Four main clinical signs characterize onychomatricoma, a recently identified tumour specific to the nail apparatus, with finger nails being much more often affected than toe nails:

• A yellow longitudinal band of variable width, leaving a single or double portion of normal pink nail on either side.

• Splinter haemorrhages involving the proximal nail region.

• A tendency toward transverse overcurvature of the nail.

• Nail avulsion exposes a villous tumour emerging from the matrix while the nail plate appears as a thickened funnel, storing the filamentous digitations fitting into the holes of its proximal extremity.

Interestingly, some clinical presentations may be confusing: for example, a longitudinal melanonychia may hide the yellow hue. A swelling at the junction of the proximal nail fold and the lateral nail fold may produce the appearance of cutaneous horn, sometimes completely separated from the nail plate. Associated onychomycosis may be misleading also. Magnetic resonance images are typical and allow confirmation of the clinical diagnosis (see p. 98).

Histological examination shows multiple 'glove finger' digitations lined with matrix epithelium oriented around antero-oblique connective tissue axes, and perforation of the nail plate by multiple cavities which (generally at the distal edge of the lunula) lose their epithelial digitations and become filled with serous fluid. The connective tissue stroma of the digitations extends deeply into the dermis and is not demarcated from healthy tissue.

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