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105 Cards in this Set
- Front
- Back
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Occurrence rate for Hemangiomas of infancy vs malformations
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40% @ birth for hemangiomas
99% for malformations |
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Name the three types of hemangiomas of infancy
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Superficial (strawberry) -- involves papillary dermis
Deep (Cavernous) -- reticular dermis and subcutaneous fat Mixed (involves dermis and subcutis) |
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Describe strawberry hemangiomas
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present at birth, usu w/in 1st 2 wks of life in 1-3 % of infants. female to male ratio 3:1; proliferate for 8-18 months and regress over 5-8 years.
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Rule of thumb for involution of hemangiomas
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30% resolve by 3rd birthday, 50% by 5th, 70% by 7th
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How can you slow down the proliferation stage of hemangiomas?
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prednisone -- usually high doses divided into bid. course should probably be longer than 2-3 weeks.
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what dose of ILK can be used in hemangiomas?
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10mg or 20mg/ml, max injection 3-5mg/kg per procedure to slow down rapidly expanding ulcerating hemangiomas
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What is Interferon alfa-2b?
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An antiangiogenic protein, can halt growth of hemangiomas
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Dose of interferon alfa-2b?
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sc injections of 1 to 3 million U/m2 per day in first month and subsequently every 48 to 72 hours. treatment lasts 3-12 months
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How can you monitor treatment response to interferon alfa-2b?
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urinary basic fibroblast growth factor levels are decreased
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Side effects of interferon alfa-2b?
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fever, neutropenia, increased serum amino-transferase levels, neurotoxicity. 5 cases of spastic diplegia reported, 3 pts with permanent deficits
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can you use argon lasers or Nd:Yag lasers with hemangiomas?
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No -- too much scarring
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What type of laser is good for hemangiomas?
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Pulsed Dye Laser (flash-lamp-pumped), short pulses are used
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What is the depth of injury for PDL?
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1.2mm
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Does early intervention with lasers help prevent proliferative stage?
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No
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What type of hemangiomas would you consider PDL for?
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periorificial location, functional impairment, risk of ulceration, cosmetic disfigurement risk
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Role of Imiquimod for hemangiomas?
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Has been used to slow down the proliferative stage. apply tiw x 4wks, with a 2wk rest period.
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What is a cavernous hemangioma?
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collection of dilated vessels deep in the dermis and sc tissue -- presents as pale, skin colored, red &/or blue mass. hyperhidrosis is seen over lesion, managed like superficial hemangiomas.
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Kasaback Merritt Syndrome
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variant of DIC (microangiopathic hemolytic anemia) in which platelets (thrombocytopenia) and clotting factors are locally consumed within a giant hemangioma; usu in the first few weeks of life.
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PHACE Syndrome?
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Posterior fossa brain malformation,
Multiple hemangiomas, arterial anomalies, coarctation of the aorta, cardiac defects, eye abnormalities |
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Name some malformation syndromes associated with hemangiomas?
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PHACE, midline abdominal and sternal defects w/ facial hemangiomas, spinal cord and vetebral abnmls with sacral hemangiomas, large facial hemangiomas linked to Dandy-Walker malformations.
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What posterior fossa brain abnormalities have been linked to large facial hemangiomas?
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hypoplastic cerebellum, posterior fossa arachnoid cysts
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What ophthalmologic abnormalities have been linked to large facial hemangiomas?
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choroidal hemangioma, microphthalmos, and strabismus
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What are vascular malformations?
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anomalies that result from inborn errors of vascular morphogenesis; grow with child, no proliferative stage
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common site of nevus flammeus?
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face and neck, seen in newborns (0.1 to 0.3%)
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Are nevus flammeus genetic?
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No, developmental anomaly
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Do nevus flammeus proliferate?
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No, there is progressive dilation of pre-existing vessels. It is a vascular ectasia rather than proliferation.
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Do port wine stains fade with time?
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No. Size usually stable throughout life.
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What % of pts who have facial port wine stains have glaucoma (without leptomeningial involvement)?
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10%
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Cobb syndrome aka?
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cutaneomeningo-spinal angiomatosis; not familial
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sturge-weber syndrome aka?
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encephalotrigeminal angiomatosis;
dominant partial trisomy, or not familial |
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rendu-osler-weber syndrome aka?
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hereditary hemorrhagic telangiectasia;
autosomal dominant |
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Fabry-Anderson Syndrome aka?
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angiokeratoma coporis diffusum;
recessive trait (X chromosome) |
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ataxia telangiectasia aka?
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cephalo-oculocutaneous telangiectasia;
autosomal recessive |
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von Hippel-Lindau Disease aka?
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angiomatosis retinae et cerebelli syndrome;
autosomal dominant |
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Associated findings with Cobb syndrome
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Angioma of vertebrae, Renal Angioma, Kyphoscoliosis
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Associated findings with Sturge Weber
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Renal Angioma, coarctation of aorta, high-arched palate, abnormally developed ears
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Associated findings with Rendu-osler-weber
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pulmonary arteriovenus anastomosis, hemorrhage from lesions in mouth/GI tract, or GU tract, anemia
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Associated findings with Fabry-Anderson
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stooped posture, slender limbs; thin weak muscles; dilated, tortuous conjunctival and retinal vessels; varicose veins and stasis edema; scant facial hair; hypogonadism
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Associated findings with Ataxia Tel.
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sinopulmonary infections; hypoplastic or absent thymus; small spleen; retarded growth; malignancies (reticulum cell sarcoma, hodgkin's disease, lyphosarcoma, gastric carcinoma).
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Associated findings with von Hippel-Lindau
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pheochromocytoma, pancreatic cysts, hepatic angiomas (renal hypernephromas 20%), polycythema (erythropoietic substance from tumor)
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What is Sturge-Weber Syndrome?
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Vascular malformations of the CNS and the face in a V1 trigeminal nerve distribution
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What stain pattern is high risk for CNS problems in Sturge-Weber?
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Full V1 alone or Full V1 + another site; bilateral distribution, nevus flammeus of the eyelids, unilateral nevus flammeus involving all three branches of the trigeminal nerve are associated with higher CNS involvement.
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Describe CNS manifestations of Sturge-Weber
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Seizures, Mental Retardation, contralateral hemiplegia or hemisensory deficits, contralateral homonymous hemanopsia
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What are some ocular involvement in Sturge Weber?
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Ipsilateral to stain, glaucoma, Buphthalmos (enlargement of ocular globe), vascular malformations of the conjunctivia, episclera, choroid, and retina
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What is Klippel-Trenaunay syndrome?
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triad of capillary and venous malformations, venous varicosity, and hyperplasia of soft tissue and possible bone in the affected area.
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What is Parkes-Weber Syndrome?
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Klippel-Trenaunay syndrome plus arteriovenous fistula. lower limb is most commonly involved.
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Treatment of Port Wine Stains?
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Lasers, Covermark, Dermablend
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What are salmon patches?
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variant of nevus flammeus -- aka stork bite or angel's kiss (40-70% of newborns); usu fade w/in a year
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cherry angioma aka?
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senile angioma -- the most common acquired cherry angioma
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what is an angiokeratoma?
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dilation of superficial dermal blood vessels and hyperkeratosis of the overlying epidermis
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what is a venous lake?
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dark blue, slightly elevated, 0.2 to 1cm dome shaped lesions composed of dilated, blood-filled vascular channel. usu on sun-exposed surfaces (vermilion border of the lip, face, and ears)
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what is lymphangioma circumscriptum?
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uncommon hamartomatous malformations consisting of dilated lymph channels which may be filled with serosanguineous fluid that communicate with deeper lymph channels. appears like "frog spawn".
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How do you treat lymphangioma circumscriptum?
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electrosurgery, surgical removal of subcut cicsterns, CO2 lasers
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what is pregnancy epulis?
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pyogenic granuloma in pregnant women -- usually seen in gingiva
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common sites for pyogenic granulomas?
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head, neck and extremities (fingers)
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what is a pyogenic granuloma?
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inflammatory and hyperplastic condition interpreted as a florid expression of granulation tissue proliferation
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How do you treat pyogenic granulomas?
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electroessication, curretage... spontaneous resolution usually w/in 6 months is seen.
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What causes Bacillary angiomatosis?
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Bartonella: B. quintana & B henselae
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what animal serves as a reservoir for bacillary angiomatosis?
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cats
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what stain is used to dx bacillary angiomatosis?
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warthin-starry stain, can also be seen on H&E as masses of bacteria aggregates
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describe bacillary angiomatosis
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lesions can be solitary or multiple, red to purple pinpoint-size papules that increase in size to form nodules and tumors. usu in AIDS pts with CD4 count less than 200.
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What is Kaposi's sarcoma?
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multiple idiopathic hemorrhagic sarcomas. vascular neoplasms.
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Name the 4 types of Kaposi's sarcoma
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Classic, African cutaneous/African lymphadenopathic, AIDS, Immunosuppressive
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Which two types of KS have links to other cancers?
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Classic and KS in AIDS pts
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Describe pathogenesis of KS
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HHV 8 has altered expression and respons to cytokines and stimulation of KS growth by the HIV-1 transactivating protein Tat.
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What doest Tat do?
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Tat promotes the growth of spindle cells of endothelial origin only in the presence of inflammatory cytokines
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How is HHV 8 transmitted
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sexually and by other means. virus is found in saliva and semen of infected persons. homosexual men, regardless of HIV status, have asymptomatic infection rate of 40%!
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Describe Classic KS
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violaceous macules and papules forming plaques -- mainly on hands, feet, or lower legs... progresses up the arms and legs; M:F (15:1), usu elderly males of jewish, greek, or italian descent. slow indolent course. Rx: observation or interferon alfa-2b injection, radiation, and/or chemo
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Most common cause of tumors in central africa
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Endemic African KS; 50% of tumors reported, 25-50% of soft tissue sarcomas in children in eastern and southern africa
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TOC for Endemic African KS
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local radiation therapy
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Difference between cutaneous and lymphatic African KS
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Cutaneous is in men (median 41yrs) with indolent disease on limbs and rare involvement of LNs. Lymphadenopathic form is Aggressive and seen in children <10y/o, poor prognosis
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KS with immunosupression is seen in what type of patients?
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usually transplant recipients; 0.1% to 5.3%; men prodominantly (67% to 80%); interval time to KS is 30 months post transplant.
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Incidence of AIDS related KS in different HIV patients?
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20% of homosexual men
3% heterosexual IV drug users 3% of transfusion recipients 3% women and/or children 1% hemophiliacs |
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most common tumor in HIV pts?
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KS
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Describe AIDS related KS
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multicentric rather than metastatic; mucous membrane involvement; starts on trunk and head/neck areas; eventually goes onto extracutaneous areas.
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Rx for AIDS related KS, limited disease
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Alitretinoin (Panretin), intralesional vinblastine, radiation therapy, laser therapy, or cryotherapy
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Treatment options for AIDS related KS
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HAART, Surgery (single lesions), LN2, Intralesional chemotherapy, Topical Retinoids, Radiation therapy, Cytoxic Therapy, Interferon
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LN2 Rx for AIDS related KS
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LN2 every 3 week intervals; blister fluid may contain HIV
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When is intralesional chemotherapy appropriate for AIDS related Ks?
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nodular lesions > 1cm or symptomatic oral lesions
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What intralesional chem is used in AIDS related KS?
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Vinblastine 0.1mg (0.5 ml of a 0.2mg/ml solution) injected per square centimeter; or you can increase concentration to 0.4 to 0.6mg/ml; max dose per visit is 2mg; done at 3wk intervals
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Indications for cytotoxic therapy for KS
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extensive disease and failure of antiretroviral therapy; or rapidly progressive disease (>10 or more new cutaneous lesions/month), lymphedema, pulmonary KS, and widespread symptomatic visceral disease.
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What is the Klein Regimen?
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weekly outpatient IV vinblastine (4-6mg) for KS
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What are Telangiectasias?
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permanently dilated small blood vessels
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Name to Primary (cause unknown) Telangiectasias
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Ataxia Telangiectasia, Generalized essential telangiectasia, HHT, Spider angiomas, Unilateral nevoid telangiectasia syndrome
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Another name for spider angioma
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Nevus Araneus, Rx is electrodessication
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HHT is the most common cause of what type of fistula?
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pulmonary arteriovenous fistula (seen in 5-15% of HHT patients). Need Chest CT, arterial blood gas measurements and finger oximetry to screen for it.
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HHT lesions are similar to lesions in what other dz?
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CREST
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Most common manifestation of HHT?
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Epistaxis
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Where do HHT lesions occur?
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Any skin area or internal organ can be involved
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How do you treat HHT?
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electrocautery of bleeding points; Tranexamic Acid (an antifibrinolytic drug) can help the local hyperfibrinolysis in lesions; may need transfusions and oral iron
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KS with immunosupression is seen in what type of patients?
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usually transplant recipients; 0.1% to 5.3%; men prodominantly (67% to 80%); interval time to KS is 30 months post transplant.
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Incidence of AIDS related KS in different HIV patients?
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20% of homosexual men
3% heterosexual IV drug users 3% of transfusion recipients 3% women and/or children 1% hemophiliacs |
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most common tumor in HIV pts?
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KS
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Describe AIDS related KS
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multicentric rather than metastatic; mucous membrane involvement; starts on trunk and head/neck areas; eventually goes onto extracutaneous areas.
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Rx for AIDS related KS, limited disease
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Alitretinoin (Panretin), intralesional vinblastine, radiation therapy, laser therapy, or cryotherapy
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Treatment options for AIDS related KS
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HAART, Surgery (single lesions), LN2, Intralesional chemotherapy, Topical Retinoids, Radiation therapy, Cytoxic Therapy, Interferon
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LN2 Rx for AIDS related KS
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LN2 every 3 week intervals; blister fluid may contain HIV
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When is intralesional chemotherapy appropriate for AIDS related Ks?
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nodular lesions > 1cm or symptomatic oral lesions
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What intralesional chem is used in AIDS related KS?
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Vinblastine 0.1mg (0.5 ml of a 0.2mg/ml solution) injected per square centimeter; or you can increase concentration to 0.4 to 0.6mg/ml; max dose per visit is 2mg; done at 3wk intervals
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Indications for cytotoxic therapy for KS
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extensive disease and failure of antiretroviral therapy; or rapidly progressive disease (>10 or more new cutaneous lesions/month), lymphedema, pulmonary KS, and widespread symptomatic visceral disease.
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Describe Unilateral Nevoid Telangiectasia Syndrome
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Numerous threadlike telangiectasias that appear in a unilateral dermatomal distribution; most occur in the trigeminal, C3, C4 or adjacent dermatomes. Two forms: acquired & congenital
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Is the congenital form of unilateral nevoid syndrome more common in males or females?
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males
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What three states does the acquired form of unilateral nevoid syndrome appear?
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1. at puberty in females 2. during pregnancy 3. alcoholic cirrhosis (all high estrogen states)
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What syndromes have telangiectatic mats?
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CREST syndrome and Scleroderma; flat collections of vessels; usu on face, lips, palms and backs of hands
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Name of rare disorder with widespread spreading telangiectases
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Generalized essential telangiectasia; primarily in women; often familial, avg onset 38y/o, health is not affected; suggested autosomal dominance; Rx TCn or 585nm PDL
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