olmsted syndrome ( os ; mutilating palmoplantar keratoderma and periorificial involvement ) is characterized by the presence of congenital , diffuse , symmetric , sharply marginated , mutilating palmoplantar keratoderma with periorificial hyperkeratosis . three siblings of age 12 , 10 , and 6 years born of parents with consanguineous marriage , full term normal delivery with normal developmental milestones were brought with complaints of hair loss and palmoplantar keratoderma at the age of 23 years . gradually lesions extended to knees and elbows . on examination , children were moderately built and nourished . there was palmoplantar keratoderma in all three siblings [ figures 1 and 2 ] . in two children , severe cheilitis , glossitis and keratotic plaques , and fissures around mouth were present . scalp showed sparse hair with keratotic papule and follicular prominences , few scattered hair were thin and grew up to 12 cm in length [ figure 3 ] . palmoplantar keratoderma clinical photograph of palmoplantar keratoderma scalp showing sparse hair with keratotic papule and follicular prominences trichoscopy showed black dots , yellow dots in plenty . tapered and thin hair , angulated hair , kinky gray hair , also had comma - shaped ,  v hair , broken hair , and vellus hair . the gap between hair follicles was more with follicular prominences [ figure 4 ] . trichoscopy findings of  v  shaped hair , broken hair , black dots , yellow dots , and follicular prominences os is a rare congenital disorder characterized by palmoplantar and periorificial keratoderma , alopecia in most cases , and severe itching . until recently , only 46 individuals had been reported , including 36 sporadic cases and four families containing ten affected individuals . the definitive mode of inheritance was still uncertain , and autosomal - dominant , x - linked - dominant , and x - linked - recessive modes of inheritance had been proposed . they suggest that this condition is inherited as an x - linked dominant trait with reduced expression in female subjects . a case of a 6-year - old indian girl in one report and two more unrelated female cases in another were reported . whereas in our case , three siblings in a single family were affected , out of which two were females . the two major ones are the symmetrical involvement of keratoderma of the palms and soles , and the symmetrical hyperkeratotic plaques around the body orifices . it starts in the neonatal period or in childhood . in our case , we have palmoplantar keratoderma , glossitis , cheilitis , and keratotic plaques around the mouth , sparse scalp hair with follicular prominences , hypopigmented macules over scalp and psoriasiform plaques were seen on elbows and knees . os patients often show nail abnormalities including dystrophic , lusterless , ridged , rough nails , hyperkeratosis , onychogryphosis , leukonychia , irregular curvatures , onycholysis , paronychia , subungual hyperkeratosis , and even absence of nails . in our case , we had paronychia and irregular dystrophy of finger and toe nails . report a case of this rare syndrome diagnosed in a 48-year - old woman , who developed several squamous cell carcinomas of limbs and adenocarcinoma of the lung . we have reported this case to highlight the trichoscopy findings , female preponderance , and rarity in occurrence .
olmsted syndrome ( os ) is a rare disorder characterized by the combination of periorificial , keratotic plaques , and bilateral palmoplantar keratoderma . 
 synonyms are mutilating palmoplantar keratoderma with periorificial keratotic plaques ( orpha659 , mim # 614594 and # 300918 ) . a number sign ( # ) 
 is used with this entry because of evidence that mutilating palmoplantar keratoderma with periorificial keratotic plaques ( os ) is caused by heterozygous mutation in the trpv3 gene on chromosome 17p13.2 . 
 we report three cases of os , two females and one male in the same family , who presented with palmoplantar keratoderma , sparse scalp hair , cheilitis , and periorificial fissures . 
 we are reporting the cases due to the rarity of occurrence and to highlight the trichoscopy findings .