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livedo reticularis; livedoid vasculopathy; mottled skin; cutis marmorata
Classification:
1) cutis marmorata
a) mottling appears on exposure to cold
b) mottling disappears on warming
2) livedo reticularis idiopathica
a) persists on warming
b) not associated with known diseases
c) occurs in persons with unstable nervous systems
d) ulceration is a complication
3) livedo reticularis symptomatica
a) persists on warming
b) frequently accompanies polyarteritis nodosa
c) ulceration is a complication
Etiology:
1) neurohumoral disease
a) pheochromcytoma
b) carcinoid syndrome
2) hematologic disease
a) polycythemia vera
b) leukemia
c) thrombocytosis
d) cold agglutinin syndrome [6]
3) hypercoagulable state (thrombophilia)
- anti-phospholipid antibody syndrome
4) paraproteinemia
- multiple myeloma & associated cryoglobulinemia type-1
- Waldenstrom's macroglobulinemia & other hyperviscosity syndromes
5) autoimmune disease (connective tissue disease)
a) systemic lupus erythematosus (SLE)
b) dermatomyositis
c) rheumatoid arthritis
1] Still disease
2] Felty syndrome
d) scleroderma
e) Sjogren's syndrome
6) infections
a) parvovirus B19
b) hepatitis C
c) syphilis
d) meningococcemia
e) pneumococcal sepsis
f) tuberculosis
7) drug reactions
a) amantadine
b) quinidine
c) warfarin
d) minocycline
8) vasculitides (vasculitis)
a) polyarteritis nodosa
b) Wegener's granulomatosis
c) microscopic polyarteritis
d) Churg-Strauss syndrome
e) Takayasu arteritis
f) temporal arteritis
9) calciphylaxis
10) cholesterol embolization
a) livedo reticularis over the legs & thighs
b) embolization generally involves digits
- ischemia, painful
c) prior vascular catheterization
11) endocarditis
12) microvascular occlusion syndromes
a) hemolytic uremic syndrome
b) thrombotic thrombocytopenic purpura
c) disseminated intravascular coagulation
d) heparin necrosis
e) paroxysmal nocturnal hemoglobinuria [2]
13) cryoglobulinemia
Epidemiology:
- mean age 45 years
- 71% women
Pathology:
1) stagnation of blood in dilated superficial capillaries & venules
2) pathologic changes in larger vessels deeper in the skin
3) hyalinizing vascular disease
4) 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]
* images [3,6]
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%
Related
livedoid vasculitis
General
sign/symptom
vascular disease (vasculopathy)
References
- DeGowin & DeGowin's Diagnostic Examination, 6th edition,
RL DeGowin (ed), McGraw Hill, NY 1994, pg 455
- Medical Knowledge Self Assessment Program (MKSAP) 11, 15, 16,
17, 18. American College of Physicians, Philadelphia 1998, 2009,
2012, 2015, 2018.
- DermNet NZ. Livedo reticularis (images)
http://dermnetnz.org/vascular/livedo-reticularis.html
- Scheinfeld NS, James WD
Medscape: Livedoid Vasculopathy
http://emedicine.medscape.com/article/1082675-overview
- 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
- Sharma K, Patel A.
Livedo Reticularis in Cold Agglutinin Disease.
N Engl J Med 2019; 381:e27
PMID: 31553839
https://www.nejm.org/doi/full/10.1056/NEJMicm1902289