|LETTER TO EDITOR
|Year : 2019 | Volume
| Issue : 2 | Page : 112-114
Dermoscopy as an additional tool in a case of woolly hair with palmoplantar keratoderma
Shilpitha Srinivas1, Kallappa C Herakal2, Srinivasa Krishnamurthy Murthy3, Shwetha Suryanarayan4
1 Department of Dermatology Venereology and Leprosy, Christian Medical College, Vellore, Tamil Nadu, India
2 Department of Dermatology Venereology and Leprosy, Navodaya Medical College Hospital and Research Center, Raichur, Karnataka, India
3 Skin and Cosmetology Center, Bengaluru, Karnataka, India
4 Department of Dermatology Venereology and Leprosy, Sambhram Institute of Medical Sciences, Kolar Gold Fields, Karnataka, India
|Date of Web Publication||28-Nov-2019|
Department of Dermatology Venereology and Leprosy, Christian Medical College, Vellore, Tamil Nadu
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Srinivas S, Herakal KC, Murthy SK, Suryanarayan S. Dermoscopy as an additional tool in a case of woolly hair with palmoplantar keratoderma. Indian J Dermatopathol Diagn Dermatol 2019;6:112-4
|How to cite this URL:|
Srinivas S, Herakal KC, Murthy SK, Suryanarayan S. Dermoscopy as an additional tool in a case of woolly hair with palmoplantar keratoderma. Indian J Dermatopathol Diagn Dermatol [serial online] 2019 [cited 2020 Feb 27];6:112-4. Available from: http://www.ijdpdd.com/text.asp?2019/6/2/112/271950
Woolly hair is a rare congenital structural anomaly of the hair. It manifests as short kinked hair and can involve the other parts of the body, including the eyebrows, eyelashes, pubic, and axillary hair. Only a few cases of woolly hair have been reported in India. Four types of woolly hair have been described-hereditary woolly hair, familial woolly hair, woolly hair nevus, and symmetrical circumscribed allotrichia. It can also be classified into syndromic or nonsyndromic forms of woolly hair.
A 6-year-old boy born to a second-degree consanguineous marriage came to the outpatient Department with a history of thickening of the skin over the palms and soles since 1st year of life. The parents gave a history of woolly hair over the scalp of the child since birth. Other family members were not affected. On cutaneous examination, the child had diffuse thickened and hyperkeratotic plaques on the palms and soles [Figure 1]a and [Figure 1]b. These plaques were more prominent on the hypothenar area of the palms and on the feet. Teeth and nails were normal. Hair examination revealed short, tightly coiled, poorly pigmented hair over the scalp [Figure 2]a. The scalp hair, palms, and soles were examined with dermalite DL4 with a ×10 using ultrasonography gel as a contact medium. Photographs were taken with Canon IXUS 133. Trichoscopy of the hair revealed a difference in hair shaft diameter [Figure 2]b. Crawling snake appearance was seen on plucking the hair and observing it under the dermoscope. Dermoscopic examination of the palms revealed brownish-to-reddish homogeneous areas with skin lines and a few white scales were visible [Figure 3]a. Light microscopy of the hair revealed mild kinking of the hair. A differential diagnosis of Naxos disease and Carvajal disease was made. Routine blood investigations, lipid profile, chest X-ray, and electrocardiogram revealed a normal picture. Echocardiography revealed situs solitus with levocardia. This was considered to be a normal finding. A 3-mm punch biopsy was done from the left foot and histopathology revealed hyperkeratosis, acanthosis, and focal hypergranulosis and sparse lymphocytic infiltrate in the dermis on ×40 with hematoxylin and eosin stain [Figure 3]b. Genetic screening could not be done. A diagnosis of Unna-Thost type of palmoplantar keratoderma (PPK) with woolly hair was made. Since the child had no significant cardiac anomaly or specific cardiac symptoms, Naxos disease was ruled out. The child was treated with capsule acitretin 10 milligram, topical clobetasol ointment 0.05% with salicylic acid 6.5% ointment, and topical urea-based cream for the upper back. The palmar and plantar lesions resolved by 70% and 30% at the end of 2 months, respectively. Whenever woolly hair is associated with PPK, a search for cardiac disease is warranted. In the case of Naxos disease, woolly hair appears from birth, and PPK appears from the 1st year of life. Arrhythmogenic right ventricular cardiomyopathy appears by adolescence. Carvajal-Huerta syndrome is a cardiocutaneous syndrome with nonepidermolytic PPK, woolly hair and dilated cardiomyopathy. As cardiac symptoms was not present in this case, symptomatic treatment was given and the patient responded to some extent to the treatment. Asymptomatic cardiac disease does not require any treatment. Dermoscopy or epiluminescence microscopy is a tool initially used to diagnose skin tumors and its use has been extended to diagnose inflammatory skin disorders, hair, nails, infections, and infestations. Trichoscopy has been found to be a useful tool to differentiate between hair shaft disorders. In trichoscopy, the hair shaft is examined in vivo for hair shaft disorders under magnification and there is no need of pulling out the hair for examination. This can aid in faster diagnosis of the hair disorder. In the case of woolly hair, it shows crawling snake appearance which is likened to the mythical character Medusa, a character in Greek mythology. Thus, trichoscopy contributed to the rapid and noninvasive diagnosis in this case.
|Figure 1: (a) Hyperkeratotic and thickened plaques on the palms. (b) Hyperkeratotic and thickened plaques on the soles with sparing of the instep of the sole|
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|Figure 2: (a) Hair is short and tightly coiled. Poorly pigmented hair over the scalp. (b) Trichoscopy of the hair in polarized light in ×10 – stars showing a difference in hair shaft diameter and arrow showing crawling snake appearance|
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|Figure 3: (a) Dermoscopic image of the palms in ×10 in nonpolarized light revealed brownish-to-reddish homogenous areas with skin lines and a few white scales were visible. The vascular structures were not visualized. (b) On light microscope examination of histopathology slide with hematoxylin and eosin staining in high power ×40 revealed hyperkeratosis, acanthosis (marked by an arrow) and focal hypergranulosis (marked by a star) and sparse lymphocytic infiltrate in the dermis|
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Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that name and initials will not be published, and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
The authors would like to acknowledge the post graduate residents in Navodaya Medical College Hospital And Research Centre for their support and help in collecting the data for this article.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]