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Vol. 5. Issue 6.
Pages 259-263 (November - December 2009)
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Vol. 5. Issue 6.
Pages 259-263 (November - December 2009)
DOI: 10.1016/S2173-5743(09)70134-0
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Primary hypertrophic osteoarthropathy (pachydermoperiostosis). Report of 2 familial cases and literature review
Osteoartropatía hipertrófica primaria (paquidermoperiostosis). Aportación de 2 casos familiares y revisión de la literatura
Norberto Gómez Rodríguez??
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Corresponding author.
, Jesús Ibáñez Ruán, Marisol González Pérez
Servicio de Reumatología, Hospital POVISA, Vigo, Pontevedra, Spain
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The primary hypertrophic osteoarthropathy (pachydermoperiostosis) is a hereditary disease characterized by skin thickening (pachydermia), finger clubbing, and proliferation of periosteum (periostitis) with subperiosteal new bone formation. We describe the cases of 2 brothers of 30 and 24 years, who consulted due to bone pain, arthralgia, and oligoarthritis. Pachydermia, hyperhidrosis, seborrhea, digital clubbing, periostosis, and non-inflammatory effusions of the knees. The first had been diagnosed with juvenile idiopathic arthritis at age 15, while the youngest also presented with a thoracic scoliosis, hypertrophic gastritis, iron deficiency anemia, and glucose intolerance by pancreatic endocrine dysfunction. In both patients, symptoms were controlled satisfactorily with etoricoxib (90mg/day) and risedronate (35mg/week).

Primary hypertrophic osteoarthropathy
Hypertrophic gastritis
Digital clubbing

La osteoartropatía hipertrófica primaria (paquidermoperiostosis) es una enfermedad hereditaria caracterizada por engrosamiento cutáneo (paquidermia), dedos en palillo de tambor y proliferación perióstica (periostosis) con neoformación ósea subcortical. Describimos los casos de 2 hermanos de 30 y 24 años, que consultaron por dolores óseos, artralgias y oligoartritis. En ambos casos se objetivó paquidermia, hiperhidrosis, seborrea, hipocratismo digital, periostosis y derrame articular en las rodillas de características no inflamatorias. El primero había sido diagnosticado de artritis idiopática juvenil a los 15 años, mientras que el más joven también presentaba una escoliosis dorsal, gastritis hipertrófica, anemia ferropénica crónica e intolerancia a la glucosa por disfunción endocrina pancreática. Los síntomas se controlaron satisfactoriamente en ambos enfermos con etoricoxib (90mg/día) y risedronato (35mg/ semana).

Palabras clave:
Osteoartropatía hipertrófica primaria
Gastritis hipertrófica
Dedos en palillo de tambor
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Reumatología Clínica (English Edition)

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