Bacillary angiomatosis successfully treated with doxycycline and erythromycin: a case report

¹Dermatovenereology, St. Paul Hospital Millennium Medical College, KolfeKeranew KK, Addis Ababa, Ethiopia, ²KADISCO General Hospital, Addis Ababa, Ethiopia

^&Corresponding author
Samia Yaheya Metena, Dermatovenereology, St. Paul Hospital Millennium Medical College, Addis Ababa, Ethiopia

Introduction    

Bacillary angiomatosis is a recently recognized bacterial infectious disease, with the first cases being described around 1983 [1,2], and is seen mainly in patients with acquired immunodeficiency syndrome [3]. The lesions of bacillary angiomatosis are very variable. They may be solitary or appear in crops, as small papules or dermal nodules [4]. The vast majority of cases are diagnosed histologically, with the identification of the causative bacteria by Warthin-Starry staining [5]. There are reports of successful treatment of bacillary angiomatosis with erythromycin and doxycycline.

Cases of bacillary angiomatosis are rarely reported in Africa. To our knowledge, there are only 7 cases reported in Africa [4,5]. This is a small number considering the high prevalence of HIV in most African countries including Ethiopia where the infection has a 4.4% prevalence. In three of the cases reported from Africa, specifically East Africa there was a significant diagnostic challenge, especially in differentiating the cases from Kaposi sarcoma [5]. This poses a question of whether these cases are not commonly reported because they are not as common or they are underdiagnosed. We report a case of bacillary angiomatosis from Ethiopia treated with doxycycline and erythromycin after which he was followed for 4 years with significant improvement and no relapse.

Patient and observation     

Patient information: we present a 27-year-old male patient with a neck mass of eight months associated with pain and skin lesions on the face and the extremities. The patient was known to have human immune deficiency virus infection 2 years before this presentation after presenting with cryptococcal meningitis and HIV dementia and was initiated on first-line anti-retroviral medications with a baseline CD4 of 22, with TDF 3TC EFV then second-line ART (AZT 3TC ATV/r) after a CD4 was dropped to 16. Patient worked in office did not have any pets and could not recall any cat scratch incident.

Clinical findings: the patient´s vital signs were stable but he was emaciated and sick-looking. He had three anterolateral neck masses measuring 6 by 5 centimeters. They were firm immobile and fused and had erythematous cutaneous nodules overlying them. He had similar cutaneous nodules over the forehead, below the nasal orifice, on the upper extremities the scrotum, and the thighs. Most of the lesions showed hemorrhagic and yellowish crusts. There were also subcutaneous nodules on the upper extremities and thighs which are more felt than seen in the pictures. He had hyper-pigmented patches on the buccal mucosa hard palates and tongue. His chest was clear and had no signs of organomegaly or fluid collection.

Timeline of the current episode

At presentation: he had three anterolateral neck masses measuring 6 by 5 centimeters. He had similar cutaneous nodules over the forehead measuring 3 by 2 centimeters and lesions below the nasal orifice, the upper extremities the scrotum, and the thighs (Figure 1 A, B). Hemoglobin was 7.8, platelet count 30,000 and WBC count 4000, CD4 count of 16, ESR 82, viral load 1,458,584, Urea 0.59 creatinine 0.59, SGPT 2.8, and SGOT 8.3. The neck X-ray showed multiple hypervascular cervical lymph nodes and the neck ultrasound showed a highly vascular lesion on the anterior neck. Chest X-ray and abdominal ultrasound were normal, previous and repeated fine needle aspiration cytology (FNAC) from neck lesions were inconclusive and biopsy from the neck mass showed granulomatous inflammation with proliferating capillaries. An excisional skin biopsy was done from the arm lesions and sent for dermatopathologic evaluation.

Two weeks after the presentation: biopsy of skin lesions showed vascular proliferation lined by plump epithelioid endothelial cells with a background of inflammatory cell infiltrate composed of lymphocytes, histiocytes, and neutrophils with nuclear dust and amphophilic granular material near them.

Diagnostic assessment: bacillary angiomatosis and Kaposi sarcoma were the two most important differential diagnoses. The clues to bacillary angiomatosis in hematoxylin and eosin-stained sections of tissue are the pyogenic granuloma-like proliferation of blood vessels with nuclear dust of neutrophils and clumps of purplish material representing a hazy colony of bacteria. These clumps of amphophilic granular material are present, particularly near neutrophils, and represent the organisms that are too small to be seen under the light microscope but readily demonstrated by a Giemsa, Warthin-Starry, or Grocottmethenamine silver stain in the setting where special stains are available (Figure 2 A, B).

Diagnosis: short of specific stains, bacillary angiomatosis was made as the best diagnosis by the dermatopathologist.

Therapeutic interventions: the patient was started with doxycycline 100mg PO twice a day.

Follow-up and outcome: three weeks after the start of treatment, the patient showed improvement in the size of lesions and ulceration (Figure 3 A, B).

Six weeks after the start of treatment: the patient continued to have an improvement in lesions and general condition but was having signs and symptoms of peptic ulcer disease (PUD).

Nine weeks after the start of treatment: doxycycline was changed to erythromycin because of gastric irritation, the patient continued to improve and was treated for a total of 6 months. He was followed for a total of 4 years and had no signs of relapse (Figure 4).

Patient´s perspective: "I did not think I would survive after becoming that ill. I thought it was non-curable."

Informed consent: an informed consent was obtained from the patient.

Discussion     

Bacillary angiomatosis is a recently recognized bacterial infectious disease, with the first cases being described around 1983 [1,2], and is seen mainly in patients with acquired immunodeficiency syndrome [3]. The average CD4 count of patients at presentation is 57/mm³ [4]. Bacillary angiomatosis has also been described in transplant patients [4], in patients with disseminated malignancy [4]. Two Bartonella species, B. henselae and B. quintana have been associated with this infection [1]. The infection is sporadic and there is no history of exposure to cats or skin injury in most cases. There are no known differences between skin infections caused by the two species of Bartonella [2].

The lesions of bacillary angiomatosis are very variable. They may be solitary or appear in crops, as small papules or dermal nodules. The most common cutaneous morphologies of bacillary angiomatosis are pyogenic granuloma-like lesions, subcutaneous nodules, and hyperpigmented indurated plaques [3]. In addition to cutaneous lesions, other organ systems may be affected and lymphadenopathy is common [3]. The vast majority of cases are diagnosed histologically, with the identification of the causative bacteria by Warthin-Starry staining [4]. Histology shows rounded masses of small vessels in superficial dermis, deep dermis, or both. Endothelial cells have enlarged vesicular nuclei; abundant pink cytoplasm and granular purple clusters of bacteria, neutrophils, and nuclear dust around bacteria [6].

Cultures are not usually taken as they are technically difficult to process. However, polymerase chain reaction (PCR) is now increasingly used [5]. There are reports of successful treatment of bacillary angiomatosis with erythromycin and doxycycline [4,5]. Antituberculous drugs, gentamicin combined with either doxycycline or ciprofloxacin are also mentioned in literature [4,5]. Surgical excision of solitary cutaneous lesions can be successful but antimicrobial therapy can accomplish the same result and also provide therapy for possible occult dissemination of the bacteria. The appropriate duration of antimicrobial therapy is uncertain, but most patients who were successfully treated have received antimicrobials for longer than 4 weeks [4,5]. The drugs are to be used for a minimum period of two months. In osseous and central nervous system infection, intravenous medication is desirable for the first 4 weeks followed by another 3 months of oral therapy. The illness is prone to relapse, especially when a shorter course of antibiotic therapy is administered. If relapse occurs, the treatment is prolonged for 3 to 6 months or even for life [4].

Although relatively uncommon, it is worth considering bacillary angiomatosis especially in patients with human immune deficiency virus infection. Kaposi´s sarcoma and pyogenic granuloma (lobular capillary hemangioma) are important differential diagnoses of bacillary angiomatosis both clinically and histopathologically. Though the proliferation of blood vessels are common finding in all the conditions mentioned above, the blood vessels in bacillary angiomatosis are lined by plump, epithelioid endothelial cells. In addition, the background inflammatory cell infiltrate is composed of lymphocytes, histiocytes, and neutrophils.

The clues to bacillary angiomatosis in hematoxylin and eosin-stained sections of tissue which were used to make a definitive diagnosis in this case were pyogenic granuloma-like proliferation of blood vessels with nuclear dust of neutrophils and clumps of purplish material representing a hazy colony of bacteria. These clumps of amphophilic granular material are present, particularly near neutrophils, and represent the organisms that are too small to be seen under the light microscope but readily demonstrated by a Giemsa, Warthin-Starry, or Grocottmethenamine silver stain in the setting where special stains are available. Cases of bacillary angiomatosis are rarely reported in Africa, to our knowledge there are only 7 cases reported in Africa [7,8]. This is a small number considering the high prevalence of HIV in most African countries including Ethiopia where the infection has a 4.4% prevalence [9].

In three of the cases reported from Africa, specifically East Africa there was a significant diagnostic challenge, especially in differentiating the cases from Kaposi sarcoma [8]. This poses a question of whether these cases are not commonly reported because they are not as common or are underdiagnosed. This case report is meant to increase awareness about this debilitating but fairly treatable disease appreciate the role of dermatology and dermatopathology in the diagnosis and treatment of life-threatening and debilitating conditions and work towards improving these services in Ethiopia and other countries where pathology services are limited.

Conclusion     

Bacillary angiomatosis poses a diagnostic challenge to many physicians including dermatologists. This is one of the dermatology cases that is life-threatening but can be easily cured once diagnosed.

Competing interests     

The authors declare no competing interests.

Authors' contributions     

Samia Yaheya metena: study concept, data curation, formal analysis, methodology, project management, and writing (original draft, review, and editing). Fuad Awel Temam: study concept, data curation, formal analysis, methodology, project management, and writing (review and editing).

Figures     

Figure 1: lesions at presentation: A) two large nodules with hemorrhagic crusts on the face; B) erythematous nodules overlying cervical lymphadenopathy

Figure 2: histologic sections of lesions at presentation; A) medium magnification, of HE stained section - blood vessels lined by plump epithelioid endothelial cells; B) high magnification of HE stained section - background inflammatory cells composed of lymphocytes, histiocytes, and neutrophils associated with amphophilic granular material at the center

Figure 3: lesions six weeks after treatment; A) decrease in size of nodules and crustation on the face; B) decrease in size of nodules and crustation on the neck

Figure 4: face lesions 4 years after treatment; completely healed face and neck lesions with no significant scarring

References