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Case Report

Hypothenar Hammer Syndrome Followed by Systemic Sclerosis

MARC LAMBERT, PIERRE-YVES HATRON, ERIC HACHULLA, and BERNARD DEVULDER

ABSTRACT.We describe the first case of bilateral hypothenar hammer syndrome (HHS) followed by systemic sclerosis (SSc) that was associated with silica exposure (Erasmus syndrome). The patient was a woman smoothing tiles in an earthenware factory who presented with bilateral digital ischemia associated with Raynaud’s phenomenon. HHS was diagnosed, based on an angiographic study showing aneurysm of the ulnar arteries and occlusions of multiple digital arteries. Pulmonary silicosis was also diagnosed on pulmonary tomodensitometry. Two years later digital swelling with acroosteolysis developed. The FANA test was positive (titer 1:640) and anticentromere antibody tests were also positive. Esophageal manometry showed dysmotility of the lower esophagus. These findings were consistent with a diagnosis of SSc. (J Rheumatol 2000;27:2516–7)

Key Indexing Terms:

HYPOTHENAR HAMMER SYNDROME
SYSTEMIC SCLEROSIS
SILICA EXPOSURE
ERASMUS SYNDROME



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