###Rationale: Ans. is 'b' i.e.. Primary systemic amyloidosis o Purpura of the eyelids following pinching (pinch purpura) is a characteristicfeature of primary systemic amyloidosiso Pinch purpura of the eyelids are seen in about 30% of the cases ofprimary systemic amyloidoses.o It occurs due to involvement of cutaneous blood vessels in primary1 systemic amyloidosis.o The most common skin lesions are pete hie or ecchymoses due to amyloid deposition within blood vessel walls with subsequent fragility and dermal hemorrhage.o These are o ften seen at sites predisposed to trauma, such as the hands or intertriginous areas.o Pinching the skin gives charaterstic purpura lesions known as "pinch purpura".o Purpura around the eyes may occur spontaneously but is also seen following proctoscopy or vomiting (postproctoscopic purpura).o Dermatological involvement occur in about 30% - 40% patients with systemic amyloidosis.o Other dermatological manifestations include waxy or translucent papules and lets frequently tense hemorrhagic bullae.o Histopathology of the cutaneous lesions reveal amyloid deposits in the dermis.o Occular findings in primary generalized amyloidosis includes :Purpura of the eyelids.B/l symmetrical small amyloid papules of the skin of the eyelids.PtosisOphthalmalgia or evidence of amyloid neuropathes affective pupillary function or both.Subconjunctival hemorrhages.

###Answer: OPTION B IS CORRECT.
###Question: Pinch purpura around eyelids is one of the most common finding in -
###Options:
A. Fabry's disease
B. Primary systemic amyloidosis
C. Porphyria cutanea tarda
D. None of the above
