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THE NAEVOID BASAL CELL CARC INOMA (GORL IN ’S SYN)

SYS i nvo lved : sk in , ske le ta l sys (60 - 70%) , endoc r ine sys , CNS

AUTOSOMAL DOMINANT

Basa l ce l l ca rc inomas o f the sk in

Ben ign cys t s o f the j aw

Anoma l ies o f r i bs & sp ine

Dyskera toses o f the pa lms o f the hand & so les o f the f ee t

CALC IF ICAT ION OF FALX CEREBR I

RADIOLOGY:

- Mul t ip le mand ibu la r & max i l l a ry odontogen ic ke ra tocys t s a re


charac te r i s t i c (65 - 75%) . Observed i n the f i r s t decade & cause
l oosen ing & l o s s o f tee th . Cys ts (<2cm) a re tw ice more
common i n mand ib le than max i l l a .

- Kyphosco l i os i s (50 %)

- Segmenta l anoma l ies o f ver tebra .

- R ib anoma l ies – b i f i d , f used , broad ant . Ends .

- Arachnodac ty ly / po lydac ty ly

- Fron ta l & b ipa r ie ta l boss ing .

DD: ca l c i f ac t i on o f f a l x + mul t ip le cys t s i n the j aw – CHARCOT-


MARIE -TOOTH DISEASE .