Cutaneous Angiomatosis: Definition and Clinical Context
Cutaneous angiomatosis refers to a spectrum of vascular proliferative disorders affecting the skin, most notably bacillary angiomatosis in immunocompromised patients and reactive angiomatoses in various clinical settings.
Bacillary Angiomatosis
Bacillary angiomatosis is the most clinically significant form of cutaneous angiomatosis, caused by Bartonella henselae or Bartonella quintana infection, primarily affecting immunocompromised patients, especially those with AIDS. 1
Clinical Presentation
Two distinct morphologic patterns occur: 1
The condition occurs predominantly in AIDS patients but can also affect other immunocompromised individuals, including organ transplant recipients 2, 3
Rare cases occur in immunocompetent children and adults 2
Diagnosis
Diagnosis is challenging because Bartonella species are fastidious organisms that rarely grow in culture from tissue. 1
Serologic testing supports diagnosis but has limitations due to cross-reactivity between B. henselae and B. quintana 1
Warthin-Starry silver stain of infected tissue is useful for confirmation, though it cannot differentiate Bartonella species 1
PCR detection of Bartonella DNA in tissue biopsy extracts provides definitive diagnosis 3
Histologically, bacillary angiomatosis shows reactive proliferation of bland endothelial cells and pericytes forming small capillary vessels 4
Treatment
All immunosuppressed patients with bacillary angiomatosis must be treated with antibiotics due to the potentially life-threatening course of the disease. 3
Erythromycin 500 mg four times daily for 2 weeks to 2 months is the recommended first-line treatment 1
Doxycycline 100 mg twice daily for 2 weeks to 2 months is an equally effective alternative 1, 5
Treatment duration of 3 months has been successfully used in pediatric cases 2
Reactive Angiomatoses
Cutaneous reactive angiomatosis represents a benign vascular disorder characterized by lobular or diffuse proliferation of small blood vessels with endothelial cell and pericyte hyperplasia, typically associated with systemic diseases. 6
Clinical Features
Presents as asymptomatic erythematous-violaceous patches on various body sites including back, palms, and elbows 6
Associated with systemic conditions, particularly chronic lymphocytic leukemia and other hematologic disorders 6
Spontaneous involution can occur over 6 months 6
Diffuse Dermal Angiomatosis (DDA)
DDA is a distinct subtype of reactive angiomatosis presenting as livedoid patches and plaques with tender ulceration. 4
Breast involvement (DDAB) is more common than previously recognized, typically affecting middle-aged women with macromastia, obesity, and smoking history 4
Predisposing conditions for DDA at other sites include advanced atherosclerotic vascular disease and arteriovenous fistula 4
Histologically shows diffuse proliferation of bland endothelial cells and pericytes forming small capillary vessels within the dermis 4
Medical therapies including isotretinoin have limited success; breast reduction surgery appears to be the most viable treatment option for DDAB in women with macromastia 4
Critical Distinctions
Cutaneous angiomatosis must be distinguished from infantile hemangiomas and vascular malformations, which are separate entities with different pathophysiology and management. 1
Infantile hemangiomas are benign vascular tumors with characteristic GLUT1 expression, distinct from reactive angiomatoses 1
Vascular malformations are congenital lesions with normal endothelial cell turnover, not proliferative disorders 1
Key Clinical Pitfalls
Do not delay antibiotic treatment in immunocompromised patients with suspected bacillary angiomatosis, as the disease can be life-threatening 3
Do not rely solely on culture for diagnosis; use serologic testing, PCR, and histopathology with special stains 1, 3
Do not confuse bacillary angiomatosis with pyogenic granuloma or Kaposi sarcoma, which have different histologic features and treatment approaches 1
Consider bacillary angiomatosis in any immunocompromised patient presenting with fever and cutaneous angioma-like lesions 3