livedo reticularis; livedoid vasculopathy; mottled skin; cutis marmorata
Jump to navigation
Jump to search
Classification
- cutis marmorata
- livedo reticularis idiopathica
- persists on warming
- not associated with known diseases
- occurs in persons with unstable nervous systems
- ulceration is a complication
- livedo reticularis symptomatica
- persists on warming
- frequently accompanies polyarteritis nodosa
- ulceration is a complication
Etiology
- neurohumoral disease
- pheochromcytoma
- carcinoid syndrome
- hematologic disease
- hypercoagulable state (thrombophilia)
- paraproteinemia
- multiple myeloma & associated cryoglobulinemia type-1
- Waldenstrom's macroglobulinemia & other hyperviscosity syndromes
- autoimmune disease (connective tissue disease)
- infections
- drug reactions
- vasculitides (vasculitis)
- calciphylaxis
- cholesterol embolization
- livedo reticularis over the legs & thighs
- embolization generally involves digits
- ischemia, painful
- prior vascular catheterization
- endocarditis
- microvascular occlusion syndromes
- cryoglobulinemia
Epidemiology
- mean age 45 years
- 71% women
Pathology
- stagnation of blood in dilated superficial capillaries & venules
- pathologic changes in larger vessels deeper in the skin
- hyalinizing vascular disease
- thrombosis & ulceration of the lower extremities[4]
Clinical manifestations
- mottling of the skin of the arms &/or legs with round patches of normal skin in a background of cyanotic-appearing skin
- macular, lesions not elevated
- more commonly affects lower extremities (bilateral in 80%)
- lesions of livedo reticularis blanch on diascopy[5]
- may be painful[4]
- ulceration (69%)
- may occur during pregnancy[4]
Laboratory
- factor V Leiden mutation (22%)
- prothrombin G20210A gene mutation (8%)
- lupus anticoagulant (18%)
- anticardiolipin Ab (29%)
- decreased protein C activity (13%)
- serum homocysteine (homocysteinemia in 14%)
- vascular biopsy show intraluminal thrombosis in 98%
More general terms
Additional terms
References
- ↑ DeGowin & DeGowin's Diagnostic Examination, 6th edition, RL DeGowin (ed), McGraw Hill, NY 1994, pg 455
- ↑ Jump up to: 2.0 2.1 Medical Knowledge Self Assessment Program (MKSAP) 11, 15, 16, 17, 18. American College of Physicians, Philadelphia 1998, 2009, 2012, 2015, 2018.
- ↑ Jump up to: 3.0 3.1 DermNet NZ. Livedo reticularis (images) http://dermnetnz.org/vascular/livedo-reticularis.html
- ↑ Jump up to: 4.0 4.1 4.2 4.3 Scheinfeld NS, James WD Medscape: Livedoid Vasculopathy http://emedicine.medscape.com/article/1082675-overview
- ↑ Jump up to: 5.0 5.1 Rashid RM, Barros BS (image) Hidden Heart Disease: 19 Dermatologic Clues You Should Know. Medscape. June 13, 2017. http://reference.medscape.com/slideshow/hidden-heart-disease-6004452
- ↑ Jump up to: 6.0 6.1 6.2 Sharma K, Patel A. Livedo Reticularis in Cold Agglutinin Disease. N Engl J Med 2019; 381:e27 PMID: https://www.ncbi.nlm.nih.gov/pubmed/31553839 https://www.nejm.org/doi/full/10.1056/NEJMicm1902289