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Chronic mucocutaneous candidiasis (CMCC)
Î SS 187. Patients who present exclusively with recurrent Candida
species infection of nails, skin, and mucous membranes should be
considered for the diagnosis of CMCC. (C)
Î SS 188. Evaluation of patients with suspected CMCC should include
NK cell numbers and functional studies and assessment of T-cell
response to Candida species. (C)
Î SS 189. Antifungal agents should be the mainstays of therapy for
CMCC. (C)
Susceptibility to trypanosomiasis
Î SS 190. Patients with sleeping sickness caused by Trypanosoma
evansi should be studied for mutation in the apolipoprotein L1
(APOL1) gene. (C)
Selective NK cell defects
Î SS 191. Patients with severe disease caused by herpesviruses or
papillomaviruses who do not have another defined immunodeficiency
should have phenotypic and functional assessments of NK cells. (C)
Î SS 192. Patients with a selective functional NK cell defect should be
screened for mutations affecting CD16 by using flow cytometry with
the anti-CD16 clone B73.1. (C)
Î SS 193. Patients with growth retardation, adrenal insufficiency, and
NKcell deficiency should be tested for the MCM4 mutation. (C)
Î SS 194. Patients with selective NK cell defects might benefit from
specific chemoprophylaxis against herpesviruses and vaccination
against HPV. (C)
Isolated congenital asplenia (ICA)
Î SS 195. Patients presenting with a family history of asplenia or sepsis
caused by encapsulated bacteria, most frequently S. pneumoniae,
should be evaluated for congenital asplenia.
Î SS 196. Patients with ICA should be started immediately on antibiotic
prophylaxis and receive vaccination for encapsulated organisms.
Defects of Innate Immunity
