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Î SS 176. Spondyloenchondrodysplasia with immune dysregulation
(SPENCD) should be suspected in patients presenting with
characteristic osseous lesions, CIDs, autoimmunity, and neurological
disorders. (C)
Î SS 177. Therapy of type 1 interferonopathies should be directed
toward infectious and autoimmune complications. (C)
Warts, hypogammaglobulinemia, immunodeficiency, and
myelokathexis syndrome (WHIM)
Î SS 178. WHIM syndrome should be suspected in patients
who manifest the principal characteristics. (C)
Î SS 179. Treatment of WHIM syndrome should include IgG
replacement, G-CSF therapy, or both to reduce the incidence of
infection. (C)
Î SS 180. Vaccination against HPV should be considered in patients with
WHIM syndrome. (C)
Î SS 181. Hematopoietic stem cell transplantation should be considered
for patients with WHIM syndrome. (C)
Epidermodysplasia verruciformis (EV)
Î SS 182. EV should be suspected in patients with diffuse verrucosis
caused by HPV. (C)
Î SS 183. Primary therapy for EV should involve avoidance of UVB and
radiation exposure and frequent dermatologic screening for skin
cancer. (C)
Î SS 184. Patients with severe viral illnesses (especially disseminated
vaccine strain measles) should be studied for mutations in STAT2. (C)
Susceptibility to herpes simplex encephalitis (HSE)
Î SS 185. Patients with HSE should be tested for one of the known
associated gene defects. (C)
Î SS 186. Antiviral prophylaxis should be considered for patients with
defects in UNC93B1, TLR3, TBK1, TRIF, and TRAF3. (F)
