RESUMENLa paquidermodactilia (pachy: grueso; dermos: piel: dactylos: dedos) es un tipo de fibromatosis digital benigna, patología rara que afecta sobre todo a varones jóvenes. Su etiología no se conoce bien, pero algunos autores defienden los traumatismos repetidos como principal desencadenante. Presentamos el caso de un varón de 16 años con engrosamiento digital difuso en ambas manos de 6 años de evolución, asintomático. El paciente refirió tener un "tic" en las manos; consiste en el roce muy frecuente entre los dedos. Se trata de una patología benigna cuyo diagnóstico es clínico, por lo que no es necesario realizar pruebas adicionales que prolonguen el número de visitas del paciente al hospital y los trastornos que ello conlleva. Se debe hacer hincapié en el control del hábito por parte del paciente, para evitar traumatismos repetidos y lograr la mejoría del cuadro. Palabras clave: fibromatosis, paquidermodactilia, tejido conectivo.
The patient, a 56-year-old woman, presented with bilateral lesions in the mandibular region that appeared after infiltration of hyaluronic acid 2 months earlier. Initially, purplish papules appeared, and they progressively increased in size and ulcerated without affecting the oral mucosa or permeability of the Stensen duct. On examination, 2 bilateral, violaceous, well-defined ulcers were seen (left and right). The patient also had a hemicranial headache in association with disease in the maxillary sinus, in which, on biopsy, necrotizing granulomas were found. Chest radiography revealed multiple cavitary lesions. Results of antineutrophil cytoplasmic antibody testing were positive for a cytoplasmic immunofluorescence pattern with specificity for proteinase 3. During her hospital stay, the patient developed a progressive saddlenose deformity. Granulomatosis with polyangiitis (Wegener's) (GPA) was diagnosed, and the patient was started on systemic glucocorticoids and methotrexate, with good response. Mucocutaneous involvement in GPA occurs in ϳ40% of patients, but it is the presenting sign in just 10%. The presence of ulcers resembling pyoderma gangrenosum is a very rare manifestation.
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