Monilethrix with Acitretin

no known treatment that successfully cures the condition. In this report we present a case of monilethrix in a 7-year-old girl treated with oral acitretin. A very good clinical and cosmetic result was obtained while treatment was continued. However, clinical symptoms recurred after discontinuation of acitretin therapy. Monilethrix is a rare genetic disease that affects hair, and is first been noticed 3 months after birth. Specifically, the hair that specifically characterized by hair shaft anomaly. Fusiform nodes grew in place of the lost hair did not grow long, it broke when it and narrow segments are seen in the hair shafts. Although several reached a certain length and her hair was thinning out in certain autosomal recessive cases have been reported, autosomal domi- areas. Her general history was unremarkable, except for premature nant inheritance is more common. [1-4] It has been reported that the birth and iron deficiency anemia. Her family history was also disease develops because of mutations in genes (chromosome unremarkable. Physical examination revealed that all systems, 12q13) that code hHb1 and hHb6, which are basic hair keratins in including growth and development, were normal. Dermatologic humans. [2,5-8] Lanugo hair in the neonatal period is normal. examination revealed sparse, stiff, dry, short, and irregular hair at Clinical signs appear when terminal hair characteristics begin to bilateral temporo-parietal areas (figure 1). Hair and skin appendform. Alopecia may be seen in the neonatal period in severe ages at other areas were normal. The only dermatologic sign in the cases. [2] The scalp in particular is affected, but the eyebrows, fingernails was slight longitudinal lines. Oral mucosa examination eyelashes, axillary hair, pubic hair, and even body hair follicles was normal. may be involved. There is no known successful treatment of the Laboratory investigations showed that the whole blood count, disease. However, some treated cases have been reported. [1] Here peripheral blood smear, all biochemical values, liver function, we present a case of monilethrix that responded to acitretin treat- serological parameters, and thyroid function were normal. The ment. erythrocyte sedimentation rate was 14 mm/h.