Other Vascular disorders Flashcards by Malindi Haggett

How do you classify telangectasia?

Primary:
- Spider telangectasia
- Hereditary benign telangectaisa
- angioma serpifinosum
- unilateral naevoid telangectaisa
- generalised essential telangectaisa
- cutaneous collagenous vasculopahty

Congential:
- CMTC
- KTS
- HHT
- AT
- Xeroderma pigmentosa
- Bloom sydrome
- Rombo syndrome
- Rothman thompson
- hypotrichosis - lymphoedema - telangectasia syndrome

Secondary:

To physical damage
- Photodamage
- Radiation
- Traumatic
- venous hypertension

Skin disease
- rosacea
- BCC
- residual Infantile haemangiomas

Hormonal
- Estrogen related (liver disease, Pregnancy, exogenous)
- corticosteroids

Medication related
- exogenous hormones
- Anti-neoplastic agents
- corticosteroids

Systemic conditions
- AICTD (lupus, scleroderma, dermatomyositis)
- GVHD
- Carcinoid syndorme
- Mastocytosis
- Cutaneous lymphoma - MF, B cell
- Angiolupoid sarcoidosis
- HIV

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What is a telangectasia?

Abmormal, small, peristently dilated blood vessel visible in the skin

Due to vascular dilatation of capillaries, venules or small AV malformaitons

Characteristics:
- erythematous to violaceous
- empty with pressure

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What type of telangectasia is shown here?

Spider telangectaisa

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What is your preferred diagnosis?

Generalised Essential Telangectasia

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What is generalised essential telangectasia?

A Primary disorder

Characterised by sheets of asymptomatic blanchable telangectasias

Often starts at the distal extermities, but is progressive and becomes more widespread

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What is a spider telangectasia

Also called a spider naevus / spider angioma

It is form of telangiectasia with a
central feeding arterial vessel.

Characterised by a localized lesion that has a slightly raised central red papule (which often becomes more prominent with time) and multiple small, radiating, dilated vessels.

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Spider telangectasia associations?

Often occur in healhty individuals

Multiple lesions may be a sign of liver disease or hormonal changes (pregnancy, OCP)

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Preferred diagnosis?

Hereditary, benign telangectasias

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Preferred diagnosis?

Unilateral naevoid telangectasia

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Preferred diagnosis?

Cutaneous collagenous vasculopathy

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Histopathology findings in Cutanoues collagenous vasculopathy?

ectatic superficial blood vessels with hyalinized and laminated, concentric concretions surrounding the basement membrane of affected vessels.

With special staining, this material is PAS-positive and diastase-resistant, and by immunohistochemistry, it reacts with antibodies against collagen type IV

the deposits have been shown to consist of collagen with abnormal banding patterns (Luse bodies)

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Diagnosis?

Naevus anaemicus

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This gentleman has a history of epistaxis and haemoptysis

HHT

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Diagnosis?

Venous lake

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Diagnosis?

angiospastic macules (bier spots)

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What is a bier spot?

Represent a **pattern of
vascular mottling **consisting of white macular areas surrounded by a red to occasionally blue cyanotic background.

Become more pronounced when the limb is in a dependant position

Benign physiological response

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Bier spots might be seen in which conditions?

pregnancy,

lymphedema,

cryoglobulinemia,

capillary malformation -arteriovenous malformation syndrome (especially type 2 due to EPHB4 mutations),

tuberous sclerosis complex, and aortic hypoplasia.

BASCULE - syndrome features Bier anemic spots, cyanosis (acral), and an urticaria-
like eruption in association with orthostatic intolerance.

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Diagnosis?

Spider angioma

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Diagnosis?

Generalised essential telangectasia

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What is angioma serpiginosum?

Rare vascular disorder with a characteristic appearance

Multiple, small, asymptomatic, non-palpable, deep-red to purple puncta occur in small clusters
and sheets.

The extremities are most commonly affected, initially with a unilateral distribution; over months to years, the involvement may become more widespread
The palms, soles, and
mucous membranes are not involved.

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What is unilateral naevoid telangectasia?

Telangiectasias in this condition are usually confined to the trigeminal or upper cervical dermatomes

Congenital and acquired forms are recognized.

It has been proposed that an increase in estrogen receptors on blood vessels in affected areas and/or an increase in estrogen levels is causative.

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What is erythromelalgia?

an episodic condition characterized by a burning
sensation, erythema, and increased skin temperature that affects the acral sites

How is erythromelalgia classified?

Three forms:

Type 1: ass with thrombocytosis
Type 2: idiopathic / primary
Type 3: ass with another underlying cause

What are the clinical features of erythromelalgia?

burning, erythema, and **warmth **of acral sites

Usually episodic
most commonly occur late in the day, last through the night

The affected areas will often appear red and swollen. Other findings include acrocyanosis, livedo reticularis, facial flushing, cutaneous
necrosis, and ulceration.

The hands are more commonly invovled than the feet in erythromelalgia

Flase - feet in 90%, hands in 25%

Type I erythromelalgia normally presents with bilateral involvement

False - often unilateral to start with

Triggers for erythromelalgia attack?

Minor increases in temp (between 32 - 36 degrees) Other factors include: exercise, standing, walking, fever, and limb dependency.

Type II erythromelalgia has high risk of ischaemic necrosis than type 1

False

What diseases are associated with type III erythromelalgia?

SLE and other AICTD Diabetes Mellitus Peripheral arterial disease Vasculitis Malignancy and myelodysplastic syndromes

What is the prognosis for erythromelalgia?

30% improve 30% are stable 30% get worse 10% resolve

Treatment of erythromelalgia

Challenging General: - cooling the affected area: fans, wet dressings, ice packs - leg elevation Specific: - Oral analgesia For Type 1: - Aspirin - Hydroxyurea - JAK inhibitors Topical - capsaicin cream 1 -2 % - amitriptalyine 1 - 2% - Lidnocaine patches Systemic: - ssris - TCAs - anticonvulsants - CCBs

What is the mutation for primary familial erythromelalgia?

SCN9A

What are the key features of carcinoid syndrome?

What is flushing?

a term used to describe transient and episodic reddening of the skin It is a visible sign of a generalized increase in cutaneous blood flow.

What is the difference between flushing and blushing?

Blushing is a subtype of flushing Emotionally triggered flushing - considered an exagerated physiological response.

List the exogenous causes of flushing?

Alcohol Durgs: * ACEi * Calcium channel blockers * Cholinergic agonists * Cyclosporine * Dupilimab * Hormonal therapy * Opiates * Gold * Nicotinic acid * Tamoxifen Foods Food additives: - MSG, sodium nitrate

Causes of flushing:

Physiologic Exgenous (medications, alcohol, food) Menopause Neurological disorders Dumping syndrome due to rapid gastric emptying Systemic disease

Clinical appraoch to the evaluation of flushing?

Treatment of idiopathic flushing?

Non-selective Beta Blockers Clonidine transthoracic endoscopic sympathomectomy...

Repeated flushing may result in fixed erythema

What is the carcinoid flush for midgut tumours?

Occur in ~ 10% of midgut tumours ONLY when there are liver mets Lasts minutes and consists of erythema and pallor and a cyanotic hue

What is the carcinoid flush for type III gastric carcinoid tumours?

Pruritic, patchy, bright red flush, admixed with white patches

Bronchial tumours are ass with prolonged (hours to days) intensely red, purple flushing

True

Hind gut carcinoid tumours do not cause flushing

T - rarely if ever ass with carcinoid syndrome and flushing (even if liver mets occur)

What is the pathogenesis of flushing?

Vasodilation / vascular SM relaxation either via: - autonomic nervous system - endogenous vasoactive agents - exogenous vasoactive agents

Flushing from alcohol can be caused by?

1/ Alcohol dehydrogenase deficency leading to elevated levels of acetylaldehyde OR 2/ fermented alcohol substance called tyramine

Sign?

Livedo Reticularis

Sign?

Livedo Racemosa

Causes of Livedo Reticularis?

Physiological: - Congenital: CMTC - Aquired: CM (often cold induced, disapears with warming) Secondary: - from factors that slow intravascular blood flow: --> Cryoglobulins --> Cryofibrinogens --> IgM paraproteins --> Polycythaemia --> thrombocytosis

Causes of Livedo Racemosa

Broadly categorised as: 1/ Intraluminal pahtology 2/ Vessel Wall pathology 3/ Other

DDx of widespread livedo racemosa?

Sneddon Syndrome Lymphocytic thrombophillic arteritis Antiphospholipid antibody syndrome (APS)

What is sneddon syndrome?

Rare disorder Characterised by **widespread livedo racemosa** and **mulitple transiet ischaemic attacks and infarctions ** Often leads to progressive neurological impairment THe Livedo Racemosa is often centrally ditributed with a large branching pattern

What is lymphocytic thrombophillic arteritis?

A Lymphocytic vascultiis Characterised by widespread blanchable Livedo + macular pigmentation + focal areas of cutaneous ulceration

What is ADA2 deficency?

ADA2 deficiency is due to homozygous or compound heterozygous mutations in **CECR1** which encodes adenosine deaminase 2 This disorder is also known as **vasculitis**, **autoinflammation**, **immunodeficiency,** and **hematologic defects syndrome** (VAIHS). a **PAN-like presentation** with **LRa **and **nodules** is accompanied by **early-onset cerebrovascular accidents** hepatosplenomegaly, fevers, hypogammaglobulinemia, and cytopenias