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Dermatology

Amyloidosis

Built from Dermatology, 5th Edition

What’s inside

12 sections · 89 slides

  1. 01

    Overview

    • What this deck covers

    1 slide

  2. 02

    Introduction & classification

    What amyloid is, its history, how common it is, and how it is grouped

    • What amyloidosis is
    • Two ways dermatologists meet amyloidosis
    • Approaching amyloidosis involving the skin
    • History of amyloid
    • How common is amyloidosis
    • Two ways to classify amyloidosis
    • Clinical classification of amyloidosis
    • Reading the chemical classification table
    • Chemical classification of amyloidoses
    • Chemical classification of amyloidoses (continued)
    • Chemical classification of amyloidoses (continued)
    • Chemical classification of amyloidoses (continued)
    • Amyloid, grouped by its precursor protein

    13 slides

  3. 03

    Pathogenesis & amyloid properties

    How normal proteins misfold into amyloid, and how pathologists identify it

    • How amyloid forms
    • What drives primary cutaneous amyloid
    • Nodular amyloidosis has a different origin
    • Amyloid's structural signature
    • Cross-beta-pleated sheet structure of amyloid fibrils
    • Histologic and ultrastructural features of amyloid
    • Special stains that identify amyloid deposits
    • Histopathologic features of amyloid deposits 2

    8 slides

  4. 04

    Macular and lichen amyloidosis

    The itchy, pigmented skin-only forms, and how they differ

    • Macular, lichen, and biphasic amyloidosis
    • Macular amyloidosis
    • Macular amyloidosis - clinical and histologic features
    • Friction and notalgia paresthetica overlap
    • Lichen amyloidosis
    • Lichen amyloidosis of the shins and calves
    • Lichen amyloidosis in a linear array
    • Lichen amyloidosis of the forearm
    • Lichen amyloidosis of the legs
    • Lichen amyloidosis of the extensor arms
    • Unusual sites and disease associations
    • Cutaneous amyloidosis in systemic sclerosis

    12 slides

  5. 05

    Familial cutaneous amyloidosis syndromes

    Inherited gene changes that predispose to skin-only amyloid deposits

    • Genes behind familial skin amyloidosis
    • Familial cutaneous amyloidosis, by gene
    • IL-31 receptor-associated amyloidosis
    • Dyschromic amyloidosis
    • Dyschromic amyloidosis cutis
    • Sipple syndrome (MEN type 2A)
    • Partington amyloidosis (X-linked)
    • Other associations and drug-triggered amyloid
    • Anakinra-associated amyloidosis at an injection site

    9 slides

  6. 06

    Nodular amyloidosis

    The rarest cutaneous form, and why it needs long-term follow-up

    • Nodular amyloidosis - clinical features
    • Nodular amyloidosis - clinical and histologic features
    • Nodular amyloidosis of the plantar surface
    • Nodular amyloidosis papules of the sole
    • Does nodular amyloidosis progress to systemic disease

    5 slides

  7. 07

    Pathology & differential diagnosis

    What biopsies show, and the look-alike conditions to rule out

    • Pathology of macular and lichen amyloidosis
    • Macular amyloidosis on histology
    • Pathology of nodular amyloidosis
    • Look-alikes for macular amyloidosis
    • Look-alikes for lichen amyloidosis
    • Look-alikes for nodular amyloidosis

    6 slides

  8. 08

    Treating cutaneous amyloidosis

    Breaking the itch-scratch cycle, since no cure yet exists

    • Principles of treating macular and lichen amyloidosis
    • Treatment of macular and lichen amyloidosis
    • Escalating therapy for macular and lichen amyloidosis
    • More treatment options, ranked by evidence
    • Treating nodular amyloidosis

    5 slides

  9. 09

    Secondary cutaneous amyloidosis

    Amyloid found by accident inside unrelated skin growths

    • Secondary cutaneous amyloidosis
    • Hypotrichosis simplex of the scalp

    2 slides

  10. 10

    Primary systemic (AL) amyloidosis

    A life-threatening plasma cell disease, and how it shows up in skin

    • AL amyloidosis - the basics
    • Oral cavity findings in AL amyloidosis
    • Mucocutaneous findings in primary systemic AL amyloidosis
    • Macroglossia and tongue papulonodules in AL amyloidosis
    • Purpura - bruising from fragile vessels
    • Periorbital purpura in AL amyloidosis
    • Waxy papules and plaques in AL amyloidosis
    • Waxy translucent facial papules in AL amyloidosis
    • Other skin and nail changes in AL amyloidosis
    • Organ involvement beyond the skin
    • Confirming the diagnosis of AL amyloidosis
    • Evaluating a patient with suspected AL amyloidosis
    • Pathology of AL amyloidosis in the skin
    • Perivascular amyloid deposits on histology
    • Differential diagnosis of AL amyloidosis
    • Treating AL amyloidosis

    16 slides

  11. 11

    Secondary systemic (AA) amyloidosis

    Amyloid triggered by long-standing inflammation or infection

    • Two forms of systemic amyloidosis, at a glance
    • AA amyloidosis
    • Treating AA amyloidosis

    3 slides

  12. 12

    Other systemic and inherited amyloidoses

    Dialysis-related and inherited forms

    • Hemodialysis-associated amyloidosis
    • Finnish-type familial amyloidosis (AGel)
    • Transthyretin (ATTR) amyloidosis
    • Treating ATTR amyloidosis
    • Key takeaways
    • References
    • References (continued)
    • References (continued)
    • Dermatology, 5th Edition (2-Volume Set)

    9 slides