28- Dermal Tumors Flashcards
This type of vascular anomaly involute spontaneously
Infantile hemangioma
This type of vascular anomaly does not involute and are persistent
Vascular malformations
Cutaneous vascular anomaly that is characterized by an abnormal arrangement of tissues
Hamartomas
Hamartomas are usually contrasted to this condition because it is described as an increase in tissue present but in an “normal” arrangement
Nevus
What are the 4 types of phakomatosis pigmentovascularis?
Cesioflammea (blue nevus, nevus flammeus)
Spirolosa (nevus spilus, pale pink vascular spot
Cesiomarmorata (blue spots, cutis marmorata)
Unclassifiable
Most common type of phakomatosis pigmentovascularis
Cesioflammea
Most commonly associated condition with phakomatosis cesioflammea
Sturge-Weber syndrome
Most commonly associated condition with phakomatosis spirulosa
Multiple granular cell tumors
Hamartoma that appears as a solitary nodular lesion on acral areas with pain and hyperhidrosis
Eccrine angiomatous hamartoma
Eccrine angiomatous hamartoma has been associated with these conditions
Spindle cell hemangioma
Verrucous hemangioma
AVM
Treatment for eccrine angiomatous hamartoma
Excision because of pain
Abnormal structures that result from an aberration in embryonic development or trauma
May be due to anatomic or functional alteration
Malformations
Types of anatomic malformations
Capillary Venous Arterial Lymphatic Combined
Congenital disorder characterized by various shapes and size macules that are paler than surrounding skin
Nevus anemicus
Nevus anemicus resembles this disease but has normal amount of melanin
May occur in neck and trunk
Vitiligo
Underlying defect of nevus anemicus
Increased sensitivity of blood vessels to catecholamines
Patch of skin that is cooler than normal skin
Nevus oligemicus
Underlying defect in nevus oligemicus
Decreased blood flow due to vasoconstriction of deep blood vessels
Presence of purplish, reticulated, vascular network with segmental distribution- usually in extremities
Cutis marmorata telangiectasia congenita
Mottling is curis marmorata becomes more distinct during
Crying
Vigorous activity
Cold
Treatment for cutis marmorata
None- lesions usually improve by 2 years old
Differential in cutis marmorata where there is progressive development of large venous ectasias involving one limb
Bockenheimer syndrome
Congenital capillary malformation present in 25% of newborns
Pink red macule on posterior midline between occipital protruberance and C5
Nevus flammeus nuchae
“Stork bite”
Port wine stain
Salmon patch type of nevus flammeus is located usually in
Glabellar region
One upper eyelid
Congenital capillary malformation that usually fades in childhood
Nevus flammeus
Port wine stain
Most common site of nevus flammeus
Unilateral distribution on the face
Nevus flammeus found in this area is associated with Sturge Weber syndrome
Ophthalmic division ( V1) of CN5
Leptomeningeal angiomatosis has this characteristic feature
Calcifications in the outer cortex
“Tram tracks” that follow brain convolutions
Sturge Weber Syndrome usually results from the persistence of
primitive embryonal vascular plexus
-during the 6th fetal week around the cephalic neural tube and facial skin area
Usually regress by 9 weeks
Other syndrome associated with nevus flammeus (port wine stain) that has overgrowth of soft tissue and underlying bone in affected extremity
Klippel-Trenaunay- Parkes- Weber
Syndrome
This syndrome associated with nevus flammeus is characterized by port wine malformations
Klippel- Trenaunay
This syndrome associated with nevus flammeus is characterized by deep AVM
Parkes- Weber
Lesional skin in nevus flammeus demonstrates overgrowth of
Vascular endothelial growth factor (VEGF) VEGF receptor (VEGF-R2)
Treatment for port wine stain
Laser therapy- recurrence common
Best record of safety and efficacy in the treatment of nevus flammeus
Flashlamp pulsed dye laser
Present as rounded, bright red or deep purple spongy nodules that occur chiefly on the head and neck and may also involve mucous membranes
Generally asymptomatic
Cavernous venous malformation
Syndrome associated with venous malformation:
Dyschondroplasia with hemagiomata
Uneven bone growth, unilateral distribution
Maffucci syndrome
Syndrome associated with venous malformation:
Enchondromatosis is present without cutaneous abnormalities
Ollier disease
Human enchondromatosis has been associated with this protein and this gene
Parathyroid hormone related protein (PTHrP)
Indian hedgehog gene (IHH)
PTHrP does this to the proliferating chondrocytes
Delays differentiation
What is the effect of IHH to chondrocytes?
Promotes proliferation
Syndrome associated with venous malformation:
Characterized by cutaneous and GI venous malformations (can rupture)
Lesions are bluish with soft nipple like center that can be emptied by pressure
Blue rubber bleb nevus syndrome
Syndrome associated with venous malformation:
Cutaneous and osseus venous and lymphatic malformations
associated with massive osteolysis “disappearing bones”
Gorham’s disease
Gorham’s sign
Vascular malformation that presents in adults as a blush purple nodule on trunk or breast
Lobular mass with dilated interconnected vascular channels filled with blood
Sinusoidal hemangioma
Venous malformation should be distinguished from
Glomuvenous malformation
Glomangioma
VM or GM?
Painful if compressed
GM
VM or GM?
Shrinks with pressure
VM
VM or GM?
Painful in the morning due to congestion
VM
VM or GM?
Inherited
GM
VM or GM?
Sporadic
VM
VM or GM?
Sclerotherapy more effective
VM
VM or GM?
Extremity involvement more common
GM
VM or GM?
Isolated blue lesion that may involve muscle
VM
VM or GM?
Increased pain with menstruation, pregnancy, OCP
VM
Triad of Klippel- Trenaunay syndrome
Nevus flammeus
Venous and lymphatic malformation
Soft tissue hypertrophy of affected extremity
(When you clip the tray- wine stains, malformed Venus, lots of tissues)
What condition is seen that warrants KTS diagnosis to be appended to Parkes-Weber?
AV fistula
Klippel- Trenaunay Syndrome (KTS) is usually seen in what body part
Lower limb (95%)
Earliest and most common presenting sign in KTS
Nevus flammeus that is confined to the skin of an extremity
Diagnostics for KTS
Color duplex UTZ- venous patency
MRI- muscle and bone
Arteriography- if AV fistula is suspected
Xray- extremities
Treatment of KTS
Flashlamp pumped pulsed dye laser- nevus flammeus
Varicosities- microfoam sclerosis, thermal ablation or surgical stripping
Edema- elevation diuretics compression stockings
Congenital AV fistulas mostly occur on which body part
Extremities
Congenital AV fistulas is usually a component of this disease
Osler-Weber- Rendu
Reddish or purple nodules observed in AV fistulas may resemble this
Kaposi sarcoma (Stewart- Bluefarb syndrome)
Congenital AV fistulas usually occur during what age
2nd-3rd decade of life
Uncommon congenital AV fistulas of scalp or face
Pulsating mass on skin and may penetrate cranium or
Solitary blue or red papule in mid adult
Cirsoid aneurysm (angioma arteriale racemosum)
Diagnosis of AV fistulas
Plethysmography
Thermography
O2 saturation of venous blood
Arteriography
Treatment for AV fistula
Traumatic- excision
Congenital- doppler guided sclerotherapy
Deep seated vesicle like papules resembling frog spawn at birth
Yellowish and exude a clear liquid
Arranged irregularly in groups but localized to one region
Superficial lymphatic malformation
Lymphangioma circumscriptum
Superficial lymphatic malformation may also be seen adjacent to this lesion that may represent twin spotting phenomenon
Cafe au lait macules
Treatment of superficial lymphatic malformation
MRI to evaluate extent of deep involvement
CO2 laser- deeper component absent
Pulsed dye, intense pulse light, sclerosants and electrosurgery
Circumsribed painless soft tissue masses that occur in neck, axilla or groin
Associated with Turner syndrome and orher aneuploidy syndromes
Cystic hygroma
This syndrome is characterized by lymphangiomatosis and chylous effusions with osteolytic changes resulting in “vanishing bones”
Gorham-Stout syndrome
This lesion can occur on face and neck
Children: back of hands, forearms, face
Palmar erythema
Also occurs in pregnant and liver disease
Spider angioma
Spider angiomas occurs in children, pregnant and those with hepatic dysfunction because
Elevated estrogen levels in blood
Treatment for spider angioma
Involute in children and pregnant (upon delivery)
Active therapy- electrodessication of central punctum or laser
Small, dark blue slightly elevated blebs that are easily compressed
Face, neck, ears, lips forearms, back of hands
Manifestations of chronic sun damage
Venous lakes (phlebectases)
Treatment of venous lakes
Electrocautery Laser ablation Fulguration Infrared coagulation Cryotherapy
Capillary aneurysms are clinically indistinguishable from
Malignant melanoma
Flesh colored solitary lesions that resemble intradermal nevus that may thrombose and grow darker and larger
Surrounded by zone of erythema
Capillary aneurysms
Fine, linear vessels coursing on the skin surface are collectively called
Telangiectasia
Cuticular telangiectasias are indicative of
Collagen vascular disease ( LE, scleroderma, dermatomyositis(
Tortuous- LE
Dilated- scleroderma, DM
Treatment for telangiectasia
Electrodessication
Laser ablation- pulse stacking
Bleeding disease that affects blood vessels of skin and mucous membranes
Mottled skin with redness that blanch on pressure. Normal blood coagulation
Differs from generalized telangiectasia due to hemorrhagic tendency
Universal angiomatosis
Fine threadlike telangiectasia in a unilateral or dermatomal distribution
Acquired- increased estrogen
Unilateral nevoid telangiectasia
Unilateral nevoid telangiectasia is usually found in
Trigeminal
C3 and C4 areas
Telangiectases that have overlying hyperkeratotic surface
Angiokeratomas
Also called telangiectatic warts
Dull red or purplish black verrucous, rounded papules usually on dorsum of fingers and toes, elbows and knees
Cold cyanotic hands and feet
Angiokeratoma of Mibelli
Multiple small vascular papules jn the scrotum or vulva
Diffuse redness of area
Histo: communicating lacunae in subpapillary layer
Angiokeratoma of Fordyce
Treatment for Fordyce angiokeratoma
Reassurance Laser ablation Shave excision Cautery Fulguration
Red brown, dome shaped dermal papules or nodules usually in head and neck ( retroauricular, scalp)
Underlying AV shunt present
Do not spontaneously regress
May recur if AV shunt not excised
Female preponderance
Angiolymphoid hyperplasia with eosinophilia (ALHE)
ALHE must be differentiated from this disease
Kimura disease
Inflammatory disease that present as massive subcutaneous swelling in preauricular and submandibular area in Asian men
Associated with allergic conditions
Kimura disease
Small, eruptive solitary sessile/pedunculated friable papule often seen in exposed surface and on gingiva
Bleed easily on slightest trauma
Secondary staph colonization common
Pyogenic granuloma
Treatment of pyogenic granuloma
Curettage or shave excision
Fulguration/ silver nitrate- destroy base
Minute, copper colored to bright red angiomatous puncta that tend to become papular
Occur in groups, forms new points in periphery while center fades
No purpura- netlike/diffuse erythema
Mostly in LE
Involution never complete
Angioma serpiginosum
Angioma serpiginosum must be differentiated from
Progressive pigmentary disease of Schamberg
- pinpoint areas if purpura (cayenne pepper spots)
Most important histo finding in angioma serpiginosum
Dilated and tortuous capillaries in dermal papillae
No inflammatory infiltrate or extravasation of RBC
