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TOC and Sample Chapters - McGraw-Hill Professional

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Section 14 The Skin in Immune, Autoimmune, <strong>and</strong> Rheumatic Disorders 305<br />

Localized Cutaneous Amyloidosis ICD-10: E85.810/E85.430<br />

■ Three varieties of localized amyloidosis that are<br />

unrelated to the systemic amyloidoses.<br />

■ Nodular amyloidosis: single or multiple, smooth,<br />

nodular lesions with or without purpura on limbs,<br />

face, or trunk (Fig. 14-4A).<br />

■ Lichenoid amyloidosis: discrete, very pruritic,<br />

brownish-red papules on the legs (Fig. 14-4B).<br />

■ Macular amyloidosis: pruritic, gray-brown,<br />

reticulated macular lesions occurring principally on<br />

A B<br />

◧ ◐<br />

the upper back (Fig. 14-5); the lesions often have<br />

a distinctive “ripple” pattern.<br />

■ In lichenoid <strong>and</strong> macular amyloidosis, the amyloid<br />

fibrils in skin are keratin derived. Although these<br />

three localized forms of amyloidosis are confined<br />

to the skin <strong>and</strong> unrelated to systemic disease, the<br />

skin lesions of nodular amyloidosis are identical<br />

to those that occur in AL, in which amyloid<br />

fibrils derive from immunoglobulin light chain<br />

fragments.<br />

Figure 14-4. Localized cutaneous amyloidosis (A) Nodular. Two plaque-like nodules, waxy, yellowish-orange with<br />

hemorrhage. (B) Lichenoid amyloidosis. Grouped confluent scaly papules of livid, violaceous color. This is a purely cutaneous<br />

disease.

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