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erythromelalgia (erythermalgia)
Episodic bilateral painful vasodilation & erythema. The antithesis of Raynaud's disease
Etiology:
1) idiopathic, genetic
2) associated with:
a) warm temperature
b) exercise
c) myeloproliferative disease
- polycythemia vera [4]
d) thrombocytosis
- essential thrombocythemia [4]
e) hypertension
f) hemiplegia
g) multiple sclerosis
h) tabes dorsalis
i) dementia paralytica
j) autoimmune disease
k) pregnancy
l) vasodilators
Epidemiology:
- up to 28% of patients with polycythemia vera [4]
Genetics:
1) may be associated with mutations in SCN9A gene
2) autosomal dominant form(s)
Clinical manifestations:
1) episodic presentation
2) bilateral extremity distribution
3) localized burning of the skin on the extremities including palms & soles
a) exacerbated by warm temperatures &/or fever
b) ameliorated by cool temperatures
4) vasodilation
a) swelling
b) warmth
c) erythema, pallor or cyanosis [4]
d) perspiration
e) burning pain
3) symptoms aggravated by dependent position
Management:
1) symptoms attenuated by aspirin
2) normalizing thrombocytosis with chemotherapy
Related
Raynaud's phenomenon
General
genetic disease of blood vessels
sign/symptom
Database Correlations
OMIM 133020
References
- Saunders Manual of Medical Practice, Rakel (ed),
WB Saunders, Philadelphia, 1996, pg 289, 1051
- DeGowin & DeGowin's Diagnostic Examination, 6th edition,
RL DeGowin (ed), McGraw Hill, NY 1994, pg 884
- Medical Knowledge Self Assessment Program (MKSAP) 16,
American College of Physicians, Philadelphia 2012
- Geriatric Review Syllabus, 9th edition (GRS9)
Medinal-Walpole A, Pacala JT, Porter JF (eds)
American Geriatrics Society, 2016