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Cutaneous Vascular Diseases, Part 1

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Presentation on theme: "Cutaneous Vascular Diseases, Part 1"— Presentation transcript:

1 Cutaneous Vascular Diseases, Part 1

2 Dr. Bracciano

3 Raynaud’s Phenomenon Intermittent constriction of the small digital arteries and arterioles Persistently cyanotic and painful Aggravated by cold weather Young middle aged women Assoc c scleroderma, dermatomyositis, LE, Mixed connective tissue diseases, Sjögren's RA, and paroxysmal hemoglobinuria.

4 Raynaud’s Phenomenon Scleroderma is the underlying condition for more than half of the patients Maybe caused by medications, ie bleomycin Simple noninvasive tests along with other clinical findings are helpful

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7 Raynaud’s Disease Primary disorder of cold sensitivity
Pallor, cyanosis, hyperemia, and numbness of the fingers Precipitated by cold. Present for 2 years with out associated disease finding Good prognosis

8 Etiology ofRaynaud’s Disease/phenomenon
Multifactorial. Increase alpha-2 sympathetic receptor activity on vessels. Endothelia dysfunction Deficiency in calcitonin gene related protein Central 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 tid Nitroglycerin 2% local application Sympathectomy 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 days Triggered by increase in environmental temperature May be secondary to myeloproliferative disease such as polycythemia vera, TTP

11 Pathophysiology is poorly understood
Responds to treatment of primary disorders Cold water immersion Serotonin antagonists

12 erythromelalgia

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14 Livedo Reticularis Mottled or reticulated pink/reddish/blue discoloration Assoc c LE, DM, scleroderma, RA Side effect of amantadine

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17 Necrotizing livedo reticularis
Assoc. with cutaneous nodules and ulcerations Results from cholesterol emboli in severe atherosclerotic disease Sneddon’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 heal Mostly 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

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22 Marshall-White Syndrome “Bier’s Spots”
Marbled mottling produced in the forearm and hand with use of a tight sphgmomanometer Consist of Bier’s spot and is associated with insomnia and tachycardia White middle age men

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24 Purpura Multifocal extravasation of blood into the skin or mucous membrane Petechiae <3mm Ecchymosis – deeper and more extensive interstitial hemorrhage Vibices (vibex) – Linear Hematoma – pool-like collection, usually walled off by facial planes

25 Purpura May result from hyper or hypocoagulable states, vascular dysfunction, and extravascular causes Complete blood count PT and PTT Family history medications

26 Thrombocytopenic Purpura
Three Large Categories: Accelerated platelet destruction, immunologic and nonimmunologic Deficient platelet production Unknown pathogenesis

27 Idiopathic Thrombocytopenic Purpura
Aka autoimmune thrombocytopenic purpura Aka Werlhof’s disease Characterized by acute or gradual onset of petechiae or ecchymosis in the skin and mm Bleeding occurs when platelet count drops below 50,000 Risk 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 weeks Most cases resolve spontaneously with minimal therapy Chronic case may result in death.

29 Idiopathic Thrombocytopenic Purpura
Chronic form most often occurs in adults Evaluate patient with Tc99M radionuclide scan to look for accessory spleen Idiopathic Thrombocytopenic Purpura is the result of platelet injury by antibodies of the IgG class Treatment include Splenectomy, systemic corticosteroid, IVIg

30 These are the kidneys from a case of idiopathic thrombocytopenic purpura. Petechiae are found throughout the renal parenchyma.

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33 Drug-Induced Thrombocytopenia
Drug induced antiplatelet antibodies May be caused by sulfonamides, digoxin, quinine, quinidine, PCN, furosemide, Lidocaine, methyldopa Remove the offending agent corticosteroids

34 Thrombotic Thrombocytopenic Purpura
Aka Moschcowitz’s syndrome Pentad 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 days Splenectomy may be required

35 Thrombotic Thrombocytopenic Purpura
Positive histologic diagnosis requires gingival biopsies looking for subendothelial hyaline deposits Exchange plasmapheresis is required for treatment. 80% patient survive if treatment is instituted.

36 Dysproteinemic Purpura
Aka Nonthrombocytopenic purpura, purpura cryoglobulinemica, cryofibrinogenemia Abnormal serum proteins behaving as cryoglobulins and cryofibrinogens Purpura 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 chronic Tx with plasmapheresis, systemic steroids, and immunosuppressors.

38 Purpura Hyperglobulinemica
Aka Waldenström's hyperglobulinemic purpura Consist of episodic showers of petechiae occurring on all parts of body, most profusely on the lower extremities Diffuse peppery distribution, resembling Schamberg’s Induced or aggravated by prolonged walking Most 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, RA Histologically: 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 purpura Perivascular 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 Purpura Large, sharply outlined 1-5 cm dark purplish red ecchymoses on dorsum of the forearm Less frequently, back of the hand

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45 Purpura Fulminans Aka purpura gangrenosa
Severe, rapidly fatal reaction occurring most commonly in children after infectious illness Sudden appearance of large ecchymotic areas, esp. prominent over the extremities, progressing to acral hemorrhagic skin necrosis is characteristic Usually 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 supportive Protein C replacement if protein C deficiency is present Fresh frozen plasma maybe useful Amputations and deaths continue in severest forms of the disease

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50 Disseminated Intravascular Coagulation
Up to 2/3 of DIC patients have skin lesions Minute, widespread petechiae, ecchymoses, ischemic necrosis of the skin and hemorrhage bullae. Elevated PT and PTT, fibrin degradation products Decrease 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 clot Massive hemorrhages into the skin produce blackish, purplish swelling. Can be a complication of pregnancy in cases of placental previa, eclampsia, and fatal death Disease is produced by excessive or inappropriate fibrinolysis

53 Blue Muffin Baby Purpuric lesions observed in newborns with congenital rubella Assoc. with many disorder that produce extramedullary erythropoiesis Generalized dark blue to magenta nonblanchable, indurated, round, oval hemispheric papules 1-7mm Evaluation 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.

55 CMV

56 Itching Purpura Aka disseminated pruriginous angiodermatitis
Orange-purplish-red petechiae evolve completely and may become confluent in 2 weeks Runs its course in 3-6 months. May become chronic Unknown etiology

57 Deep Vein Thrombosis Almost always affects femoral vein
Can cause reversible ischemia or frank gangrene Patients may develop, either abruptly or gradually, severe pain, extensive edema, and cyanosis of an extremity, L leg> R Significant 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 condition Pulmonary Embolism has 40% mortality When assoc. with cancer, DVT is the first sign in 35% of the cases Patient younger than 40 with DVT prompt for search for cancer

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60 Superficial Thrombophlebitis
Painful induration with erythema Often linear or branching configuration forming cords May be assoc. with a hypercoagulable state Need to be evaluated for possibility of deep venous disease Treatment is directed at the underlying cause Elevation 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 wall First 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 associated Treatment of the symptom: hot moist dressing and NSAID Runs its course for 3-6 months.

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65 Calciphylaxis End-stage renal disease patients with metastatic calcification are most exclusively affected by this disease. Reticulated violaceous, mottled patches Progress into ecchymosis, central necrosis, and ulceration Gangrene and self amputation of the digits has been reported

66 Calciphylaxis The pathogenic mechanism is related to predisposing sensitizing conditions that create a conductive environment for calcium precipitation 50%+ morbidity and mortality Death is usually caused by staphylococcal sepsis after infection Hyperbaric oxygen has used with some success

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69 calciphylaxis

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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 purpura Deficiency in Vitamin C

76 Achenbach’s Syndrome Aka Paroxysmal Hand Hematoma
Spontaneous focal hemorrhage into palm or volar surface Transitory localized pain followed by rapid swelling and bluish discoloration Acute nature with rapid resolution

77 Painful Bruising Syndrome
Aka Autoerythrocyte Sensitization Aka Gardner-Diamond Syndrome Distinctive localized purpuric reaction Young and middle-aged women with some emotional disturbance Acquired platelet dysfunction with eosinophilia

78 Painful Bruising Syndrome
Localized purpuric reaction, recurrent, characterized by extremely painful and tender, ill-defined ecchymoses Emotional upset is the precipitating factor Intracutaneous injections of erythrocyte stroma evoke lesions,WB, PRBC,WRBC Some believe the symptom to be artifactual. psychotherapy

79 Psychogenic Purpura Similar purpura as Painful bruising syndrome
Absence of erythrocytes sensitivity Secretan’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 extremities Similar histologic finding Schamberg's Majocchi's Gougerot-Blum

81 Schamberg Diseases Aka progressive pigmentary dermatosis
Grains of cayenne pepper Slow proximal extension Lesions seldom itch Favors lower shins and ankles

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84 Majocchi’s Disease Aka purpura annularis telangiectodes
Bluish annular macules 1-3cm in diameter with telangiectatic puncta Fading of central area Begins symmetrically at lower extremities Involution requires as long as a year, and may prolong indefinitely Asymptomatic.

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90 Gougerot-Blum Pigmented purpuric lichenoid dermatitis
Minute, rust-colored lichenoid papules that fuse into plaques of various hues Legs, thighs, and lower trunk Differentiate from Schamberg based on distribution and lichenoid papular elevations.

91 Ducas and Kapetanakis’ pigmented purpura
Scaly and papular eruption Histologically present with distinguished from others by presence of spongiosis Must be distinguished from mycosis fungoides

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93 Histology Purpura Majocchi-Schamberg is characterized by slight alteration of superficial capillaries with hemorrhage and perivascular lymphocytic infiltrate.

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96 Luckily, Dave's computer was equipped with an airbag and he was able to walk away from this system crash See you next time…


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