Abstract
Monilehtrix is a rare inherited hair shaft disorder with considerable variations in age of onset severity and course. We present a 6-year-old girl with monilethrix and discuss different aspects of the disease and its treatment.
Highlights
Monilethrix or beading of hair is a mostly autosomal dominant inherited hair shaft disorder with high penetrance but variable expressivity [1]
Light microscopy showed beaded appearance of hair with nodes separated by internodes (Figure 3)
Monilethrix is a mostly autosomal dominant inherited hair shaft disorder caused by mutations in the hHB1, hHB3 and hHB6 keratin gene on chromosome 12q11-q13
Summary
Monilethrix or beading of hair is a mostly autosomal dominant inherited hair shaft disorder with high penetrance but variable expressivity [1]. It usually starts in the first few month of life and persists throughout life. It is characterized by short and fragile hair [2]
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