Purpura

Purpura is the extravasation of red blood cells from the blood vessels into the skin, subcutaneous tissue, or mucous membranes. It’s characterized by discoloration that’s easily visible through the epidermis, usually purplish or brownish red. Purpuric lesions include petechiae, ecchymoses, and hematomas. (See Identifying purpuric lesions, page 662.) Purpura differs from erythema in that it doesn’t blanch with pressure because it involves blood in the tissues, not just dilated vessels.

Purpura results from damage to the endothelium of small blood vessels, a coagulation defect, ineffective perivascular support, capillary fragility and permeability, or a combination of these factors. These faulty hemostatic factors, in turn, can result from thrombocytopenia or another hematologic disorder, an invasive procedure, or the use of an anticoagulant.

Additional causes are nonpathologic. Purpura can be a consequence of aging, when loss of collagen decreases connective tissue support of upper skin blood vessels. In an elderly or cachectic person, skin atrophy and inelasticity and loss of subcutaneous fat increase susceptibility to minor trauma, causing purpura to appear along the veins of the forearms, hands, legs, and feet. Prolonged coughing or vomiting can produce crops of petechiae in loose face and neck tissue. Violent muscle contraction, as occurs in seizures or weight lifting, sometimes results in localized ecchymoses from increased intraluminal pressure and rupture. High fever, which increases capillary fragility, can also produce purpura.

Gender Cue: This condition is more common in women and particularly in individuals with large areas of subcutaneous fat, such as the breasts, abdomen, buttocks, thighs, and calves.

History and physical examination

Ask the patient when he first noticed the lesion and whether he has noticed other lesions on his body. Does he or his family have a history of a bleeding disorder or easy bruising? Find out what medications he’s taking, if any, and ask him to describe his diet. Ask about recent trauma or transfusions and the development of associated signs, such as epistaxis, bleeding gums, hematuria, and hematochezia. Ask about systemic complaints that may suggest infection, such as fever. If the patient is female, ask about heavy menstrual flow.

Inspect the patient’s entire skin surface to determine the type, size, location, distribution, and severity of purpuric lesions. Inspect the mucous membranes. Remember that the same mechanisms that cause purpura can also cause internal hemorrhage, although purpura isn’t a cardinal indicator of this condition.

Medical causes

Amyloidosis

This disorder produces purpura that appears either spontaneously on dependent areas of the skin or following minor trauma, coughing, or straining. The eyelids and mucous membranes are commonly affected.

Autoerythrocyte sensitivity

With this syndrome, painful ecchymoses appear either singly or in groups, usually preceded by local itching, burning, or pain. Common associated findings include epistaxis, hematuria, hematemesis, and menometrorrhagia. Abdominal pain, diarrhea, nausea, vomiting, syncope, headache, and chest pain are also common.

Cholesterol emboli

Purpura due to cholesterol emboli are most commonly found in the lower extremities of patient with atherosclerotic vascular disease and usually occur after anticoagulation therapy or an invasive arterial procedure such as angiogram or cardiac catheterization but may occur spontaneously. Associated findings include livedo reticularis, cyanosis, gangrene, nodules and ulceration of the skin.

Dermatoses (pigmented)

This group of disorders, thought to result from chronic stasis, produces benign, chronic purpura, mainly on dependent areas.

Disseminated intravascular coagulation

This disorder can cause varying degrees of purpura, depending on its severity and underlying cause. Rarely, the patient develops life-threatening purpura fulminans, with symmetrical cutaneous and subcutaneous lesions on the arms and legs, or he may have cutaneous oozing, hematemesis, or bleeding from incision or needle insertion sites. Other findings include acrocyanosis; nausea; dyspnea; seizures; severe muscle, back, and abdominal pain; and signs of acute tubular necrosis, such as oliguria.

Dysproteinemias

With multiple myeloma, petechiae and ecchymoses accompany other bleeding tendencies: hematemesis, epistaxis, gum bleeding, and excessive bleeding after surgery. Similar findings occur with cryoglobulinemia, which may also produce malignant maculopapular purpura. Hyperglobulinemia typically begins insidiously with occasional outbreaks of purpura over the lower legs and feet. These outbreaks eventually become more frequent and extensive, involving the entire lower leg and possibly the trunk. The purpura usually occurs after prolonged standing or exercise and may be heralded by skin burning or stinging. Leg edema, knee or ankle pain, and low-grade fever may precede or accompany the purpura, which gradually fades over 1 to 2 weeks. Persistent pigmentation develops after repeated outbreaks.

Easy bruising syndrome

This syndrome is characterized by recurrent bruising on the legs, arms, and trunk, either spontaneously or following minor trauma. Bruising may be preceded by pain and is more common in women than in men, especially during menses.

Ehlers-Danlos syndrome (EDS)

Besides petechiae, this syndrome is marked by easy bruising, epistaxis, gum bleeding, hematuria, melena, menorrhagia, and excessive bleeding after surgery. EDS characteristically produces soft, velvety, hyperelastic skin; hyperextensible joints; increased skin and blood vessel fragility; and repeated dislocations of the temporomandibular joint.

Fat emboli

Petechiae that occur on the upper body a few days after a major injury are caused by fat emboli.

Idiopathic thrombocytopenic purpura (ITP)

Chronic ITP typically begins insidiously, with scattered petechiae that are usually found on the distal arms and legs. Deep-lying ecchymoses may also occur. Other findings include epistaxis, easy bruising, hematuria, hematemesis, and menorrhagia.

Leukemia

This disease produces widespread petechiae on the skin, mucous membranes, retina, and serosal surfaces that persist throughout the course of the disease. Confluent ecchymoses are uncommon. The patient may also exhibit swollen and bleeding gums, epistaxis, and other bleeding tendencies. Lymphadenopathy and splenomegaly are common.

Acute leukemias also produce severe prostration and high fever and may cause dyspnea, tachycardia, palpitations, and abdominal or bone pain. Confusion, headache, seizures, vomiting, papilledema, and nuchal rigidity may occur late in the disease. Chronic leukemias begin insidiously with minor bleeding tendencies, malaise, fatigue, pallor, low-grade fever, anorexia, and weight loss.

Liver disease

Liver disease may cause purpura, particularly ecchymoses, and other bleeding tendencies. Associated findings include hepatomegaly, ascites, right-upper-quadrant pain, jaundice, nausea, vomiting, and anorexia.

Lymphomas

T-cell (Hodgkin’s) lymphomas initially may produce erythematous patches with some scaling. These lesions, which may be psoriasiform or parapsoriasiform, then become interspersed with nodules. Pruritus and discomfort are common. Later, tumors and ulcerations form, and nontender lymphadenopathy develops.

B-cell (non-Hodgkin’s) lymphoma may produce a scaling dermatitis with pruritus, which usually begins on the legs and then affects the entire body. Small pink-to-brown nodules and diffuse pigmentation also occur. B-cell lymphomas typically produce painless peripheral lymphadenopathy, usually affecting the cervical nodes first. Other findings in both types of lymphoma include fever, fatigue, malaise, weight loss, and hepatosplenomegaly.

Meningococcemia

Transmitted by droplet inhalation, it’s most common in children and caused by Neisseria meningitidis. Cutaneous and oropharyngeal petechia and purpura are initially discrete but become confluent, developing into hemorrhagic bullae and ulcerations. Fulminant infection results in extensive purpura and ecchymosis with irregular borders (purpura fulminans) most notably on the extremities. These lesions may develop necrotic centers. This disease is usually fatal if not recognized and treated early. Prognosis is poor when purpura or ecchymosis is present at the time of diagnosis. Associated symptoms include spiking fevers, chills, myalgia and arthralgia, and recent upper respiratory tract infection. Rapid progression of symptoms leads to headache, neck stiffness and nuchal rigidity. Septic shock ensues within hours on onset of symptoms with altered mental status and hypotension.

Myeloproliferative disorders

These disorders, which include polycythemia vera, paradoxically can cause hemorrhage accompanied by ecchymoses and ruddy cyanosis. The oral mucosa takes on a deep purplish red hue, and slight trauma causes swollen gums to bleed. Other findings include pruritus, urticaria, and such nonspecific signs and symptoms as lethargy, weakness, fatigue, and weight loss. The patient typically complains of headache, a sensation of fullness in the head, and rushing in the ears; dizziness and vertigo; dyspnea; paresthesia of the fingers; double or blurred vision and scotoma; and epigastric distress. He may also experience intermittent claudication, hypertension, hepatosplenomegaly, and impaired mentation.

Nutritional deficiencies

With vitamin C deficiency (scurvy), the characteristic pattern of purpura is perifollicular petechiae, which coalesce to form ecchymoses, in the “saddle area” of the thighs and buttocks. Additional hemorrhaging occurs in arm and leg muscles (with phlebothrombosis), viscera, joints (with limb and joint pain), and nail beds. Related findings include scaly dermatitis; pallor; tender, swollen, bleeding gums and loosened teeth; dry mouth; and poor wound healing. Nonspecific symptoms include weakness, lethargy, and anorexia. Irritability, depression, insomnia, and hysteria may also develop.

Vitamin K deficiency produces abnormal bleeding tendencies, such as ecchymosis, gum bleeding, epistaxis, hematuria, and GI and intracranial bleeding.

Vitamin B₁₂ deficiency can cause varying degrees of purpura. GI findings include anorexia, nausea, vomiting, weight loss, abdominal discomfort, and jaundice. Dyspnea, peripheral neuropathies, ataxia, glossitis and, occasionally, depression also occur.

Folic acid deficiency also can cause varying degrees of purpura. The patient may be irritable and forgetful and complain of fatigue, weakness, dyspnea, palpitations, nausea, anorexia, headaches, and fainting spells. Additional findings include pallor, slight jaundice, and glossitis.

Rocky Mountain spotted fever

This illness is contracted through the bite of an infected tick and is most common among children between ages 5 and 10. Initial skin lesions are small pink macules that evolve into blatant petechia and palpable purpura. Hemorrhagic macules may develop. The palms and soles are particularly affected. Extensive cutaneous necrosis occurs due to disseminated intravascular coagulation in a small percentage of patients experiencing gangrene of the extremities, necessitating amputation. Associated signs and symptoms include fever, severe headache, generalized myalgia, photophobia, nausea and vomiting. Late in the course of the illness, shock and death may occur.

Septicemia

Thrombocytopenia or the effects of toxins in acute infection can lead to purpura, especially in the form of petechiae. Associated findings include fever, chills, headache, tachycardia, lethargy, diaphoresis, and anorexia. Signs and symptoms specific to the area of infection—for example, cough, wound drainage, and urinary burning—also occur.

Stasis

Chronic stasis usually affects the elderly, producing dusky reddish purpura on the legs after prolonged standing.

Systemic lupus erythematosus

This chronic inflammatory disorder may produce purpura accompanied by other cutaneous findings, such as scaly patches on the scalp, face, neck, and arms; diffuse alopecia; telangiectasia; urticaria; and ulceration. The characteristic butterfly rash appears in the disorder’s acute phase. Common associated signs and symptoms include nondeforming joint pain and stiffness, Raynaud’s phenomenon, seizures, psychotic behavior, photosensitivity, fever, anorexia, weight loss, and lymphadenopathy.

Thrombotic thrombocytopenic purpura

Generalized purpura, hematuria, vaginal bleeding, jaundice, and pallor are among the usual presenting signs and symptoms in this disorder. Most patients have fever, and some also experience fatigue, weakness, headache, nausea, abdominal pain, arthralgias, and hepatosplenomegaly. Possible neurologic effects include seizures, paresthesia, cranial nerve palsies, vertigo, and altered level
of consciousness. Renal failure may also occur.

Trauma

Traumatic injury can cause local or widespread purpura.

Vasculitis

Palpable purpura is commonly caused by allergic vasculitis (leukocytoclastic vasculitis) of which Henoch-Schönlein purpura is one subtype. Most common in adolescents and children, lesions can be found anywhere on the body but are most prevalent on the lower extremities and buttocks. The purpura tends to be smooth, bordered, and circular in nature. Systemic signs and symptoms include fever, arthralgias, abdominal pain, GI bleeding and nephritis.

Other causes

Diagnostic tests

Invasive procedures, such as venipuncture and arterial catheterization, may produce local ecchymoses and hematomas due to extravasated blood.

Drugs

The anticoagulants heparin and warfarin can produce purpura. Administration of warfarin can result in painful areas of erythema that become purpuric then necrotic with an adherent black eschar. The lesions develop between the 3rd and 10th day of drug administration.

Surgery and other procedures

Any procedure that disrupts circulation, coagulation, or platelet activity or production can cause purpura. These include pulmonary and cardiac surgery, radiation therapy, chemotherapy, hemodialysis, multiple blood transfusions with platelet-poor blood, and use of plasma expanders such as dextran.

Special considerations

Reassure the patient that purpuric lesions aren’t permanent and will fade if the underlying cause can be successfully treated. Warn him not to use cosmetic fade creams or other products in an attempt to reduce pigmentation. If he has a hematoma, apply pressure and cold compresses initially to help reduce bleeding and swelling. After the first 24 hours, apply hot compresses to help speed absorption of blood.

Prepare the patient for diagnostic tests. These may include a peripheral blood smear, bone marrow examination, and blood tests to determine platelet count, bleeding and coagulation times, capillary fragility, clot retraction, one-stage prothrombin time, activated partial thromboplastin time, and fibrinogen levels.

Pediatric pointers

Neonates commonly exhibit petechiae, particularly on the head, neck, and shoulders, after vertex deliveries. Thought to result from the trauma of birth, these petechiae disappear within a few days. Other causes in infants include thrombocytopenia, vitamin K deficiency, and infantile scurvy.

The most common type of purpura in children is allergic purpura. Other causes in children include trauma, hemophilia, autoimmune hemolytic anemia, Gaucher’s disease, thrombasthenia, congenital factor deficiencies, Wiskott-Aldrich syndrome, acute ITP, von Willebrand’s disease, and the rare but life-threatening purpura fulminans, which usually follows bacterial or viral infection.

As a child grows and tests his motor skills, the risk of accidents multiplies, and ecchymoses and hematomas commonly occur. However, when you assess a child with purpura, be alert for signs of possible child abuse: bruises in different stages of resolution, from repeated beatings; bruise patterns resembling a familiar object, such as a belt, hand, or thumb and finger; and bruises on the face, buttocks, or genitalia, areas unlikely to be injured accidentally.

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Book Source Details

• Book Title: Professional Guide to Signs & Symptoms (Fifth Edition)
• Author(s): Springhouse
• Year of Publication: 2006
• Copyright Details: Professional Guide to Signs & Symptoms (Fifth Edition), Copyright © 2006 Lippincott Williams & Wilkins.

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