Dermatology - Cutaneous Signs of Systemic Disease

From Iusmicm

Contents 1 Cutaneous Signs of Systemic Disease 1.1 Rheumatology 1.1.1 Lupus erythematosus 1.1.1.1 Acute lupus erythematosus 1.1.1.2 Chronic (discoid) lupus erythematosus 1.1.2 Dermatomyositis 1.1.3 Scleroderma 1.1.4 Vasculitis 1.1.5 Aphthous ulcer 1.1.6 Behcet's 1.2 Endocrinology 1.2.1 Hypothyroidism (Myxedema) 1.2.2 Hirsutism 1.2.3 Diabetes Mellitus 1.3 Pulmonary 1.3.1 Sarcoidosis 1.4 Gastroenterology 1.4.1 Metabolic Disorders 1.4.1.1 Hyperlipidemia 1.4.1.2 Porphyria cutanea tarda 1.4.2 Chronic liver diasease 1.4.3 Inflammatory bowel disease 1.5 Hematology / Oncology 1.5.1 Leukemia 1.5.2 Lymphoma 1.5.3 Bone marrow transplantation 1.6 Psychiatry 1.6.1 Factitial 1.6.2 Neurotic excoriations 1.7 Cases 1.7.1 Case 1 1.7.2 Case 2 1.8 Casse 3

[edit] Cutaneous Signs of Systemic Disease

[edit] Rheumatology

[edit] Lupus erythematosus

[edit] Acute lupus erythematosus

• Cutaneous symptoms:
  • NB: lupus erythematous lesions are blanchable
  • Distribution is along photon exposure
  • Look for a malar rash
  • Violaceous = purple lesion; often means there are lymphocytes in the epidermis.

• Systemic symptoms:
  • circulating antibodies
  • immunoglobulin deposition in skin and other organs
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[edit] Chronic (discoid) lupus erythematosus

• Cutaneous symptoms:
  • Erythema with telangiectasis
  • Scaling, follicular plugging
  • Scarring that leads to alopecia
  • Hypopigmentation on the inside and hyperpigmentation on the outside.
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• Systemic symptoms:
  • Discoid LE usually remains localized to the skin
  • 5% will develop systemic symptoms like immunoglobulin

[edit] Dermatomyositis

• Cutaneous symptoms:
  • Gottron's papules over joints
  • Periungual erythema and "shaggy" cuticles
  • Periorbital heliotrope eruption: erythema / violaceous around the eyes
    • Spares the upper lip
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[edit] Scleroderma

• Cutaneous symptoms:
  • Called "localized scleroderma"
  • Light macules / papules
  • Hard to see; feels like hard scar tissue
  • Shows atrophy of the epidermis
  • Scarring alopecia

**Follows a dermatome?

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• Systemic symptoms:
  • Usually started in hands
  • Rounded off digits
  • Increased deposition of collage in the heart / lungs / kidneys / GI tract / joints / skin
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[edit] Vasculitis

• Cutaneous symptoms:
  • Palpable purpura

**Biopsy to diagnose vasculitis

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• Systemic symptoms:
  • Henoch-Schonlein purpura associated with renal or bowel involvement
  • Type 3 Ab reaction (immune complex) against the endothelium of the vasculature
    • Antibodies bind to an antigen and form a complex
    • The complex activates platelets (clotting) and the complement pathway (inflammation)
      • C3a and C5a are important complement factors that lead to inflammation
      • Vasoactive amines increase permeability and lead to erythema
    • Microthrombi are formed because of the activation of clotting on the deposits of immune complexes.
    • Endothelial damage occurs because of the inflammation that leads to enzyme release (neuts, basophils, etc.)
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• Hypersensitivity vasculitis:
  • Has 5 criteria; 3 or more required for dx:

1. Age >16
2. Use of a possible offending drug in temporal relation to the symptoms
3. Palpable purpura
4. Maculopapular rash
5. Biopsy of a skin lesion showing neutrophils around an arteriole or venule

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[edit] Aphthous ulcer

• Come in minor and major forms
• Little eronsions on the oral mucosa
• Minor are not usually a sign of systemic disease
• Think LE and HIV
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[edit] Behcet's

• Results in erythema nodosum
• Think HIV
• Erythema nodosum: type 3 hypersensitivity in the fat tissue
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[edit] Endocrinology

[edit] Hypothyroidism (Myxedema)

• Myxedema: "Swelling of the skin and underlying tissues giving a waxy consistency, typical of patients with underactive thyroid glands"
  • Lateral eyebrow loss in myxedema is classic board question.
  • Mucopolysaccharides in the skin.

• Cutaneous symptoms:
  • Puffy skin
  • Yellowish tint
  • Loss of lateral eyebrows
  • Dry, course, brittle hair
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• Systemic symptoms:
  • Typical hypothyroid stuff

[edit] Hirsutism

• Excess androgen production
  • Can be from ovary, adrenals, or drug-induced
  • Mind the racial differences and normal variation

• Cutaneous symptoms:
  • Lots of hair
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• Systemic symptoms:
  • may be an endocrine tumor

[edit] Diabetes Mellitus

• Cutaneous symptoms:
  • Necrobiosis lipoidica: well-demarcated areas of epidermal atrophy
    • Yellowing ulcerations
    • Telangiectasias
    • Anterior lower legs
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[edit] Pulmonary

[edit] Sarcoidosis

• Cutaneous symptoms:
  • Nasal / facial papules / plaques
  • Fingernail infections
  • Intra-scapular papules
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• Systemic symptoms:
  • Think lung issues
  • Distal digit bone deterioration
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[edit] Gastroenterology

[edit] Metabolic Disorders

[edit] Hyperlipidemia

• Cutaneous symptoms:
  • Xanthomas: firm, flesh-colored to yellowish, papules / plaques
    • At the eye, a xanthoma is called a xanthelasma
  • Xanthelasmas usually DO NOT indicate an underlying lipid issue
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[edit] Porphyria cutanea tarda

• Cutaneous symptoms:
  • Blisters / bullae and scarring on dorsum of hand
  • Hypertrichosis
    • Can be dx by demonstrating fluorescent serum
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• Systemic issues:
  • Liver disease
  • Acquired porphyria

[edit] Chronic liver diasease

• Cutaneous symptoms:
  • Spider telangiectasias
  • Palmar erythema
  • Jaundice
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[edit] Inflammatory bowel disease

• Cutaneous symptoms:
  • Ulcerations characterized by their rolled violaceous border
  • Called pyoderma gangrenosum: 50% have an underlying disease (think IBD, LE)
    • DO NOT DEBRIDE!
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[edit] Hematology / Oncology

[edit] Leukemia

• Cutaneous symptoms:
  • Leukemia cutis: purpuric or flesh-colored papules and plaques
  • Characteristically round
  • 10-50% of monocytic leukemia pts manifest leukemia cutis
  • 6-20% of lymphocytic and granulocytic leukemias manifest leukemia cutis
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[edit] Lymphoma

• Cutaneous symptoms:
  • Lymphoma cutis: cutaneous collections of T cells
    • appears as macules / pathces, plaques, OR nodules
    • Can be present for years, then start evolving into plaques and nodules.
    • Mycosis fungoides type...?
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[edit] Bone marrow transplantation

• Cutaneous symptoms:
  • GVHD:
    • Acute: Light erythema macules that spread down the head along the cheeks
    • Chronic: pink macules at the joints / tips of hands, depigmentation plaques
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[edit] Psychiatry

[edit] Factitial

• Cutaneous symptoms:
  • Look for odd borders, weird patterns, strange distributions, et cetera.
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[edit] Neurotic excoriations

• Cutaneous symptoms:
  • Excoriations made by pt's own manipulation: an uncontrolled cycle of re-damaging the skin.
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[edit] Cases

[edit] Case 1

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[edit] Case 2

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• Discoid lupus erythematosus

[edit] Casse 3

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• Spider telangiectasias
• Jaundice
• Palmar erythema
• Liver failure