Friday, December 07, 2012

Cryoglubulinemia


Disorder
Vasculitis
Epidemiology/Etiology
Clinical/Laboratory Findings
 
 
 
 
Cryoglobulinemia
Small vessel vasculitis involving skin, GI tract, renal vessels
Adults
Association with HCV, type I MPGN
Cryoglobulins: immunoglobulins that gel at cold temperatures
Palpable purpura, acral cyanosis of nose and ears and Raynaud's phenomenon (reverses when in warm room)

Serum Sickness


Disorder
Vasculitis
Epidemiology/Etiology
Clinical/Laboratory Findings
 
 
 
 
Serum sickness
Small vessel vasculitis involving immunocomplex deposition in skin vessels
Children and adults
Complication of treatment of rattlesnake envenomation with horse-or sheep-based antivenin
Fever, urticaria with vasculitis, arthralgia, GI pain with melena

Infectious Vasculitis


Disorder
Vasculitis
Epidemiology/Etiology
Clinical/Laboratory Findings
 
 
 
 
Infectious vasculitis
Small vessel vasculitis involving skin vessels
Children and adults
Involves all microbial pathogens
Rocky Mountain spotted fever: tick transmission of Rickettsia rickettsiae; organisms invade endothelial cells; petechiae on palms spread to trunk
Disseminated meningococcemia due to Neisseria meningitidis
Capillary thrombosis produces hemorrhage into skin and confluent ecchymoses

Vascular tumors and tumor-like conditions


Tumor/Condition
Clinical Findings
Angiomyolipoma
Kidney hamartoma composed of blood vessels, muscle, and mature adipose tissue
Association with tuberous sclerosis
Angiosarcoma
Liver angiosarcoma associated with exposure to polyvinyl chloride, arsenic, thorium dioxide
Bacillary angiomatosis
Benign capillary proliferation involving skin and visceral organs in AIDS patients
Simulates Kaposi sarcoma in AIDS
Caused by Bartonella henselae, a gram-negative bacillus
Capillary hemangioma
Facial lesion in newborns that regresses with age
Cavernous hemangioma
Most common benign tumor of liver and spleen
May rupture if large
Cystic hygroma
Lymphangioma in the neck associated with Turner's syndrome
Glomus tumor
Derive from arteriovenous shunts in glomus bodies
Painful red subungual nodule in a digit
Hereditary telangiectasia (AD)
Dilated vessels on skin and mucous membranes in mouth and gastrointestinal tract
Chronic iron deficiency anemia
Kaposi sarcoma
Malignant tumor arising from endothelial cells or primitive mesenchymal cells
Associated with human herpesvirus type 8
Raised, red-purple discoloration that progresses from a flat lesion to a plaque to a nodule that ulcerates
Common sites include skin, mouth, and gastrointestinal tract
Lymphangiosarcoma
Malignancy of lymphatic vessels
Arises out of longstanding chronic lymphedema after modified radical mastectomy
Pyogenic granuloma
Vascular, red pedunculated mass that ulcerates and bleeds easily
Post-traumatic or associated with pregnancy (relation to estrogen); usually regress postpartum
Spider telangiectasia
Arteriovenous fistula (disappears when compressed)
Associated with hyperestrinism (e.g., cirrhosis, pregnancy)
Sturge-Weber syndrome (AD)
Nevus flammeus ("birthmark") on face in distribution of ophthalmic branch of cranial nerve V (trigeminal)
Ipsilateral malformation of pia mater vessels overlying occipital and parietal lobes (leptomeningeal angiomatosis, AV malformation)
Von Hippel-Lindau syndrome (AD)
Cavernous hemangiomas in cerebellum and retina
Increased incidence of pheochromocytoma and bilateral renal cell carcinomas

Source: Goljan