Clinical and immunoserological features of 7 cases of anti-p200 pemphigoid / 中华皮肤科杂志

Objective:To analyze clinical and immunoserological features of patients with anti-p200 pemphigoid.Methods:Clinical data were collected from patients with confirmed anti-p200 pemphigoid in Hospital of Dermatology, Chinese Academy of Medical Sciences from January 2015 to October 2021, and their clinical and immunoserological characteristics were retrospectively analyzed.Results:Seven patients with anti-p200 pemphigoid were included. Indirect immunofluorescence on salt-split skin (IIF-SSS) showed that serum IgG antibodies of the 7 patients were located in the dermis of the salt-split skin, and Western blot analysis with dermal extracts as substrates revealed a protein band with a relative molecular mass of 200 000. Four patients presented with classic bullous pemphigoid-like skin lesions, 2 initially presented with eczematous lesions, and 1 presented with linear IgA bullous dermatosis-like skin lesions. Circulating IgG antibodies could recognize the recombinant laminin γ1 C-terminal region in 6 cases. Four patients received different doses of systemic glucocorticoids, 1 of whom was resistant to high-dose systemic glucocorticoids (equivalent to 1.4 mg·kg -1·d -1 prednisone) ; 2 responded well to minocycline and dapsone; 1 was lost to follow-up. Four patients achieved complete remission and discontinued the treatment at a mean follow-up of 22.5 months; 2 received complete remissiona on minimal therapy at a mean follow-up of 8 months. Conclusion:Patients with anti-p200 pemphigoid presented with heterogeneous clinical manifestations, and the recombinant C-terminal fragment of laminin γ1 can serve as a reliable antigen substrate for the detection of autoantibodies in patients with anti-p200 pemphigoid; some patients can eventually achieve complete remission off treatment.

Anti-laminin γ1 pemphigoid: a case report / 中华皮肤科杂志

A 55-year-old male patient presented with tense bullae on the extremities and trunk.Histological examination revealed subepidermal vesicles and superficial dermal infiltration of eosinophils and lymphocytes.The patient was primarily diagnosed with bullous pemphigoid.However,serum autoantibodies of the patient bound to the dermal side of salt-split skin,and no serum antibodies against BP180,BP230 or type Ⅶ collagen were detected by enzyme-linked immunosorbent assay.Hence,the diagnoses of bullous pemphigoid and epidermolysis bullosa acquisita were excluded.As Western blot and immunoprecipitation analysis showed,there existed antibodies capable of binding to a dermal antigen with a relative molecular mass of 200 000 in the serum of the patient.Based on the above findings,the patient was diagnosed as anti-laminin γ1 (p200) pemphigoid.

Anti-p200 Pemphigoid / 대한피부과학회지

Anti-p200 pemphigoid is an autoimmune subepidermal bullous disease characterized by circulating and tissue-bound autoantibodies directed against a 200 kD protein of the human dermis. We report a 78-year-old male who was presented with bullous eruptions on the trunk, extremities, which clinically resemble bullous pemphigoid, epidermolysis bullosa aquisita, linear IgA dermatosis or dermatitis herpetiformis. Oral muscosa of the lower lip was also affected. Histopathological examination of a skin biopsy specimen from the trunk revealed subepidermal blister and infiltration predominantly by neutrophils. Direct immunofluorescence revealed linear deposits of IgG, C3 and IgA at the basement membrane zone. Indirect immunofluorescence using salt-split skin showed that IgG antibodies bound on the dermal side. Immunoblotting with dermal extracts showed that the patient's IgG autoantibodies reacted with a 200 kD protein. The patient showed good response to dapsone.

Anti-p200 Pemphigoid / 대한피부과학회지

Anti-p200 pemphigoid is an autoimmune subepidermal bullous disease characterized by circulating and tissue-bound autoantibodies directed against a 200 kD protein of the human dermis. We report a 78-year-old male who was presented with bullous eruptions on the trunk, extremities, which clinically resemble bullous pemphigoid, epidermolysis bullosa aquisita, linear IgA dermatosis or dermatitis herpetiformis. Oral muscosa of the lower lip was also affected. Histopathological examination of a skin biopsy specimen from the trunk revealed subepidermal blister and infiltration predominantly by neutrophils. Direct immunofluorescence revealed linear deposits of IgG, C3 and IgA at the basement membrane zone. Indirect immunofluorescence using salt-split skin showed that IgG antibodies bound on the dermal side. Immunoblotting with dermal extracts showed that the patient's IgG autoantibodies reacted with a 200 kD protein. The patient showed good response to dapsone.

Penfigoide anti p-200 / Anti-p200 pemphigoid

Se efectúa una revisión del penfigoide ampollar anti-P200 desde el punto de vista clínico, histopatológico, inmunológico como su tratamiento y múltiples diagnósticos diferenciales.

A review of the anti-P200 pemphigoid from the clinical, histopathological, immunological are updated, as well assess to its treatment and various differentials diagnosis.

Two Cases of Anti-p200 Pemphigoid / 대한피부과학회지

Anti-p200 pemphigoid is a recently defined subepidermal immunobullous disease. Most anti-p200 pemphigoid patients show the clinical features of bullous pemphigoid, dermatitis herpetiformis and linear IgA disease. It is characterized by the binding of circulating IgG autoantibodies to the dermal side of 1 M NaCl split skin, and by reactivity of these autoantibodies to a unique 200-kD antigen on immunoblot of dermal extract. On immunoelectron microscopic examination, these autoantibodies deposit at the lamina lucida-lamina densa interface. Herein, we report two cases of anti-p200 pemphigoid, as well as their favorable response to treatment with systemic corticosteroid, colchicine and dapsone.

A Korean Case of Anti-p200 Pemphigoid

Anti-p200 pemphigoid is a newly defined autoimmune subepidermal blistering disease, which is characterized by the presence of IgG autoantibodies to the dermal side of 1M NaCl split skin as well as by the reactivity of these antibodies to a novel 200-kDa antigen on immunoblot analysis of a dermal extract. We describe a 49-year-old Korean male who presented with a bullous eruption on the whole body, which clinically resembled bullous pemphigoid or epidermolysis bullosa acquisita. A histopathological examination of a lesional skin biopsy specimen showed an area of dermal-epidermal separation and mixed dermal inflammatory infiltrates consisting of lymphocytes, neutrophils, and eosinophils. Direct immunofluorescence showed a linear deposition of IgG and C3 along the basement membrane zone. Indirect immunofluorescence demonstrated circulating IgG autoantibodies directed against the dermal side of the 1M NaCl split skin. Immunoblot analysis of dermal extracts revealed the patient's sera recognized the 200-kDa antigen. This is the first Korean case of an anti-p200 pemphigoid who showed good response to the treatment with systemic corticosteroids and dapsone.

Immunofluorescence studies on the skin sections of recessive dystrophic epidermolysis bullosa patients with anti-P_ (200) pemphigoid sera / 中国免疫学杂志

Objective:In order to determine the nature of P 200 autoantigen Methods:12 cases of anti-P 200 pemphigoid sera were collected The skin sections from 6 cases of recessive dystrophic epidermolysis bullosa were studied with these sera by indirect immunofluorescence Results:All the 12 anti-P 200 pemphigoid sera could react with basement membrane zone (BMZ)of 5 cases of recessive dystrophic epidermolysis bullosa, while epidermolysis bullosa acquisita sera were negative in these skins In addition, in a case of recessive dystrophic epidermolysis bullosa, epidermolysis bullosa acquisita sera react with both BMZ and intracytoplasmic deposition of type Ⅶ collagen, while no anti-P 200 pemphigoid sera showed this reactivity Conclusion:These results suggested that the 200 kD antigen is not a component of type Ⅶ collagen, but a specific autoantigen