Chapter 35 Cutaneous Vascular Diseases

Chapter 35 Cutaneous Vascular Diseases

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

Classification cutaneous small-vessel disease
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

Classification cutaneous small-vessel disease
Erythema elevatum diutinum Granuloma faciale Reactive Hansen’s disease Septic vasculitis

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

Large-vessel vasculitis
Giant-cell arteritis Takayasu’s arteritis

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

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

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

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

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

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

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

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

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

Subtypes of Small-Vessel Vasculitis

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

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

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

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

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

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

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

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

Urticarial vasculitis
Distinctive syndrome involving hypocomplementemia, arthritis, arthralgia, angioedema, abdominal or chest pain ,or both, and in some cases pulmonary and renal involvement

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

Underlying diseases may be associated Treatment options include those used to treat leukocytoclastic vasculitis Additionally plaquenil 200 mg bid

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 Most common skin eruptions include erythematous macules, erythematous papules, urticarial lesions and erythematous nodules

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

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

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

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

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 papules take on a doughy to firm consistency and develop red to purple

Most patients are asymptomatic; pruritis arthralgias and pain have been reported The prominence of eosinophils; the chronicity of the process, which results in perivascular dermal fibrosis; and the admixture of plasma cells and many lymphocytes are the hallmarks of EED TOC Dapsone

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

Granuloma faciale Intralesional corticosteroids Cryotherapy
Topical corticosteroids Dapsone, colchicine, antimalarials Topical PUVA and gold injections Dermabrasion, pulsed dye laser, electrosurgery, and cryosurgery

Granuloma faciale

Serum sickness A clinical syndrome resulting from circulating immune complexes that may occur after primary exposure to heterologous antisera or drugs Patient may develop fever, lymphadenopathy, arthralgia, proteinuria and skin lesions Urticarial or morbilliform, and tend to marginate along the demarcation of the palms and soles from the dorsa of the extremities (Wallace’s line)

Serum sickness Has been a complication of wasp venom immunotherapy, streptokinase therapy, and intravenous immune globulin , and with the use of antibiotics such as penicillin. Minocycline, rifampicin, cefprozil, and cefacor

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

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

Polyarteritis nodosa cutaneous 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

Polyarteritis nodosa internal 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

Polyarteritis nodosa laboratory 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%

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

Polyarteritis nodosa epidemiology
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

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

Polyarteritis nodosa treatment
Untreated classic PAN has a 5 year survival rate of 13% Death usually occurs from renal failure or cardiovascular or GI complications Tx with corticosteroids and cytotoxic agents has increased the survival rate to more than 90% cyclophosphamide

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

Wegener’s 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

Fever, weight loss and malaise occur m:f=1.3:1 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

The early detection of Wegener’s 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

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

Untreated WG has a mean survival time of 5 months and a90% mortality over 2 years Cyclophosphamide Trimethoprim-sulfamethoxazole

Allergic granulomatosis (Churg-Strauss syndrome)
AKA necrotizing angiitis with granulomata Characterized by distinctive initial manifestation of asthma A debilitated asthmatic begins after 2 to 12 years to experience attacks of fever and eosinophilia After a few more months or years, diffuse angiitis involves the lung, heart , liver, spleen , kidneys, intestine, and pancreas

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

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

Lethal midline granuloma
Clinical term describing those entities that produce progressive destructive ulcerations of the nose, sinuses, palate, or pharynx-the central part of the face First sign may be a chronic mucoid nasal discharge that later becomes purulent Eventually there is necrosis of the facial bones and base of the skull Patients are most often men 20-50yrs

Lethal midline granuloma
Three main histologic and clinical divisions 1. Lymphoma 2. Nonneoplastic granulomatous tissue reaction 3. Wegener’s granulomatosis Types 1 and 2 respond to radiation therapy

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 Horton’s disease Characterized by a necrotizing panarteritis with granulomas and giant cells

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

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

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

Giant-cell arteritis treatment
Prednisone Disease is quite responsive

Giant-cell arteritis

Takayasu’s 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

Takayasu’s 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

Malignant atrophic papulosis
Degos’ disease Potentially fatal obliterative arteritis syndrome Most frequently in men 20-40 Patients survive untreated an average of 2 years

Clinically it is characterized by the presence of pale rose, rounded, edematous papules Occurring mostly n 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

Wedge-shaped necrosis brought on by the occlusion of arterioles and small arteries account for the clinical lesions Etiology is unknown Inherited forms have been reported Corticosteroids have not proven beneficial Acetylsalicylic acid and persantine Anticoagulation therapy

Thromboangiitis obliterans (Buerger’s 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

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

A characteristic tapering of the arteries with “corkscrew” collateral circulation is found in Buerger’s disease Cessation of smoking Other forms of therapy are only palliative Serial amputations are often necessary

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

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

Treatment with bypass of the affected artery or sympathectomy or both

Mucocutaneous lymph node syndrome (Kawasaki’s 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

Mucocutaneous lymph node syndrome (Kawasaki’s disease) diagnosis
Six criteria have to be identified Fever Conjunctival congestion Oropharangeal lesion Hand and foot lesions An exanthem lymphadenopathy

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

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

Mucocutaneous lymph node syndrome (Kawasaki’s 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

Mucocutaneous lymph node syndrome (Kawasaki’s disease) treatment
IVGG is the cornerstone of treatment Antiplatelet therapy with aspirin is recommended

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

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

telangiectasia

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

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

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

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

Hereditary hemorrhagic telangiectasia (Osler’s 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

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

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

Hereditary hemorrhagic telangiectasia (Osler’s disease) treatment
Several methods have been recommended Epistaxis has been reduced by estrogen therapy Dermoplasty of the bleeding nasal septum Aminocaproic acid

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

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

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

Hereditary sclerosing poikiloderma and 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

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

Poikiloderma congenitale
AKA Thomson’s 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

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

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

Venous diseases of the extremities

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

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

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

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

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

Venous ulcer treatment
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

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

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

Venous ulcer Aspirin Oral zinc sulfate Stanazol grafting

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

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

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

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, Kaposi’s sarcoma and malignant lymphomas Infectious ulcers

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

lymphedema Primary lymphedema Congenital lymphedema (Milroy’s disease)
Lymphedema praecox Lymphedema tarda Syndromes assoc with primary lymphedema Yellow nail syndrome Turner’s syndrome Noonan’s syndrome Pes cavus Phakomatosis pigmentovascularis

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

lymphedema Secondary lymphedema Postradiation therapy
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

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

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

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

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

Nonne-Milroy-Meige Syndrome (hereditary lymphedema)
If long-standing a verrucous appearance to the affected extremity develops Treatment is difficult Pratt procedure Kondoleon operation

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

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

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

Secondary lymphedema

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

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

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

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

Factitial lymphedema When cause by blunt trauma to the hand or forearm it is referred to as Secretan’s syndrome and l’ oedeme bleu Effective care requires psychiatric intervention

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

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

The end

Chapter 35 Cutaneous Vascular Diseases

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