Scanty, short scalp hair along with keratotic papular lesions on the scalp of a 7-year-old boy.

A 7-year-old boy presented in our skin OPD with scanty, brittle, and short scalp hair since birth. There was no history of consanguinity in his parents and no family member had similar disease. On light microscopic examination, there was characteristic beading and internodal thinning of hair shaft. There were multiple, keratotic papules on the entire scalp which was extending to the forehead and neck [Figure 1]. Detailed physical examination was done and no dental defects or mental retardation was found.

Figure 1

Keratotic papular lesions with scanty, short, brittle hair on the scalp

Monilethrix is a rare condition, characterized by the presence of beaded shaft of the scalp hair. Hair shaft shows a beaded appearance due to alternate zones of spindle-like thickening and thinning placed about 0.7 to 1 mm apart. Swelling represents normal part of the hair, whereas narrowing part is abnormal. It is usually inherited as an autosomal dominant trait and rarely can be autosomal recessive.[1] These changes are usually associated with a follicular hyperkeratosis or keratosis pilaris.[2] Its pedigree presentation is suggestive of hereditary transmission. One case of monilethrix with pedigree was reported by Solomon and Green.[3] Many therapies like oral steroids, retinoids, and immunosuppressive drugs are suggested.[3] In our case, there was no similar illness in family members. It was a sporadic case. We have given low dose isotretinoin to the patient (0.5 mg/kg body weight) and the patient is on follow-up.