Cutaneous Vascular Diseases, Part 1
Download
Report
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
See you next time…