keratosis follicularis spinulosa decalvans ( kfsd ) is a hereditary disorder of the hair follicle which presents with scarring alopecia and follicular papules affecting the scalp and other areas of the body . it usually follows an x - linked pattern of inheritance , but sporadic and autosomal dominant cases can also be seen . our first case was an 18-year - old female who complained of progressive hair loss over the scalp since 10 years and over the eyebrows since 5 years . on examination of the scalp , she had multiple patches of cicatricial alopecia with sparse hair in the frontal , parietal , and temporal areas of the scalp . sparse hair over the eyebrows , eyelashes , axillae , and pubic area were noted [ figure 1 ] . she also had generalized dry skin and multiple follicular papules over upper and lower limbs and buttocks [ figure 2 ] . follicular papules and alopecia seen over the scalp along with sparse eyebrows and eyelashes follicular keratotic papules over dorsum of hand our second case was a 26-year - old male with decreased hair growth over scalp and body . he had no family history of similar complaints . on examination , he had diffuse alopecia of the scalp with sparse hair over eyebrows , eyelashes , and beard area . multiple keratotic papules were noted over the scalp , upper limbs , and abdomen [ figures 3 and 4 ] . sparse hair and papular lesions noted over the scalp follicular papules seen over abdomen the third case was a 23-year - old male with complaints of decreased hair over scalp and body with skin lesions over the trunk . he gave a history of similar but milder complaints being present in his younger brother suggesting x - linked inheritance . on examination , he had alopecia over the crown area of the scalp [ figure 5 ] , with sparse hair over the eyebrows and axillae , multiple follicular papules over the trunk and all four limbs . scarring alopecia seen over scalp plantar keratoderma seen over the foot scalp biopsies from all three cases showed variable amounts of acanthosis and papillomatosis and dilated hair follicles filled with keratin plugs and entrapped hair shafts [ figures 7 and 8 ] . dilated follicular infundibulum filled with keratin plug ( h and e , 40 ) entrapped hair shafts within hair follicle ( h and e , 40 ) keratosis pilaris atrophicans ( kpa ) is a group of cutaneous disorders which are characterized by follicular hyperkeratosis along with scarring . kfsd ( omim 308800 ) is a rare x - linked disorder of the hair follicle . the term was first used by siemens in 1926 when he reported the disorder in a bavarian family . the candidate gene suggested is the membrane - bound transcription factor protease site 2 ( mbtps2 ) gene which is required for cleavage of sterol regulatory element - binding proteins ( srebps ) . altered srebp cleavage impairs cholesterol and lipid homeostasis in the skin causing defective epidermal differentiation . males are more commonly and severely affected than female heterozygotes in families having an x - linked inheritance pattern . the age of onset is early in life with keratotic papules appearing initially on the face and later on the trunk and limbs . as the disease progresses , cicatricial alopecia ensues on the scalp , eyebrows , and eyelashes along with the loss of hair in axillary and pubic areas . the most important differential diagnoses include ichthyosis follicularis alopecia photophobia ( ifap ) syndrome and graham little piccardi lasseur syndrome ( glpls ) . however , the alopecia in ifap syndrome is of the nonscarring type while that in kfsd is scarring . furthermore , ocular features like photophobia and corneal dystrophy are essential for the diagnosis of ifap . glpls is a variant of lichen planopilaris which presents with scarring alopecia of the scalp , nonscarring alopecia of axillae and pubic region , and keratotic follicular spinous papules over a body . the distinction from kfsd is made mainly on histopathology which reveals changes suggestive of lichen planus including vacuolar degeneration and interface dermatitis . systemic retinoids like isotretinoin and etretinate are beneficial in the early stages of the disease as they downregulate follicular hyperkeratosis and inflammation . dapsone was found to be useful due to its inhibition of leukocyte chemotaxis and stabilization of lysosomal enzymes . the authors certify that they have obtained all appropriate patient consent forms . in the form the patient(s ) has / have given his / her / their consent for his / her / their images and other clinical information to be reported in the journal . the patients understand that their names and initials will not be published and due efforts will be made to conceal their identity , but anonymity can not be guaranteed . the authors certify that they have obtained all appropriate patient consent forms . in the form the patient(s ) has / have given his / her / their consent for his / her / their images and other clinical information to be reported in the journal . the patients understand that their names and initials will not be published and due efforts will be made to conceal their identity , but anonymity can not be guaranteed .