Moniliform blepharosis: A characteristic sign of lipoid proteinosis

A 28-year-old man presented to the dermatology outpatient department with a coarse-looking face, waxy papules, verrucous plaques and scars over the body. He also had hoarse voice since childhood.

Cutaneous examination revealed a row of waxy, yellow–white, beaded papules along the margin of upper eyelid [Figure 1]. The eyelashes had grown out normally, but the papules appeared to be encircling them. This is referred to as moniliform blepharosis because it appears like a string of beads. It is a pathognomonic feature of lipoid proteinosis, an autosomal recessive hyaline deposition disorder, and is caused by mutations in the extracellular matrix protein 1 gene.

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Figure 1:: Row of waxy, yellow–white, beaded papules along the margin of the upper eyelid

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