defining characteristics
TONS of BCCs as child, megaloblastomas, palmar pits, bifid ribs, frontal bossing of scalp
disease development
Deficiency in patch (negative regulator of Shh) causes constitutive action of positive regulator (Shh), increasing incidence of BCC
Shh binds and inhibits patch; without patch, smoothened causes increased transcription of GLI-1,2,3, causing increased cell proliferation
potential causes
epidemiology
risk factors
labimaging
conventional treatment
Vismodegib (targets Shh to decrease tumor size; but still residual tumor so perhaps development of resistance)
complications
Loss of taste (and ultimately anorexia) is a side effect of tx
