Autoimmune Vasculitis Flashcards
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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
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Acute Cutaneous Lupus Erythematosus
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Localized Scleroderma
- Also known as morphea
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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
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Dermatomyositis
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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
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Dermatomyositis
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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)
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Chronic Cutaneous Lupus Erythematosus
Discoid Lupus
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Systemic Scleraderma
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Linear Morphea
- Discrete indurated linear bands
- May cross joint lines and lead to contractures
- Underlying subcutaneous tissue and muscle sometimes involved
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Acute Cutaneous Lupus Erythematosus
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Dermatomyositis
- Gottron’s Papules
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Cutaneous Vasculitis
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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
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Lupus Erythematosus
- Multisystem autoimmune disorder of unkown origin; likely the result of envrionmental factors (UV light, viruses, drugs, and hormones)
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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
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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
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Cutaneous Vasculitits
- Henoch-Schonlein Purpura
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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
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Dermatomyositis
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Cutaneous Vasculitis
Classification
- Large Vessel
- Medium Vessel
- Small and medium vessel
- Small vessel
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Raynaud’s
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Raynaud’s
- Systemic Scleroderma
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Chronic Cutaneous Lupus Erythematosus
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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
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Dermatomyositis
-Heliotrope Rash
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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
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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
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Chronic Cutaneous Lupus Erythematosus
- Scale
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Subacute Cutaneous Lupus Erythematosus
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Systemic Scleraderma
- Initially presents with edema and induration of hands and feet
- Raynaud's phenomenon
- Progresses to bilateral sclerosis on the fingers, hands, and face
- Eventually results in atrophy of skin and subcutaneous tissue
- Dyspigmentation; salt-and-pepper appearance
- Nail fold capillary dilatation and telangiectasias are common
- Calcinosis
- Reduced oral aperature
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Subacute Cutaneous Lupus Erythematosus
- Systemic manifestations rarely severe if present (arthralgias and fatigue common)
- Prognosis: intermittent recurrences over long periods of time, 15% develop SLE
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Vitiligo
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Chronic Cutaneous Lupus Erythematosus
- Discoid rash refers to erythematous, rasied patches, with adherent scale and follicular plugging
- Face, scalp, ears and neck commonly involved
- Results in alopecia, dyspigmentation, atrophy, and scarring
- Prognosis: disease can be largely controlled,
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Localized scleroderma
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Systemic Scleraderma
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Subacute Cutaneous Lupus Erythematosus
- Most common in white females
- Erythematous, scaly papules that evlove into hyperkeratotic papulosquamous or annular/polycyclic plaques
- Dyspigmentation and telangiectasias often present
- Photosensitivity prominent
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Dermatomyositis
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Localized Scleroderma
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Dermatomyositis
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Neonatal Lupus
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Dermatomyositis
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Raynaud's
- Limited Systemic Scleraderma and CREST syndrome
- CREST is an older term used to describe a unique subset of limited systemic scleraderma with the following features:
C-calcinosis cutis
R- Raynaud's
E- esophageal dysmotility
S- sclerodactyly
T- Telangiectasia
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Systemic Scleraderma
- "sausage fingers"
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Henoch-Schonlein Purpura
- Palpable purpura
- Often preceded by streptococcal infection
- Most common vasculitis in children
- DX: Presence of 2 of the 4
- Palpable purpura
- Age
- Bowel angina or diffuse abdominal pain
- Arteriole or venule wall granulocytes on biopsy
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Subacute Cutaneous Lupus Erythematosus
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Acute Cutaneous Lupus Erythematosus
- Prognosis dictated by SLE activity; HTN, nephritis Systemic vasculitis, and CNS disease
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Urticarial Vasculitis
- Presents as an eruption of erythematous, pruritic wheals resembling hives
- Often painful or burning in nature lasting more than 24 hours, resolves with purpura or hyperpigmentation
- Pts. may have photosensitivity, lymphadenopathy, arthralgia, angioedema, fever, abdominal pain, renal dx, dyspnea, pleural and pericardial effusions
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Discoid Lupus
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Cutaneous Vasculitis
- Signs and Symptoms:
- Small Vessel: Palpable purpura or infiltrated erythema (urticaria); Pustules, vesicles, petechiae and urticarial papules
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Linear Scleroderma
(En coup de Sabre)
- Multisystem autoimmune disorder characterized by sclerosis of the skin, blood vessels, and visceral organs, including the GI tract, lungs, heart, musculoskeletal, and kidneys.
- Often diagnosed between 35-64 years of age
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Bullous Pemphigoid
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Acute Cutaneous Lupus Erythematosus
ACLE
- Butterfly rash
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Acute Cutaneous Lupus Erythematosus
- Butterfly rash on malar and nasal bridge
- May have associated edema
- Diffuse morbiliform or maculopapular rash, particularly on the ears and chest
- Photosensitivity
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Systemic Scleroderma
- DX: One major or two minor
- Major: Proximal sclerosis
- Minor: Sclerodactyly -finger fixed semiflexed position with tightened skin; digital pitting scars, erosions, or loss of substance of the finger pad; basilar lung fibrosis or pulmonary fibrosis
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Cutaneous Vasculitis
