Vascular, Lymphatic and Systemic Conditions

Venous Lakes

Venous lakes are benign vascular lesions that result from dilated venules. They commonly affect the lips, face, and ears. The photo above shows a venous lake on the lower lip of an elderly man.

Keratosis Pilaris Atrophicans

Keratosis pilaris atrophicans (KPA) is a group of inherited disorders with three subtypes including (a) keratosis pilaris atrophicans faciei (KPAF), (b) atrophoderma vermiculatum (AV), and (c) keratosis follicularis spinulosa decalvans (KFSD). KPAF and AV present mainly on the face with KFSD often appearing on the eyebrow and AV most commonly seen on the cheeks, sparing the eyebrows and scalp. KFSD can affect the face, scalp, and trunk. Inheritance pattern can be autosomal dominant (KPAF, AV), recessive (AV), or X-linked (KFSD). Here, a patient is emotionally bothered by persistent erythema from keratosis pilaris atrophicans.

Pernicious Anemia

Pernicious anemia is a disease where large, immature, nucleated cells (megaloblasts, which are forerunners of red blood cells) circulate in the blood, and do not function as blood cells; it is a disease caused by impaired uptake of vitamin B-12 due to the lack of intrinsic factor (IF) in the gastric mucosa. It was termed "pernicious" because before it was learned that vitamin B-12 could treat the anemia, most people that developed the disease died from it. In the image above, bone marrow aspirate is seen from a patient with untreated pernicious anemia. Megaloblastic maturation of erythroid precursors is shown. Two megaloblasts occupy the center of the slide with a megaloblastic normoblast above.

ali serrakh

Diabetes also affects the blood vessels and alters the flow of blood. Without adequate blood flow, it takes longer for a sore or cut to heal. Poor blood flow in the arms and legs is called "peripheral vascular disease." Peripheral vascular disease is a circulatory disorder that affects blood vessels away from the heart. If you have an infection that will not heal because of poor blood flow, you are at risk for developing ulcers or gangrene (the death of tissue due to a lack of blood).

Telangiectasias after Treatment

Post-treatment erythema immediately after intense pulsed light treatment.

Telangiectasias Before Treatment

Prominent facial telangiectasias prior to treatment with intense pulse light.

Cherry Angioma

Picture of cherry angioma, chest.

Vascular Malformations on Hand

Vascular malformations. These are congenital malformations that consist of capillary, venous, arterial, or lymphatic abnormalities. There are often combined alformations that comprise different types of vessels. Examples of vascular malformations include port-wine stains (capillary malformation), cystic hygroma (lymphatic malformation), and venous malformations. Vascular malformations are present at birth and grow proportionately with the child. Some vascular malformations may not manifest themselves until adolescence or adulthood. These figures represent venous malformations on the hand and foot.

Vascular Malformations on Foot

Vascular malformations. These are congenital malformations that consist of capillary, venous, arterial, or lymphatic abnormalities. There are often combined alformations that comprise different types of vessels. Examples of vascular malformations include port-wine stains (capillary malformation), cystic hygroma (lymphatic malformation), and venous malformations. Vascular malformations are present at birth and grow proportionately with the child. Some vascular malformations may not manifest themselves until adolescence or adulthood. These figures represent venous malformations on the hand and foot.

Necrobiosis Lipoidica Diabeticorum

Necrobiosis lipoidica diabeticorum: A dull red raised area on the skin that evolves into a shiny scar with a violet border, most often on the shin. There is telangiectasia with blood vessels easily visible under the skin. The area be itchy and painful and crack open. Treatment may include whirlpool therapy, occlusive dressings, topical steroids, aspirin, and pentoxifylline. Necrobiosis lipoidica diabeticorum (NLD) is a degenerative disease of the connective tissue in the skin. More than half of people with NLD have diabetes. Hence the name. Also known as necrobiosis lipoidica and as necrobiosis.

Twin-to-Twin Transfusion

Twin-to-twin “transfusion”. Twins who develop with some form of common circulation in utero may show a temporary difference of cutaneous color related to oddities of hemodynamics. In the figure, one sees twin neonates, of whom the one on the right is uniformly and abnormally erythematous, whereas the other is abnormally pallid. The one looks plethoric, the other, anemic. In the course of time, restoration of normal blood counts and color will develop in both.

Spider Angioma

Spider angioma. Vascular papule with radiating arterioles on the cheek of a child.

Salmon Patch on Newborn

Salmon patch. Salmon patch on the glabella of a newborn.

Pyogenic Granuloma (Chin)

Pyogenic Granuloma (PG) can be regarded as a benign vascular tumor or as a reactive vascular process arising at sites of previous trauma or irritation. PG is also known as lobular capillary hemangioma, granuloma telangiectaticum, and granuloma gravidarum when predenting on the gingiva of pregnant women. It commonly occurs in areas of trauma including the face and fingers. Pyogenic granuloma overlying a dermal nevus.

Pyogenic Granuloma (Hand)

Pyogenic Granuloma (PG) can be regarded as a benign vascular tumor or as a reactive vascular process arising at sites of previous trauma or irritation. PG is also known as lobular capillary hemangioma, granuloma telangiectaticum, and granuloma gravidarum when predenting on the gingiva of pregnant women. It commonly occurs in areas of trauma including the face and fingers. Shown is pyogenic granuloma on a hand.

Pyogenic Granuloma

Pyogenic granuloma. Sudden appearance of a vascular nodule on the cheek of an infant.

Meningococcemia

Meningococcemia. Stellate necrotic areas on the legs of a child with meningococcemia.

Klippel-Trénaunay-Weber Syndrome

Klippel-Trénaunay-Weber syndrome. This is a condition in which vascular malformations, varicosities, and phlebectasia cover an entire limb or other body area. There may be associated skeletal abnormalities including macrodactyly and syndactyly. A combination of port-wine stain and vascular malformations may be present from birth. The osteohypertrophy develops during the first several years of life. These figures are the front and back views of an infant who has the beginning of the angiomatous process. At this stage, the condition appears almost like a nevus flammeus. Over time, more massive vascularization and enlargement of the limb will develop. Meta Description : Picture of Klippel-Trénaunay-Weber syndrome. This is a condition in which vascular malformations, varicosities, and phlebectasia cover an entire limb or other body area. There may be associated skeletal abnormalities including macrodactyly and syndactyly. A combination of port-wine stain and vascular malformations may be present from birth. The osteohypertrophy develops during the first several years of life. These figures are the front and back views of an infant who has the beginning of the angiomatous process. At this stage, the condition appears almost like a nevus flammeus. Over time, more massive vascularization and enlargement of the limb will develop.

Kawasaki's Disease

Kawasaki's disease. Blotchy erythema on the trunk of a child with Kawasaki's disease.

Hereditary Hemorrhagic Telangiectasia on Eye

Hereditary hemorrhagic telangiectasia. Telangiectases on the bulbar conjuctiva.

Henoch-Schonlein Purpura

Henoch-Schonlein purpura. Hemorrhagic macules, papules, and urticarial lesions on the foot of a child with Henoch-Schonlein purpura. Henoch-Schonlein purpura (HSP) is a form of blood vessel inflammation or vasculitis. HSP occurs most commonly in children, but people of all age groups can be affected. Classically, HSP causes skin rash, pain in the abdomen, and joint inflammation (arthritis). The rash of skin lesions appears in gravity-dependent areas, such as the legs. The joints most frequently affected with pain and swelling are the ankles and the knees. The treatment of HSP is directed toward the most significant area of involvement. Joint pain can be relieved by antiinflammatory medications such as aspirin or ibuprofen (Motrin).

Erythema Nodosum (Leg)

Erythema nodosum. These are tender, red nodules on the pretibial area of an adolescent. Erythema nodosum is a type of skin inflammation that is located in a certain portion of the fatty layer of skin. Erythema nodosum (also called EN) results in reddish, painful, tender lumps most commonly located in the front of the legs below the knees. The tender lumps, or nodules, of erythema nodosum range in size from 1 to 5 centimeters. The nodular swelling is caused by a special pattern of inflammation in the fatty layer of skin. Erythema nodosum can be self-limited and resolve on its own in three to six weeks. Upon resolution, it may leave only a temporary bruised appearance or leave a chronic indentation in the skin where the fatty layer has been injured.

Facial Telangiectasias

Facial telangiectasias are dilated vessels appearing superficially in the dermis mostly on the alae nasi. Telangiectasias are also common in scars and various skin lesions. Shown here is a middle-aged male with multiple facial telangiectasias.

Cutis Marmorata Telangiectatica Congenita

Cutis marmorata telangiectatica congenita. Vascular lesions resolve with depressed scars.

Ataxia Telangiectasia (Legs)

Ataxia telangiectasia. Hirsutism on the lower legs and ecchymoses secondary to ataxia and numerous falls. Ataxia telangiectasia is a progressive neurodegenerative genetic disease characterized by cerebellar ataxia (incoordination and lack of balance), ocular telangiectasia ("red eyes" due to widening of small blood vessels in the conjunctiva), immune defects, and a predisposition to malignancy. A-T becomes evident in early childhood, usually in the first decade of life. The hallmarks of A-T are lack of balance and slurred speech (due to the ataxia) and telangiectasias (tiny red "spider" veins), which appear in the whites of the eyes or on the surface of the ears and cheeks. People with A-T are predisposed to leukemia and lymphoma. They are also extremely sensitive to radiation exposure. Most people with A-T have a defective immune system, making them susceptible to recurrent sinus and respiratory infections. Other features of the disease may include diabetes mellitus, premature graying of the hair, difficulty swallowing (which causes choking and drooling), and slowed growth. Children with A-T usually have and maintain normal or above normal intelligence. There is no cure for A-T and, currently, there is no known therapy to slow the progression of the disease. Treatment is symptomatic and supportive.

Ataxia Telangiectasia (Ear)

There are telangiectasias on the ear of this child. Ataxia telangiectasia is a progressive neurodegenerative genetic disease characterized by cerebellar ataxia (incoordination and lack of balance), ocular telangiectasia ("red eyes" due to widening of small blood vessels in the conjunctiva), immune defects, and a predisposition to malignancy. A-T becomes evident in early childhood, usually in the first decade of life. The hallmarks of A-T are lack of balance and slurred speech (due to the ataxia) and telangiectasias (tiny red "spider" veins), which appear in the whites of the eyes or on the surface of the ears and cheeks. People with A-T are predisposed to leukemia and lymphoma. They are also extremely sensitive to radiation exposure. Most people with A-T have a defective immune system, making them susceptible to recurrent sinus and respiratory infections. Other features of the disease may include diabetes mellitus, premature graying of the hair, difficulty swallowing (which causes choking and drooling), and slowed growth. Children with A-T usually have and maintain normal or above normal intelligence. There is no cure for A-T and, currently, there is no known therapy to slow the progression of the disease. Treatment is symptomatic and supportive.

Ataxia Telangiectasia

Ataxia-telangiectasia: A progressive neurodegenerative genetic disease characterized by cerebellar ataxia (incoordination and lack of balance), ocular telangiectasia ("red eyes" due to widening of small blood vessels in the conjunctiva), immune defects, and a predisposition to malignancy. Chromosomal breakage is a feature. Ataxia-telangiectasia (A-T) cells are abnormally sensitive to killing by ionizing radiation.

Angiokeratoma

Angiokeratomas are telangiectasias with keratotic elements. They present in different clinical scenarios including (a) solitary or multiple angiokeratomas occurring predominantly on lower extremities; (b) angiokeratoma of Fordyce affecting the scrotum and the vulva; (c) angiokeratoma of Mibelli, an autosomal dominant disorder affecting dorsum of hands and feet, elbows, and knees;(d) angiokeratoma corporis diffusum associated with Fabry’s disease, an X-linked recessive disorder characterized by a-galactosidase-A deficiency and affecting the lower abdomen, buttocks, and genitalia; and (e) angiokeratoma circumscriptum usually grouped on one extremity. In this photo is an angiokeratoma on the thigh of a young child.

Angiokeratoma Circumscriptum

Angiokeratoma circumscriptum. This vascular ectasia may be present at birth but has been reported to develop in childhood and even adulthood. There is a higher incidence in females. Lesions are most commonly found on the lower extremities but may be found elsewhere. Lesions consist of small nodules or larger plaques characterized by a dark red to purple color and a warty, hyperkeratotic scale sometimes in a linear distribution. These lesions may bleed when traumatized.

Urticaria

Urticaria. This is a close-up view of wheals with white-to-light-pink color centrally and peripheral erythema. These are the classic lesions of hives, or urticaria. Some hives are caused by allergies to such things as foods, medications, and insect stings, but the large majority of cases are not allergic, and no specific cause for them is ever found. It is characteristic that they are transient and highly pruritic. The goal of treating most cases of ordinary urticaria is to relieve symptoms while the condition goes away by itself.

Erythema Nodosum

Erythema nodosum: An inflammatory reaction deep in the skin characterized by the presence of tender red lumps or nodules ranging in size from 1 to 5 centimeters most commonly located over the shins but occasionally involving the arms or other areas. The causes of erythema nodosum include medications (sulfa-related drugs, birth control pills, estrogens, iodides and bromides), strep throat, cat scratch disease, fungal diseases, infectious mononucleosis, sarcoidosis, Behcet's disease, inflammatory bowel disease (Crohn's disease and ulcerative colitis), and normal pregnancy. In many cases, no cause can be determined. Erythema nodosum may be self-limited and go away on its own in 3 to 6 weeks. If treatment is needed, the underlying condition is treated and simultaneously treatment is directed toward the erythema nodosum. This can include antiinflammatory drugs and cortisone by mouth or injection. Colchicine is sometime used effectively to reduce inflammation.

Polyarteritis Nodosa

Polyarteritis nodosa is an autoimmune disease (immune system attacking its own body) characterized by spontaneous inflammation of the arteries (arteritis) of the body. Because arteries are involved, the disease can affect any organ of the body, most commonly muscles, joints, intestines, nerves, kidneys, and skin. Treatment is directed toward decreasing the inflammation of the arteries by suppressing the immune system. Medications used to treat polyarteritis nodosa include high-dose intravenous and oral cortisone medications such as prednisone, as well as immunosuppressive drugs such as cyclophosphamide (Cytoxan) or azathioprine (Imuran).

Dermatomyositis

Dermatomyositis: A chronic inflammatory disease of skin and muscle which is associated with patches of slightly raised reddish or scaly rash. The rash can be on the bridge of the nose, around the eyes, or on sun-exposed areas of the neck and chest. Classically, however, it is over the knuckles. When the characteristic inflammation of the muscle (myositis) occurs without skin disease, the condition is referred to as polymyositis. Dermatomyositis is one of a group of acquired muscle diseases called inflammatory myopathies. The disease has a subacute (somewhat short and relatively severe) onset. It affects both children and adults. Females are more often affected than males. Dermatomyositis is characterized by a rash accompanying, or more often, preceding muscle weakness. The most common symptom is muscle weakness, usually affecting those muscles that are closest to the trunk of the body (proximal). Eventually, patients have difficulty rising from a sitting position, climbing stairs, lifting objects, or reaching overhead. In some cases, distal muscles (those not close to the trunk of the body) may be affected later in the course of the disease. Trouble with swallowing (dysphagia) may occur. Occasionally, the muscles ache and are tender to touch. Some patients develop hardened bumps of calcium deposits under the skin. Patients may also feel fatigue and discomfort and have weight loss or a low-grade fever. Treatment commonly involves a steroid drug called prednisone. For patients in whom prednisone is not effective, other immunosuppressants such as azathioprine and methotrexate may be prescribed. Recently, a drug called intravenous immunoglobulin was shown to be effective and safe in the treatment of the disease. Physical therapy is usually recommended to preserve muscle function and avoid muscle atrophy. Most cases of dermatomyositis respond to therapy. The disease is usually more severe and resistant to therapy in patients with cardiac or pulmonary problems. Both polymyositis and dermatomyositis can sometimes be associated with cancers, including lymphoma, breast, lung, ovarian, and colon cancer. The cancer risk is reported to be much greater with dermatomyositis than polymyositis. (See polymyositis).

Hereditary Hemorrhagic Telangiectasia

Hereditary hemorrhagic telangiectasia: Abbreviated HHT. A genetic disease characterized by the presence of arteriovenous malformations (AVMs) which involve direct connections between arteries and veins without the usual intervening capillaries. These AVMs can vary in size from a pinhead to a pea. The tiniest AVMs are called telangiectases. Those telangiectases that are close to the surface of skin and mucous membranes tend to rupture easily and bleed after even slight trauma. Recurrent nosebleeds are common, particularly at night, beginning at about the age of 12, due to the rupture of telangiectases within the nose. Large AVMs can bleed in the gastrointestinal (GI) tract, brain, spine, lung, liver and other sites and create major, sometimes life-threatening, problems. HHT is inherited as an autosomal dominant trait. Most patients with HHT have a parent with the disease. Every child born to a person with HHT has a 50% risk of inheriting their affected parent's mutation and having the disease. HHT can be caused by mutation in one of two different genes: the endoglin gene (ENG) on chromosome 9 or the activin receptor gene (ACTRL1) on chromosome 12. HHT has been genetically subdivided into HHT1 (due to ENG mutation) and HHT2 (due to ACTRL1 mutation). Some families with HHT may also have mutations in another, as yet unidentified, gene. HHT is also known as Osler-Rendu-Weber and Rendu- Osler-Weber disease or syndrome. Although his name was never given to the disorder, it was first described in 1865 by the British physician Benjamin Guy Babbington (who also invented the laryngoscope).

Lymphangioma

The term lymphatic malformation is the new terminology for what was formerly called “lymphangioma.” These typical lesions comprise multiple, grouped, small macroscopic vesicles filled with clear or serosanguineous fluid (“frog-spawn”). However, these are not true vesicles but microcystic lesions (lymphangioma) as opposed to a macrocystic lesion (cystic hygroma), which is located deep in the dermis and subcutis and appears as a large soft subcutaneous tumor often distorting the face or an extremity. The microcystic LM is present at birth or appears in infancy or even in childhood. It does not disappear spontaneously. Bacterial infection may occur. LM may occur as an isolated solitary lesion, or cover large areas (up to 10 20 cm); it may be associated with a capillary venous lymphatic (CVL) malformation. The lesion can be excised, if feasible, or treated with sclerotherapy.

Hemangioma After Laser Treatment

There is a marked lightening and flattening of the hemangioma after multiple pulsed dye laser treatments. Hemangiomas are caused by many tiny blood vessels bunched together and vary in severity. Typically, this birthmark can be just that, a mark, or it can grow larger and larger until treated. Hemangiomas can grow very rapidly through the first year of a child's life. Most hemangiomas will go away on their own; roughly 50% resolve by age five, 70% by age seven and 90% by age nine. Reasons to treat hemangioma include problems with functions (such as sight, eating, hearing or defecation), ulceration or pain. Hemangiomas can be treated in different ways, each of which carries its own risks. Corticosteroid medication, which can be injected or taken orally, is one option for treating hemangiomas. Risks associated with corticosteroid medication include high blood pressure, high blood sugar, poor growth, or cataracts. If corticosteroids fail, there are other medications that may be an option. Certain hemangiomas can also be treated with lasers to stop them from growing. Risks associated with that treatment include ulceration and scarring. In some cases, a hemangioma can also be removed with surgery. Other times, a combination of these approaches is the most beneficial treatment.

Hemangioma of Infancy (HI)

Formerly known as strawberry, cherry, capillary hemangioma. HI is the most common tumor of infancy. Hemangiomas of infancy are not present at birth but appear postnatally; grow rapidly during the first year (proliferating phase), undergo slow spontaneous regression during childhood (involution phase), and remain stable thereafter. This bright red nodular plaque is frightening to the parents, and caution is needed to prevent scarring from the treatment itself. Since most of these lesions disappear spontaneously with only 20% showing residual atrophy or depigmentation, a wait and see strategy is recommended.

Hemangioma 2

This left upper eyelid hemangioma, though in its early growth phase, is a lesion that may threaten the child's vision. Hemangiomas are caused by many tiny blood vessels bunched together and vary in severity. Typically, this birthmark can be just that, a mark, or it can grow larger and larger until treated. Hemangiomas can grow very rapidly through the first year of a child's life. Most hemangiomas will go away on their own; roughly 50% resolve by age five, 70% by age seven and 90% by age nine. Reasons to treat hemangioma include problems with functions (such as sight, eating, hearing or defecation), ulceration or pain. Hemangiomas can be treated in different ways, each of which carries its own risks. Corticosteroid medication, which can be injected or taken orally, is one option for treating hemangiomas. Risks associated with corticosteroid medication include high blood pressure, high blood sugar, poor growth, or cataracts. If corticosteroids fail, there are other medications that may be an option. Certain hemangiomas can also be treated with lasers to stop them from growing. Risks associated with that treatment include ulceration and scarring. In some cases, a hemangioma can also be removed with surgery. Other times, a combination of these approaches is the most beneficial treatment.

Hemangioma 1

Hemangioma: A birth irregularity where a localized tissue mass grows rich in small blood vessels. Capillary hemangiomas are composed nearly entirely of tiny capillary vessels. Cavernous hemangiomas are composed of blood- filled "lakes" and channels. An hemangioma may be visible through the skin as a birthmark, known colloquially as a "strawberry mark." Most hemangiomas that occur at birth disappear after a few months or years.

Lymphedema

Lymphedema: A common chronic, debilitating condition in which excess fluid called lymph collects in tissues and causes swelling (edema) in them. Lymphedema (edema due to lymphatic fluid) may occur in the arms or legs. This often happens after lymph vessels or lymph nodes in the axilla (armpit) or groin are removed by surgery or damaged by radiation, impairing the normal drainage of lymphatic fluid. Lymphedema may also due to a mass such as a tumor pressing on the lymphatic vessels. Congenital lymphedema: In many other cases, lymphedema is evident at birth and is due to a congenital malformation (that is, a birth defect) of the lymphatic system. Congenital lymphedema can be found associated with the Noonan and Turner syndromes and a number of forms of lymphedema are clearly due to genetic factors. Genetic factors in lymphedema: Hereditary lymphedema is heterogeneous and usually occurs as an autosomal dominant trait. The most common form of congenital primary hereditary lymphedema is Milroy disease which is sometimes caused by mutations (changes) in the vascular endothelial growth factor receptor-3 gene (VEGFR-3, or FLT4) The lymphedema-distichiasis (LD) syndrome is a less common cause of hereditary lymphedema. In LD there is lymphedema (primarily of the limbs, with variable age at onset) together with distichiasis, (double rows of eyelashes). There may also be heart defects, cleft palate, extradural cysts, and photophobia. A gene for LD was mapped by linkage studies to chromosome band 16q24.3. In families with LD, mutations have been found in the FOXC2 (MFH-1) gene. FOXC2 is a member of the forkhead/winged-helix family of transcription factors, whose members are involved in diverse developmental pathways. (Fang et al. Am J Hum Genet 67:1382-1388, 2000)

Livedo Reticularis

Livedo reticularis: A mottled purplish discoloration of the skin. Livedo reticularis can be a normal condition that is simply more obvious when a person is exposed to the cold. Livedo reticularis can also be an indicator of impaired circulation. Livedo reticularis has been reported in association with: Autoimmune diseases such as systemic lupus erythematosus. Abnormal antibodies referred to as phospholipid antibodies. Examples of these antibodies are the cardiolipin antibody and falsely positive testing for syphilis. A syndrome featuring phospholipid antibodies with multiple brain strokes. It is felt that the blood of these patients has a propensity to clot which may predispose to stroke.

Acute Systemic Lupus

Acute systemic lupus erythematosus. Bright red, sharply defined erythema with slight edema and minimal scaling in a “butterfly pattern” on the face. This is the typical “malar rash.” Note also that the patient is female and young.

Systemic Lupus Erythematosus 2

Systemic lupus erythematosus. Erythematous, edematous plaques appear in a "butterfly" distribution on the face. Lupus is an autoimmune disease characterized by acute and chronic inflammation of various tissues of the body. Common complaints and symptoms include fatigue, low-grade fever, loss of appetite, muscle aches, arthritis, ulcers of the mouth and nose, facial rash ("butterfly rash"), unusual sensitivity to sunlight (photosensitivity), inflammation of the lining that surrounds the lungs (pleuritis) and the heart (pericarditis), and poor circulation to the fingers and toes with cold exposure (Raynaud's phenomenon). Complications of organ involvement can lead to further symptoms that depend on the organ affected and severity of the disease. There is no permanent cure for SLE. The goal of treatment is to relieve symptoms and protect organs by decreasing inflammation and/or the level of autoimmune activity in the body.

Systemic Lupus Erythematosus 1

Systemic lupus erythematosus: A chronic inflammatory condition caused by an autoimmune disease. An autoimmune disease occurs when the body's tissues are attacked by its own immune system. Patients with lupus have unusual antibodies in their blood that are targeted against their own body tissues. Lupus can cause disease of the skin, heart, lungs, kidneys, joints, and nervous system. When only the skin is involved, the condition is called discoid lupus. When internal organs are involved, the condition is called systemic lupus erythematosus (SLE). Up to 10% of persons with discoid lupus (lupus limited to the skin) eventually develop the systemic form of lupus (SLE). SLE is eight times more common in women than men. The causes of SLE are unknown. However, heredity, viruses, ultraviolet light, and drugs may all play a role. Eleven criteria have been established for the diagnosis of SLE: Malar (over the cheeks of the face) "butterfly" rash Discoid skin rash: patchy redness that can cause scarring Photosensitivity: skin rash in reaction to sunlight exposure Mucus membrane ulcers: ulceration of the lining of the mouth, nose or throat Arthritis: 2 or more swollen, tender joints of the extremities Pleuritis/pericarditis: inflammation of the lining tissue around the heart or lungs, usually associated with chest pain with breathing Kidney abnormalities: abnormal amounts of urine protein or cellular elements Brain irritation: manifested by seizures (convulsions) and/or psychosis Blood count abnormalities: low counts of white or red blood cells, or platelets Immunologic disorder: abnormal immune tests include anti-DNA or anti-Sm (Smith) antibodies, falsely positive blood test for syphilis, anticardiolipin antibodies, lupus anticoagulant, or positive LE prep test Antinuclear antibody: positive ANA antibody testing The treatment of SLE is directed toward decreasing inflammation and/or the level of autoimmune activity. Persons with SLE can help prevent "flares" of disease by avoiding sun exposure and by not abruptly discontinuing medications.

Port-Wine Stain after Laser Treatment

There is a marked resolution of the port-wine stain after multiple treatments with pulsed dye laser. A port-wine stain is a mark on the skin that resembles port wine (porto) in its rich ruby red color. Due to an abnormal aggregation of capillaries, a port wine stain is a type of hemangioma. A port wine stain on the face is a sign of the Sturge-Weber syndrome.

Port-Wine Stain (Neck)

This is an extensive port-wine stain on the right side of the neck of a young female. A port-wine stain is a mark on the skin that resembles port wine (porto) in its rich ruby red color. Due to an abnormal aggregation of capillaries, a port wine stain is a type of hemangioma. A port wine stain on the face is a sign of the Sturge-Weber syndrome.

Port-Wine Stain (Lip)

Port-wine stains (PWS) are low-flow capillary malformations. They represent the most common type of vascular malformations. Any area of the body can be affected. However, the head and neck areas are most commonly affected. Port-wine stain on the lower mucosal and cutaneous lip.

Port Wine Stain

Port wine stain: A mark on the skin that resembles port wine (porto) in its rich ruby red color. Due to an abnormal aggregation of capillaries, a port wine stain is a type of hemangioma. A port wine stain on the face is a sign of the Sturge-Weber syndrome.

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