Unilateral Hypertrophic Osteoarthropathy in a Patient With a Vascular Graft Infection

Abstract

Pachydermodactyly is a superficial benign fibromatosis of unknown etiology; it is rare, more frequent in adolescent males, and characterized by painless swelling of the proximal interphalangeal joints(PIP) of the hands. Histologic examination of the skin shows epidermal hyperplasia and increased number of dermal fibroblasts and collagen fibers.We report the case of a 16-year-old adolescent boy who presented swelling of the lateral and dorsal regions of all the metacarpophalangeal and PIP joints of the left hand and PIP and metacarpophalangeal joints of the second and fifth fingers of the right hand, with 3 years of evolution and no arthritis or functional impairment. Results of complementary diagnostic examinations were normal, with the exception of hand ultra sound that showed skin thickening, without synovial proliferation or joint effusion. Skin biopsy confirmed pachydermodactyly. The patient under went aesthetic surgery with good outcome, without recurrence.This rare condition should be distinguished from idiopathic juvenile arthritis and other entities such as knuckle pads syndrome.

Hypertrophic Osteoarthropathy: Clinical and Imaging Features

“…HOA secondary to vascular graft infection is treated by surgical removal of the infected prosthesis and intravenous antibiotic therapy (13). The prognosis of vascular graft infection is reportedly poor, however, with an overall survival of only 58% and a posttreatment complication rate of 57% (11).…”

“…Secondary HOA can also be further classified into generalized forms due to systemic diseases and localized forms limited to one or two extremities (Fig 1), usually due to prominent endothelial injury (eg, arterial aneurysm). In addition, HOA may be limited to the cyanotic limb in the setting of a patent ductus arteriosus complicated by pulmonary hypertension, and may manifest unilaterally in the setting of arterial graft infection (11)(12)(13) or a functioning arteriovenous fistula (14).…”

Coexistence of hypertrophic osteoarthropathy and myelofibrosis

“…1 HOA includes skin manifestations such as thickened skin on face and scalp, and coarse facial features along with clubbing, periostosis, acroosteolysis, and painful joint motion. 6 HOA may be associated with various disorders including intrathoracic malignancies, cyanotic heart diseases, gastrointestinal tumors and inflammatory bowel diseases.…”

“…6 HOA may be associated with various disorders including intrathoracic malignancies, cyanotic heart diseases, gastrointestinal tumors and inflammatory bowel diseases. 1 One of the rare causes of secondary HOA is myelofibrosis.…”

“…Primary HOA is a rare and hereditary condition observed mainly in children and adolescents. 1 Secondary HOA may be seen in the various systemic disorders including inflammatory and malign diseases. Clinical findings develop as a result of an increase in peripheral blood flow as well as abnormal fibroblast activity and proliferation.…”