1 / 172

Cutaneous Vascular Diseases

Cutaneous Vascular Diseases. JoAnne M. LaRow, DO June 7, 2004. Vasculitis. Clinicopathologic process characterized by inflammation and necrosis of blood vessels Blood vessel size is useful in classifying these disorders. Idiopathic cutaneous small-vessel vasculitis

josie
Download Presentation

Cutaneous Vascular Diseases

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript


  1. Cutaneous Vascular Diseases JoAnne M. LaRow, DO June 7, 2004

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

  3. Idiopathic cutaneous small-vessel vasculitis Henoch-Schönlein purpura Acute hemorrhagic edema of infancy Urticarial vasculitis Essential mixed cryoglobulinemia Waldenström’s hypergammaglobulinemic purpura Collagen vascular associated Rheumatoid nodules with vasculitis Hyperimmunoglobulinemia D syndrome Familial Mediterranean fever Classificationcutaneous small-vessel disease

  4. Erythema elevatum diutinum Granuloma faciale Reactive Hansen’s disease Septic vasculitis Classificationcutaneous small-vessel disease

  5. Polyarteritis nodosa Benign cutaneous forms Systemic form (including microscopic variant) Granulomatous vasculitis Limited Wegener’s granulomatosis Wegener’s granulomatosis Allergic granulomatosis (Churg-Strauss) Medium-vessel necrotizing vasculitis

  6. Large-vessel vasculitis • Giant-cell arteritis • Takayasu’s arteritis

  7. Cutaneous small-vessel vasculitis(leukocytoclastic 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

  8. Cutaneous small-vessel vasculitis(leukocytoclastic 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

  9. Cutaneous small-vessel vasculitis(leukocytoclastic 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

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

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

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

  13. 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

  14. 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

  15. 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

  16. 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

  17. Subtypes of Small-Vessel Vasculitis

  18. 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

  19. 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

  20. 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

  21. 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

  22. 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

  23. Acute Hemorrhagic edema of infancy • AKA Finkelstein’s 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

  24. 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

  25. 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 Kawasaki’s disease • Clinically most urgent to exclude meningococcemia

  26. Acute Hemorrhagic edema of infancy

  27. Multiple erythematous, nummular & targetoid plaques on an infant’s thighs

  28. 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

  29. 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

  30. 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

  31. Several erythematous urticarial plaques on the foot & ankle

  32. 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

  33. 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

  34. 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

  35. 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

  36. 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

  37. 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

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

  39. 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

  40. 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 variable;can last 5-35 yrs;periods of waxing & waning;TOC:Dapsone

  41. Erythema elevatum diutinum

  42. 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

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

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

  45. 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

  46. 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

More Related