Acroosteolysis - Nail and Distal Digit
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.
M89.549 – Osteolysis, unspecified hand
27201004 – Acroosteolysis
- Renal osteodystrophy
- Idiopathic nonfamilial acroosteolysis
- Familial acroosteolysis