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A Case Report of Bacillary Angiomatosis in a Patient Infected with Human Immunodeficiency Virus

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A Case Report of Bacillary Angiomatosis in a Patient Infected with Human Immunodeficiency Virus Continuing Medical Education A Case Report of Bacillary Angiomatosis in a Patient Infected With Human Immunodeficiency Virus Cindy F. Hoffman, DO; Dimitria Papadopoulos, DO; Debbie M. Palmer, DO; Craig Austin, MD; Charles A. Gropper, MD GOAL To gain a complete and detailed understanding of bacillary angiomatosis OBJECTIVES Upon completion of this activity, dermatologists and general practitioners should be able to: 1. Discuss the clinical manifestations of bacillary angiomatosis. 2. Explain the histologic findings of bacillary angiomatosis. 3. Describe the treatment for bacillary angiomatosis. CME Test on page 210. This article has been peer reviewed and Medicine is accredited by the ACCME to provide approved by Michael Fisher, MD, Professor of continuing medical education for physicians. Medicine, Albert Einstein College of Medicine. Albert Einstein College of Medicine designates Review date: February 2002. this educational activity for a maximum of 1.0 hour This activity has been planned and implemented in category 1 credit toward the AMA Physician’s in accordance with the Essential Areas and Policies Recognition Award. Each physician should claim of the Accreditation Council for Continuing Medical only those hours of credit that he/she actually spent Education through the joint sponsorship of Albert in the educational activity. Einstein College of Medicine and Quadrant This activity has been planned and produced in HealthCom, Inc. The Albert Einstein College of accordance with ACCME Essentials. A man infected with human immunodeficiency angiomatosis, and the patient responded to antibi- virus (HIV) presented with a few-month history of otic therapy. We provide an overview of bacillary an enlarging friable growth on the medial area of angiomatosis, a rare disorder that affects immuno- the left foot and a one-week history of bilateral compromised patients with CD4 cell counts less lower extremity edema. Clinical and histologic than 100/µL. examination led to a diagnosis of bacillary Case Report From the Department of Dermatology, Saint Barnabas Hospital, A 45-year-old black man whose medical history Bronx, New York. Dr. Hoffman is Director of the Dermatology included human immunodeficiency virus (HIV) Residency Program and Associate Attending Physician. infection, endocarditis, congestive heart failure, Drs. Papadopoulos and Palmer are Dermatology Residents. intravenous drug abuse, end-stage renal disease, Dr. Austin is Assistant Clinical Professor of Dermatopathology. Dr. Gropper is Chief of Dermatology. sinusitis, and pneumonia presented with a few- Reprints: Cindy F. Hoffman, DO, Third Ave and 183rd St, month history of a progressively enlarging growth on Bronx, NY 10457 (e-mail: [email protected]). the left foot (a growth that bled with mild trauma) VOLUME 69, MARCH 2002 175 Bacillary Angiomatosis A well-circumscribed hemorrhagic nodule on the medial area of the left foot. and a one-week history of bilateral lower extremity A biopsy specimen taken from the nodule on edema. The patient’s medications included meth- the medial area of the left foot contained a lobe of adone, temazepam, zolpidem, calcium carbonate, proliferating capillaries, venules, and neutrophils calcitriol, folic acid, multivitamin, ferrous and an interstitial bacillary deposit. A Warthin- sulfate, and trimethoprim-sulfamethoxazole. Starry silver stain tested positive for bacilli. On physical examination, the patient was An initial diagnosis of cellulitis was made, and afebrile and had normal mucous membranes, brittle the patient was treated with amoxicillin. After a fingernails, generalized xerosis, “track marks” on final diagnosis of bacillary angiomatosis was made, the left upper extremity, bilateral lower extremity the antibiotic regimen was changed to eryth- pitting edema that was tender and warm to the romycin 500 mg 4 times a day. The hemorrhagic touch, and a 1-cm well-circumscribed hemorrhagic nodule cleared after 8 weeks of therapy. The nodule on the medial area of the left foot (Figure). patient was then lost to follow-up. Results of laboratory studies were a leukocyte count of 7.3ϫ103/µL (reference range, 4.8–11ϫ103/µL), Comment a CD4 lymphocyte count of 107 cumulative cells First described in 1983 by Stoler et al,1 bacillary (reference range, 400–1770 cumulative cells), a angiomatosis is an angioproliferative disease that CD8 lymphocyte count of 2292 cumulative cells often occurs with severe immunodeficiency, as in (reference range, 240–1200 cumulative cells), and advanced acquired immunodeficiency syndrome.2,3 no growth of any organisms in 3 serial serum blood This disease has been found in patients with acute cultures. Findings from bilateral lower extremity myeloblastic leukemia, chronic lymphocytic x-rays and Doppler ultrasounds were normal. The leukemia, and organ transplants.2-4 Bacillary differential diagnoses that were considered included angiomatosis usually manifests as cutaneous tumors pyogenic granuloma, hemangioma, Kaposi sarcoma, but also may manifest as systemic disease.4 The papular angiokeratoma, arteriovenous malforma- name of this disease was derived from the prolifer- tion, hemangiopericytoma, vasculitis, thrombo- ating blood vessels seen in histologic specimens phlebitis, and an infectious etiology. and from the presence of numerous bacillary organ- 176 CUTIS® Bacillary Angiomatosis isms detected with Warthin-Starry silver stain or mixed inflammatory cell infiltrate predominated electron microscopy.2 by vessel-surrounding neutrophils.2,7 Blood vessels Although Bartonella infections are not uncom- can proliferate superficially or deep.7 Superficially mon, bacillary angiomatosis is a rare (or underdiag- proliferating vessels resemble a pyogenic granu- nosed) disorder—1.2 cases per 1000 patients loma or papular angiokeratoma7; deep-proliferating infected with HIV.2 Patients affected most are vessels resemble a histiocytoid hemangioma with those with CD4 cell counts less than 100/µL.2 a proliferation of small blood vessels lined by Regular use of antibiotics may account for the rar- protuberant endothelial cells adhering to one ity or underdiagnosis of this disease in patients another in an “epithelioid” pattern.7 The granular infected with HIV.2 material found beside blood vessels is bacteria, The first reported case of infection with organ- which stains black with Warthin-Starry silver isms of Rochalimaea genus, now known as stain.7 Bacteria also may be detected with Bartonella, occurred during World War I and was immunohistochemistry, using anti–Bartonella- termed trench fever.2 Transmission was through species immune serums.4 Positive factor XIIIa the bite of the human body louse.5 In 1990, dermal dendrocytes have been found in bacillary Relman et al6 recognized a relation between angiomatosis.7 Fibrous long-spacing collagen, a trench fever and Rochalimaea quintana, now distinct ultrastructural collagen present in normal known as Bartonella quintana, the cause of bacil- tissue, is abundant in various tumors, including lary angiomatosis. Transmission through the body those of bacillary angiomatosis.8 This collagen is louse vector accounts for the association of found adjacent to Bartonella organisms and to B quintana infection with homelessness.2 In 1992, endothelial cells.8 another member of the genus Bartonella, B henselae, The diagnosis of bacillary angiomatosis is estab- also was recognized as an etiologic agent of this lished by clinical and histologic examination of illness.2 B henselae is transmitted to humans affected tissues.2 Culture of Bartonella species from through direct contact with cats or cat fleas, serum is insensitive, and very few isolates are avail- Ctenocephalides felis.2 B quintana is responsible for able worldwide.4 Polymerase chain reaction ampli- most cases of subcutaneous infection, deep soft- fication is another method used in detecting tissue disease, and lytic bone lesions.5 Liver and Bartonella species in biopsy specimens.4 lymph node involvement have been associated Bacillary angiomatosis can be cured with with B henselae infection.5 Bartonella species are appropriate antibiotic therapy.4,5 Erythromycin fastidious gram-negative bacteria.3 2 g/d is the treatment of choice, and doxycycline Bacillary angiomatosis can present typically 100 mg twice a day is alternative therapy.2,4 Treat- and atypically. Typical presentation is an inflam- ment of immunocompromised patients should be matory disease most often involving the skin.2 continued for 2 to 3 months.2,4 Not uncommonly, One or multiple skin lesions can develop, and patients with bacillary angiomatosis relapse after these can be localized in cutaneous and subcuta- withdrawal of antibiotic therapy.4 Precautionary neous tissues.2 The usual lesion is an angiomatous measures that should be taken with immunocom- papule or nodule resembling a pyogenic granu- promised patients include avoidance of contact loma or a subcutaneous nodule with or without with cats, fleas, and lice.4 Permethrin 1% dusting ulceration.7 Atypical presentation resembles powder, the agent of choice for delousing, should Kaposi sarcoma or papular angiokeratoma.7 Other be applied to clothing and bedding.4 areas of involvement could include the oral, anal, Maintaining a high level of clinical suspicion is conjunctival, and gastrointestinal mucosal sur- the most important aspect of diagnosing bacillary faces, as well as the brain, respiratory tract, liver, angiomatosis in immunocompromised patients. As spleen, bone, bone marrow, and lymph nodes.2,5 this disease can be cured,
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