Pathology Associates Of Lexington, P.A.
Pathology Associates Of Lexington, P.A.
Pathology Associates Of Lexington, P.A.
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Vasculidities & vasculopathies
Cerebral & aortic anurysms are an important cause of sudden adult death. Arterial lesions tend to produce ischemic injury (painful areas...headaches), infarcts (causing abdominal pain, blood in stool, peripheral neuropathy, myalgias) and with organ damage (liver, pancreas, skeletal or myocardial muscle) causing elevated serum enzymes and/or other factors (ESR, CRP) of various types. Capillary, venule, and vein inflammation often leads to skin bleeding (purpura), blood in sputum (hemoptysis), blood in urine (hematuria), intestinal blood loss (hematochezia), and evidences of small bleedings elsewhere. Pathological diagnosis is more likely with larger, well-selected specimens (a thoracoscopic wedge biopsy including an area of hemorrhage is likely much better than blind, transbronchial lung biopsy). And, many entities are primarily cutaneous and fewer are primarily systemic & with some cutaneous manifestation. The classifications of the American College of Rheumatology 9research based) and also the Chapel Hill Consensus Conference give positive predictive value performance of from 17-29%. And, as in most other medical diseases (dermatoses, hepatitides, & colitides), there are morphologic & vessel-size overlap syndromes or situations. It may take expert skill to make the correct diagnosis; here is a link to the JohnS Hopkins Vasculitis Center, HERE.

Types of aneurysms and/or dissections:

  • degenerative: (the below 3 types of degeneration are often mixed together to various degrees7)
    1. age-related loss of elastica:
    2. atherosclerotic: usually hypertension associated [FA10-74].
    3. cystic medial necrosis:
  • traumatic:
  • inherited defects:
  • Marfans syndrome: often have pectus excavatum or protrusion, joint hypermobility, cardiac chordae elongation & floppy valves
  • autoimmune or connective tissue disorders:
    1. rheumatoid arthritic
  • infectious:
    1. syphilitic
Large-vessel vasculitis
  • embolic (clot; atheroemboli)
  • giant-cell (temporal or cranial) arteritis (granulomatous)
  • Takayasu arteritis (type I: aortic arch syndrome), types I-IV. (granulomatous)
  • syphilitic aortitis
  • tropical aortitis
  • sarcoidosis
  • Cojan's syndrome
  • Wiskott-Aldrich syndrome
  • non-luetic infectious aneurysms (salmonella, staph., enterococci).
  • atherosclerotic aortic aneurysm
  • inflammatory abdominal aortic aneurysm
Medium-sized vessel vasculitis (suspect this when no evidence of other than arterial involvement...negative for purpura, hemoptysis, hematuria)
  • temporal arteritis (like this, there can be intracranial arteritis of similar type causing pain, TIA, stroke).
  • polyarteritis nodosa (PAN)(negative for MCLN; negative lungs)
  • embolic vasculitis
  • Kawasaki disease (must have mucocutaneous lymph node syndrome [MCLN]): usual age 2 or less, unresponsive fever, bilateral nonsuppurative conjunctivitis, strawberry tongue, oropharyngeal injection/red, followed by red palms and soles, followed by desquamation...an acute vasculitis of unknown cause that occurs predominantly in infants and young children and produces coronary artery aneurysms in approximately 15% to 25% of those affected.
  • Behcet's disease
  • familial Medierranian fever
  • cutaneous polyarteritis nodosa
  • ANCA associated Wegener's granulomatosis, Churg-Strauss syndrome, microscpic polyangiitis
  • overlap syndromes of PAN, Churg-Strauss, and hypersensitivity vasculitis.
  • Buerger's disease (thromboangiitis obliterans): younger adults and almost exclusively cigarette smokers (but can also be associated with marijuana, affecting distal extremity arteries (finger & toe amputations) with thrombosis which may contain giant cells [? L12-15636 ?].
  • primary angiitis of the CNS
  • thromboangiitis obliterans (synonym for Buerger's)
  • MIVOD (mesenteric inflammatory veno-occlusive disease): 1st noted 1994; is a spectrum of venulitis to phlebitis which can be lymphocytic, necrotizing, granulomatous, or mixed & it has a thrombotic component; no arterial component and causes intestinal ischemia which can be surgical, even fatal; no medication or ANCA associations; one case with anti-phospholipid syndrome (APS).
  • vasculitis of various connective tissue diseases
  • isolated granulomatous phlebitis: arteries spared & colon perf. due to ischemic colitis [a variant of MIVOD?]
  • idiopathic enterocolic lymphocytic phlebitis: stenosing cecal mass with colon ischemia [a variant of MIVOD?]
  • lepromatous arteritis
  • Degos vasculopathy: very rare and ,early, has edematous intimal thickening in which one case had IgM & C3 complexes; then to medial thickening; then to stenosing mural stenosis (without inflammatory cells).
  1. Small-vessel vasculitis (DFA immuno-testing on biopsies can help) [check out a differential diagnosis table]
    • pauci-immune (few/no immune deposits) small-vessel vasculitis
      • Wegener's granulomatosis...Diff. Dx: granulomas and no asthma
      • microscopic polyangiitis (old terms, microscopic polyarteritis, microscopic periarteritis): an aggressive systemic necrotizing vasculitis with few or no immune deposits needing rapid diagnosis...key effects in kidneys (necrotizing and crescentic glomerulonephritis)  and lungs (hemorrhagic pulmonary capillaritis)...Diff. Dx: no asthma & no granulomas
      • Churg-Strauss (allergic angiitis and granulomatosis) Syndrome (CSS)...Diff. Dx group: asthmatics, granulomas and blood eosinophilia (ring granulomas around germinal centers, granulomas with central "eosinophilic abscesses"; eosinophilic vasculitis)
      • primary angiitis (granulomatous) of CNS (no skin lesions)
      • drug-induced ANCA-positive vasculitis
      • isolated retinal vasculitis
      • arteritis & venulitis secondary to other (such as intestinal-adhesional ischemic ulcerations causing some secondary granulomatous enteritis [vs. lumenal contents]) lesions: usually close (1-2 cm.) to the primary lesion and inflammatory character...even granulomatous...tends to reflect that of the primary lesion.
    • skin:
      • septic:
        1. clinical lesions: may be as red macules, petechiae, hemorrhagic vesicles & bullae, hemorrhagic crusts, and ulcers
        2. micro:
          • early lesions: superficial & deep perivascular & interstitial polys and lymphocytes
          • fully developed lesions: as with "early" but heavier; plus, thrombi; papillary dermal edema to vessiculation to necrosis.
          • late lesions: perivascular as with early except polys mostly interstitial; plus thrombi
      •   lymphocytic3:
        1. micro: often does not give much differential-diagnosis help except to bracket [LMC-03-409]
        2. diseases:
          • venular with typical lymphs:
            1. frost bite (chilblains/pernio)...50% of cases
            2. rickettsial lesions (epidermis can necrose)
            3. drug reactions
            4. allograft rejection
            5. idiopathic
            6. livedo vasculopathy
            7. Behcet's disease (?)
            8. collagen vascular disease (?)
            9. resolving leukocytoclastic vasculitis
            10. plus interface dermatitis:
            11. plus ballooning degeneration of keratinocytes:
            12. plus psoriasiform epidermal hyperplasia, spongiosis, or focal epidermal necrosis:
            13. plus extravascular necrosis:
            14. plus panniculitis:
          • venular with atypical lymphs:
            1. lymphomatoid papulosis
            2. mycosis fungoides (rarely)
            3. adult T-cell leukemia/lymphoma (rarely)
            4. disseminated pagetoid reticulosis (rarely)
            5. angiocentric lymphoma
            6. lymphomatoid drug eruptions (rarely)
          • arteriolar:
            1. Sneddon's syndrome
            2. Degos disease (Kohlmeier-Degos disease): very rare & predominantly skin vasculopathy manifested as "malignant atrophic papulosis" affecting skin, GI tract, and central nervous system, HERE.
      • leukocytoclastic (immune-complex small-vessel) vasculitis...angiitis(leukocytoclastic [LCV] or hypersensitivity vasculitis; palpable purpura): lesions tend to be in dependent locations due to hydrostatic pressure effect on damaged vessels; post-capillary venulitis is much more common than capillaritis and arteriolitis.[S-01-8683]
        • Henoch-Schonlein purpura (HSP) : IgA deposits dominate (usually children; palpable purpura, arthralgias, colicky GI symptoms, and glomerulonephritis)
        • acute generalized pustulosis: predilection for distal extremities; has leukocytoclastic vasculitis
        • cryoglobulinemic vasculitis: cryoproteins detectible in blood...may reflect hepatitis C, other infections, collagen vascular disease, arthralgia-purpura-weakness syndrome of Peetom-Melzer, and hematologic malignancies.
        • Bechet's disease: oral aphthous ulcers and skin vasculitis in absence of collagen vascular disease or IBD [S-01-4004?].
        • Goodpasture's syndrome (anti-GBM disease)
        • lupus vasculitis: ANA serological positivities (can develop a systemic vasculitis resembling CPN)
        • scleroderma: fibrinoid acute phase followed by obliterative sclerosis
        • dermatomyositis: vasculitis most common in childhood variant
        • mixed connective tissue disease (MCTD): similar to lupus, scleroderma, or MCTD.
        • rheumatoid vasculitis: usually in long-standing cases with high levels of RF (can vary from small vessel to arteritis)
        • serum sickness vasculitis
        • drug-induced immune-complex  serum-sickness-like vasculitis (old terminology, hypersensitivity vasculitis...which was usually in those over 20 years of age, and the acute phase of disease caused Ag-Ab complexes and reduced complement in serum...skin is usual site, with palpable purpura and leukocytoclastic-type of vasculitis acutely and lymphocytic type later). [S06-6687]
        • infection-induced immune-complex vasculitis (such as HIV or HCV) & HBV has produced cutaneous vasculitis, PAN, and cryoglobulinemic vasculitis also as secondary vasculitis.
        • urticaria, prolonged (>24 hour) lesions:
          • urticarial vasculitis (see peteciae, purpura, and/or siderotic hyperpigmentation in lesions):
            • hypocomplementemic (congenital or acquired) tend systemic sxs
            • normocomplementemic (congenital or acquired) urticarial vasculitis
          • late-phase (eosinophilic) urticaria5: non-LCV, non-fibrinonecrotic vasculitis (vasculocentric eos) in greater-than-24hour urticarial lesions.
          • late-phase (polys & eos) urticaria: non-LCV, non-fibrinonecrotic vasculitis (vasculocentric polys & eos) in greater-than-24-hour urticarial lesions.
        • erythema elevatum diutinum (EED)8: is a rare chronic form of fibrosing vasculitis. Clin = firm, red to brown or violaceous nodular lesions developing over joints of the elbows, knees, hands, and feet, often in a symmetric pattern. Associated with HIV/AIDS, hematologic disorders (IgA monoclonal gammopathy, multiple myeloma), celiac disease, Wegener’s granulomatosis, connective tissue disorders (especially systemic lupus erythematosus), and recurrent streptococcal infections. Dapsone is the first-line therapy for EED.
        • myelodysplastic syndromes (MDS)
        • hyperimmunoglobulin D
        • paraneoplastic or neoplasm-associated vasculitis (especially lymphomas...lymphomatoid granulomatosis)
        • ulcerative-colitis-associated (IBD) vasculitis
        • Crohn's-disease-associated (IBD) vasculitis
        • biliary-cirrhosis-associated vasculitis
        • retroperitoneal-fibrosis-associated vasculitis
  2. Vasculopathies:
    • arterial:
    • Heath-Edwards grading (for all [both primary and secondary pressure-induced change] hypertensive pulmonary vascular disease [HPVD] ) by H&E and elastic tissue stain4:
      1. Grade I: revert to double elastic layer of fetal pulmonary arterioles...reversible.
      2. Grade II: above plus addition of cellular intimal proliferation...reversible.
      3. Grade III: above plus addition of medial muscular thickening/hypertrophy...reversible [LMC-06-10582].
      4. Grade IV: above plus either fibrinoid mural change (may be preceeded by mural eos & polys) or angiomatoid or plexiform change...irreversible.
      • primary:
        1. primary pulmonary hypertension (PPH): Is diagnosed when secondary causes are ruled out; ll four grades of H-E change can be seen in PPH4.
        2. primary (idiopathic) arteriopathy of amyloidosis.
      • secondary:
        1. hypertensive pulmonary vascular disease (HPVD): a variety of pilmonary arterial pressure elevating diseases ...such as COPD & LV heart failure...cause grade I, II, & III changes, while grade IV change is rare in the absence of a left to right shunt4.
        2. diabetic arteriopathy.
        3. hypertensive arteriopathy.
        4. amyloidosis.
    • venous:
    • capillary:

REFERENCES:

  1. 20th Edition Cecil Textbook of Medicine.
  2. 4th Edition Dermatology in General Medicine.
  3. Carlson, Mihm, and LeBoit text.
  4. Personal communications with pulmonary and/or cardiovascular pathology experts (such as Jess Edwards who trained JBC).
  5. "late-phase urticaria" June 2007 update HERE.
  6. Diseases A to Z vasculitis file HERE.
  7. Dr. John B. Carter, personal communications.
  8. via Derm 101 website.

(posted 2000; latest minor update 12 June 2015)

 
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