Title: GZ / The Grenz Zone Season 2 / 33 Vascular Growths
Tags: GZ DermnetNZ

Q: What is the difference between a vascular tumor and a vascular malformation?
Vascular tumor is proliferation of [normal or abnormal] cells or structures whereas vascular malformations involve an [normal or abnormal] development of blood vessels
A: normal-appearing; abnormal
Back Extra: Vascular tumor examples: hemangiomas or pyogenic granuloma
Vascular malformation examples: port-wine stains or lymphangiomas

Q: What are the 2 categories of hemangiomas?
[...]
A: Congenital & Infantile
Back Extra: 90% of lesions will go away by 9 years old
Congenital hemangiomas can be classified into rapidly involuting congenital hemangiomas (RICH) and noninvoluting congential hemangiomas (NICH)

What does the PHACES syndrome stand for?
P: Posterior fossae malformations (e.g. Dandy Walker)
H: Hemangiomas
A: Arterial anomalies (e.g. aneurysms)
C: Cardiac anomalies (e.g. atrial/ventricular septal defects)
E: Eye abnormalities (e.g. cataracts, retinal vascular changes)
S: Sternal clefting or Supraumbilical raphe

Q: Infantile Hemangiomas are extremely common and present as a [onset] superficial bright red papule that eventually involutes
A: rapidly growing
Back Extra: Up to 50% start with a precursor lesion such as a red patch or telangiectasia
Can have a deeper component that takes on a blue hue
Grow rapidly during the first 5 months of life, plateau between 9-12 months, then slowly involute

Q: Diagnosis of an infantile hemangioma is usually [...]
A: clinical
Back Extra: Reasons for aggressive workup/treatment:
Ulceration
Threaten function by involving the periocular area, nose, ears, lips, or genitals
Extensive in the beard area (risk of airway involvement)
Large/segmental V1 nerve distribution = ↑ risk of PHACES syndrome (get brain MRI & echo)
Large/segmental in lumbosacral area = ↑ risk of LUMBAR/SACRAL syndrome

Q: Treatment of infantile hemangioma is topical [...] or oral [...]
A: timolol; propanolol
Back Extra: Rule out arterial and cardiac anomalies first if PHACES concern
Can also get pediatric neurology/cardiology & ophthalmology consults
Pearl: Neonates with a red patch in V1 distribution need to be followed closely on a weekly basis, since early hemangiomas can look exactly like a port wine stain one day and then develop the classic elevation of hemangiomas within days to weeks

Q: Some risk factors for infantile hemangiomas include [male or female], premature birth, advanced maternal age, placental abnormalities, and pre-eclampsia
A: female sex

Q: Nevus Flammeus (Port-Wine Stain) are congenital [specific blood vessel] malformations that are present at birth
A: capillary
Back Extra: Associated with Sturge-Weber Syndrome

Q: Clinically, nevus flammeus presents as a well-demarcated [color] patch that grows slowly with the patient
A: pink to red
Back Extra: DO NOT SELF RESOLVE. Can become thickened and bumpy or papular later in life

Q: Sturge-Weber syndrome has a triad of a port-wine stain in a [...] distribution, leptomeningeal angiomatosis (seizures), and [eye abnormality]
A: V1; glaucoma

Q: Treatment of nevus flammeus includes getting a pediatric [3 answers] consult
A: neurology, ophtalmology, and radiology
Back Extra: Pearl: Do NOT mix up the association between V1 port wine stains with Sturge-Weber syndrome and the V1 hemangioma’s association with PHACES syndrome.

Q: Nevus simplex is the most common vascular malformation and presents as a pink, [blanching or nonblanching] patch that is often located in the midline of the occiput
A: blanchable
Back Extra: Compared to a port-wine stain, nevus simplex is lighter in color, is less well-demarcated, and usually resolves if it is on the face

Q: Pyogenic granuloma present as a solitary friable, [color] papule that grows relatively quickly over the course of weeks to a few months
A: red
Back Extra: Tend to appear on the trauma-prone sites of the gingiva, lips, fingers, or face
Triggers include: minor trauma, pregnancy, medications (e.g. isotretinoin, OCPs)

Q: Angiokeratomas present as a small [color] papule with rough scale
A: red to purple
Back Extra: Angio = superficial vascular nature, keratoma = hyperkeratotic look

The 5 types of angiokeratomas are:
1. Solitary or multiple angiokeratomas
2. Angiokeratomas of Fordyce
3. Angiokeratoma corpus diffusum
4. Angiokeratoma circumscriptum
5. Angiokeratoma of Mibelli
Back Extra: - Solitary or multiple angiokeratomas: tend to favor lower extremities
- Angiokeratomas of Fordyce: classically appear on the scrotum or vulva of older patients
- Angiokeratoma corpus diffusum: clustered in a bathing suit distribution. Associated with Fabry's disease
- Angiokeratoma circumscriptum: coalescing angiokeratomas developing into a plaque in children
- Angiokeratoma of Mibelli: on the hands and feet of teenagers

Q: Angiokeratomas of Fordyce tend to classically appear on the [...] of older patients
A: scrotum or vulva

Q: Angiokeratoma corporis diffusum presents with angiokeratomas in a [distribution] and is associated with Fabry's disease
A: bathing suit distribution
Back Extra: Recall, Fabry's disease is a deficiency in alpha-galactosidase enzyme that presents with classical triad of angiokeratomas, hypohidrosis, and peripheral neuropathy

Q: Angiokeratoma circumscriptum presents as coalescing angiokeratomas developing into a plaque in [children or adults]
A: children

Q: Angiokeratoma of Milbelli presents as angiokeratomas in teenagers on the [body distribution]
A: hands & feet

Q: On histology, angiokeratomas present as dilated vessels in the [specific dermal layer] with an acanthotic or thickened epidermis
A: papillary dermis
Back Extra: Pearl: At first glance, angiokeratomas basically look like a bloody seborrheic keratosis on path

Q: Tufted angioma classically presents as [color] plaques on the neck of children less than [age]
A: pink-red; 1 years old
Back Extra: Associated with Kassabach-Merritt phenomenon (KMP): when platelets are trapped and destroyed in vascular lesions, leading to rapid lesion growth and coagulopathies
Pearl: KMP is classically seen in tufted angiomas or kaposiform hemangioendotheliomas (KHE). Remember that these two lesions TAKHE away platelets, with TAKHE spelled T-A-K-H-E and standing for Tufted Angioma and Kaposiform HemangioEndothelioma.

Q: Glomus Tumor classically presents as a solitary [painful or asymptomatic] red papule on the finger
A: painful
Back Extra: Can cause significant nail dystrophy

Q: Angiolymphoid Hyperplasia with Eosinophilia (ALHE) presents as [solitary or grouped] pink to red-brown dome shaped papules classically by the [body location] but can be anywhere on the head and neck in young to middle-aged adults
A: grouped; ear

Q: On histology, angiolymphoid hyperplasia with eosinophilia presents as proliferation of vessels in the [specific skin layer] with large epithelioid endothelial cells and a background of [2 specific cell types] inflammation
A: dermis; lymphocytic and eosinophilic
Back Extra: Treatment consists of excisionaly surgery (most common), cryotherapy, intralesional kenalog, imiquimod
Inflamed dermis with increased vascularity
Enlarged and epithelioid endothelial cells with lymphocytes forming follicles and eosinophils

Q: Kaposi's Sarcoma is caused by [specifc virus] and is considered a low-grade vascular tumor
A: HHV-8
Back Extra: Stain for HHV-8 on histology for confirmation because it is present is 100% of lesions

The 4 types of Kaposi's Sarcoma are:
1. Classic
2. African endemic
3. Iatrogenically immunocompromised
4. AIDS-associated
Back Extra: Classic: slow enlargement of macules into vascular plaques and nodules on the legs of older men with a Mediterranean background
African endemic: young African males in endemic areas. Can be fatal
Iatrogenically immunocompromised: Typically skin-limited. Occurs in patients on immunosuppressive medications for organ transplants or autoimmune disease
AIDS-associated: caused by HIV

Q: Treatment of Kaposi's Sarcoma includes [...], alitretinoin, and systemic chemotherapy
A: cryotherapy
Back Extra: Alitretinoin = topical retinoid
Systemic chemo = for progressive cases with internal organ involvement

Q: Angiosarcoma is a [low-grade or high-grade] malignant vascular tumor that classically presents with a progressive enlarging bruise-like patch, plaque, or nodule on the [2 body locations]
A: high-grade; face or scalp
Back Extra: Presents in elderly Caucasian males
5 year survival of < 20%

Q: Stewart-Treves syndrome is the development of [malignant vascular tumor] in an area of chronic lymphedema
A: angiosarcoma
Back Extra: Usually takes at least 4 years of problems with lymphedema before the angiosarcoma develops
Think of a patient with a history of mastectomy and chronic lymphedema of their arm from the axillary lymph node dissection, and they start to develop purpuric papules and plaques of that limb.