AL amyloidosis - Nail and Distal Digit
See also in: Overview,External and Internal Eye,Oral Mucosal LesionSynopsis
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Nail changes in amyloidosis are much less common than cutaneous manifestations. Nail dystrophy is secondary to nail matrix and bed amyloid deposition leading to faulty nail plate production. Nail dystrophy usually slowly worsens over time. In about one-half of cases, cutaneous manifestations precede nail changes. In about 25% of cases, nail changes may be the presenting sign of amyloidosis.
The most common underlying diagnosis in patients with nail changes is myeloma-associated systemic amyloidosis, followed by an underlying monoclonal gammopathy; in some cases, amyloidosis is idiopathic.
For nail dystrophy due to amyloidosis, there is a slight male predominance. It usually presents in the sixth or seventh decade (range: 49-81 years).
The severity of cardiac dysfunction at the time of diagnosis impacts the survival of patients with systemic AL amyloidosis. Patients without cardiac impairment survive for many years, while patients who are diagnosed after advanced heart damage have a median survival of 3-6 months.
Related topics: multiple myeloma, monoclonal gammopathy
Codes
E85.81 – Light chain (AL) amyloidosis
SNOMEDCT:
23132008 – AL amyloidosis
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Last Updated:10/02/2024
