Ann Dermatol.  2016 Feb;28(1):102-106. 10.5021/ad.2016.28.1.102.

A Case of Pemphigus Herpetiformis with Only Immunoglobulin G Anti-Desmocollin 3 Antibodies

• Hong WJ
• Hashimoto
• Kim SC

• KMID: 2429490
• DOI: http://doi.org/10.5021/ad.2016.28.1.102

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.

Keyword

Anti-desmocollin 3 antibody; Pemphigus herpetiformis

MeSH Terms

Aged
Antibodies*
Autoantibodies
Biopsy
Blister
Complement C3
Dapsone
Desmogleins
Desmosomal Cadherins
Diagnosis
Enzyme-Linked Immunosorbent Assay
Eosinophils
Erythema
Female
Fluorescent Antibody Technique, Direct
Humans
Hydrogen-Ion Concentration
Immunoblotting
Immunoglobulin G*
Immunoglobulins*
Keratinocytes
Leg
Lymphocytes
Mouth Mucosa
Neutrophils
Pemphigus*
Physical Examination
Prednisolone
Skin
Antibodies
Autoantibodies
Complement C3
Dapsone
Desmogleins
Desmosomal Cadherins
Immunoglobulin G
Immunoglobulins
Prednisolone

Figure

• 

  Fig. 1 Clinical and histopathologic findings. Clinical features. Annular erythematous plaques with grouped peripheral tense vesicobullae on the (A) trunk and (B) legs. (C) Histopathological features. Intraepidermal blister with infiltrates of neutrophils, eosinophils and lymphocytes (H&E, ×200).

• 

  Fig. 2 Immunologcal findings. DIF shows (A) immunoglobulin G (IgG) and (B) complement 3 (C3) deposition along the cell surface of keratinocytes. (C) Results of immunoblotting of normal human epidermal extracts. Pemphigus vulgaris (PV) control serum reacted with the 160-kDa desmoglein (Dsg) 1 and the 130-kDa Dsg3 (lane 1), paraneoplastic pemphigus (PNP) control serum reacted with the 210-kDa envoplakin and the 190-kDa periplakin (lane 2), bullous pemphigoid (BP) control serum reacted with the 230-kDa BP230 and the 180-kDa BP180 (lane 3), anti-desmocollin (Dsc) monoclonal antibody (mAb) (lane 4) and the patient serum (lane 5) reacted strongly with the 110-kDa a-form and the 100-kDa b-form Dsc3. DIF: direct immunofluorescence.

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