Nail dystrophy as initial presentation of systemic amyloidosis

Abstract

Nail dystrophy as initial presentation of systemic amyloidosisSystemic amyloidosis is a rare group of diseases that result from the extracellular deposition of amyloid protein. In patients with systemic amyloidosis, the most common skin symptoms are petechiae and ecchymoses; nail dystrophy as the initial symptom is rare, with only one previous report.A 50-year-old Chinese man developed bilateral fingernail and toenail lesions without apparent cause, manifested as thickening and destruction of nails followed by petechia developing under and around his fingernails, face and on both upper limbs (Fig. 1). After scratching, erythematous blisters appeared accompanied by swelling of the hands. There was no significant hair loss, sclerodermallike thickening, pigmentary changes or bullous lesions. He went to a nearby hospital and was diagnosed with contact dermatitis or eczema. After treatment, there was no significant improvement so he came to our hospital.No obvious abnormalities in myocardial enzyme, C3, C4, ANA, echocardiography, abdominal ultrasound and urological ultrasound. CT scan of the head also showed no Source(s) of support: None. Conflicting Interest: None.

Nail Bed: An Unusual Site of Diagnosis for a Heart Failure Cause

“…Amyloidosis is a systemic disease with multiple clinical manifestations because of the deposit of amyloid substance in many tissues and, in rare cases, nail involvement can be the unique cutaneous sign to confirm the disease and avoid overlooked diagnosis. Multiple nail changes were related to amyloidosis, such as longitudinal striations [1-3], onychorrexis [1, 2], splinter hemorrhage [3], pterygium simulating lichen planus [1, 4], pachyonychia [4], and destruction of the nail plate [5]. Our case did not show longitudinal striations as most cases did.…”

“…These 5 cases occurred mainly in male patients (4:1) as the first presentation of systemic amyloidosis (all cases), and it can be related to multiple myeloma (3:2 with no underlying disease). Some studies indicated the improvement of the nail’s lesions as a predictor of treatment response [1, 5].…”

Nail changes in systemic amyloidosis

“…Nail involvement is rarely described and can be the unique sign of the disease. 3 It is usually characterized by brittleness, increased fragility, trachyonychia, longitudinal ridging, and subungual splinter hemorrhages. 3,4 Distal onycholysis and anonychia have also been described.…”

“…3 It is usually characterized by brittleness, increased fragility, trachyonychia, longitudinal ridging, and subungual splinter hemorrhages. 3,4 Distal onycholysis and anonychia have also been described. The main differential diagnosis is lichen planus.…”

“…The most common of them are periorbital purpura, macroglossia and waxy, translucent or purpuric papules. Nail involvement is rarely described and can be the unique sign of the disease 3 . It is usually characterized by brittleness, increased fragility, trachyonychia, longitudinal ridging, and subungual splinter hemorrhages 3,4 .…”