Transcript Case 3

Phlebologisch-Lymphologische
Fallvorstellungen
Felizitas Pannier
Department of Dermatology
University of Cologne
Private Practice Phlebology & Dermatology
Germany
Case 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 1
Doppler- 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 PickHerxheimer
Therapy:
14 days intravenously applied Ceftriaxon
Significant improvement of the skin
changes.
6 months later only positive IgGantibodies against Borrelia burgdorferi
were found.
Acrodermatitis chronica atrophicans PickHerxheimer
A differential diagnosis for Chronic venous
insufficiency
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Blue discoloration and skin atrophy in the ankle region, as
well as edema are not only found in chronic venous
insufficiency or other vascular diseases. In particular if
venous investigations are normal, chronic infection by
Burrelia burgdorferi has to be included in the differential
diagnosis
Case 2
64 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 2
Duplex: recurrent varicose veins
of the GSV at both legs
Laboratory routine findings:
normal, a burrelia burgdorferi
serology and antinuclear
antibodies were negative
Case 1
Histology:
thick collagen fibre bundles
and a perivascular, mostly
lymphohistiocytic
infiltration
Diagnosis:
Morphea (circumscript
scleroderma
Case 2
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Morphea with Koebner-phenomenon after
inflammation, trauma and operation is well
discribed in literature.
However we could only find one paper concerning
morphea after stripping of the saphenous veins
The authors describe three cases. Non of them
showed development of morphea in the area of
non operated insufficient veins.
The question occurs if aside of the operation
trauma, the varicose vein itself functions as an
isomorphic trigger factor for morphea.
This phenomenon is also known from Vitiligo
Case 3
65 year old patient
Atrial fibrillation
Holidays in India 4 months ago
After injury at the beach of left 1st toe
2 x 3 cm ulceration
No healing, slight improvement with local
antibiotics and antimycotics
 Ulceration of unknown origin
Case 3
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Venous or arterial ulcer?
Embolism in atrial fibrillation?
Local infection?
Case 3
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Histology: histiocytic cutanous inflammation
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Laboratory tests: normal
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Bacterial culture: Stapylococcus aureus pos.
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Mykologic culture: negative
Parasitology: positive Leishmania titer 1:256
Skin biopsy: pos. Leishmania culture
Leishmania species: Leishmania donovani
Leishmaniosis
Different types
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Cutaneous Leishmaniosis (Orientbeule):
Leishmania tropica
Mukokutaneous Leishmaniosis / Leishmaniosis of
the New World
Leishmania brasiliensis
Visceral Leishmaniosis (Kalar Azar)
Leishmania donovani
Diagnosis
Leishmaniosis:
cutaneous infection with the species for
visceral Leishmaniosis
Tratment:
Local?
 Systemic?
Paramomycinsulfat
Antimon intraläsional
Ambisome (1. choice)
Pentamidin
Case 3
Before treatment
Case 3
Local treatment:
Paramomycinsulfat 15%
in Uera pura 10% in
Vaselinum album
worsening of the
ulceration
Case 3
Ulcer after the 1st
cycle of Ambisome
Follow up: complete
healing, no recurrence
Case 4
71 year old man with polycythaemia vera
- since 8 years 1500mg Hydroxycarbamid/d
- since 6 months ulcerations left ankle
- Duplex: PTS both legs, ABI normal
Indications for Hydroxycarbamid
Myeloproliferative Diseases
- CML
- Polycythaemia Vera
- Thrombozytämia
- Chronisc idiopathic Myelofibrosis
Dermatologic side effects of
Hydroxycarbamid
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Pigmentation
Erythema
Shingles
Skin- and Nail atrophy
Alopezia
Dermatomyositis-like skin changes
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rarely:
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Skinulceration
Ulcera at diagnosis
3 months after reduction of
Hydroxycarbamid from1500mg/d to
500mg/d
Similar case
80 year old woman with Polycythaemia vera
- since 1 1/2 years 1000mg Hydroxycarbamid / d
- since 9 months ulcerations on both legs
- Duplex: venous and arterial normal
- high blood pressure
12 weeks after stop of
Hydroxycarbamid treatment
Ulcera at diagnosis
Case 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 5
Angiography:
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 5
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Echocardiographic examination revealed aortic,
tricuspid and mitral valve insufficiency I°-II°
No childhood history suggesting scarlet or
rheumatic fever
In the first-line treatment the patient abstained
from ergotamine and nicotine abuse
Intravenous infusion of prostaglandine was
administered
Rapid and complete improvement of
arteriospasm in-between 8 days was noted,
confirmed by further duplex examination
Case 5
Ergotamine 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 ergotaminealkaloid producing mushroom.
Because of the burning pain the
disease was called St. Anthony’s
fire
Case 5
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In this special case peripheral vasospasm was combined
with cardiac valve insufficiency of three valves.
This combination is very rarely reported in literature.
The question stays if aside of vasospastic complications
cardiac valve insufficiency can also be caused by
ergotamine abuse.
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1. Austin S, El-Hayek A, M Comoanos, D Tamulonis: Mitral Valve
Disease associated with Long-Term Ergotamine Use: Southern
Medical Journal 1993; 86 (10): 1179-81
5. Piquemal R, J Emmerich, J Guilmot, J. Fiessinger: Successful
Treatment for Ergotism with Iloprost. Angiology 1998; 49 (6):
493-7
13. Wilke A, H Hesse, G Hufnagel, B Maisch: Mitral, aortic and
tricuspid valvular disease associated with ergotamin therapy for
migraine. Eur Heart J. 1997; 18 (4): 701
Case 6
50 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 6
Soft tumor
No fibrosis
Stemmer´s sign negative
No pitting edema
Case 6
Multiple Café au lait maculae
Diagnosis:
Morbus Recklinghausen
(Neurofibromatosis Type 1)
Autosomal dominant
Family history negative
No neurological tumors
Case 7
11 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 7
3 years before first symptoms
bothsided dislocated mandibular
fracture after bycicle accident
Asymmetric face
X-ray: deformation of the left
mandible (ramus ascendens)
Case 7
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Frey-Syndrome (auriculotemporal syndrome) is
characterized by local sweating and reddening of the cheek
after eating. Most frequent reasons are parotis operations
or trauma.
Pathophysiology of Frey-Syndroms consists of aberrant
regeneration of parasympatisc nerve fibers. The
auriculotemporal nerve, a branch of the trigeminus nerve
innervates as well the glandula parotis with parasympathic
fibers as subcutaneous blood vessels and sweat glands with
sympathic fibers.
After trauma of the nerve the regeneration can lead to
parasympathic innervation of the blood vessels which was
meant for the parotis gland.
In this case Frey syndrome occured with reddening alone
and without sweating, a variation of the syndrome.
Case 8
Sclerotherpy
- complications and risks 
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Allergic reaction
Skin necrosis
Phlebitis
Pigmentation
Matting
Nerve damage
Visual disturbances
Migraine like neurological disturbances
Thromboembolic complications
ESAF Study
side effects
pain
hematoma
phlebitis
induration
pigmentation
itching
Metallic taste
Rabe et al. 2007, n = 108
liquid
n = 53
6
7
6
3
4
2
3
(%)
11
13
11
6
8
4
6
foam
n = 55
7
3
7
4
5
1
-
(%)
13
5
13
7
9
2
-
French Study (n= 12.173 sessions)
-thromboembolic complicationsComplication
Liquid
Foam
both
DVT (V. fem. sup. 6 ml foam in GSV)
0
1
0
Muscle vein thrombosis
0
2
0
Perforator thrombosis
0
3
0
Thrombophlebitis
0
3
0
Guex et al. Dermatol Surg 2005; 31: 123-128
Late-onset phlebitis
B. D.: female, 53 years old
07.03.2008: foam sclerotherapy
tributary left medial lower leg
1% polidocanol, 1:5, 2ml
no 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 phlebitis
phlebitis
tributary after sclerotherapy
Late-onset phlebitis
G. M.: female, 57 years old
09.04.2008: foam sclerotherapy of tributary varicose veins left thigh
1% and 0.5% polidocanol, 1:5, 4 ml, compression stocking
16.04.2008: all treated veins occluded, no side effects
28.04.2008: acute phlebitis of GSV at distal lower leg, duplex: GSV distally
occluded, no DVT. No history of phlebitis or DVT.
Late-onset phlebitis
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Phlebitis in a superficial vein
First symptoms 2 – 6 weeks after foam
sclerotherapy
Short segments
No clinical signs of phlebitis in between
Late-onset phlebitis
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Patients after successful foam sclerotherapy
No initial thromboembolic reaction
Phlebitis in surrounding veins after 2 – 6 weeks
Theory 1:
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Insufficient sclerosing effect
Progression of thrombus after an interval
Theory 2:
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Active foam has been kept enclosed in the treated and occluded vein.
After some weeks thrombolysis of thrombus material may occur and
minimal doses of still active polidocanol are set free.
These low amounts are not able to sclerose the vein but cause local
irritation of veins in the surroundings of the initially treated vessel.
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K.Parsi et al EJVES 2009; 38: 220-228: in vitro low concentrations of
polidocanol have procoagulant activities
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