3 Raynaud’s PhenomenonIntermittent constriction of the small digital arteries and arteriolesPersistently cyanotic and painfulAggravated by cold weatherYoung middle aged womenAssoc c scleroderma, dermatomyositis, LE, Mixed connective tissue diseases, Sjögren's RA, and paroxysmal hemoglobinuria.
4 Raynaud’s PhenomenonScleroderma is the underlying condition for more than half of the patientsMaybe caused by medications, ie bleomycinSimple noninvasive tests along with other clinical findings are helpful
7 Raynaud’s Disease Primary disorder of cold sensitivity Pallor, cyanosis, hyperemia, and numbness of the fingersPrecipitated by cold.Present for 2 years with out associated disease findingGood prognosis
8 Etiology ofRaynaud’s Disease/phenomenon Multifactorial.Increase alpha-2 sympathetic receptor activity on vessels.Endothelia dysfunctionDeficiency in calcitonin gene related proteinCentral thermoregulatory defects
9 TX of Raynaud’s Disease/phenomenon Treatment includes avoidance of the aggravating factor, ie cold. (not just hands)Vasodilating drugs, nifedipine, mg tid; prazosin 1-3 mg tidNitroglycerin 2% local applicationSympathectomy in severe disease
10 Erythromelalgia Aka erythermalgia and acromelalgia Rare form of paroxysmal vasodilation affects the feet with burning. Infrequently upper ext.Burning may last from minutes to daysTriggered by increase in environmental temperatureMay be secondary to myeloproliferative disease such as polycythemia vera, TTP
11 Pathophysiology is poorly understood Responds to treatment of primary disordersCold water immersionSerotonin antagonists
17 Necrotizing livedo reticularis Assoc. with cutaneous nodules and ulcerationsResults from cholesterol emboli in severe atherosclerotic diseaseSneddon’s syndrome, association of LR with the development of a cerebrovascular lesion
18 Livedoid Vasculitis Aka - atrophie blanche Aka - PURPLE (Painful purpuric ulcer with reticular pattern of the lower extremity)Characterized clinically by early, focal, painful purpuric lesions of the lower extremities that frequently ulcerate and slowly healMostly represents an idiopathic disorder but may be assoc. with systemic disease
19 Treatment Low Dosage of Aspirin 325mg qd Nifedipine 10mg TID Pentoxifylline 400mg BID-TID
22 Marshall-White Syndrome “Bier’s Spots” Marbled mottling produced in the forearm and hand with use of a tight sphgmomanometerConsist of Bier’s spot and is associated with insomnia and tachycardiaWhite middle age men
24 PurpuraMultifocal extravasation of blood into the skin or mucous membranePetechiae <3mmEcchymosis – deeper and more extensive interstitial hemorrhageVibices (vibex) – LinearHematoma – pool-like collection, usually walled off by facial planes
25 PurpuraMay result from hyper or hypocoagulable states, vascular dysfunction, and extravascular causesComplete blood countPT and PTTFamily historymedications
26 Thrombocytopenic Purpura Three Large Categories:Accelerated platelet destruction, immunologic and nonimmunologicDeficient platelet productionUnknown pathogenesis
27 Idiopathic Thrombocytopenic Purpura Aka autoimmune thrombocytopenic purpuraAka Werlhof’s diseaseCharacterized by acute or gradual onset of petechiae or ecchymosis in the skin and mmBleeding occurs when platelet count drops below 50,000Risk greatly increased for serious hemorrhage when count goes below 10,000
28 Idiopathic Thrombocytopenic Purpura Acute variety occurs in children following season viral illness in 50% of the patient.Lag between illness and onset of purpura is 2 weeksMost cases resolve spontaneously with minimal therapyChronic case may result in death.
29 Idiopathic Thrombocytopenic Purpura Chronic form most often occurs in adultsEvaluate patient with Tc99M radionuclide scan to look for accessory spleenIdiopathic Thrombocytopenic Purpura is the result of platelet injury by antibodies of the IgG classTreatment include Splenectomy, systemic corticosteroid, IVIg
30 These are the kidneys from a case of idiopathic thrombocytopenic purpura. Petechiae are found throughout the renal parenchyma.
33 Drug-Induced Thrombocytopenia Drug induced antiplatelet antibodiesMay be caused by sulfonamides, digoxin, quinine, quinidine, PCN, furosemide, Lidocaine, methyldopaRemove the offending agentcorticosteroids
34 Thrombotic Thrombocytopenic Purpura Aka Moschcowitz’s syndromePentad of thrombocytopenia, hemolytic anemia, renal abnormalities, fever, CNS disturbance.Delay in diagnosis may lead to a mortality rate as high as 90%Exchange plasmapheresis, 3 to 5 liters of plasma for 4 to 10 daysSplenectomy may be required
35 Thrombotic Thrombocytopenic Purpura Positive histologic diagnosis requires gingival biopsies looking for subendothelial hyaline depositsExchange plasmapheresis is required for treatment. 80% patient survive if treatment is instituted.
36 Dysproteinemic Purpura Aka Nonthrombocytopenic purpura, purpura cryoglobulinemica, cryofibrinogenemiaAbnormal serum proteins behaving as cryoglobulins and cryofibrinogensPurpura most apt to occur on exposed surfaces after cold exposure
37 Occurs most frequently in multiple myeloma and macroglobulinemia of monoclonal IgM, IgG, or Bence Jones cryoglobulin.Purpura tends to be chronicTx with plasmapheresis, systemic steroids, and immunosuppressors.
38 Purpura Hyperglobulinemica Aka Waldenström's hyperglobulinemic purpuraConsist of episodic showers of petechiae occurring on all parts of body, most profusely on the lower extremitiesDiffuse peppery distribution, resembling Schamberg’sInduced or aggravated by prolonged walkingMost useful lab test is protein electrophoresis
39 Purpura Hyperglobulinemica Hyperglobulinemic purpura occurs most commonly in women.Frequently seen with Hepatitis C and Sjögren's syndrome, keratoconjunctivitis sicca, RAHistologically: dermal vessels with perivascular infiltrate of mononuclear cells.Benign and chronic course. May be assoc. with connective tissue diseases.Steroids are usually not of benefit
40 Waldenström's Macroglobulinemia Bleeding from mucous membrane of the mouth and nose, lymphadenopathy, hepatomegaly, retina hemorrhage, and RARELY purpuraPerivascular infiltrate containing lymphocytes and neutrophils and eosinophils
41 Waldenström's Macroglobulinemia Plasmapheresis until adequate dose of chlorambucil is administered.Cyclophosphamide and corticosteroids are treatment options as well
42 Drug- and Food Induced Purpura Drug induced purpura may occur without platelet destruction.Cocaine induced thrombosis with infarctive skin lesions is assoc. with skin popping.Rumpel-Leede sign: distal shower of petechiae that occurs immediately after the release of pressure from a tourniquet release. Associated with capillary fragility.Topical EMLA can induce purpura in 30m.
43 Solar PurpuraLarge, sharply outlined 1-5 cm dark purplish red ecchymoses on dorsum of the forearmLess frequently, back of the hand
45 Purpura Fulminans Aka purpura gangrenosa Severe, rapidly fatal reaction occurring most commonly in children after infectious illnessSudden appearance of large ecchymotic areas, esp. prominent over the extremities, progressing to acral hemorrhagic skin necrosis is characteristicUsually follows some acute infectious disease such as scarlet fever, strep pharyngitis, and meningococcal meningitis, varicella.
46 Purpura Fulminans Assoc. with Protein C or S deficiency in Neonates Management is supportiveProtein C replacement if protein C deficiency is presentFresh frozen plasma maybe usefulAmputations and deaths continue in severest forms of the disease
50 Disseminated Intravascular Coagulation Up to 2/3 of DIC patients have skin lesionsMinute, widespread petechiae, ecchymoses, ischemic necrosis of the skin and hemorrhage bullae.Elevated PT and PTT, fibrin degradation productsDecrease platelets, decreased fibrinogen
51 Disseminated Intravascular Coagulation All patient needs to receive vitamin K replacement to exclude vitamin K deficiency.FFP and platelets may be useful
52 Fibrinolysis Syndrome An acute hemorrhagic state brought by inability of the blood to clotMassive hemorrhages into the skin produce blackish, purplish swelling.Can be a complication of pregnancy in cases of placental previa, eclampsia, and fatal deathDisease is produced by excessive or inappropriate fibrinolysis
53 Blue Muffin BabyPurpuric lesions observed in newborns with congenital rubellaAssoc. with many disorder that produce extramedullary erythropoiesisGeneralized dark blue to magenta nonblanchable, indurated, round, oval hemispheric papules 1-7mmEvaluation with biopsy, TORCH serology, CBC, viral culture.
54 Fig. 1. Blueberry muffin baby Fig. 1. Blueberry muffin baby. A, Extensive lesions of dermal erythropoiesis in infant with erythroblastosis fetalis. B, Facial lesions in infant with congenital cytomegalovirus infection.
56 Itching Purpura Aka disseminated pruriginous angiodermatitis Orange-purplish-red petechiae evolve completely and may become confluent in 2 weeksRuns its course in 3-6 months. May become chronicUnknown etiology
57 Deep Vein Thrombosis Almost always affects femoral vein Can cause reversible ischemia or frank gangrenePatients may develop, either abruptly or gradually, severe pain, extensive edema, and cyanosis of an extremity, L leg> RSignificant superficial vein thrombosis is a risk factor for DVT
58 Deep Vein Thrombosis Pulmonary Embolism is a major concern Malignant Neoplasms are the most common underlying conditionPulmonary Embolism has 40% mortalityWhen assoc. with cancer, DVT is the first sign in 35% of the casesPatient younger than 40 with DVT prompt for search for cancer
60 Superficial Thrombophlebitis Painful induration with erythemaOften linear or branching configuration forming cordsMay be assoc. with a hypercoagulable stateNeed to be evaluated for possibility of deep venous diseaseTreatment is directed at the underlying causeElevation and local heat promotes clot dissolution, 8-12 weeks
61 Mondor’s Disease 3:1 = women:men Age range 30-60 Sudden appearance of a cord like thrombosed vein along the anterior-lateral chest wallFirst red and tender and subsequently change into a painless tough, fibrous band.
62 Mondor’s Disease Cause is multifactorial Incidence L=R No systemic symptoms associatedTreatment of the symptom: hot moist dressing and NSAIDRuns its course for 3-6 months.
65 CalciphylaxisEnd-stage renal disease patients with metastatic calcification are most exclusively affected by this disease.Reticulated violaceous, mottled patchesProgress into ecchymosis, central necrosis, and ulcerationGangrene and self amputation of the digits has been reported
66 CalciphylaxisThe pathogenic mechanism is related to predisposing sensitizing conditions that create a conductive environment for calcium precipitation50%+ morbidity and mortalityDeath is usually caused by staphylococcal sepsis after infectionHyperbaric oxygen has used with some success
71 On low magnification, basophilic alteration of a fibrous septum can be seen Fibrin thrombi are also present within many of the blood vessels of the subcutaneous adipose tissue (Figure 3). Higher magnification identifies calcium deposition within the fibrous septum, primarily on elastic fibers (Figure 4, Figure 5). Higher magnification of the same area with Verhoeff-van Gieson stain confirms the presence of fragmented elastic fibers (Figure 6). Special stain for calcium (von-Kossa) identifies calcium deposition both within the septum of the fat lobule (Figure 7) and within the walls of blood vessels (Figure 8).
72 Fibrin thrombi are also present within many of the blood vessels of the subcutaneous adipose tissue
73 Higher magnification of the same area with Verhoeff-van Gieson stain confirms the presence of fragmented elastic fibers
74 Special stain for calcium (von-Kossa) identifies calcium deposition both within the septum of the fat lobule
75 Scorbutic Purpura Bleeding gums Perifollicular purpura Corkscrew hairs Cutaneous purpuraDeficiency in Vitamin C
76 Achenbach’s Syndrome Aka Paroxysmal Hand Hematoma Spontaneous focal hemorrhage into palm or volar surfaceTransitory localized pain followed by rapid swelling and bluish discolorationAcute nature with rapid resolution
77 Painful Bruising Syndrome Aka Autoerythrocyte SensitizationAka Gardner-Diamond SyndromeDistinctive localized purpuric reactionYoung and middle-aged women with some emotional disturbanceAcquired platelet dysfunction with eosinophilia
78 Painful Bruising Syndrome Localized purpuric reaction, recurrent, characterized by extremely painful and tender, ill-defined ecchymosesEmotional upset is the precipitating factorIntracutaneous injections of erythrocyte stroma evoke lesions,WB, PRBC,WRBCSome believe the symptom to be artifactual.psychotherapy
79 Psychogenic Purpura Similar purpura as Painful bruising syndrome Absence of erythrocytes sensitivitySecretan’s syndrome: factitial lymphedema of the hand or L’oedeme bleu: factitial lymphedema of the arm may a ha purpuric component related to repetitive trauma
80 Pigmentary Purpuric Eruption Pigmented purpuric eruptions of the lower extremitiesSimilar histologic findingSchamberg'sMajocchi'sGougerot-Blum
81 Schamberg Diseases Aka progressive pigmentary dermatosis Grains of cayenne pepperSlow proximal extensionLesions seldom itchFavors lower shins and ankles
84 Majocchi’s Disease Aka purpura annularis telangiectodes Bluish annular macules 1-3cm in diameter with telangiectatic punctaFading of central areaBegins symmetrically at lower extremitiesInvolution requires as long as a year, and may prolong indefinitelyAsymptomatic.
90 Gougerot-Blum Pigmented purpuric lichenoid dermatitis Minute, rust-colored lichenoid papules that fuse into plaques of various huesLegs, thighs, and lower trunkDifferentiate from Schamberg based on distribution and lichenoid papular elevations.
91 Ducas and Kapetanakis’ pigmented purpura Scaly and papular eruptionHistologically present with distinguished from others by presence of spongiosisMust be distinguished from mycosis fungoides