Autoimmune Vasculitis Flashcards Preview

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Flashcards in Autoimmune Vasculitis Deck (57)
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1

Linear Morphea (En coup de sabre)

- Differs from other forms of liner morphea by presenting in childhood

- Atrophic depression resembling a stroke from a sword

- May be extensive and cause hemifacial atrophy 

- Neurologic abnormalities may occur

2

Acute Cutaneous Lupus Erythematosus 

2

Localized Scleroderma

- Also known as morphea

2

Cutaneous Vasculitis 

- Term referring to a specific pattern of blood vessel inflammation

- Skin involvement may occur alone or as part of a systemic disorder

- Arteries and veins in any organ may be affected in systemic disease 

3

Dermatomyositis

4

Dermatomyositis

-DX: Two major criteria or one major and two minor combined with biposy showing changes consistent with DM

- Major: heliotrope rash, Gottron's papules, and Gottron's sign

- Minor: macular violaceous erythemas (scalp, malar eminences, forehead, chin, V sign, shawl sign, extensor arms, periungal areas, holster sign, dorsal hands, medial malleoli) periungal nail fold telangiectasia poikiloderma, mechanic's hands, cutaneous calcinosis, cutaneous ulcers, and pruritis

4

Dermatomyositis

5

Localized Scleroderma

Classification: 

- Raynaud's and internal involvement does not occur

- Plaque morphea (variants include morphea en plaque, guttate, keloidal or nodular, and atrophoderma of pasini and pierini)

-Linear scleroderma (variants include linear morphea, en coup de sabre or frontoparietal linear morphea, and Parry-Romberg syndrome)

- Generalized Morphea

- Bullous Morphea

- Deep Morphea (variants include eosinophilic fascitis, morphea profunda, and disabling pansclerotic morphea)

7

Chronic Cutaneous Lupus Erythematosus

Discoid Lupus 

9

Systemic Scleraderma 

9

Linear Morphea

- Discrete indurated linear bands

- May cross joint lines and lead to contractures

- Underlying subcutaneous tissue and muscle sometimes   involved 

10

Acute Cutaneous Lupus Erythematosus 

10

Dermatomyositis

- Gottron's Papules 

10

Cutaneous Vasculitis 

11

Dermatomyositis

-Heliotrope Rash 

- Multisystem disorder affecting the skin and skeletal muscles but may affect other organ systems such as the joints, esophagus, heart, and lungs

- Believed to be an autoimmune process targeting the endothelium of endomysial capillaries leading to vascular compromise. 

- Genetic predisposition

12

Lupus Erythematosus

- Multisystem autoimmune disorder of unkown origin; likely the result of envrionmental factors (UV light, viruses, drugs, and hormones)

13

Plaque Morphea

- Begin as poorly defined areas of non-pitting edema

- Circumscribed, purplish or lilac indurated plaques develop

- Eventually the surface becomes ivory colored, smooth, and shiny

- Over several years, skin becomes atrophic often with residual hyperpigmentation

13

Vitiligo

- Acquired loss of pigmentation most likely due to formation of Ab to epidermal melanocytes

- Two main types: 

- Type A: symmetrical well-defined white macules and patches progressing slowly over many years; halo nevi, Koebner's phenomenon, and immunologic diseased may coexist 

- Type B: segmental or asymmetric distribution 

13

Cutaneous Vasculitits

- Henoch-Schonlein Purpura 

14

Systemic Scleraderma

- Other findings: 

- Musculoskeletal: arthralgia, myalgia, weakness, loss of ROM

- GI tract: reflux dx, dysphagia

- Lungs: Dyspnea, restrictive lung dx, pulmonary HTN

- Cardiovascular: conduction abnormalities, CHF, effusions, myocardial fibrosis

- Kidneys: HTN, renal crisis, chronic renal insifficiency

15

Dermatomyositis

 

16

Cutaneous Vasculitis 

Classification 

- Large Vessel

- Medium Vessel

- Small and medium vessel

- Small vessel 

17

Raynaud's 

19

Raynaud's 

- Systemic Scleroderma 

21

Chronic Cutaneous Lupus Erythematosus

 

23

Cutaneous Leukocytoclastic Vasculitis

- Usually localized to the skin, but may manifest in other organs

- Palpable purpura, hemorrhagic macules and/or vesicles, urticarial lesions

- Lesions may coalesce to form plaques, ulceration possible, but uncommon 

- Most frequently found on lower legs

- Fever, malaise, burning pain, pruritis may be present

24

Dermatomyositis

 

-Heliotrope Rash 

25

Bullous Pemphigoid

- Autoimmune disorder characterized by subepidermal blistering

- Mostly a disease of the elderly 

- May initially present as urticarial lesions with severe pruritis

- Classic lesions are large, tense bullae with good structural integrity (compared to flaccid, easily ruptured bullae of pemphigus)

- Negative Nikolsky's sign

- Bullae rupture leaving an eroded base that heals rapidly without scarring 

26

Cutaneous Leukocytoclastic Vasculitis/Angiitis

- AKA: Cutaneous Small Vessel Vasculitis and hypersensitivity, vasculitis or drug/infection-induced vasculitis

- S/S: Medication at dx onset, palpable purpura, maculopapular rash

- Causes include medications, streptococcus and numerous other infections, Henoch-Schonlein purpura, connective tissue disease, malignancies, serum sickness, HCV

 

27

Chronic Cutaneous Lupus Erythematosus

- Scale