Idiopathic Thrombocytopenic Purpura With Black Oral Mucosal Lesions LT Amy E. Helms, MC, USNR; Robert I. Schaffer, MD Idiopathic thrombocytopenic purpura (ITP) is an shiny black papules were observed on the right buc- acquired autoimmune disorder in which autoanti- cal mucosa (Figures 1 and 2). There were 2 ecchy- bodies are made against platelets, causing accel- moses, approximately 3 to 4 cm in diameter—one erated platelet destruction. History and physical on the right arm and one on the left leg. Multiple examination are most often normal except for red hemorrhagic nonblanching macules 1 to 2 mm petechiae, commonly seen in the lower extremi- in diameter were present on the anteromedial lower ties. Hemorrhagic bullae of mucous membranes legs and dorsa of his feet and were barely visible. can indicate the presence of severe thrombocy- The patient’s spleen was palpable just below the left topenia. We report a case of ITP in a 33-year-old central margin with inspiration. No hepatomegaly man who presented with insidious onset of black or lymphadenopathy was detected. oral mucosal lesions. Differential diagnosis included metastatic mela- Cutis. 2007;79:456-458. noma because of the shiny black appearance of the papules. Acute leukemia and idiopathic thrombocy- topenic purpura (ITP) were considered in light of the petechial and ecchymotic lesions, suggesting a hem- Case Report orrhagic diathesis. Actinomycosis, histoplasmosis, A 33-year-old man presented with a 2-week history of aspergillosis, and cryptococcosis were included in “black spots” in his mouth. The first lesion appeared the differential diagnosis because oral granuloma- on the left buccal mucosa, with 4 more black papules tous papules also occur in these diseases.1 However, appearing on the right buccal mucosa in the next sev- considering the patient’s general appearance and eral days. He felt congested and noticed blood-tinged lack of fever or malaise, these diagnoses were consid- mucus when blowing his nose. He was seen at an ered less likely causes of the black papules. urgent care center and was given a 10-day course of Laboratory results revealed that hemoglobin, amoxicillin for what had been diagnosed as sinusitis. hematocrit, and white blood cell counts, as well The patient then noticed a bruise on his right arm as prothrombin and partial thromboplastin times, and another on his left leg. After noting tiny red spots were all within reference range. The platelet count, on his legs and feet, he notified a family physician, however, was drastically low at 3000/mL (reference who gave him methylprednisolone. When this treat- range, 150–450 3103/ mL). Peripheral smear showed ment did not alleviate his eruption, he was referred to normal size platelets. The patient was diagnosed a dermatologist. with ITP and sent to the emergency room where Physical examination revealed a well-nourished, his internist and a hematologist examined him. well-developed, cooperative man in no acute dis- In view of the acute onset of the lesions and the tress. His vital signs were stable, and he had a nor- patient’s age, a bone marrow biopsy was consid- mal temperature. An irregular 8-mm black papule ered unnecessary and treatment was initiated. He was seen on the left buccal mucosa, and 4 smooth received 1 U of platelets, intravenous immunoglob- ulin G, and prednisone starting at 80 mg/d. After 2 weeks, his platelet count was 130,000/mL. Accepted for publication June 6, 2006. Dr. Helms is from Uniformed Services University of the Comment Health Sciences, Bethesda, Maryland. Dr. Schaffer is in ITP, also known as immune thrombocytopenic pur- private practice, Warren, Ohio. The authors report no conflict of interest. pura, is an acquired disease in which autoantibod- Reprints: LT Amy E. Helms, MC, USNR, 1971 Kennedy Dr, ies are made against platelets, causing accelerated McLean, VA 22102 (e-mail: [email protected]). platelet destruction.2 ITP is classified as primary or 456 CUTIS® Idiopathic Thrombocytopenic Purpura the symptoms and signs of bleeding are not. Occurring most commonly in dependent regions, petechiae are not palpable and they are numer- ous in the mucous membranes where hemorrhagic bullae can occur when severe thrombocytopenia is present. Signs and symptoms are predictable from the commonly recognized pattern of bleeding associated with congenital platelet function disor- ders. Purpura, menorrhagia, epistaxis, and gingival bleeding are common; gastrointestinal bleeding and hematuria are less common. Hemorrhagic bul- lae of mucous membranes can occur with severe thrombocytopenia.8,10 Figure 1. A dark hemorrhagic and hyperkeratotic pap- Laboratory findings usually are suitable indi- ule on the left buccal mucosa. Photograph courtesy of cators of ITP. Isolated thrombocytopenia is the Dr. Stephen E. Helms, Warren, Ohio. primary abnormality. Hemoglobin concentration usually is normal unless substantial hemorrhage associated with thrombocytopenia has resulted in anemia.8 The white blood cell count typically is within reference range. Other conditions such as infection with human immunodeficiency virus or hepatitis C, chronic liver disease with hypersplenism, myelodysplastic syndromes, systemic lupus erythematosus, and chronic diffuse intravascular coagulation can mimic ITP.3,11-13 Excluding other causes of throm- bocytopenia is the most efficient way of diagnosing ITP. The absence of systemic symptoms, as well as the duration of the bleeding signs, helps to rule out secondary forms and other diagnoses.3 The diag- nosis of ITP is mainly based on history, physical Figure 2. Multiple smooth, shiny, black papules on the right buccal mucosa. Photograph courtesy of examination, and complete blood cell count, with Dr. Stephen E. Helms, Warren, Ohio. examination of the peripheral smear. Bone marrow aspiration is recommended to establish a diagnosis secondary to an underlying disease and may be acute in patients older than 60 years and to rule out or chronic.3 ITP occurs most often between the ages myelodysplastic syndromes.2 of 18 and 40 years and is twice as common in women Treatment of ITP should be individualized. than in men.4 It occurs in approximately 38 per mil- Glucocorticoid therapy (ie, prednisone [1–2 mg/kg lion adults per year.5 per day]) is the appropriate initial therapy for Approximately 30% to 40% of adults with ITP moderate to severe thrombocytopenia and symp- have no symptoms.5-7 This form of the disease has tomatic purpura. Intravenous immunoglobulin G an insidious onset and rarely results in spontaneous therapy (2 g/kg given over 2–5 days) is indicated for resolution.8 Patients with ITP usually present with patients with platelet counts less than 30,000/mL platelet counts greater than 50,000/mL. Individuals and patients with severe life-threatening bleeding.2,8 with platelet counts between 30,000 and 50,000/mL Platelet transfusions and hospitalization usually are might experience excessive bruising from minimal reserved for patients with platelet counts less than trauma. When platelet counts are between 10,000 20,000/mL. Splenectomy is considered when platelet and 30,000/mL, petechiae or ecchymoses suddenly counts remain below 30,000/mL after 4 to 6 weeks of develop. Patients with platelets below 10,000/mL are medical treatment.2 Anti-D immunoglobulin therapy at risk of internal hermorrhage.9 currently is used only for Rh-positive patients with The majority of adults present with a long- severe thrombocytopenia unresponsive to oral agents standing history of petechiae and purpura, and many but is being studied for initial therapy. It is given as a adults are diagnosed incidentally as a result of rou- single dose (75 mg/kg) and may be repeated based on tine platelet counting.5 Although the remainder of platelet response.3 Most patients with mild to moder- the history and physical examination are normal, ate asymptomatic thrombocytopenia (those who can VOLUME 79, JUNE 2007 457 Idiopathic Thrombocytopenic Purpura maintain platelet counts .30,000/mL) can be safely 7. Stasi R, Stipa E, Masi M, et al. Long-term observation followed with no treatment.2,9 of 208 adults with chronic idiopathic thrombocytopenic purpura. Am J Med. 1995;98:436-442. 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