Working Diagnosis:
Atypical primary vasospasm
Treatment:
Given lack of efficacy from nifedipine, recommendations to primary care provider were given for initiation of alpha blocker (low dose doxazosin or prazosin) vs sildenafil or endothelin antagonist.
Patient is currently considering medication options and activity modification for symptom management.
Outcome:
Patient is cleared to return to swimming, modifying her activity based on symptoms.
Author's Comments:
Primary vasospasm can be thought of as an exaggerated response to the body's normal vasospasm in cold temperatures. This may also be triggered by emotional stress.
May affect 3-5% of the global population with a shift in higher prevalence in colder climates, and may be familial. Females are disproportionately affected greater than males, 7:1.
"Triphasic" color pattern changes of pallor, cyanosis and rubor are characteristic for vasospasm.
Primary vasospasm must be differentiated from secondary vasospasm, which may have additional complications.
In our patient, this phonomania was not entirely temperature dependent. There were no identified factors to attribute this vasospastic process to. Our patient's vasospastic process was discoordinated, involving patchy areas in both feet. Typically primary vasospasm is thought of as a symmetric process. Generally this affects the hands primarily; however in our athlete the feet were most affected.
Editor's Comments:
Interesting case to be sure, with impressive visuals.
References:
1- Maverakis E et. al. International consensus criteria for the diagnosis of Raynaud's phenomenon. J Autoimmun. 2014 Feb-Mar;48-49:60-5. PMID: 24491823; PubMed Central PMCID: PMC4018202.
2- Linnemann B, Erbe M. Raynaud's phenomenon - assessment and differential diagnoses. Vasa. 2015 May;44(3):166-77. doi: 10.1024/0301-1526/a000426. Review. PubMed PMID: 26098320.
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