Cutaneous Vascular Diseases, Cutaneous Vascular Diseases, Part 1Part 1

Dr. BraccianoDr. Bracciano

Raynaud’s PhenomenonRaynaud’s Phenomenon Intermittent constriction of the small digital Intermittent constriction of the small digital

arteries and arteriolesarteries and arterioles Persistently cyanotic and painfulPersistently cyanotic and painful Aggravated by cold weatherAggravated by cold weather Young middle aged womenYoung middle aged women Assoc c scleroderma, dermatomyositis, LE, Assoc c scleroderma, dermatomyositis, LE,

Mixed connective tissue diseases, Sjögren's Mixed connective tissue diseases, Sjögren's RA, and paroxysmal hemoglobinuria.RA, and paroxysmal hemoglobinuria.

Raynaud’s PhenomenonRaynaud’s Phenomenon Scleroderma is the underlying condition for Scleroderma is the underlying condition for

more than half of the patientsmore than half of the patients Maybe caused by medications, ie Maybe caused by medications, ie

bleomycinbleomycin Simple noninvasive tests along with other Simple noninvasive tests along with other

clinical findings are helpfulclinical findings are helpful

Raynaud’s DiseaseRaynaud’s Disease Primary disorder of cold sensitivityPrimary disorder of cold sensitivity Pallor, cyanosis, hyperemia, and numbness Pallor, cyanosis, hyperemia, and numbness

of the fingersof the fingers Precipitated by cold.Precipitated by cold. Present for 2 years with out associated Present for 2 years with out associated

disease findingdisease finding Good prognosisGood prognosis

Etiology ofRaynaud’s Etiology ofRaynaud’s Disease/phenomenonDisease/phenomenon

Multifactorial. Multifactorial. Increase alpha-2 sympathetic receptor Increase alpha-2 sympathetic receptor

activity on vessels. activity on vessels. Endothelia dysfunctionEndothelia dysfunction Deficiency in calcitonin gene related Deficiency in calcitonin gene related

proteinprotein Central thermoregulatory defectsCentral thermoregulatory defects

TX of Raynaud’s TX of Raynaud’s Disease/phenomenonDisease/phenomenon

Treatment includes avoidance of the Treatment includes avoidance of the aggravating factor, ie cold. (not just hands)aggravating factor, ie cold. (not just hands)

Vasodilating drugs, nifedipine, 10-20 mg Vasodilating drugs, nifedipine, 10-20 mg tid; prazosin 1-3 mg tidtid; prazosin 1-3 mg tid

Nitroglycerin 2% local applicationNitroglycerin 2% local application Sympathectomy in severe diseaseSympathectomy in severe disease

ErythromelalgiaErythromelalgia Aka erythermalgia and acromelalgiaAka erythermalgia and acromelalgia Rare form of paroxysmal vasodilation Rare form of paroxysmal vasodilation

affects the feet with burning. Infrequently affects the feet with burning. Infrequently upper ext.upper ext.

Burning may last from minutes to daysBurning may last from minutes to days Triggered by increase in environmental Triggered by increase in environmental

temperaturetemperature May be secondary to myeloproliferative May be secondary to myeloproliferative

disease such as polycythemia vera, TTPdisease such as polycythemia vera, TTP

Pathophysiology is poorly understoodPathophysiology is poorly understood Responds to treatment of primary disordersResponds to treatment of primary disorders Cold water immersionCold water immersion Serotonin antagonistsSerotonin antagonists

erythromelalgiaerythromelalgia

Livedo ReticularisLivedo Reticularis

Mottled or reticulated Mottled or reticulated pink/reddish/blue pink/reddish/blue discolorationdiscoloration

Assoc c LE, DM, Assoc c LE, DM, scleroderma, RAscleroderma, RA

Side effect of Side effect of amantadineamantadine

Necrotizing livedo reticularisNecrotizing livedo reticularis Assoc. with cutaneous nodules and Assoc. with cutaneous nodules and

ulcerationsulcerations Results from cholesterol emboli in severe Results from cholesterol emboli in severe

atherosclerotic diseaseatherosclerotic disease Sneddon’s syndrome, association of LR Sneddon’s syndrome, association of LR

with the development of a cerebrovascular with the development of a cerebrovascular lesionlesion

Livedoid VasculitisLivedoid Vasculitis Aka - atrophie blancheAka - atrophie blanche Aka - PURPLE (Painful purpuric ulcer with Aka - PURPLE (Painful purpuric ulcer with

reticular pattern of the lower extremity)reticular pattern of the lower extremity) Characterized clinically by early, focal, Characterized clinically by early, focal,

painful purpuric lesions of the lower painful purpuric lesions of the lower extremities that frequently ulcerate and extremities that frequently ulcerate and slowly healslowly heal

Mostly represents an idiopathic disorder but Mostly represents an idiopathic disorder but may be assoc. with systemic diseasemay be assoc. with systemic disease

TreatmentTreatment Low Dosage of Aspirin 325mg qdLow Dosage of Aspirin 325mg qd Nifedipine 10mg TIDNifedipine 10mg TID Pentoxifylline 400mg BID-TIDPentoxifylline 400mg BID-TID

Marshall-White SyndromeMarshall-White Syndrome “Bier’s Spots” “Bier’s Spots”

Marbled mottling produced in the forearm Marbled mottling produced in the forearm and hand with use of a tight and hand with use of a tight sphgmomanometersphgmomanometer

Consist of Bier’s spot and is associated with Consist of Bier’s spot and is associated with insomnia and tachycardiainsomnia and tachycardia

White middle age menWhite middle age men

PurpuraPurpura Multifocal extravasation of blood into the Multifocal extravasation of blood into the

skin or mucous membraneskin or mucous membrane Petechiae <3mmPetechiae <3mm Ecchymosis – deeper and more extensive Ecchymosis – deeper and more extensive

interstitial hemorrhageinterstitial hemorrhage Vibices (vibex) – LinearVibices (vibex) – Linear Hematoma – pool-like collection, usually Hematoma – pool-like collection, usually

walled off by facial planeswalled off by facial planes

PurpuraPurpura May result from hyper or hypocoagulable May result from hyper or hypocoagulable

states, vascular dysfunction, and states, vascular dysfunction, and extravascular causesextravascular causes

Complete blood countComplete blood count PT and PTTPT and PTT Family historyFamily history medicationsmedications

Thrombocytopenic PurpuraThrombocytopenic Purpura Three Large Categories:Three Large Categories:

– Accelerated platelet destruction, immunologic Accelerated platelet destruction, immunologic and nonimmunologic and nonimmunologic

– Deficient platelet productionDeficient platelet production– Unknown pathogenesisUnknown pathogenesis

Idiopathic Thrombocytopenic Idiopathic Thrombocytopenic PurpuraPurpura

Aka autoimmune thrombocytopenic purpuraAka autoimmune thrombocytopenic purpura Aka Werlhof’s diseaseAka Werlhof’s disease Characterized by acute or gradual onset of Characterized by acute or gradual onset of

petechiae or ecchymosis in the skin and mmpetechiae or ecchymosis in the skin and mm Bleeding occurs when platelet count drops Bleeding occurs when platelet count drops

below 50,000below 50,000 Risk greatly increased for serious Risk greatly increased for serious

hemorrhage when count goes below 10,000hemorrhage when count goes below 10,000

Idiopathic Thrombocytopenic Idiopathic Thrombocytopenic PurpuraPurpura

Acute variety occurs in children following Acute variety occurs in children following season viral illness in 50% of the patient.season viral illness in 50% of the patient.

Lag between illness and onset of purpura is Lag between illness and onset of purpura is 2 weeks2 weeks

Most cases resolve spontaneously with Most cases resolve spontaneously with minimal therapyminimal therapy

Chronic case may result in death.Chronic case may result in death.

Idiopathic Thrombocytopenic Idiopathic Thrombocytopenic PurpuraPurpura

Chronic form most often occurs in adultsChronic form most often occurs in adults Evaluate patient with Tc99M radionuclide Evaluate patient with Tc99M radionuclide

scan to look for accessory spleenscan to look for accessory spleen Idiopathic Thrombocytopenic Purpura is the Idiopathic Thrombocytopenic Purpura is the

result of platelet injury by antibodies of the result of platelet injury by antibodies of the IgG classIgG class

Treatment include Splenectomy, systemic Treatment include Splenectomy, systemic corticosteroid, IVIgcorticosteroid, IVIg

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

Drug-Induced ThrombocytopeniaDrug-Induced Thrombocytopenia

Drug induced antiplatelet antibodiesDrug induced antiplatelet antibodies May be caused by sulfonamides, digoxin, May be caused by sulfonamides, digoxin,

quinine, quinidine, PCN, furosemide, quinine, quinidine, PCN, furosemide, Lidocaine, methyldopaLidocaine, methyldopa

Remove the offending agentRemove the offending agent corticosteroidscorticosteroids

Thrombotic Thrombocytopenic Thrombotic Thrombocytopenic PurpuraPurpura

Aka Moschcowitz’s syndromeAka Moschcowitz’s syndrome Pentad of thrombocytopenia, hemolytic Pentad of thrombocytopenia, hemolytic

anemia, renal abnormalities, fever, CNS anemia, renal abnormalities, fever, CNS disturbance.disturbance.

Delay in diagnosis may lead to a mortality Delay in diagnosis may lead to a mortality rate as high as 90%rate as high as 90%

Exchange plasmapheresis, 3 to 5 liters of Exchange plasmapheresis, 3 to 5 liters of plasma for 4 to 10 daysplasma for 4 to 10 days

Splenectomy may be requiredSplenectomy may be required

Thrombotic Thrombocytopenic Thrombotic Thrombocytopenic PurpuraPurpura

Positive histologic diagnosis requires Positive histologic diagnosis requires gingival biopsies looking for gingival biopsies looking for subendothelial hyaline depositssubendothelial hyaline deposits

Exchange plasmapheresis is required for Exchange plasmapheresis is required for treatment. 80% patient survive if treatment treatment. 80% patient survive if treatment is instituted.is instituted.

Dysproteinemic PurpuraDysproteinemic Purpura Aka Nonthrombocytopenic purpura, purpura Aka Nonthrombocytopenic purpura, purpura

cryoglobulinemica, cryofibrinogenemiacryoglobulinemica, cryofibrinogenemia Abnormal serum proteins behaving as Abnormal serum proteins behaving as

cryoglobulins and cryofibrinogenscryoglobulins and cryofibrinogens Purpura most apt to occur on exposed surfaces Purpura most apt to occur on exposed surfaces

after cold exposureafter cold exposure

Occurs most frequently in multiple Occurs most frequently in multiple myeloma and macroglobulinemia of myeloma and macroglobulinemia of monoclonal IgM, IgG, or Bence Jones monoclonal IgM, IgG, or Bence Jones cryoglobulin.cryoglobulin.

Purpura tends to be chronicPurpura tends to be chronic Tx with plasmapheresis, systemic steroids, Tx with plasmapheresis, systemic steroids,

and immunosuppressors.and immunosuppressors.

Purpura HyperglobulinemicaPurpura Hyperglobulinemica Aka Waldenström's hyperglobulinemic Aka Waldenström's hyperglobulinemic

purpurapurpura Consist of episodic showers of petechiae Consist of episodic showers of petechiae

occurring on all parts of body, most profusely occurring on all parts of body, most profusely on the lower extremitieson the lower extremities

Diffuse peppery distribution, resembling Diffuse peppery distribution, resembling Schamberg’sSchamberg’s

Induced or aggravated by prolonged walkingInduced or aggravated by prolonged walking Most useful lab test is protein electrophoresisMost useful lab test is protein electrophoresis

Purpura HyperglobulinemicaPurpura Hyperglobulinemica Hyperglobulinemic purpura occurs most Hyperglobulinemic purpura occurs most

commonly in women.commonly in women. Frequently seen with Hepatitis C and Frequently seen with Hepatitis C and

Sjögren's syndrome, keratoconjunctivitis Sjögren's syndrome, keratoconjunctivitis sicca, RAsicca, RA

Histologically: dermal vessels with Histologically: dermal vessels with perivascular infiltrate of mononuclear cells.perivascular infiltrate of mononuclear cells.

Benign and chronic course. May be assoc. Benign and chronic course. May be assoc. with connective tissue diseases.with connective tissue diseases.

Steroids are usually not of benefitSteroids are usually not of benefit

Waldenström's Waldenström's MacroglobulinemiaMacroglobulinemia

Bleeding from mucous membrane of the Bleeding from mucous membrane of the mouth and nose, lymphadenopathy, mouth and nose, lymphadenopathy, hepatomegaly, retina hemorrhage, and hepatomegaly, retina hemorrhage, and RARELY purpuraRARELY purpura

Perivascular infiltrate containing Perivascular infiltrate containing lymphocytes and neutrophils and lymphocytes and neutrophils and eosinophilseosinophils

Waldenström's Waldenström's MacroglobulinemiaMacroglobulinemia

Plasmapheresis until adequate dose of Plasmapheresis until adequate dose of chlorambucil is administered. chlorambucil is administered.

Cyclophosphamide and corticosteroids are Cyclophosphamide and corticosteroids are treatment options as welltreatment options as well

Drug- and Food Induced PurpuraDrug- and Food Induced Purpura

Drug induced purpura may occur without Drug induced purpura may occur without platelet destruction.platelet destruction.

Cocaine induced thrombosis with infarctive Cocaine induced thrombosis with infarctive skin lesions is assoc. with skin popping.skin lesions is assoc. with skin popping.

Rumpel-Leede sign: distal shower of Rumpel-Leede sign: distal shower of petechiae that occurs immediately after the petechiae that occurs immediately after the release of pressure from a tourniquet release of pressure from a tourniquet release. Associated with capillary fragility.release. Associated with capillary fragility.

Topical EMLA can induce purpura in 30m.Topical EMLA can induce purpura in 30m.

Solar PurpuraSolar Purpura Large, sharply outlined 1-5 cm dark Large, sharply outlined 1-5 cm dark

purplish red ecchymoses on dorsum of the purplish red ecchymoses on dorsum of the forearmforearm

Less frequently, back of the handLess frequently, back of the hand

           

          

Purpura FulminansPurpura Fulminans Aka purpura gangrenosaAka purpura gangrenosa Severe, rapidly fatal reaction occurring Severe, rapidly fatal reaction occurring

most commonly in children after infectious most commonly in children after infectious illnessillness

Sudden appearance of large ecchymotic Sudden appearance of large ecchymotic areas, esp. prominent over the extremities, areas, esp. prominent over the extremities, progressing to acral hemorrhagic skin progressing to acral hemorrhagic skin necrosis is characteristicnecrosis is characteristic

Usually follows some acute infectious Usually follows some acute infectious disease such as scarlet fever, strep disease such as scarlet fever, strep pharyngitis, and meningococcal meningitis, pharyngitis, and meningococcal meningitis, varicella.varicella.

Purpura FulminansPurpura Fulminans Assoc. with Protein C or S deficiency in Assoc. with Protein C or S deficiency in

NeonatesNeonates Management is supportiveManagement is supportive Protein C replacement if protein C Protein C replacement if protein C

deficiency is presentdeficiency is present Fresh frozen plasma maybe usefulFresh frozen plasma maybe useful Amputations and deaths continue in Amputations and deaths continue in

severest forms of the diseaseseverest forms of the disease

Disseminated Intravascular Disseminated Intravascular CoagulationCoagulation

Up to 2/3 of DIC patients have skin lesionsUp to 2/3 of DIC patients have skin lesions Minute, widespread petechiae, ecchymoses, Minute, widespread petechiae, ecchymoses,

ischemic necrosis of the skin and ischemic necrosis of the skin and hemorrhage bullae.hemorrhage bullae.

Elevated PT and PTT, fibrin degradation Elevated PT and PTT, fibrin degradation productsproducts

Decrease platelets, decreased fibrinogenDecrease platelets, decreased fibrinogen

Disseminated Intravascular Disseminated Intravascular CoagulationCoagulation

All patient needs to receive vitamin K All patient needs to receive vitamin K replacement to exclude vitamin K replacement to exclude vitamin K deficiency.deficiency.

FFP and platelets may be usefulFFP and platelets may be useful

Fibrinolysis SyndromeFibrinolysis Syndrome An acute hemorrhagic state brought by An acute hemorrhagic state brought by

inability of the blood to clotinability of the blood to clot Massive hemorrhages into the skin produce Massive hemorrhages into the skin produce

blackish, purplish swelling.blackish, purplish swelling. Can be a complication of pregnancy in Can be a complication of pregnancy in

cases of placental previa, eclampsia, and cases of placental previa, eclampsia, and fatal deathfatal death

Disease is produced by excessive or Disease is produced by excessive or inappropriate fibrinolysisinappropriate fibrinolysis

Blue Muffin BabyBlue Muffin Baby Purpuric lesions observed in newborns with Purpuric lesions observed in newborns with

congenital rubellacongenital rubella Assoc. with many disorder that produce Assoc. with many disorder that produce

extramedullary erythropoiesisextramedullary erythropoiesis Generalized dark blue to magenta Generalized dark blue to magenta

nonblanchable, indurated, round, oval nonblanchable, indurated, round, oval hemispheric papules 1-7mmhemispheric papules 1-7mm

Evaluation with biopsy, TORCH serology, Evaluation with biopsy, TORCH serology, CBC, viral culture.CBC, viral culture.

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.

CMVCMV

Itching PurpuraItching Purpura Aka disseminated pruriginous Aka disseminated pruriginous

angiodermatitisangiodermatitis Orange-purplish-red petechiae evolve Orange-purplish-red petechiae evolve

completely and may become confluent in 2 completely and may become confluent in 2 weeksweeks

Runs its course in 3-6 months. May Runs its course in 3-6 months. May become chronicbecome chronic

Unknown etiologyUnknown etiology

Deep Vein ThrombosisDeep Vein Thrombosis Almost always affects femoral veinAlmost always affects femoral vein Can cause reversible ischemia or frank Can cause reversible ischemia or frank

gangrenegangrene Patients may develop, either abruptly or Patients may develop, either abruptly or

gradually, severe pain, extensive edema, gradually, severe pain, extensive edema, and cyanosis of an extremity, L leg> Rand cyanosis of an extremity, L leg> R

Significant superficial vein thrombosis is a Significant superficial vein thrombosis is a risk factor for DVTrisk factor for DVT

Deep Vein ThrombosisDeep Vein Thrombosis Pulmonary Embolism is a major concernPulmonary Embolism is a major concern Malignant Neoplasms are the most common Malignant Neoplasms are the most common

underlying conditionunderlying condition Pulmonary Embolism has 40% mortalityPulmonary Embolism has 40% mortality When assoc. with cancer, DVT is the first When assoc. with cancer, DVT is the first

sign in 35% of the casessign in 35% of the cases Patient younger than 40 with DVT prompt Patient younger than 40 with DVT prompt

for search for cancerfor search for cancer

Superficial ThrombophlebitisSuperficial Thrombophlebitis

Painful induration with erythemaPainful induration with erythema Often linear or branching configuration Often linear or branching configuration

forming cordsforming cords May be assoc. with a hypercoagulable stateMay be assoc. with a hypercoagulable state Need to be evaluated for possibility of deep Need to be evaluated for possibility of deep

venous diseasevenous disease Treatment is directed at the underlying Treatment is directed at the underlying

causecause Elevation and local heat promotes clot Elevation and local heat promotes clot

dissolution, 8-12 weeksdissolution, 8-12 weeks

Mondor’s DiseaseMondor’s Disease 3:1 = women:men3:1 = women:men Age range 30-60Age range 30-60 Sudden appearance of a cord like Sudden appearance of a cord like

thrombosed vein along the anterior-lateral thrombosed vein along the anterior-lateral chest wallchest wall

First red and tender and subsequently First red and tender and subsequently change into a painless tough, fibrous band.change into a painless tough, fibrous band.

Mondor’s DiseaseMondor’s Disease Cause is multifactorialCause is multifactorial Incidence L=RIncidence L=R No systemic symptoms associatedNo systemic symptoms associated Treatment of the symptom: hot moist Treatment of the symptom: hot moist

dressing and NSAIDdressing and NSAID Runs its course for 3-6 months.Runs its course for 3-6 months.

CalciphylaxisCalciphylaxis End-stage renal disease patients with End-stage renal disease patients with

metastatic calcification are most exclusively metastatic calcification are most exclusively affected by this disease.affected by this disease.

Reticulated violaceous, mottled patchesReticulated violaceous, mottled patches Progress into ecchymosis, central necrosis, Progress into ecchymosis, central necrosis,

and ulcerationand ulceration Gangrene and self amputation of the digits Gangrene and self amputation of the digits

has been reportedhas been reported

CalciphylaxisCalciphylaxis The pathogenic mechanism is related to The pathogenic mechanism is related to

predisposing sensitizing conditions that predisposing sensitizing conditions that create a conductive environment for create a conductive environment for calcium precipitationcalcium precipitation

50%+ morbidity and mortality50%+ morbidity and mortality Death is usually caused by staphylococcal Death is usually caused by staphylococcal

sepsis after infectionsepsis after infection Hyperbaric oxygen has used with some Hyperbaric oxygen has used with some

successsuccess

calciphylaxiscalciphylaxis

On low magnification, basophilic alteration On low magnification, basophilic alteration of a fibrous septum can be seen Fibrin of a fibrous septum can be seen Fibrin thrombi are also present within many of the thrombi are also present within many of the blood vessels of the subcutaneous adipose blood vessels of the subcutaneous adipose tissue (tissue (Figure 3Figure 3). Higher magnification ). Higher magnification identifies calcium deposition within the identifies calcium deposition within the fibrous septum, primarily on elastic fibers (fibrous septum, primarily on elastic fibers (Figure 4Figure 4, , Figure 5Figure 5). Higher magnification ). Higher magnification of the same area with Verhoeff-van Gieson of the same area with Verhoeff-van Gieson stain confirms the presence of fragmented stain confirms the presence of fragmented elastic fibers (elastic fibers (Figure 6Figure 6). Special stain for ). Special stain for calcium (von-Kossa) identifies calcium calcium (von-Kossa) identifies calcium deposition both within the septum of the fat deposition both within the septum of the fat lobule (lobule (Figure 7Figure 7) and within the walls of ) and within the walls of blood vessels (blood vessels (Figure 8Figure 8). ).

Fibrin thrombi are also present within many of the blood vessels of the subcutaneous adipose tissue

Higher magnification of the same area with Verhoeff-van Gieson stain confirms the presence of fragmented elastic fibers

Special stain for calcium (von-Kossa) identifies calcium deposition both within the septum of the fat lobule

Scorbutic PurpuraScorbutic Purpura Bleeding gumsBleeding gums Perifollicular purpuraPerifollicular purpura Corkscrew hairsCorkscrew hairs Cutaneous purpuraCutaneous purpura Deficiency in Vitamin CDeficiency in Vitamin C

Achenbach’s SyndromeAchenbach’s Syndrome Aka Paroxysmal Hand HematomaAka Paroxysmal Hand Hematoma Spontaneous focal hemorrhage into palm or Spontaneous focal hemorrhage into palm or

volar surfacevolar surface Transitory localized pain followed by rapid Transitory localized pain followed by rapid

swelling and bluish discolorationswelling and bluish discoloration Acute nature with rapid resolutionAcute nature with rapid resolution

Painful Bruising SyndromePainful Bruising Syndrome Aka Autoerythrocyte SensitizationAka Autoerythrocyte Sensitization Aka Gardner-Diamond SyndromeAka Gardner-Diamond Syndrome Distinctive localized purpuric reactionDistinctive localized purpuric reaction Young and middle-aged women with some Young and middle-aged women with some

emotional disturbanceemotional disturbance Acquired platelet dysfunction with Acquired platelet dysfunction with

eosinophiliaeosinophilia

Painful Bruising SyndromePainful Bruising Syndrome Localized purpuric reaction, recurrent, Localized purpuric reaction, recurrent,

characterized by extremely painful and characterized by extremely painful and tender, ill-defined ecchymosestender, ill-defined ecchymoses

Emotional upset is the precipitating factorEmotional upset is the precipitating factor Intracutaneous injections of erythrocyte Intracutaneous injections of erythrocyte

stroma evoke lesions,WB, PRBC,WRBCstroma evoke lesions,WB, PRBC,WRBC Some believe the symptom to be artifactual.Some believe the symptom to be artifactual. psychotherapypsychotherapy

Psychogenic PurpuraPsychogenic Purpura Similar purpura as Painful bruising Similar purpura as Painful bruising

syndromesyndrome Absence of erythrocytes sensitivityAbsence of erythrocytes sensitivity Secretan’s syndrome: factitial lymphedema Secretan’s syndrome: factitial lymphedema

of the hand or L’oedeme bleu: factitial of the hand or L’oedeme bleu: factitial lymphedema of the arm may a ha purpuric lymphedema of the arm may a ha purpuric component related to repetitive traumacomponent related to repetitive trauma

Pigmentary Purpuric EruptionPigmentary Purpuric Eruption

Pigmented purpuric eruptions of the lower Pigmented purpuric eruptions of the lower extremitiesextremities

Similar histologic findingSimilar histologic finding Schamberg'sSchamberg's Majocchi'sMajocchi's Gougerot-BlumGougerot-Blum

Schamberg DiseasesSchamberg Diseases Aka progressive pigmentary dermatosisAka progressive pigmentary dermatosis Grains of cayenne pepperGrains of cayenne pepper Slow proximal extensionSlow proximal extension Lesions seldom itchLesions seldom itch Favors lower shins and anklesFavors lower shins and ankles

Majocchi’s DiseaseMajocchi’s Disease Aka purpura annularis telangiectodesAka purpura annularis telangiectodes Bluish annular macules 1-3cm in diameter Bluish annular macules 1-3cm in diameter

with telangiectatic punctawith telangiectatic puncta Fading of central areaFading of central area Begins symmetrically at lower extremitiesBegins symmetrically at lower extremities Involution requires as long as a year, and Involution requires as long as a year, and

may prolong indefinitelymay prolong indefinitely Asymptomatic. Asymptomatic.

Gougerot-BlumGougerot-Blum Pigmented purpuric lichenoid dermatitisPigmented purpuric lichenoid dermatitis Minute, rust-colored lichenoid papules that Minute, rust-colored lichenoid papules that

fuse into plaques of various huesfuse into plaques of various hues Legs, thighs, and lower trunkLegs, thighs, and lower trunk Differentiate from Schamberg based on Differentiate from Schamberg based on

distribution and lichenoid papular distribution and lichenoid papular elevations.elevations.

Ducas and Kapetanakis’ Ducas and Kapetanakis’ pigmented purpurapigmented purpura

Scaly and papular eruptionScaly and papular eruption Histologically present with distinguished Histologically present with distinguished

from others by presence of spongiosisfrom others by presence of spongiosis Must be distinguished from mycosis Must be distinguished from mycosis

fungoidesfungoides

HistologyHistology Purpura Majocchi-Schamberg is Purpura Majocchi-Schamberg is

characterized by slight alteration of characterized by slight alteration of superficial capillaries with hemorrhage and superficial capillaries with hemorrhage and perivascular lymphocytic infiltrate. perivascular lymphocytic infiltrate.

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equipped with an airbag and he was able to walk away from this system

crash

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