a 32-year - old male came to us with complaints of cystic lesions in both lids [ figs . 1 , 2a and b ] . he was diagnosed to have lipoid proteinosis elsewhere and was completely screened for systemic involvement . a buccal mucosal biopsy taken elsewhere showed deposition of pas - positive hyaline material [ fig . 3 ] . there were verrucous lesions in both upper and lower lids with cauliflower shaped lesion in the lower puncta of both eyes . . external photograph of the eye external photograph closer view ( a ) upper lid ( b ) lower lid histopathology picture depicting pas positive hyaline material uws is an autosomal recessive disorder , first described by urbach and wiethe in 1929 . the incidence of hyalinosis cutis et mucosae seems to be fairly high in south africa . it is a multi - system disease caused due to mutations in the gene encoding extracellular matrix protein 1 on chromosome 1q21 . skin lesions generally appear as nodules on the face , lips ( at earlier stages ) , and later become hyperkeratotic . schirmer 's test in both eyes was normal suggesting no involvement of lacrimal gland in our case . various ocular manifestations such as dry eyes , open angle glaucoma , drusen in the macula , retinitis pigmentosa , uveitis and subluxation of the lens has been reported along with lipoid proteinosis . however , our patient had no abnormality in his eyes . the most common radiological hallmark is the presence of bean or comma shaped intracranial calcifications in the temporal lobes in the amygdala , which is more evident in the patients who have lipoid proteinosis for a long duration . patients with neurological manifestations present with a migraine , seizures , mental retardation , anxiety , depression , and panic attacks . though rare , systemic manifestations of lipoid proteinosis include life - threatening situations like acute respiratory distress or seizures . awareness among ophthalmologists about this rare entity is crucial for appropriate management of these patients .