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Acroosteolysis - Nail and Distal Digit
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Acroosteolysis - Nail and Distal Digit

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Contributors: Bertrand Richert MD, Robert Baran MD
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Synopsis

Acroosteolysis describes the occurrence of destructive changes of the distal phalangeal bone. Individuals cleaning or inspecting autoclaves for polymerization of vinyl chloride (VC) to polyvinyl chloride (PVC) have a high incidence of the development of acroosteolysis. The disease is also characterized by Raynaud phenomenon, sclerodactyly, and papular fibrotic lesions on the wrists and dorsa of the hands. Fingertips appear bulbous and are associated with shortening of the distal phalanges and pseudo clubbing. Progressive destruction of the bone produces peg-shaped phalanges. Inhalation of the vinyl chloride monomer is the method of exposure. The prevalence of acroosteolysis secondary to the VC monomer is 3–4% in maintenance workers.

The acroosteolysis secondary to PVC is the transverse variety. In transverse acroosteolysis, there are linear osteolytic bands in the distal phalanx, while longitudinal acroosteolysis shows "whittling" or penciling of the tufts resulting from their gradual resorption. Both types lead to acronecrosis. Interestingly, as time passes, the transverse bands reach the base of the distal phalanx. Cessation of exposure to the toxic vapors stops the development of the defect, and progressive recovery appears associated with bony callus.

Systemic symptoms may include fatigue, myalgias, cold hands and feet, paresthesias, reduced libido, and difficulty with hand grip. Other systemic exam findings include hepatosplenomegaly, thrombocytopenia, obstructive pulmonary disease, cryoglobulinemia, and cryofibrinogenemia. Raynaud phenomenon is usually the first symptom to occur in acroosteolysis secondary to PVC.

A rare autosomal dominant variant, sometimes called Band Acroosteolysis, is associated with Hadju-Cheney Syndrome, caused by a NOTCH2 mutation. Symptoms of Hadju-Cheney Syndrome can include short stature, generalized osteoporosis, bowing of the long bones, and vertebrae anomalies.

For more information, see OMIM.

Codes

ICD10CM:
M89.549 – Osteolysis, unspecified hand

SNOMEDCT:
27201004 – Acroosteolysis

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Transverse Acroosteolysis:
  • Renal osteodystrophy
  • Idiopathic nonfamilial acroosteolysis
  • Familial acroosteolysis
Longitudinal Acroosteolysis:

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Therapy

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Last Updated: 03/29/2017
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Acroosteolysis - Nail and Distal Digit
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Acroosteolysis : Fingers, Lytic bone lesions, Nail clubbing, Shortened distal phalanx, Vinyl chloride exposure, Myalgia
Clinical image of Acroosteolysis
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