Abstract
Pemphigus is an autoimmune blistering disease of the skin, mucous membranes, or both. There are two main categories of pemphigus: pemphigus foliaceus (PF) and pemphigus vulgaris (PV). PV is further subdivided into mucosal dominant and mucocutaneous types, according to the extent of cutaneous lesions. These classes of pemphigus have distinct histopathologic and serologic findings, with most cases falling into these subtypes. We report 4 cases that clinically showed blisters and erosions in the skin only, without mucosal involvement. Histologic examination of cutaneous lesions demonstrated suprabasilar acantholysis, a typical finding for PV. These patients had predominant anti-desmoglein 1 (Dsg1) IgG autoantibodies as well as anti-Dsg3 IgG autoantibodies, as determined by enzyme-linked immunosorbent assay. The desmoglein compensation theory posits that this rare phenotype can be produced by pathogenically weak anti-Dsg3 IgG in the presence of potent anti-Dsg1 IgG autoantibodies. Thus, cutaneous type PV without apparent mucosal involvement is observed as a rare clinical and histologic expression of pemphigus. This expression can be a transient phenotype that may develop from, or evolve into, other subtypes of pemphigus.
| Original language | English |
|---|---|
| Pages (from-to) | 839-845 |
| Number of pages | 7 |
| Journal | Journal of the American Academy of Dermatology |
| Volume | 52 |
| Issue number | 5 |
| DOIs | |
| Publication status | Published - 2005 May |
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ASJC Scopus subject areas
- Dermatology
Cite this
Cutaneous type pemphigus vulgaris : A rare clinical phenotype of pemphigus. / Yoshida, Kazue; Takae, Yujiro; Saito, Hitoshi; Oka, Hiroshi; Tanikawa, Akiko; Amagai, Masayuki; Nishikawa, Takeji.
In: Journal of the American Academy of Dermatology, Vol. 52, No. 5, 05.2005, p. 839-845.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Cutaneous type pemphigus vulgaris
T2 - A rare clinical phenotype of pemphigus
AU - Yoshida, Kazue
AU - Takae, Yujiro
AU - Saito, Hitoshi
AU - Oka, Hiroshi
AU - Tanikawa, Akiko
AU - Amagai, Masayuki
AU - Nishikawa, Takeji
PY - 2005/5
Y1 - 2005/5
N2 - Pemphigus is an autoimmune blistering disease of the skin, mucous membranes, or both. There are two main categories of pemphigus: pemphigus foliaceus (PF) and pemphigus vulgaris (PV). PV is further subdivided into mucosal dominant and mucocutaneous types, according to the extent of cutaneous lesions. These classes of pemphigus have distinct histopathologic and serologic findings, with most cases falling into these subtypes. We report 4 cases that clinically showed blisters and erosions in the skin only, without mucosal involvement. Histologic examination of cutaneous lesions demonstrated suprabasilar acantholysis, a typical finding for PV. These patients had predominant anti-desmoglein 1 (Dsg1) IgG autoantibodies as well as anti-Dsg3 IgG autoantibodies, as determined by enzyme-linked immunosorbent assay. The desmoglein compensation theory posits that this rare phenotype can be produced by pathogenically weak anti-Dsg3 IgG in the presence of potent anti-Dsg1 IgG autoantibodies. Thus, cutaneous type PV without apparent mucosal involvement is observed as a rare clinical and histologic expression of pemphigus. This expression can be a transient phenotype that may develop from, or evolve into, other subtypes of pemphigus.
AB - Pemphigus is an autoimmune blistering disease of the skin, mucous membranes, or both. There are two main categories of pemphigus: pemphigus foliaceus (PF) and pemphigus vulgaris (PV). PV is further subdivided into mucosal dominant and mucocutaneous types, according to the extent of cutaneous lesions. These classes of pemphigus have distinct histopathologic and serologic findings, with most cases falling into these subtypes. We report 4 cases that clinically showed blisters and erosions in the skin only, without mucosal involvement. Histologic examination of cutaneous lesions demonstrated suprabasilar acantholysis, a typical finding for PV. These patients had predominant anti-desmoglein 1 (Dsg1) IgG autoantibodies as well as anti-Dsg3 IgG autoantibodies, as determined by enzyme-linked immunosorbent assay. The desmoglein compensation theory posits that this rare phenotype can be produced by pathogenically weak anti-Dsg3 IgG in the presence of potent anti-Dsg1 IgG autoantibodies. Thus, cutaneous type PV without apparent mucosal involvement is observed as a rare clinical and histologic expression of pemphigus. This expression can be a transient phenotype that may develop from, or evolve into, other subtypes of pemphigus.
UR - http://www.scopus.com/inward/record.url?scp=18144396142&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=18144396142&partnerID=8YFLogxK
U2 - 10.1016/j.jaad.2005.01.106
DO - 10.1016/j.jaad.2005.01.106
M3 - Article
C2 - 15858475
AN - SCOPUS:18144396142
VL - 52
SP - 839
EP - 845
JO - Journal of the American Academy of Dermatology
JF - Journal of the American Academy of Dermatology
SN - 0190-9622
IS - 5
ER -