Clinical and pathological analysis of 20 patients with pachydermodactly and follow-up of 6 patient / 中华风湿病学杂志

ABSTRACT

Objective To improve the understanding of the clinical features, differential diagnosis and prognosis of pachydermodactyly. Methods The clinical data of 20 patients with pachydermo dactyly and follow-up data of 6 patient were analyzed. Results Seventeen out of 20 patients were male. The average onset age was (15±2) years. All of them had asymptomatic, firm swellings which developed insidiously on both sides of the proximal interphalangeal (PIP) joints. All the patients indicated that they were in the compulsive habit of interlacing their fingers and squeezing them. For all patients, biochemical examinations were normal and there was no rheumatoid factor or antinuclear antibodies. X-ray and MRI showed that the thickening of soft tissue around PIP joints without changes in bones or joints structure. Histopathologic findings from the swellings reveMed marked hyperkeratosis, with slight epidermal hyperplasia, and a markedly thickened dermis with whorled, densely aggregated bundles of coarse collagen. Even though 8 patients in the group had gotten rid of the aforesaid habit for 3 to 22 years, the swellings of their PIP joints did not resolve. Conclusion Pachy-dermodactyly is a benign digital fibromatosis which usually involves the PIP joints. It can be distinguished from juvenile rheumatoid arthritis and knuckle pad.