Bullous Pemphigoid VS Pemphigus Vulgaris

Dr.Faust

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https://www.bmj.com/content/357/bmj.j2169
这是一个关于desmosome和hemidesmosome异同的非常好的图片链接网址



Nikolsky sign

• Painful, flaccid suprabasal/intraepidermal bullae (Nikolsky sign+)--Pemphigus Vulgaris
• Blisters that are resistant to shear stress and do not readily rupture (Nikolsky sign-)--bullous pemphigoid, dermatitis herpetiformis, and porphyria cutanea tarda
  

Pemphigus Vulgaris

• Autoantibodies against desmosomal proteins in skin cells
• Painful flaccid bullae in oropharynx and skin that rupture easily
• Postinflammatory hyperpigmentation
• Treatment: corticosteroids
  

Bullous Pemphigoid

• Autoantibodies against basement-membrane hemidesmosomal proteins
• Widespread blistering with pruritus
• Less severe than pemphigus vulgaris
• Rarely affects oral mucosa
• Can be drug-induced (e.g., middle-aged or elderly patient on multiple medications)
• Treatment: corticosteroids
  

Case
A 60-year-old man comes to the physician because of tender blisters on his arm and flank, worsening over the last few months. His past medical history includes hypertension and hyperlipidemia. He is currently taking atorvastatin and hydrochlorothiazide for these conditions. Physical examination shows blisters and flaccid bullae; a few have ruptured, leaving red, sore, and denuded areas. Pathologic examination shows acantholysis and a few lymphocytes.
Which of the following findings is most likely in this patient?

A.Eosinophils within bullae
The patient has pemphigus vulgaris.
Eosinophils within bullae are seen in bullous pemphigoid.
Although bullous pemphigoid can also cause multiple bullae, these lesions are typically pruritic and tense, not tender and flaccid.
[5.4% chose this answer.]

B.IgA deposits in the dermal papillae
IgA deposits in the dermal papillae are seen in dermatitis herpetiformis, which causes pruritic vesicles and papules to appear on the skin in a grouped arrangement, particularly on the extensor surfaces.
Dermatitis herpetiformis is highly associated with celiac disease and does not typically cause blistering.
[5.6% chose this answer.]

C.IgG antibodies against hemidesmosomes
IgG antibodies against hemidesmosomes are seen in bullous pemphigoid, which causes pruritic and tense blisters, which are not seen in this patient.
[28.1% chose this answer.]

D.Negative Nikolsky sign
The Nikolsky sign describes blisters that rupture with mild rubbing.
The blisters of pemphigus vulgaris have a positive Nikolsky sign.
In contrast, a negative Nikolsky sign is seen in blisters from bullous pemphigoid, dermatitis herpetiformis, and porphyria cutanea tarda.
[5.4% chose this answer.]

E.Oral mucosal lesions
Oral mucosal lesions are commonly seen in pemphigus vulgaris. Pemphigus vulgaris is an autoimmune condition that causes tender and flaccid blisters. It is caused by autoantibodies against desmosomes, resulting in impaired cell-to-cell keratinocyte adhesion that causes blistering.
The typical age of onset is 40-60 years and it can be fatal if not treated appropriately.
[54.8% correctly chose this answer.]

本题来自amboss qbank