The Medusa Head: Dermoscopic Diagnosis of Woolly Hair Syndrome

Abstract

Sir, Hair abnormalities are extremely common in genoder-matoses. Hair changes may be a significant finding or even the initial presentation of a syndrome giving the clue to the diagnosis, in diseases such as trichothiodystrophy, Netherton syndrome, or Woolly hair syndrome. Hair shaft abnormalities encompass a group of congenital or acquired alterations, which involve the hair shaft. They often lack macroscopic features, which would enable easy diagnosis in medical practice. Thus, the usual diagnostic method is light microscopy and every time about 50 hairs are plucked to decrease the risk of missing a hair with the characteristic abnormality under light microscopy.[1] Dermoscopy is a method based on epiluminescence microscopy, which is used to visualize living skin in magnification. Videodermoscopy of hair and scalp (trichoscopy) is a new method, which allows viewing hair shafts in vivo in many-fold magnification without the need of pulling hair for diagnostic purposes of examination.[2 3] We report here a case of a 25-year-old male, who presented with abnormal hair on scalp since infancy. He complained that the hairs were easily broken, slow-growing, and that they seldom required cutting. He was born out of a first-degree consanguineous marriage and there was a history of similar problem in his sister. His medical history was unremarkable. On physical examination, his scalp hairs were evenly pigmented, coarse-textured, and extremely curly [Figure 1]. His eyebrows, eyelashes, body hair, and skin were normal in appearance. Nails, teeth, and genitals were normal. Figure 1 Evenly pigmented, coarse-textured, and extremely curly scalp hair Two-dimensional color echocardiography and electrocardiography were performed, but abnormal findings were not observed. Microscopic examination of hair was essentially normal. Hematological and biochemical parameters were also normal. Trichoscopy of hair shafts demonstrated a “crawling snake” appearance, with short wave cycles under 20× magnification [Figure 2a and b]. We liken this appearance to the head of the mythical character Medusa, a character in Greek mythology, whose scalp hair was made of living snakes.[4] Apart from that, the examination demonstrated broken hair shafts. Figure 2 (a) Crawling snake appearance of hair under dermoscopy (20×); (b) Evenly pigmented hair with difference in hair shaft diameter and “crawling snake” appearance In woolly hair syndrome, the term “woolly hair” refers to an abnormal variant of fine, tightly curled hair that often exhibits decreased pigmentation. In 1974, Hutchinson et al.[5] classified woolly hair into three variants: woolly hair nevus, autosomal dominant hereditary woolly hair, and autosomal recessive familial woolly hair. Autosomal recessive variant can be syndromic and associated with palmoplantar hyperkeratosis and cardiovascular abnormalities. Our case was likely to be autosomal recessive type of woolly hair syndrome without systemic involvement. Woolly hair syndrome is most pronounced in childhood and becomes less severe with adulthood. Very few cases of familial wooly hair syndrome have been reported in non-negroid races. We report this case to emphasis on the value of trichoscopy in diagnosing woolly hair syndrome, without using light microscopy. This can be applied to most of the hair shaft disorders where characteristic light microscopy features may be visualized by trichoscopy without the need of plucking hairs for diagnostic purposes.