Transcript Cutaneous Vascular Diseases, Part 1

Cutaneous Vascular Diseases,
Part 1
Dr. Bracciano
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.

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

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

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

TX of Raynaud’s
Disease/phenomenon
Treatment includes avoidance of the
aggravating factor, ie cold. (not just hands)
 Vasodilating drugs, nifedipine, 10-20 mg
tid; prazosin 1-3 mg tid
 Nitroglycerin 2% local application
 Sympathectomy in severe disease

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

Pathophysiology is poorly understood
 Responds to treatment of primary disorders
 Cold water immersion
 Serotonin antagonists

erythromelalgia

Livedo Reticularis



Mottled or reticulated
pink/reddish/blue
discoloration
Assoc c LE, DM,
scleroderma, RA
Side effect of
amantadine
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

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

Treatment
Low Dosage of Aspirin 325mg qd
 Nifedipine 10mg TID
 Pentoxifylline 400mg BID-TID

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

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

Purpura
May result from hyper or hypocoagulable
states, vascular dysfunction, and
extravascular causes
 Complete blood count
 PT and PTT
 Family history
 medications

Thrombocytopenic Purpura

Three Large Categories:
– Accelerated platelet destruction, immunologic
and nonimmunologic
– Deficient platelet production
– Unknown pathogenesis
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

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.

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

These are the kidneys from a case of idiopathic
thrombocytopenic purpura. Petechiae are found
throughout the renal parenchyma.
Drug-Induced Thrombocytopenia
Drug induced antiplatelet antibodies
 May be caused by sulfonamides, digoxin,
quinine, quinidine, PCN, furosemide,
Lidocaine, methyldopa
 Remove the offending agent
 corticosteroids

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

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.

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

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.

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

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

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

Waldenström's
Macroglobulinemia
Plasmapheresis until adequate dose of
chlorambucil is administered.
 Cyclophosphamide and corticosteroids are
treatment options as well

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.

Solar Purpura
Large, sharply outlined 1-5 cm dark
purplish red ecchymoses on dorsum of the
forearm
 Less frequently, back of the hand

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.

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

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

Disseminated Intravascular
Coagulation
All patient needs to receive vitamin K
replacement to exclude vitamin K
deficiency.
 FFP and platelets may be useful

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

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.

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.
CMV
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

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

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

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

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.

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.

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

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

calciphylaxis

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).
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 (vonKossa) identifies
calcium deposition
both within the
septum of the fat
lobule
Scorbutic Purpura
Bleeding gums
 Perifollicular purpura
 Corkscrew hairs
 Cutaneous purpura
 Deficiency in Vitamin C

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

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

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

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

Pigmentary Purpuric Eruption
Pigmented purpuric eruptions of the lower
extremities
 Similar histologic finding
 Schamberg's
 Majocchi's
 Gougerot-Blum

Schamberg Diseases
Aka progressive pigmentary dermatosis
 Grains of cayenne pepper
 Slow proximal extension
 Lesions seldom itch
 Favors lower shins and ankles

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.

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.

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

Histology

Purpura Majocchi-Schamberg is
characterized by slight alteration of
superficial capillaries with hemorrhage and
perivascular lymphocytic infiltrate.
Luckily, Dave's
computer was
equipped with an
airbag and he was
able to walk away
from this system
crash
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