lipoid proteinosis , also known as hyalinosis cutis et mucosae ( omim 247100 ) , was first described by urbach and wiethe in 1929 . since then , over 250 cases of this autosomal recessive disorder have been described . it is characterized by the deposition of an amorphous hyaline substance ( glycoprotein ) in the mucous membrane and skin . hoarseness and thickening of vocal cords are the most characteristic symptoms present from infancy or early childhood.3 
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 4 another classic and most easily recognizable sign is the beaded eyelid papules . the other cutaneous changes may include waxy , yellow papules and nodules with generalized skin thickening . the mucosa of the pharynx , tongue , soft palate , tonsils and lips is also infiltrated.6 
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 7 other features may include epilepsy and calcification in the temporal lobes or hippocampi . 
 
 since suturing is difficult because of rigid mucosa in these patients , the use of cryosurgery seems to offer advantages for removing oral nodular lesions . therefore , cryosurgery ( n2o ) was performed to remove and reshape the lip lesions in a case of this rare syndrome . the patient was a 24-year - old female from shahrekord , central iran , who sought medical care at the department of oral medicine , isfahan university of medical sciences in 2003 . the patient was completely edentulous and showed the classic signs of lipoid proteinosis ( figure 1 ) . during oral examination , the patient had six sisters and one brother , and two of her sisters ( 25 and 11 years old ) suffered from the same syndrome . the results of complete blood analysis and routine urinalysis were all within the normal limits . the biopsy of lip lesions was performed with a scalpel under local anesthesia and examined by a pathologist to confirm the diagnosis . suturing after biopsy was difficult because the mucosa was rigid and the sutures cut into it . the patient suffered from some nodules on upper lip . after applying local anesthetic spray on the site , cryosurgery ( n2o , c 502 t , emd co ; tehran , iran ) was performed ( 63c for one minute ) , as this treatment has been used for similar superficial lesions . the surgery was done in two sessions ; half of the lip was involved in each ( figure 2a , b ) . the superficial layers were fallen by day 3 . after the operation , the patient had pain for about a week with a visual analogue scale of up to five . follow up , no new nodules were formed ; however , some fissures were created on her lip ( figure 2c ) . after the treatments , the appearance and the emotional state of this young female had improved . lipoid proteinosis is a rare autosomal recessive disorder accompanied by hoarseness as well as infiltration and thickening of the skin and certain mucous membranes . recently , lipoid proteinosis was mapped to 1q21 and pathogenetic loss - of - function mutations were identified in the extracellular matrix protein 1 ( ecm1 ) gene.2 
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 4 some treatments for this disorder have already been reported . a case of lipoid proteinosis was successfully treated with dimethyl sulfoxide , however , in three other cases , it showed no beneficial effect . retinoids have shown encouraging results in cutaneous lesions in previous studies.12 
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 13d - penicillamine had a favorable effect in a patient with this syndrome . microlaryngoscopy and dissection of the vocal cords , dermabrasion , chemical skin peeling and blepharoplasty were performed in some cases.5 
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 1 lasers are used in many fields of medicine and dentistry . carbon dioxide laser surgery of thickened vocal cords and beaded eyelid papules has proved to be helpful in some studies.2 
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 1 nd : yag and er : yag lasers were applied to remove oral lesions in one case of this syndrome . cryosurgery is the use of very low temperatures to destroy tissue . immediately following cryosurgery , tissues are virtually indistinguishable from the normal , but latent damage is produced which progresses to severe destruction of the tissue . it is a safe , effective method of treatment for many benign soft tissue lesions in the oral cavity . as suturing was difficult in this patient and mucosa was rigid , the use of cryosurgery seemed to offer some advantages in removing oral lesions in this case . it seems that surgical methods like cryosurgery and laser1 
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 1 have some advantages over conventional surgery for removing oral lesions of lipoid proteinosis syndrome as there is no bleeding and no need to suture , and it is more comfortable for the patient . the described approach is considered an early report of n2o cryosurgery being used in removal and reshaping of lip lesions of this rare syndrome .