Phlebologisch-Phlebologisch-LymphologischeLymphologischeFallvorstellungenFallvorstellungen

Felizitas PannierFelizitas PannierDepartment of DermatologyDepartment of Dermatology

University of CologneUniversity of ColognePrivate Practice Phlebology & Private Practice Phlebology &

DermatologyDermatologyGermanyGermany

Case 1Case 1•56-years old female patient

•Referred to our phlebology department for treatment of chronic venous insufficiency

•Cramps, feeling of heaviness and swelling of the ankle region every evening

•No past history of varicose veins, phlebitis or deep venous thrombosis

•Walking distance was not restricted

•Often walked in the forests

•On the lateral aspect of the right lower limb, in the ankle region an area of blue discoloration, atrophy of the skin and subcutaneous tissue

Case 1Case 1Doppler- and duplex-ultrasound investigation: normal superficial and deep venous system, normal ABI

Serological investigation showed positive IgG and IgM-antibodies against Borrelia burgdorferi

Diagnosis:Acrodermatitis chronica atrophicans Pick-Herxheimer

Therapy:14 days intravenously applied Ceftriaxon

Significant improvement of the skin changes.6 months later only positive IgG-antibodies against Borrelia burgdorferi were found.

Acrodermatitis chronica atrophicans Pick-Acrodermatitis chronica atrophicans Pick-HerxheimerHerxheimer

A differential diagnosis for Chronic venous A differential diagnosis for Chronic venous insufficiencyinsufficiency

Blue discoloration and skin atrophy in the ankle Blue discoloration and skin atrophy in the ankle region, as well as edema are not only found in region, as well as edema are not only found in chronic venous insufficiency or other vascular chronic venous insufficiency or other vascular diseases. In particular if venous investigations are diseases. In particular if venous investigations are normal, chronic infection by Burrelia burgdorferi has normal, chronic infection by Burrelia burgdorferi has

to be included in the differential diagnosisto be included in the differential diagnosis

Case 2Case 264 year old woman

7 years ago after stripping of GSV she had developed a linear atrophy and fibrosis on both legs associated to the stripping channel 2-3 weeks after the operation

Skin changes started at the incision areas, then spreading along the stripping chanel

Later she developed lesions in the area of the non stripped, but insufficient short saphenous veins on both legs

Case 2Case 2Duplex: recurrent varicose veins of the GSV at both legs

Laboratory routine findings: normal, a burrelia burgdorferi serology and antinuclear antibodies were negative

Case 1Case 1Histology:thick collagen fibre bundles and a perivascular, mostly lymphohistiocytic infiltration

Diagnosis:Morphea (circumscript scleroderma

Case 2Case 2

Morphea with Koebner-phenomenon after Morphea with Koebner-phenomenon after inflammation, trauma and operation is well inflammation, trauma and operation is well discribed in literature.discribed in literature.

However we could only find one paper However we could only find one paper concerning morphea after stripping of the concerning morphea after stripping of the saphenous veins The authors describe three saphenous veins The authors describe three cases. Non of them showed development of cases. Non of them showed development of morphea in the area of non operated morphea in the area of non operated insufficient veins.insufficient veins.

The question occurs if aside of the operation The question occurs if aside of the operation trauma, the varicose vein itself functions as trauma, the varicose vein itself functions as an isomorphic trigger factor for morphea.an isomorphic trigger factor for morphea.

This phenomenon is also known from VitiligoThis phenomenon is also known from Vitiligo

Case 3Case 365 year old patient65 year old patient

Atrial fibrillationAtrial fibrillation

Holidays in India 4 months agoHolidays in India 4 months ago

After injury at the beach of left 1st toe After injury at the beach of left 1st toe 2 x 3 cm ulceration2 x 3 cm ulceration

No healing, slight improvement with No healing, slight improvement with local antibiotics and antimycoticslocal antibiotics and antimycotics

Ulceration of unknown originUlceration of unknown origin

Case 3Case 3

Venous or arterial ulcer?Venous or arterial ulcer? Embolism in atrial fibrillation?Embolism in atrial fibrillation? Local infection?Local infection?

Case 3Case 3

Histology: histiocytic cutanous inflammationHistology: histiocytic cutanous inflammation Laboratory tests: normalLaboratory tests: normal Bacterial culture: Stapylococcus aureus pos.Bacterial culture: Stapylococcus aureus pos. Mykologic culture: negativeMykologic culture: negative Parasitology: positive Leishmania titer 1:256Parasitology: positive Leishmania titer 1:256 Skin biopsy: pos. Leishmania cultureSkin biopsy: pos. Leishmania culture Leishmania species:Leishmania species: Leishmania donovani Leishmania donovani

LeishmaniosisLeishmaniosisDifferent typesDifferent types

Cutaneous Leishmaniosis (Orientbeule):Cutaneous Leishmaniosis (Orientbeule):

Leishmania tropicaLeishmania tropica Mukokutaneous Leishmaniosis / Mukokutaneous Leishmaniosis /

Leishmaniosis of the New WorldLeishmaniosis of the New World

Leishmania brasiliensisLeishmania brasiliensis Visceral Leishmaniosis (Kalar Azar)Visceral Leishmaniosis (Kalar Azar)

Leishmania donovaniLeishmania donovani

DiagnosisDiagnosis

Leishmaniosis:Leishmaniosis:

cutaneous infection with the species for cutaneous infection with the species for

visceral Leishmaniosisvisceral Leishmaniosis

Tratment:Tratment:

Local? Local? Systemic?Systemic?ParamomycinsulfatParamomycinsulfat Ambisome (1. choice) Ambisome (1. choice)

Antimon intraläsionalAntimon intraläsional Pentamidin Pentamidin

Case 3Case 3

Before treatmentBefore treatment

Case 3Case 3

Local treatment: Local treatment: Paramomycinsulfat Paramomycinsulfat 15% in Uera pura 10% 15% in Uera pura 10% in Vaselinum albumin Vaselinum albumworsening of the worsening of the ulcerationulceration

Case 3Case 3

Ulcer after the 1st Ulcer after the 1st cycle of Ambisome cycle of Ambisome

Follow up: complete Follow up: complete healing, no healing, no recurrencerecurrence

Case 4Case 471 year old man with polycythaemia vera 71 year old man with polycythaemia vera - since 8 years 1500mg Hydroxycarbamid/d - since 8 years 1500mg Hydroxycarbamid/d

- since 6 months ulcerations left ankle- since 6 months ulcerations left ankle- Duplex: PTS both legs, ABI normal- Duplex: PTS both legs, ABI normal

Indications for HydroxycarbamidIndications for Hydroxycarbamid

Myeloproliferative DiseasesMyeloproliferative Diseases

- CML- CML

- Polycythaemia Vera- Polycythaemia Vera

- Thrombozytämia- Thrombozytämia

- Chronisc idiopathic Myelofibrosis- Chronisc idiopathic Myelofibrosis

Dermatologic side effects of Dermatologic side effects of HydroxycarbamidHydroxycarbamid

PigmentationPigmentation ErythemaErythema ShinglesShingles Skin- and Nail atrophySkin- and Nail atrophy AlopeziaAlopezia Dermatomyositis-like skin changesDermatomyositis-like skin changes rarely:rarely: Skinulceration Skinulceration

Ulcera at diagnosis

3 months after reduction of Hydroxycarbamid from1500mg/d to 500mg/d

80 year old woman with Polycythaemia 80 year old woman with Polycythaemia vera vera - since 1 1/2 years 1000mg - since 1 1/2 years 1000mg Hydroxycarbamid / d Hydroxycarbamid / d - since 9 months ulcerations on both legs - since 9 months ulcerations on both legs - Duplex: venous and arterial normal- Duplex: venous and arterial normal- high blood pressure- high blood pressure

Similar case

12 weeks after stop of Hydroxycarbamid treatment

Ulcera at diagnosis

Case 5Case 5

41-year old female patient

Since 2 years red, cold and painful right foot

Resting pain

Doppler- and Duplex: arterial occlusive disease in the right iliac and femoral artery

Migraine since she was 18 years old. Since 8 years ergotamine up to every second day

Case 5Case 5Angiography: filiform stenosis of the right external iliac artery and of the femoral artery on the left side

In general the arteriogram showed diffuse arterial spasm supporting the clinical diagnosis of egotism.

Case 5Case 5

Echocardiographic examination revealed Echocardiographic examination revealed aortic, tricuspid and mitral valve aortic, tricuspid and mitral valve insufficiency I°-II°insufficiency I°-II°

No childhood history suggesting scarlet or No childhood history suggesting scarlet or rheumatic feverrheumatic fever

In the first-line treatment the patient In the first-line treatment the patient abstained from ergotamine and nicotine abstained from ergotamine and nicotine abuseabuse

Intravenous infusion of prostaglandine was Intravenous infusion of prostaglandine was administeredadministered

Rapid and complete improvement of Rapid and complete improvement of arteriospasm in-between 8 days was noted, arteriospasm in-between 8 days was noted, confirmed by further duplex examinationconfirmed by further duplex examination

Case 5Case 5Ergotamine is known since the 6. century B.C.

In the middle ages many hundred thousand people might have died of egotism epidemy. It was caused by food contamination with Claviceps purpurea. This is an ergotamine-alkaloid producing mushroom.

Because of the burning pain the disease was called St. Anthony’s fire

Case 5Case 5 In this special case peripheral vasospasm was In this special case peripheral vasospasm was

combined with cardiac valve insufficiency of three combined with cardiac valve insufficiency of three valves.valves.

This combination is very rarely reported in This combination is very rarely reported in literature.literature.

The question stays if aside of vasospastic The question stays if aside of vasospastic complications cardiac valve insufficiency can also be complications cardiac valve insufficiency can also be caused by ergotamine abuse. caused by ergotamine abuse. 1. Austin S, El-Hayek A, M Comoanos, D Tamulonis: Mitral 1. Austin S, El-Hayek A, M Comoanos, D Tamulonis: Mitral

Valve Disease associated with Long-Term Ergotamine Use: Valve Disease associated with Long-Term Ergotamine Use: Southern Medical Journal 1993; 86 (10): 1179-81Southern Medical Journal 1993; 86 (10): 1179-81

5. Piquemal R, J Emmerich, J Guilmot, J. Fiessinger: 5. Piquemal R, J Emmerich, J Guilmot, J. Fiessinger: Successful Treatment for Ergotism with Iloprost. Angiology Successful Treatment for Ergotism with Iloprost. Angiology 1998; 49 (6): 493-71998; 49 (6): 493-7

13. Wilke A, H Hesse, G Hufnagel, B Maisch: Mitral, aortic 13. Wilke A, H Hesse, G Hufnagel, B Maisch: Mitral, aortic and tricuspid valvular disease associated with ergotamin and tricuspid valvular disease associated with ergotamin therapy for migraine. therapy for migraine. Eur Heart J. 1997; 18 (4): 701Eur Heart J. 1997; 18 (4): 701

Case 6Case 650 year old man

Right leg swelling since adolescence

Episodes of cellulitis

Diagnosis of lymphedema in a lymphologic hospital

Treatment:Compression stockings Lymphatic drainage

No improvement

Case 6Case 6Soft tumor

No fibrosis

Stemmer´s sign negative

No pitting edema

Case 6Case 6

Multiple Café au lait maculae

Diagnosis:Morbus Recklinghausen(Neurofibromatosis Type 1)Autosomal dominant

Family history negativeNo neurological tumors

Case 7Case 711 year old girl

After eating sweet and sour things within a few seconds intense präauricular reddening and warming

Resolving within minutes after stopping eating

No sweating in this region

Normal chemosensoric function of fascialis nerve

Case 7Case 73 years before first symptoms bothsided dislocated mandibular fracture after bycicle accident

Asymmetric face

X-ray: deformation of the left mandible (ramus ascendens)

Case 7Case 7

Frey-Syndrome (auriculotemporal syndrome) is Frey-Syndrome (auriculotemporal syndrome) is characterized by local sweating and reddening of the characterized by local sweating and reddening of the cheek after eating. Most frequent reasons are parotis cheek after eating. Most frequent reasons are parotis operations or trauma. operations or trauma.

Pathophysiology of Frey-Syndroms consists of Pathophysiology of Frey-Syndroms consists of aberrant regeneration of parasympatisc nerve fibers. aberrant regeneration of parasympatisc nerve fibers. The auriculotemporal nerve, a branch of the The auriculotemporal nerve, a branch of the trigeminus nerve innervates as well the glandula trigeminus nerve innervates as well the glandula parotis with parasympathic fibers as subcutaneous parotis with parasympathic fibers as subcutaneous blood vessels and sweat glands with sympathic fibers. blood vessels and sweat glands with sympathic fibers.

After trauma of the nerve the regeneration can lead After trauma of the nerve the regeneration can lead to parasympathic innervation of the blood vessels to parasympathic innervation of the blood vessels which was meant for the parotis gland. which was meant for the parotis gland.

In this case Frey syndrome occured with reddening In this case Frey syndrome occured with reddening alone and without sweating, a variation of the alone and without sweating, a variation of the syndrome.syndrome.

Case 8Case 8

SclerotherpySclerotherpy- complications and risks -- complications and risks -

Allergic reactionAllergic reaction Skin necrosisSkin necrosis PhlebitisPhlebitis PigmentationPigmentation MattingMatting Nerve damageNerve damage Visual disturbancesVisual disturbances Migraine like neurological disturbances Migraine like neurological disturbances Thromboembolic complicationsThromboembolic complications

ESAF StudyESAF Studyside effectsside effects

Rabe et al. 2007, n = 108

liquid liquid

n = 53n = 53(%)(%) foam foam

n = 55n = 55(%)(%)

painpain 66 1111 77 1313

hematomahematoma 77 1313 33 55

phlebitisphlebitis 66 1111 77 1313

indurationinduration 33 66 44 77

pigmentationpigmentation 44 88 55 99

itchingitching 22 44 11 22

Metallic tasteMetallic taste 33 66 -- --

French Study (French Study (n= 12.173 sessionsn= 12.173 sessions))-thromboembolic complications--thromboembolic complications-

ComplicationComplication LiquidLiquid FoamFoam bothboth

DVT (V. fem. sup. 6 ml foam in GSV)DVT (V. fem. sup. 6 ml foam in GSV) 00 11 00

Muscle vein thrombosisMuscle vein thrombosis 00 22 00

Perforator thrombosisPerforator thrombosis 00 33 00

ThrombophlebitisThrombophlebitis 00 33 00

Guex et al. Dermatol Surg 2005; 31: 123-128

Late-onset phlebitisLate-onset phlebitis

B. D.: female, 53 years old

07.03.2008: foam sclerotherapy tributary left medial lower leg1% polidocanol, 1:5, 2mlno adverse reaction after 1 week at control

4 weeks later: phlebitis GSV, 10 cm, Duplex: vein not compressible, no DVT,no history of phlebitis or DVT

Resolved with compression stocking after 10 days

Late-onset phlebitisLate-onset phlebitis

phlebitis

tributary after sclerotherapy

Late-onset phlebitisLate-onset phlebitis

G. M.: female, 57 years old09.04.2008: foam sclerotherapy of tributary varicose veins left thigh1% and 0.5% polidocanol, 1:5, 4 ml, compression stocking16.04.2008: all treated veins occluded, no side effects28.04.2008: acute phlebitis of GSV at distal lower leg, duplex: GSV distallyoccluded, no DVT. No history of phlebitis or DVT.

Late-onset phlebitisLate-onset phlebitis

Phlebitis in a superficial veinPhlebitis in a superficial vein First symptoms 2 – 6 weeks after foam First symptoms 2 – 6 weeks after foam

sclerotherapysclerotherapy Short segmentsShort segments No clinical signs of phlebitis in betweenNo clinical signs of phlebitis in between

Late-onset phlebitisLate-onset phlebitis

Patients after successful foam sclerotherapyPatients after successful foam sclerotherapy No initial thromboembolic reactionNo initial thromboembolic reaction Phlebitis in surrounding veins after 2 – 6 weeksPhlebitis in surrounding veins after 2 – 6 weeks Theory 1:Theory 1:

Insufficient sclerosing effectInsufficient sclerosing effect Progression of thrombus after an intervalProgression of thrombus after an interval

Theory 2: Theory 2: Active foam has been kept enclosed in the treated and Active foam has been kept enclosed in the treated and

occluded vein.occluded vein. After some weeks thrombolysis of thrombus material may After some weeks thrombolysis of thrombus material may

occur and minimal doses of still active polidocanol are set free.occur and minimal doses of still active polidocanol are set free. These low amounts are not able to sclerose the vein but cause These low amounts are not able to sclerose the vein but cause

local irritation of veins in the surroundings of the initially local irritation of veins in the surroundings of the initially treated vessel.treated vessel.

K.Parsi et al EJVES 2009; 38: 220-228K.Parsi et al EJVES 2009; 38: 220-228: in vitro low concentrations of : in vitro low concentrations of polidocanol have procoagulant activitiespolidocanol have procoagulant activities

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