ABSTRACT
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Subject(s)
Humans , Male , Female , Skin Diseases, Vascular/classification , Skin Diseases, Vascular/etiology , Skin Diseases, Vascular/pathology , Biopsy/instrumentation , Biopsy/methods , Biopsy , Medical History Taking/methods , Laser Therapy/instrumentation , Laser Therapy/methods , Laser Therapy , Lasers, Dye/therapeutic use , Lasers, Dye , Skin Diseases/diagnosisABSTRACT
INTRODUCTION: Bullous pemphigoid is the most frequent blistering disease and has been found associated to several neurological diseases, including amyotrophic lateral sclerosis. CASE REPORT: A 63-year-old male with bulbar-onset amyotrophic lateral sclerosis who presented clinical and histological signs and symptoms of bullous pemphigoid. CONCLUSIONS: The association does not seem to occur by chance and we suggest an autoimmune pathogenetic mechanism consisting in a crossed reaction between bullous pemphigoid antigen 1 and the protein dystonin, which is involved in the organisation/integrity of the neuronal cytoskeleton.
Subject(s)
Amyotrophic Lateral Sclerosis , Pemphigoid, Bullous , Amyotrophic Lateral Sclerosis/complications , Amyotrophic Lateral Sclerosis/immunology , Humans , Male , Middle Aged , Pemphigoid, Bullous/etiology , Pemphigoid, Bullous/immunologyABSTRACT
Introducción. El penfigoide ampolloso es la enfermedad ampollosa más frecuente, y se ha encontrado asociada convarias enfermedades neurológicas, entre ellas la esclerosis lateral amiotrófica. Caso clínico. Varón de 63 años con esclerosis lateral amiotrófica de inicio bulbar, que presentó en su evolución un cuadro clínico e histológico de penfigoide ampolloso.Conclusión. La asociación no parece casual y se propone un mecanismo patogénico autoinmune consistente en una reacción cruzada entre el antígeno 1 del penfigoide ampolloso y la proteína distonina, implicada en la organización/integridad del citoesqueleto neuronal
Introduction. Bullous pemphigoid is the most frequent blistering disease and has been found associated to several neurological diseases, including amyotrophic lateral sclerosis. Case report. A 63-year-old male with bulbar-onset amyotrophic lateral sclerosis who presented clinical and histological signs and symptoms of bullous pemphigoid. Conclusions. The association does not seem to occur by chance and we suggest an autoimmune pathogenetic mechanism consisting in a crossed reaction between bullous pemphigoid antigen 1 and the protein dystonin, which is involved in the organisation/integrity of theneuronal cytoskeleton
Subject(s)
Humans , Male , Aged , Pemphigoid, Bullous/complications , Amyotrophic Lateral Sclerosis/complications , Pemphigoid, Bullous/drug therapy , Pemphigoid, Bullous/immunology , Pemphigoid, Bullous/etiology , Amyotrophic Lateral Sclerosis/drug therapy , Amyotrophic Lateral Sclerosis/immunology , Cross Reactions , Antigens, Ly/immunology , Adrenal Cortex Hormones/therapeutic use , Autoimmune Diseases/immunologyABSTRACT
Clinical description of a case of FMH in a 77 years-old man, heavy smoker and diabetic. At the beginning its appearance mimic a small polyp of the middle third of the right vocal cord. The patient refused any treatment at first, but one year later, when seen again in the office, a mass covering-up the right hemilarynx was detected and a total laryngectomy was advised and done. In spite of the correctness of the follow-up in the next six months, the unhappy commit suicide.
