Compare of most common type 天皰瘡 & 類天皰瘡 

 

 

Pemphigus vulgaris

Bullous pemphigoid

 

Suprabasal epi. Cleft 表皮內 

Subepidermal epi. Cleft 表皮下

PE

Nikolsky’s sign(+)~拿棉棒推水泡推得動,表示很淺層

Nikolsky’s sign(-)

Antigen

Dsg3(), Dsg1, Acantholytic

BP1(BP230), BP2(BP180) lamina lucida

DIF

Intercellular IgG, C3 Ab

Linear C3, IgG Ab along DEJ

Age

50-60 Y/O

Elderly

S/S

Superficial flaccid blister, involve skin and mucosa

Erosion/ulcer of oral mucosa

Large tense bullae

Symmetric, no mucosa involvement, flexure region

Prognosis

Poor

Good

1st line treatment

Systemic steroid

Systemic steroid

 

Dermatitis Herpetiformis

Pemphigus Foliaceus

Paraneoplastic Pemphigus

S/S

Chronic diarrheaà

   Gluten sensitivity

群聚性水泡,很癢,多在四肢伸側,背部

Oral mucosa normal

Seborrhic area

Rare mucosal involve

Intractable painful mucosal erosion

Polymorphic skin rash

DIF

Granular IgA deposit papillary dermis

Intercellular IgG, Dsg1

Subcorneal separation

Intercellular IgG, C3 Ab (pemphigus)

Granular to linear C3 deposit on BMZ (pemphigoid)

Others

Dapsone treatment

PV還淺

Cancer-Non-HD lymphoma, CLL

           

 

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