidermal bullae, neurotrophil at
dermal-epidermal junction
DD : other bullous diseases

Tx: -Systemic:
- prednisone started 50-100mg/d
clear /combined with
Azathioprine
150mg/d
- Dapsone 100-150mg/dmilder
cases
-Topical steroid very mild cases

Def : a chronic, recurrent ,intensely pruritic

eruption occurring symetrically on the
extremities and the trunk and comprising tiny
vesicle,papules and urticarial plagues that are
arrange in groups.
associated with gluten-sensitive enteropathy
and Ig A deposit in skin
Age of onset 20-60 years,most common:30-40years
History:
-pruritus ,intense ,episodic,burning/stinging of the
skin
- ingestion iodides and gluten overload
exacerbating factors

Skin lesions :
- erythematous papules/wheal-like
plaques, tiny firm-topped vesicles,
sometimes hemorrhagic bullae
- a group lesions herpetiformis
- distribution strikingly symetric
- scratching excoriations & crusts
Predilection : extensor elbows, knees
typical, buttock, scapular & sacral area

Histopathology:
mycroabcesses (polymorphonuclear
cells &
eosinophils) at tips of the
dermal papillae
DX : clinical appearance that
accompanied by
severe pruritus
DD: -allergic contact dermatitis
- atopic dermatitis
- papular urticaria
- bullous pemphigoid
- pemphigoid gestationis

Tx : systemic :
- Dapsone 100-150mg/d
reduction
50-25mg

50mg (2x/w)
- Sulfapyridine:1-1,5gr/d dapson
contraindicated
Diet : gluten-free

Pemphigoid Gestationis
Def : pruritic & polymorphic inflamatory
bullous dermatosis of pregnancy and
post partum period, mainly on
abdomen
usualy begins :
- 4-7month of pregnancy,
- immediate post partum periode
Tx : - prednison 20-40mg/d
- antihistamine & topical steroid mild