Cutaneous Vascular Diseases

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Cutaneous Vascular Diseases

• JoAnne M. LaRow, DO
• June 7, 2004

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Vasculitis

• Clinicopathologic process characterized by
  inflammation and necrosis of blood vessels
• Blood vessel size is useful in classifying these
  disorders

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Classificationcutaneous small-vessel disease

• Idiopathic cutaneous small-vessel vasculitis
• Henoch-Schönlein purpura
• Acute hemorrhagic edema of infancy
• Urticarial vasculitis
• Essential mixed cryoglobulinemia

• Waldenströms hypergammaglobulinemic purpura
• Collagen vascular associated
• Rheumatoid nodules with vasculitis
• Hyperimmunoglobulinemia D syndrome
• Familial Mediterranean fever

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Classificationcutaneous small-vessel disease

• Erythema elevatum diutinum
• Granuloma faciale
• Reactive Hansens disease
• Septic vasculitis

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Medium-vessel necrotizing vasculitis

• Polyarteritis nodosa
• Benign cutaneous forms
• Systemic form (including microscopic variant)

• Granulomatous vasculitis
• Limited Wegeners granulomatosis
• Wegeners granulomatosis
• Allergic granulomatosis (Churg-Strauss)

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Large-vessel vasculitis

• Giant-cell arteritis
• Takayasus arteritis

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Cutaneous small-vessel vasculitis(leukocytoclasti
c vasculitis)

• Palpable purpura is the hallmark
• Pinpoint to several centimeters
• Early on lesion may not be palpable
• Papulonodular, vascular, bullous, pustular or
  ulcerated forms may develop
• Predominate on the ankles and lower legs
• Affect mainly dependent areas

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Cutaneous small-vessel vasculitis(leukocytoclasti
c vasculitis)

• Mild pruritis, fever, malaise, arthralgia and/or
  myalgia may occur
• Typically resolve in 3 to 4 weeks
• Residual postinflammatory hyperpigmentation may
  be seen
• Self-limiting
• May recur or become chronic
• Hemorrhagic vesicles or bullae may develop

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Cutaneous small-vessel vasculitis(leukocytoclasti
c vasculitis)

• Urticaria-like lesions are next most common
• They have less evanescence than ordinary hives
• Usually resolve after a few days
• Edema, especially of the ankles, is usually noted
• Arthralgias may be seen
• Major renal manifestation is glomerulonephritis
• May have gastrointestinal involvement

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histology

• Angiocentric segmental inflammation, endothelial
  cell swelling, fibrinoid necrosis of blood vessel
  walls and a cellular infiltrate composed of
  neutrophils showing fragmentation of nuclei

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pathogenesis

• Many forms of small-vessel vasculitis are felt to
  be caused by circulating immune complexes
• These lodge in vessel walls and activate
  compliment

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etiolology

• Types of antigens inducing immune complexes vary
• Some infectious agent and drugs are well defined

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Clinical evaluation

• Detailed history and physical examination
• History should focus on possible infectious
  disorders, prior associated diseases, drugs
  ingested, and a thorough review of systems
• CBC, strep throat culture or ASO titer, Hep B C
  serologies and ANA are a reasonable initial screen

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treatment

• Initial treatment should be nonaggressive
• Rest and elevation of the legs
• Analgesics, a good diet, and avoidance of trauma
  or cold
• Any identified antigen or drug should be
  eliminated

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• A variety of systemic treatments may be required
  for severe, intractable or recurrent disease
• For disease limited to the skin NSAIDs,
  antihistamines, colchicine and dapsone
• Systemic corticosteroids for those with systemic
  manifestations or necrotic lesions
• Immunosuppressive agents for rapidly progressive
  course and severe systemic involvement

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• A. classical purpuric papules papules on lower
  leg
• B. CSVV evolving to form confluent hemorrhagic
  plaque on posteroir ankle
• C. lesions in various stages of evolution

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Subtypes of Small-Vessel Vasculitis
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Henoch-Schönlein Purpura(HSP) (anaphylactoid
purpura)

• Characterized by intermittent purpura,
  arthralgia, abdominal pain, and renal disease
• Typically purpura appears on the extensor
  surfaces of the extremities
• Become hemorrhagic within a day and fades in 5
  days
• New crops appear over a few weeks

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Henoch-Schönlein purpura(HSP)

• Primarily occurs in male children
• Peak age 4-8 years
• Adults may be affected
• A viral infection or streptococcal pharyngitis
  are the usual triggering event
• In about 40 of the cases the cutaneous
  manifestations are preceded by mild fever,
  headache, joint symptoms, and abdominal pain for
  up to 2 weeks

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Henoch-Schönlein Purpura(HSP)

• May be pulmonary hemorrhage
• Abdominal pain and GI bleeding may occur at any
  time
• GI radiographs may show spiking or a marbled
  cobblestone appearance
• Renal manifestations may occur in 25 or more

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Henoch-Schönlein Purpura(HSP)

• The long-term prognosis in children with gross
  hematuria is very good however, progressive
  glomerular disease and renal failure may develop
  in a small percentage
• IgA, C3 and fibrin depositions have been
  demonstrated in biopsies of both involved and
  uninvolved skin by immunofluorescence techniques

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Henoch-Schönlein purpura(HSP)

• Treatment is supportive
• Duration of illness is typically 6 to 16 weeks
• Between 5 and 10 of patients will have
  persistent or recurrent disease
• Antispasmodics, antibiotics, and antiinflammatory
  drugs, including systemic corticosteroids
• Plamaphoresis in severe cases

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Acute Hemorrhagic edema of infancy

• AKA Finkelsteins disease, Seidlmayer syndrome,
  and purpura en cocarde avec oedema
• Affects children under the age of 2 with a recent
  history of an upper respiratory illness, a course
  of antibiotics of both
• Children are often nontoxic in appearance
• No extracutaneous involvement
• Spontaneously resolves with 1-3 weeks

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Acute Hemorrhagic edema of infancy

• Abrupt onset of large cockade, annular, or
  targetoid purpuric lesions involving the face,
  ears, and extremities
• Early in the course there may first be acral
  edema, may be nontender and asymmetrical
• Low-grade fever is common, and involvement of
  internal organ systems is rare
• Routine lab tests are nondiagnostic

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Acute Hemorrhagic edema of infancy

• Considered a variant of leukocytoclastic
  vasculitis with many similarities to HSP
• Spontaneous recovery within a few weeks
• DDX includes meningococcemia, HSP, erythema
  multiforme, urticaria and Kawasakis disease
• Clinically most urgent to exclude meningococcemia

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Acute Hemorrhagic edema of infancy
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• Multiple erythematous, nummular targetoid
  plaques on an infants thighs

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Urticarial vasculitis

• Recurrent episodes of painful, persistent
  urticaria /or angioedema
• May be associated with constitutional symptoms
  arthritis
• Pts with hypocomplementemia are more likely to
  have systemic involvement
• Associated disorders are autoimmune connective
  tissue dx viral infections

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Urticarial vasculitis

• Three clinical features distinguish the skin
  lesions of urticarial vasculitis from urticaria
• 1. Lesion are usually painful rather than
  pruritic
• 2. Lesions last longer than 24 hours
• 3. On healing there is postinflammatory
  hyperpigmentation

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Clinical features

• Similar in hypocomplementemic
  normocomplementemic variants
• Erythematous indurated wheals, angioedema, or
  macular erythema
• Extracutaneous manifestations include fever,
  malaise, lymphadenopathy, hepatosplenomegaly,
  arthralgias glomerulonephritis,
  hepatosplenomegaly, arthritis
  glomerulonephritis, GI ( nausea, vomiting,
  diarrhea, abdominal pain), respiratory (laryngeal
  edema, SOB, COPD), ocular (conjunctivitis,
  episcleritis, uveitis)-more common in
  hypocomplementemic variant

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• Several erythematous urticarial plaques on the
  foot ankle

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Urticarial vasculitis-tx

• Pts with hypocomplementemic UV respond to oral
  corticosteroids
• Hydroxychloroquine sulfate, colchicine, dapsone,
  NSAIDs or pentoxifylline
• Some pts require a combination of therapies with
  antihistamines

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Hyperimmunoglobulinemia D syndrome

• Characterized by recurrent high-spiking fevers
  with abdominal distress, diarrhea, vomiting,
  headache, and arthralgias
• Up to 79 of HID syndrome will have cutaneous
  findings
• Outside of neonatal period associated with
  papulopustules on face, scalp, cold abscesses,
  dermatitis, recurrent pneumonias with
  pneumatocele formation, osteopenia retention of
  deciduous teeth
• Genetic linkage on chromosome 4 has been reported

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Hyperimmunoglobulin E syndrome

• Multisystem immunologic disorder
• Vesicles or papulopustular eruption or recurrent
  pustules occurs early in infancy with crusting on
  face, scalp, neck upper torso
• Usually associated with high levels of IgE (over
  2000 IU/ml) eosinophilia
• Elevated levels of IgE may not be present in
  early infancy may fluctuate independent of
  severity of dx

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Hyperimmunoglobulinemia D syndrome

• Lymphadenopathy and splenomegaly are common
• Age of onset usually under 10 years
• Marked elevations in serum IgD are characteristic
• No preferred treatment
• Colchicine
• dapsone

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Familial Mediterranean fever

• A periodic fever syndrome that may be confused
  with HID syndrome
• Has been reported to affect Sephardic Jews,
  Armenians, and individuals of Arabian descent
• Onset usually under 10 years
• Cutaneous findings consist of erysipelas-like
  erythema showing a sharp border

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Familial Mediterranean fever

• Affects the lower extremities on the dorsa of the
  feet , over the ankles, and sometimes the knees
• Erysipelas-like erythema is considered
  characteristic, however this occurs in only 3 to
  46 of patients
• Arthralgias, peritonitis, and constipation may
  occur
• No lymphadenopathy, and no elevation of IgD

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Familial Mediterranean fever

• On skin biopsy there is most frequently
  leukocytoclastic vasculitis
• Defect at chromosome 16 polymorphic locus RT70
• Tx - colchicine

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Erythema elevatum diutinum

• A rare condition considered to be a chronic
  fibrosing leukocytoclastic vasculitis
• Classically multiple yellow papules develop over
  the joints, particularly the elbows, knees,
  hands, and feet
• May involve the buttocks and areas over the
  Achilles tendon
• With time the p
• Initially noduleas are soft mobile
• Papules take on a doughy to firm consistency and
  develop red to purple

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Erythema elevatum diutinum

• Symmetric, persistent, red-purple, red-brown, or
  yellowish papules, plaques or nodules
• Favor extensor surfaces of joints-especially
  hands knees, buttocks achilles tendon
• Mucous membranes usually spared
• Face ears usually affected
• Lesions may involute leave hyper-or
  hypopigmented atrophic scars
• Lesions may be painful, aching, burning, or
  asymptomatic
• Dx course is variablecan last 5-35 yrsperiods
  of waxing waningTOCDapsone

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Erythema elevatum diutinum
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histology

• Acute lesions show necrotizing LCV with
  neutrophils in upper mid-dermis may have
  prominent eos
• Chronic lesions show fibrosis, capillary
  proliferation, macrophage, plasma cell
  lymphocytic infiltrate
• Older lesions may have intracellular
  extracellular cholesterol deposits-giving the
  appearance of yellow xanthomas

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• Top multiple symmetric red-brown nodules
  plaques on extensor surface of digits
• Bottom red-brown palques nodules of concha,
  antihelix of the ear

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• Early stage dense perivascular infiltrate of
  neutrolphils admixed with lymphs histiocytes
• Late-stage minimal inflammatory infiltrate
  marked perivascular fibrous thickening

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Granuloma faciale

• Characterized by brownish-red, infiltrated
  papules, plaques, and nodules
• Involves facial areas, particularly the nose
• Typically healthy middle aged white men
• Pathology of GF is identical to EED

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Granuloma faciale

• Often resistant to tx
• Intralesional corticosteroids(first line
  non-scarring option
• Cryotherapy, dermabrasion, electrosurgery,
  surgical excision
• Topical corticosteroids
• Dapsone, colchicine, antimalarials
• Topical PUVA and gold injections

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Granuloma faciale
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• Red-brown plaque on the face
• Note prominent follicular openings

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• Left dense diffuse dermal infiltrate with a
  Grenz zone
• Right high power of polymorphous infiltrate of
  lymphocytes, eos, neuts plasma cells

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Polyarteritis nodosa

• Characterized by necrotizing vasculitis affecting
  the small and medium-sized muscular arteries of
  such caliber as the hepatic and coronary vessels
  and the arteries in the subcutaneous tissue, and
  sometimes adjacent veins
• Two major forms the benign cutaneous and the
  systemic

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Polyarteritis nodosa

• Microscopic polyangiitis is considered to be a
  subset of systemic PAN
• Segmental necrotizing and crescentic
  glomerulonephritis associated with extrarenal
  vasculitis involving small-size vessels without
  granulomas or asthma

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Polyarteritis nodosacutaneous manifestations

• Skin involvement in up to 40 of patients with
  systemic PAN
• Wide range of findings
• 15 5 to 10 mm subcutaneous nodules occurring
  singly of in groups distributed along the course
  of blood vessels
• Skin above is normal or slightly erythematous
• Often painful, may pulsate or ulcerate

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• Top petechiae multiple purpuric papules with
  central necrosis on plantar surface
• Bottom confluent hemorrhagic plaques on the
  medial plantar aspect of the foot

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Polyarteritis nodosainternal manifestations

• Classic systemic periarteritis may involve the
  vessels throughout the entire body
• Hypertension, tachycardia,fever, edema and weight
  loss are the cardinal signs of the disease
• Mononeuritis multiplex, most often manifested as
  foot drop, is the hallmark of PAN

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Polyarteritis nodosalaboratory findings

• Leukocytosis as high as 40,000 may occur with
  neutrophilia to 80
• Thrombocytosis and progressive normocytic anemia
  and elevated sed rate may be found
• Hypergammaglobulinemia with macroglobulins may be
  present
• Hepatitis C studies should be performed
• Urinary abnormalities seen in 70

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Polyarteritis nodosalab

• Patients with microscopic polyangiitis have
  positive titers for peripheral antimyeloperoxidase
  (P-ANCA) as opposed to the cytoplasmic (C-ANCA)
  form found in systemic PAN

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Polyarteritis nodosaepidemiology

• 4 X more common in men than women
• Mean age 45 yrs
• Seen in IV drug abusers and in assoc with SLE,
  inflammatory bowel disease, hairy cell leukemia,
  and familial Mediterranean fever

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Polyarteritis nodosapathology

• Histology is that of an inflammatory necrotizing
  and obliterative panarteritis that attacks the
  small and medium-sized arteries

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Polyarteritis nodosatreatment

• Untreated classic PAN has a 5 year survival rate
  of 13
• Death usually occurs from renal failure or
  cardiovascular or GI complications
• Tx with high-dose corticosteroids are initial TOC
• Cytotoxic agents such as may be
  addedcyclophosphamide

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Cutaneous polyarteritis nodosa

• Remarkable for an absence of visceral involvement
• Patients usually have recurrent skin, joint, and
  muscle involvement without involvement of vital
  organs
• Cutaneous findings similar to those described
  for the systemic form
• Most patient respond well to aspirin, prednisone,
  methotrexate, alone or in combination

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Wegeners granulomatosis

• Syndrome consisting of necrotizing granulomas of
  the upper and lower respiratory tract,
  generalized necrotizing angiitis affecting the
  medium-sized blood vessels, and focal necrotizing
  glomerulitis
• The commonest initial manifestation is the
  occurrence of rhinorrhea, severe sinusitis, and
  nasal mucosa ulcerations, with one or several
  nodules in the nose, larynx, trachea, or bronchi

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Wegeners granulomatosis

• Fever, weight loss and malaise occur
• mf1.31
• The strawberry gums appearance of hypertrophic
  gingivitis is characteristic
• Cutaneous findings occur in 45 of patients
• Nodules may appear in crops, especially along the
  extensor surface of the extremities
• Firm,slightly tender, flesh-colored or violaceous
  nodules may later ulcerate

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Wegeners granulomatosis

• The early detection of Wegeners granulomatosis
  has improved since the discovery of the assoc
  with C-ANCA
• Focal necrotizing glomerulitis occurs in 85 of
  patients
• Other organs frequently involved include the
  joints, eyes and CNS

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• Left purpuric plaques on the distal fingers
  Right ulceration of the tongue

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Wegeners granulomatosis

• Histologically the cutaneous lesions may
  demonstrate a leukocytoclastic vasculitis with or
  without granulomatous inflammation

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Wegeners granulomatosis

• Untreated WG has a mean survival time of 5 months
  and a 90 mortality over 2 years
• Prognosis improves when corticosteroids are
  combined with cytotoxic drugs-most commonly
  cyclophosphamide
• Combined tx results in marked resolution of
  symptoms in gt 90 of pts, a 75 remission rate,
  a 87 survival rate in pts between 6 months 24
  yrs

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Churg-Strauss Syndrome

• AKA allergic granulomatosis
• Asthma allergic rhinitis precede vasculitic
  phase
• Most commonly present with respiratory
  symptoms-asthma or cough or pneumonitis
• 3 clinical phases occur 1) prodrome asthma,
  allergic rhinitis, peripheral blood eosinoiphilis
  eosinophilic infiltrative dx 2.) vasculitis,
  characterized by arthritis, myositis cardiac,
  pulmonary, nervous system, GI, renal, ocular dx
  or genitourinary dx 3.) post-vasculitic
  phase-dominated by allergic rhinitis, asthma, HTN
  peripheral nerve damage

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CSS

• Cutaneous lesions are 1 of the most common
  features of the vasculitic phase (70)
• Skin lesions occur in 40-50 of pts
• Most common skin findings are palpavle purpura
  infiltrated nodules
• Less common are necrotizing livedo reticularis,
  migratory erythema, new onset Raynauds
  phenomenon digital ischemia, aseptic pustules
  or vesicles or infiltrated papules
• Extracutaneous manifestations wt loss, myalgias,
  arthralgias, mononeuritis multiplwex, GI
  symptoms, cardiomyopathy

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CSS

• A fatal outcome is most likely in untreated
  patients, with congestive heart failure resulting
  from myocarditis the most frequent cause of death
• Cutaneous lesions are present in 2/3 of patients
• Nodules may appear on the extensor surface of the
  extremities and on the scalp
• Firm nontender papules may be present on the
  fingertips
• Purpura and hemorrhagic bullae are present

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CSS

• Lab is significant for peripheral eosinophilia,
  which correlates with disease severity
• Frequently for P-ANCA and less frequently for
  C-ANCA, and tend to correlate with disease
  severity
• Cause is unknown
• Several drugs have been implicated in
  precipitating it
• Zafirlukast, Azithromycin, freebase cocaine
• Toc prednisone alone clinical remission in gt90
  of pts

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CSS

• Crusted, firm papules of the elbow

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Giant-cell arteritis

• Systemic disease of people over the age of 50
• Its best known location is in the temporal
  artery, where it is known as temporal arteritis,
  cranial arteritis, and Hortons disease
• Characterized by a necrotizing panarteritis with
  granulomas and giant cells

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Giant-cell arteritis

• Unilateral headache and exquisite tenderness in
  the scalp over the temporal arteries
• Fever, anemia, and a high sed rate are usually
  present
• Rarely fatal
• Has been shown to involve the vessels of the
  coronary arteries, breast, uterus, legs, abdomen
  and hand

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Giant-cell arteritis

• Cutaneous manifestations may only be inflammatory
• Affected artery becomes hard, pulsating, tender,
  tortuous bulge under red or cyanotic skin
• Another manifestation is gangrene of the scalp

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Giant-cell arteritis

• Polymyalgia rheumatica has a significant clinical
  association
• Prompt treatment may forestall serious disease
• ERS rates are elevated in more than 90 of pts
• Temporal artery biopsy is diagnostic
• MRI may be of value

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Giant-cell arteritistreatment

• Prednisone
• Disease is quite responsive

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Giant-cell arteritis
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Takayasus arteritis

• AKA aortic arch syndrome and pulseless disease
• Thromboobliterative process of the great vessels
  stemming from the aortic arch
• Generally occurs in young women
• Radial and carotid pulses are typically
  obliterated
• Skin changes are due to disturbed circulation

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Takayasus arteritis

• May be loss of hair and atrophy of the skin and
  its appendages, with underlying muscle atrophy
• Corticosteroids as in GCA
• Methotrexate
• With active medical and surgical intervention the
  aggressive course of this disease can be modified

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Malignant atrophic papulosis

• Degos disease
• Potentially fatal obliterative arteritis syndrome
• Rare vaso-occlusive disorder predominantly
  affects skin, GI tract CNS
• Cutaneous lesions begin as crops of small 2-5 mm
  erythematous papules on trunk or extremities
• These evolve over a 2-4 week interval with
  development of central depression ultimately a
  porcelain white scar, often a rim of
  telangiectasias an appearance similar to
  atrophie blanche
• Cutaneous findings typically preced systemic
  manifestations

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Malignant atrophic papulosis

• Occurring mostly on the trunk
• Similar lesions may occur on the bulbar
  conjunctiva and the oral mucosa
• Anemic infarcts involve the intestines
• Death is usually due to fulminating peritonitis
  caused by multiple perforations of the intestine
• No proven tx some cases respond to ASA with or
  without pentoxifylline

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Malignant atrophic papulosis

• Wedge-shaped necrosis brought on by the occlusion
  of arterioles and small arteries account for the
  clinical lesions
• A sparse perivascular lymphocytic infiltrate with
  an atrophic but slightly hyperkeratotic overlying
  epidermis
• Dermis usually is edematous with mucin deposits
  slight sclerosis occasionally necrosis
• Etiology is unknown
• Inherited forms have been reported

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Thromboangiitis obliterans(Buergers disease)

• An obliterative vascular disease affecting the
  medium and small sized arteries, especially those
  of the feet and hands
• Most often seen in men 20 to 40 who smoke heavily
• Vasomotor changes in early cases may be
  transitory or persistent,producing blanching,
  cyanosis, burning, and tingling

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Thromboangiitis obliterans(Buergers disease)

• Pain is a constant symptom, coming only at first
  after exercise and subsiding on resting
• Instep claudication is the classic complaint
• There is a strong association with cigarette
  smoking
• Exposure to cold and dampness may have etiologic
  importance
• arteriography should be done

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Thromboangiitis obliterans(Buergers disease)

• A characteristic tapering of the arteries with
  corkscrew collateral circulation is found in
  Buergers disease
• Cessation of smoking
• Other forms of therapy are only palliative
• Serial amputations are often necessary

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Thromboangiitis obliterans(Buergers disease)
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Arteriosclerosis obliterans

• An occlusive arterial disease most prominently
  affecting the abdominal aorta and the small an
  medium sized arteries of t he lower extremities
• Symptoms are due to ischemia of tissues
• Intermittent claudication manifest by pain,
  cramping, numbness, and fatigue in the muscle on
  exercise these are relieved by rest

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Arteriosclerosis obliterans

• May be rest pain at nighttime when in bed
• Impaired to absent pulses may be found on
  physical exam, confirming the diagnosis
• Feet, esp the toes, may be red and cold
• Diabetes and smoking play a role in the
  progression of the disease
• Claudication and diminished blood pressure in the
  affected extremity are findings that may lead to
  an earlier diagnosis and thus curative surgical
  intervention

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Arteriosclerosis obliterans

• Treatment with bypass of the affected artery or
  sympathectomy or both

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Mucocutaneous lymph node syndrome(Kawasakis
disease)

• Irritable, febrile infants and children (or
  rarely adults) with erythema multiforme-like,
  scarlatiniform, or morbilliform skin lesions
  accompanied by stomatitis, cheilitis, edema of
  the hands and feet, conjunctival congestion and
  cervical lymphadenitis
• Peak age at 6 months

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Mucocutaneous lymph node syndrome(Kawasakis
disease)diagnosis

• Six criteria have to be identified
• Fever
• Conjunctival congestion
• Oropharangeal lesion
• Hand and foot lesions
• An exanthem
• lymphadenopathy

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Mucocutaneous lymph node syndrome(Kawasakis
disease)

• To make the diagnosis a patient should have a
  fever above 38.3 C for 5 days plus 4 of the 5
  following criteria
• Peripheral extremity changes
• Polymorphous exanthem
• Nonpurulent bilateral conjunctival injection
• Changes in the lips and oral cavity
• Acute, nonpurulent cervical adenopathy

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Mucocutaneous lymph node syndrome(Kawasakis
disease)

• An early finding is the appearance of an
  erythematous , desquamating perianal eruption,
  usually within the first week of symptoms
• disease last 10 to 20 days the subsides
• 1 to 2 may die from MI

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Mucocutaneous lymph node syndrome(Kawasakis
disease)pathology

• Coronary arterial disease occurs and
  thrombocythemia may occur
• In combination vessel occlusion may occur and the
  subsequent MI, which occur as the child is
  recovering from the acute illness

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Mucocutaneous lymph node syndrome(Kawasakis
disease)treatment

• IVGG is the cornerstone of treatment
• Antiplatelet therapy with aspirin is recommended

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Mucocutaneous lymph node syndrome(Kawasakis
disease)
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• Diffuse erythematous macules papules on trunk
  confluent erythematous of axilla

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Telangiectasia

• Fine linear vessels coursing on the surface of
  the skin
• May occur in normal skin at any age
• Both sexes
• Anywhere on the skin and mucous membranes
• Prominent in areas of chronic actinic damage
• Nifedipine and felodipine

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Telangiectasia

• Seen in association with many conditions
• Altered capillary patterns on the fingernail
  folds are indicative of collagen vascular
  disease.
• In rosacea best treated with the tip of the
  epilating needle
• Sclerosing
• lasers

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Telangiectasia
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Generalized essential telangiectasia

• Characterized by the dilation of veins and
  capillaries over a large segment of the body
  without preceding or coexisting skin lesions

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Generalized essential telangiectasia

• Characteristic features include
• Widespread cutaneous involvement
• Progression or permanence of the lesions
• Accentuation by dependent positioning
• Absence of coexisting epidermal or dermal changes

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Generalized essential telangiectasia

• Develops most frequently in women in their 40s
  and 50s
• Initial onset is on the lower legs
• Spreads to the upper legs, abdomen, and arms
• Cause is unknown

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Generalized essential telangiectasia
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Unilateral nevoid telangiectasia

• Fine threadlike telangiectasia develop in a
  unilateral, sometimes dermatomal distribution
• Rare in men
• Tends to be assoc with increased levels of
  estrogen
• Has been assoc with Hep C
• The most commonly accepted theory is an increased
  level of estrogen receptors in involved skin

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Hereditary hemorrhagic telangiectasia(Oslers
disease)

• AKA Osler-Weber-Rendu disease
• Characterized by small tufts of dilated
  capillaries scattered over the mucous membranes
  and the skin
• Develop mostly on the lips, tongue, palate, nasal
  mucosa, ear, palms, fingertips, nailbeds and
  soles
• Frequent nose bleeds and melena are experienced

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Hereditary hemorrhagic telangiectasia(Oslers
disease)

• Epistaxis is the most frequent and persistent
  sign
• GI bleeding is the presenting sign in up to 25
  of cases
• Telangiectasias tend to increase in number in
  middle age, the first appearance on the
  undersurface of the tongue and floor of the mouth
  is at puberty

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Hereditary hemorrhagic telangiectasia(Oslers
disease)

• The disease is inherited as an autosomal dominant
  trait
• The vascular abnormalities found in HHT consists
  of direct arteriovenous connections without an
  intervening capillary bed
• Germline mutations in one of two different genes,
  endoglin or ALK-1, can cause HHT

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Hereditary hemorrhagic telangiectasia(Oslers
disease)treatment

• Several methods have been recommended
• Epistaxis has been reduced by estrogen therapy
• Dermoplasty of the bleeding nasal septum
• Aminocaproic acid

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Bloom syndrome(Bloom-Torre-Machacek syndrome)

• Transmitted as an autosomal recessive trait
• Characterized by telangiectatic erythema in the
  butterfly area of the face, photosensitivity and
  dwarfism
• Telangiectatic erythematous patches resembling
  lupus erythematosus develop in the first two
  years of life
• Exacerbation in summer months

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Bloom syndrome(Bloom-Torre-Machacek syndrome)

• The stunted growth is characterized by normal
  body proportions, no endocrine abnormalities, and
  low birth weight at full term
• The gene (BML) defect has been localized to
  15q26.1, resulting in a deficient ATP-dependent
  DNA-helicase activity

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Bloom syndrome(Bloom-Torre-Machacek syndrome)

• About ¼ patients under age 20 develop a neoplasm
• Regular use of sunscreen is recommended

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Bloom syndrome(Bloom-Torre-Machacek syndrome)
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Hereditary sclerosing poikilodermaand
mandibuloacral dysplasia

• Heritable, widespread poikilodermatous and
  sclerotic disorder
• Skin changes consist of generalized poikiloderma
  with hyperkeratotic and sclerotic cutaneous bands
  extending across the antecubital spaces, axillary
  vaults, and popliteal fossae
• The is no treatment

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Scleroatrophic syndrome of Huriez

• Characterized by
• Scleroatrophy
• Ridging or hypoplasia of the nails
• Lamellar keratoderma of the hands, and to a
  lesser extent, the soles
• The is a risk of development of aggressive
  cutaneous squamous cell carcinoma

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Poikiloderma congenitale

• AKA Thomsons disease and Rothmund-Thomson
  syndrome
• Autosomal recessive, rare
• Occurs predominantly in girls
• Begins at 3 to 6 months of age with tense, pink,
  edematous patches on the cheeks, hands, feet, and
  buttocks

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Poikiloderma congenitale

• Short stature, small hands, absence or sparseness
  of eyebrows and eyelashes, alopecia of the scalp,
  and congenital bone defects are frequently
  observed
• Sensitivity to sunlight
• SCC and BCC of the skin occasionally occur, and
  osteosarcoma of bone has been reported
• Patients have abnormal DNA helicase activity, as
  do patients with Werner and Bloom syndromes

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Leg ulcers

• Normal wound repair consists of three
  phases-inflammation, proliferation, and
  remodeling-that occur in a predictable sequence
• Abnormalities in any one of these components can
  produce delayed or ineffectual wound healing

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Venous diseases of the extremities
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Stasis dermatitis

• AKA dermatitis hemostatica and erythromelia
• A blotchy red mottling and a yellowish or light
  brown pigmentation of the lower 1/3 of the lower
  legs due to venous insufficiency
• Frequent finding in the elderly
• Often associated with obesity and other disease
  states

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Stasis dermatitis

• Approach to mgmt should be twofold
• Relief of symptoms
• Treatment of the underlying cause

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Venous ulcer

• AKA varicose ulcer, stasis ulcer
• Chronic venous insufficiency in the deep veins of
  the legs leads to shunting the venous return into
  the superficial veins, in which pressure, oxygen
  content, and flow rate are increased which result
  in dermatitis
• Edema and fibrosis develop and ulceration with
  minor trauma
• Varicose veins are usually present

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Venous ulcer

• Cause of varicose veins is multifactorial with
  inherited tendency
• Prolonged standing promotes the development
• Prolonged intraabdominal pressure contributes
• Venous ulcers usually occur on the lower medial
  aspect of the leg
• Usually there is a preceding stasis dermatitis
  with lipodermatosclerosis

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Venous ulcer

• In most cases the diagnosis of venous ulceration
  can be made on clinical grounds
• Biopsy may be necessary to exclude neoplasm

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Venous ulcertreatment

• Primarily to improve venous return
• Elevation of leg above heart
• Elastic support and exercise to improve calf
  muscle strength are recommended
• Avoidance of long cramped sitting, or prolonged
  standing is advisable
• Avoidance of trauma
• Compression therapy is the mainstay of tx

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Venous ulcer

• Occlusive permeable biosynthetic wound dressing
  have been shown very effective
• Cultured epidermal allografts
• Various skin substitutes
• In the acute setting, graded compression with
  Unna boots and graded elastic bandages
• Metronidazole
• Culture and chronic oral antibiotics may be
  required

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Venous ulcer

• Risk factors that predict failure to heal within
  24 weeks of limb-compression therapy include a
  large wound area, history of venous ligation or
  stripping, history of hip or knee replacement,
  ankle brachial index of less than 0.80, fibrin on
  50 or more of the wounds surface, and the
  presence of the ulcer for an extended time

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Venous ulcer

• Aspirin
• Oral zinc sulfate
• Stanazol
• grafting

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Ischemic ulcer

• Mostly located on the lateral surface of the
  ankle or digits
• Initial red, painful plaques breaks down into
  painful superficial ulcer with a surrounding zone
  of purpuric erythema
• Patients at risk are those e with long standing
  hypertension or other signs and risk factors of
  arteiosclerotic disease

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Ischemic ulcer

• Thinning of the skin, absence of the hair,
  decreased or absent pulses, pallor on elevation
  ,coolness of the extremity, dependent rubor,
  claudication on exercise, and pain on elevation
  relieved on dependency
• Diagnosis can be confirmed by exam and careful
  pulses in the legs

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Ischemic ulcer

• If ABI is less than 0.75 arterial insufficiency
  exists, less than 0.5 substantial insuff.
• Topical abx, protection from injury, avoidance or
  cold, smoking and tight socks
• Consult vascular surgeon
• Prevent infection
• Hyberbaric oxygen

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Leg ulcers of other causes

• Diabetic microangiopathy
• Hepatopoietic ulcers with sickle cell anemia
• Maximally treat underlying disease
• Leg ulcers from collagen vascular disease
• SCC, BCC, MM, Kaposis sarcoma and malignant
  lymphomas
• Infectious ulcers

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lymphedema

• Swelling of soft tissues in which an excess
  amount of lymph has accumulated
• Chronic lymphedema is characterized by
  long-standing nonpitting edema

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Lymphedema

• Primary lymphedema
• Congenital lymphedema (Milroys disease)
• Lymphedema praecox
• Lymphedema tarda
• Syndromes assoc with primary lymphedema
• Yellow nail syndrome
• Turners syndrome
• Noonans syndrome
• Pes cavus
• Phakomatosis pigmentovascularis

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Lymphedema

• Cutaneous disorders sometimes assoc with primary
  lymphedema
• Yellow nails
• Capillary hemangiomas
• Xanthomatosis and chylous lymphedema
• Congenital absence of nails

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Lymphedema

• Secondary lymphedema
• Postmastectomy lymphedema
• Melphalan lymphedema
• Melphalan isolated limb perfusion
• Malignant occlusion with obstruction
• Extrinsic pressure
• Factitial lymphedema

• Postradiation therapy
• Following recurrent lymphangitis/cellulitis
• Lymphedema of upper limb in recurrent eczema
• Granulomatous disease
• Rosaceous lymphedema
• Primary amyloidosis

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Lymphedema

• Most prevalent worldwide cause is filariasis
• In US most common cause is postsurgical

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Lymphedema praecox

• Develops in females between 9 and 25
• Puffiness appears around the ankles and then
  extends upwards
• Leg becomes painful, with a dull, heavy sensation
• Primary lymphedema is caused by a defect in the
  lymphatic system

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Lymphedema praecox

• Lymphangiography demonstrates
• Hypoplastic lymphatics in 87
• Aplasia in 5
• And hyperplasia with varicose dilation in 8
• Lymphedema distichiasis syndrome

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Nonne-Milroy-Meige Syndrome(hereditary
lymphedema)

• Characterized by unilateral or bilateral
  lymphedema present at birth and inherited as an
  autosomal dominant trait
• Edema in painless and pits on pressure
• Persists throughout life
• Not assoc with any other disorder
• Most frequently is unilateral

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Nonne-Milroy-Meige Syndrome(hereditary
lymphedema)

• If long-standing a verrucous appearance to the
  affected extremity develops
• Treatment is difficult
• Pratt procedure
• Kondoleon operation

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Primary lymphedema associated with yellow nails
and pleural effusion(Yellow Nail Syndrome)

• Primary lymphedema is confined mostly to the
  ankles, although other areas my be involved
• Nails show a distinct yellowish discoloration and
  thickening
• Recurrent pleural effusion requiring
  thoracocentesis may by a feature

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Phakomatosis pigmentovascularis

• Bielsa et al reported a pt with a generalized
  nevus spilus assoc. with a nevus anemicus and
  primary lymphedema
• Believed findings are not coincidental

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Secondary lymphedema

• In some malignant diseases involvement of the
  lymph nodes will produce blockage and lymphedema
• Frequently seen after mastectomy and the removal
  of axillary nodes

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Secondary lymphedema
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Postmastectomy lymphangiosarcoma(Stewart-Treves
syndrome)

• This type may arise in chronic postmastectomy
  lymphedema
• Lesions are bluish or reddish nodules arising on
  the arms
• Numerous localized lesions of lymphangiosarcoma
  may occur
• Pulmonary metastasis is frequent
• Prognosis is extremely poor

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• Note tumor mass behind the ear widespread
  purple discoloration elsewhere

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• Grouped papules on the edematous arm of a woman
  with Stewart-Treves syndrome

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• Infiltration of the dermis by ill-defined
  vascular spaces hyperchromatic, atypical
  endothelial cells

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Isolated limb perfusion

• Melphalan when used for treatment of malignancies
  with isolated limb perfusion has been reported to
  result in an increased chance of developing
  regional toxicity and lymphedema

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Inflammatory lymphedema

• The inflammatory reaction is caused by recurrent
  bouts of acute cellulitis and lymphangitis
• Chills, fever, and swelling and redness of the
  involved extremity are severe and may last for as
  long as 3 to 4 days
• Recurrent attacks of streptococcal infections
  increases the likelihood of lymphedema

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Factitial lymphedema

• AKA hysterical edema
• Lymphedema can be produced by wrapping an elastic
  bandage, cord, or shirt around an extremity
  and/or holding the extremity in a dependent and
  immobile state
• Self-inflicted causes are usually difficult to
  prove

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Factitial lymphedema

• When cause by blunt trauma to the hand or forearm
  it is referred to as Secretans syndrome and l
  oedeme bleu
• Effective care requires psychiatric intervention

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lymphedemaevaluation

• Diagnosis is usually base on a classic
  presentation
• In the early stages of the disease, may require
  further investigation

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treatment

• Most cases are treated conservatively
• Compression therapy, physical therapy, pneumatic
  pumps, and compressive garments
• Volume reducing surgery and lymphatic
  microsurgery are rarely performed
• Best to refer to a center versed in the treatment
  of these complicated conditions

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The end