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.
Subject(s)
Aged , Female , Humans , Antibodies , Autoantibodies , Biopsy , Blister , Complement C3 , Dapsone , Desmogleins , Desmosomal Cadherins , Diagnosis , Enzyme-Linked Immunosorbent Assay , Eosinophils , Erythema , Fluorescent Antibody Technique, Direct , Hydrogen-Ion Concentration , Immunoblotting , Immunoglobulin G , Immunoglobulins , Keratinocytes , Leg , Lymphocytes , Mouth Mucosa , Neutrophils , Pemphigus , Physical Examination , Prednisolone , SkinABSTRACT
Introdução: O tratamento para o câncer invasivo do pênis basea-se no tratamento do tumor primário, geralmente com amputação e na linfadenectomia regional, tratamentos que apresentam alto índice de morbidade. A presença de metástase linfonodal constitui o mais importante fator prognóstico identificado e sua avaliação por métodos clínicos e laboratoriais são falhos. Objetivos: O presente estudo visa identificar os fatores prognósticos do tumor primário e a importância das Proteínas da Placa Desmossômicas: Desmogleina, Plakoglobina e Desmoplakina e da Involucrina e a relação destes com as variáveis clínicas, demográficas e anatomopatológicas, bem como sua relação com sobrevida câncer específica e global. População e Métodos: Foram avaliados 288 pacientes portadores de carcinoma espinocelular do pênis submetidos a tratamento do tumor primário e a linfadenectomia radical ou aqueles pacientes com 5 anos de seguimento sem linfadenectomia. Resultados: A imunorreatividade da Involucrina se correlacionou com o Grau de diferenciação tumoral (p <0,001); a presença de infiltração peri-neural (p=0,002) e infiltração de corpos cavernosos (p=0,021) e a presença de coilocitose (p=0,006) e o tipo Histológico (p=0,043). A imunorreatividade da Desmoplakina se correlacionou com Antecedentes Venéreos (p=0,,13) e o Estadiamento Clinico T (p=0,002) e o Tipo Histológico (p=0,070). A imunorreatividade da Desmogleina não se correlacionou apenas a infiltração de corpos cavernosos (p=0,022) e não houve correlação da imunorreatividade da Plakoglobina com as variáveis clínico-demográficas ou anatomopatológicas. ...
Subject(s)
Humans , Male , Penile Neoplasms , Immunohistochemistry , Desmosomal Cadherins , Lymphatic Metastasis , PrognosisABSTRACT
BACKGROUND: Pemphigus foliaceus(PF) and pemphigus vulgaris(PV) have different target antigens which belong to the desmoglein(DG) subfamily of the desmosomal cadherins; DG in the case of PF and PV antigen(PVA) in the case of PV. OBJECTIVE: Because DG is also a normal major component of the intercellular adhesive core, we investigated the immunohistochemical distribution of DG in PF to compare and contrast the findings with those of PV. METHODS: Immunohistochemical analysis using streptavidin-biotin complex method with anti-DG monoclonal antibody was done in six cases of PF and six cases of PV, as well as two samples of normal skin as control. RESULTS: Both disorders showed abnormal intense diffuse cytoplasmic staining patterns in the lesional skin. Contrary to PF, showing complete loss of normal, rim-like, membranous staining, two of six cases of PV showed the relatively well preserved normal staining pat-terns in the upper epidermis. CONCLUSION: The differences in the expression of DG in the lesional skins between PF and PV suggest that PVA is distinct from DG, although an overlapping of antigens between PF and PV could exist.