A Case of Pemphigus Herpetiformis with Only Immunoglobulin G Anti-Desmocollin 3 Antibodies.
نویسندگان
چکیده
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.
منابع مشابه
Paraneoplastic pemphigus herpetiformis with IgG antibodies to desmoglein 3 and without mucosal lesions.
BACKGROUND Pemphigus herpetiformis (PH) is a rare clinical entity that combines the clinical features of dermatitis herpetiformis and the immunopathologic features of pemphigus. The target antigen is usually desmoglein 1, with exceptional cases manifesting autoantibodies against desmoglein 3. More recently, it has been found that many patients with PH also demonstrate autoantibodies against des...
متن کاملγδ T-cell lymphoma mimicking Sézary syndrome.
11. Kozlowska A, Hashimoto T, Jarzabek-Chorzelska M, Amaga A, Nagata Y, Strasz Z, Jablonska S. Pemphigus herpetiformis with IgA and IgG antibodies to desmoglein 1 and IgG antibodies to desmocollin 3. J Am Acad Dermatol 2003; 48: 117-122. 12. Bruckner AL, Fitzpatrick JE, Hashimoto T, Weston WL, Morelli JG. Atypical IgA/IgG pemphigus involving the skin, oral mucosa, and colon in a child: a novel ...
متن کاملPemphigus herpetiformis with progression to pemphigus foliaceus: a case report.
Pemphigus herpetiformis (PH) is an uncommon variant of pemphigus that accounts for an estimated 6% to 7.2% of all cases of this skin disorder. The term pemphigus herpetiformis was coined by Jablonska et al. in 1975 to describe a entity that was clinically similar to dermatitis herpetiformis, showed acantholysis on biopsy, and responded to sulfapyridine. The authors considered that PH was a vari...
متن کاملConcomitant pemphigus herpetiformis and sarcoidosis
Dsg: desmoglein H&E: hematoxylin-eosin IL: interleukin PH: pemphigus herpetiformis INTRODUCTION Pemphigus herpetiformis (PH) is a rare variant of pemphigus and accounts for approximately 7% of cases. PH combines the clinical features of dermatitis herpetiformis with the histologic features of pemphigus. Clinically, the plaques are herpetiform or even annular. PH follows a more benign course wit...
متن کاملDetection of IgA autoantibodies to desmogleins by an enzyme-linked immunosorbent assay: the presence of new minor subtypes of IgA pemphigus.
OBJECTIVE To examine the frequency of antidesmoglein 1 (Dsg1) and antidesmoglein 3 (Dsg3) IgA autoantibodies in IgA pemphigus. DESIGN We developed an enzyme-linked immunosorbent assay against recombinant Dsg1 and Dsg3 to detect IgA autoantibodies. PATIENTS Twenty-two patients with IgA pemphigus were studied. Among them, 10 patients had subcorneal pustular dermatosis type, 9 patients had int...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- Annals of dermatology
دوره 28 1 شماره
صفحات -
تاریخ انتشار 2016