28- Dermal Tumors Flashcards by Jenica Dela Cruz

This type of vascular anomaly involute spontaneously

Infantile hemangioma

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This type of vascular anomaly does not involute and are persistent

Vascular malformations

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Cutaneous vascular anomaly that is characterized by an abnormal arrangement of tissues

Hamartomas

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Hamartomas are usually contrasted to this condition because it is described as an increase in tissue present but in an “normal” arrangement

Nevus

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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

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Most common type of phakomatosis pigmentovascularis

Cesioflammea

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Most commonly associated condition with phakomatosis cesioflammea

Sturge-Weber syndrome

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Most commonly associated condition with phakomatosis spirulosa

Multiple granular cell tumors

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Hamartoma that appears as a solitary nodular lesion on acral areas with pain and hyperhidrosis

Eccrine angiomatous hamartoma

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Eccrine angiomatous hamartoma has been associated with these conditions

Spindle cell hemangioma
Verrucous hemangioma
AVM

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Treatment for eccrine angiomatous hamartoma

Excision because of pain

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Abnormal structures that result from an aberration in embryonic development or trauma

May be due to anatomic or functional alteration

Malformations

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Types of anatomic malformations

Capillary
Venous
Arterial
Lymphatic 
Combined

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Congenital disorder characterized by various shapes and size macules that are paler than surrounding skin

Nevus anemicus

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Nevus anemicus resembles this disease but has normal amount of melanin

May occur in neck and trunk

Vitiligo

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Underlying defect of nevus anemicus

Increased sensitivity of blood vessels to catecholamines

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Patch of skin that is cooler than normal skin

Nevus oligemicus

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Underlying defect in nevus oligemicus

Decreased blood flow due to vasoconstriction of deep blood vessels

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Presence of purplish, reticulated, vascular network with segmental distribution- usually in extremities

Cutis marmorata telangiectasia congenita

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Mottling is curis marmorata becomes more distinct during

Crying
Vigorous activity
Cold

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Treatment for cutis marmorata

None- lesions usually improve by 2 years old

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Differential in cutis marmorata where there is progressive development of large venous ectasias involving one limb

Bockenheimer syndrome

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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

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Salmon patch type of nevus flammeus is located usually in

Glabellar region

One upper eyelid

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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

VM or GM? Shrinks with pressure

VM or GM? Painful in the morning due to congestion

VM or GM? Inherited

VM or GM? Sporadic

VM or GM? Sclerotherapy more effective

VM or GM? Extremity involvement more common

VM or GM? Isolated blue lesion that may involve muscle

VM or GM? Increased pain with menstruation, pregnancy, OCP

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