A case of pemphigus herpetiformis with only immunoglobulin g anti-desmocollin 3 antibodies

Won Jin Hong, Takashi Hashimoto, Soo Chan Kim

Research output: Contribution to journalArticle

3 Citations (Scopus)

Abstract

Pemphigus represents a group of autoimmune blistering diseases caused by autoantibodies against desmogleins (Dsgs), a class of desmosomal cadherins. Recently, several pemphigus patients only with desmocollin (Dsc) 3-specific antibodies have been reported. Here, we report a case of pemphigus herpetiformis (PH), where only anti-Dsc3-specific antibodies but not anti-Dsg antibodies were detected. A 76-year-old woman presented with a 3-year history of blister formation. Physical examination revealed pruritic erythemas with vesicles on the trunk and legs, but no lesions of the oral mucosa. A skin biopsy specimen revealed intraepidermal blister containing neutrophils, eosinophils, and lymphocytes. Direct immunofluorescence (IF) showed immunoglobulin G (IgG) and complement 3 (C3) depositions on the keratinocyte cell surfaces. Indirect IF showed IgG anti-keratinocyte cell surface antibodies. These findings hinted at a diagnosis of pemphigus. However, repeated enzyme-linked immunosorbent assays (ELISAs) for both anti-Dsg1 and 3 antibodies proved to be negative. Immunoblotting of normal human epidermal extracts revealed Dsc antibodies, and recently established ELISAs using human Dsc1-Dsc3 recombinantly expressed in mammalian cells detected anti-Dsc3 antibodies. Based on these clinical, histopathological, and immunological findings, the patient was diagnosed as PH with only anti-Dsc3 antibodies. Treatment with corticosteroid prednisolone and steroid-sparing agent dapsone accomplished complete clinical remission of the patient.

Original languageEnglish
Pages (from-to)102-106
Number of pages5
JournalAnnals of Dermatology
Volume28
Issue number1
DOIs
Publication statusPublished - 2016 Feb

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Desmocollins
Pemphigus
Immunoglobulins
Antibodies
Anti-Idiotypic Antibodies
Blister
Keratinocytes
Desmosomal Cadherins
Desmogleins
Immunoglobulin G
Enzyme-Linked Immunosorbent Assay
Dapsone
Direct Fluorescent Antibody Technique
Complement C3
Mouth Mucosa
Erythema
Indirect Fluorescent Antibody Technique
Prednisolone
Immunoblotting
Eosinophils

All Science Journal Classification (ASJC) codes

  • Dermatology

Cite this

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title = "A case of pemphigus herpetiformis with only immunoglobulin g anti-desmocollin 3 antibodies",
abstract = "Pemphigus represents a group of autoimmune blistering diseases caused by autoantibodies against desmogleins (Dsgs), a class of desmosomal cadherins. Recently, several pemphigus patients only with desmocollin (Dsc) 3-specific antibodies have been reported. Here, we report a case of pemphigus herpetiformis (PH), where only anti-Dsc3-specific antibodies but not anti-Dsg antibodies were detected. A 76-year-old woman presented with a 3-year history of blister formation. Physical examination revealed pruritic erythemas with vesicles on the trunk and legs, but no lesions of the oral mucosa. A skin biopsy specimen revealed intraepidermal blister containing neutrophils, eosinophils, and lymphocytes. Direct immunofluorescence (IF) showed immunoglobulin G (IgG) and complement 3 (C3) depositions on the keratinocyte cell surfaces. Indirect IF showed IgG anti-keratinocyte cell surface antibodies. These findings hinted at a diagnosis of pemphigus. However, repeated enzyme-linked immunosorbent assays (ELISAs) for both anti-Dsg1 and 3 antibodies proved to be negative. Immunoblotting of normal human epidermal extracts revealed Dsc antibodies, and recently established ELISAs using human Dsc1-Dsc3 recombinantly expressed in mammalian cells detected anti-Dsc3 antibodies. Based on these clinical, histopathological, and immunological findings, the patient was diagnosed as PH with only anti-Dsc3 antibodies. Treatment with corticosteroid prednisolone and steroid-sparing agent dapsone accomplished complete clinical remission of the patient.",
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A case of pemphigus herpetiformis with only immunoglobulin g anti-desmocollin 3 antibodies. / Hong, Won Jin; Hashimoto, Takashi; Kim, Soo Chan.

In: Annals of Dermatology, Vol. 28, No. 1, 02.2016, p. 102-106.

Research output: Contribution to journalArticle

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