IJD
Indian Journal of Dermatology
  Publication of IADVL, WB
  Official organ of AADV
Indexed with Science Citation Index (E) , Web of Science and PubMed
 
Users online: 1897  
Home About  Editorial Board  Current Issue Archives Online Early Coming Soon Guidelines Subscriptions  e-Alerts    Login  
    Small font sizeDefault font sizeIncrease font size Print this page Email this page


 
Table of Contents 
E-IJD CASE REPORT
Year : 2015  |  Volume : 60  |  Issue : 5  |  Page : 524
Woolly hair with systematized epidermal nevus


Department of Dermatology, Venereology and Leprology, Government Medical College Kota, Rajasthan, India

Date of Web Publication4-Sep-2015

Correspondence Address:
Sarita Kalwaniya
Mirdha Nagar, Didwana Road, Kuchaman City, Nagaur, Rajasthan - 341 508
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.159658

Rights and Permissions

   Abstract 

Woolly hair is a hair shaft disorder characterized by fine and tightly curled hair. Woolly hair could be syndromic (associated with systemic disease) or non-syndromic (not associated with any systemic disease). Woolly hair is described in association with many skin, dental, ophthalmic and cardiac anomalies but association of woolly hair with bilateral systematized epidermal nevus described in our case is first of its kind.


Keywords: Hair shaft disorder, systematized epidermal nevus, woolly hair


How to cite this article:
Kalwaniya S, Morgaonkar M, Jain SK, Nyati A. Woolly hair with systematized epidermal nevus. Indian J Dermatol 2015;60:524

How to cite this URL:
Kalwaniya S, Morgaonkar M, Jain SK, Nyati A. Woolly hair with systematized epidermal nevus. Indian J Dermatol [serial online] 2015 [cited 2021 Jul 23];60:524. Available from: https://www.e-ijd.org/text.asp?2015/60/5/524/159658

What was known?
Woolly hair is a hair shaft disorder which is reported in isolation or with multiple associated skin, cardiac, dental and ophthalmic abnormalities. Localized variant of woolly hair (Woolly hair nevus) is frequently described in association with a linear epidermal nevus.



   Introduction Top


Woolly is tightly coiled, fine and often hypopigmented hair. It was first described by Gossage in 1907 and later classified by Hutchinson et al., in 1974 into woolly hair nevus, autosomal dominant hereditary woolly hair and autosomal recessive familial woolly hair. [1] Generalized woolly hair can occur in isolation or it can have syndromic association. Association of generalized woolly hair is also described with cutaneous anomalies, such as keratosis pilaris or palmoplantar keratoderma, and extracutaneous anomalies, especially cardiac or of senses. Localized variant of woolly hair (Woolly hair nevus) is frequently described in association with a linear epidermal nevus; however, no such association is described with generalized woolly hair. We describe the first case of generalized woolly hair in association with bilateral systematized epidermal nevus.


   Case Report Top


A 6-year-old boy presented with abnormally curly scalp hairs and patches of darker skin over whole body, most evident over neck, trunk and arms. According to history given by parents, the child was born full term of non-consanguineous marriage with dark linear and whorled patches over his whole body including scalp and without hairs over scalp. When scalp hairs first grew, they were abnormally curly and slow growing.

On examination the child had whorled and streaked linear hyperpigmented verrucous plaques affecting the whole body including scalp. Scalp hairs were coarse, lusterless, dry and tightly curled [Figure 1]. Seborrhoeic dermatitis was also present over scalp. Nail and teeth were normal. Investigations revealed normal hematological and biochemical parameters. Light microscopy of scalp hair revealed thin and hypopigmented hair [Figure 2]. There was no evidence of trichorrhexis nodosa. X-ray chest, electrocardiogram and echocardiography were normal. The child had normal IQ without any eye problem. The patient had a younger sister who had absolutely normal hair and skin. Also, parents of the patient did not have similar complaint.
Figure 1: Clinical image of the patient showing woolly hair and systemised epidermal nevus (a, b). Close view of neck (c, d) and head (e) better shows the findings

Click here to view
Figure 2: Hair microscopy-arrow showing hypopigmented and thin woolly hair lying alongside a normal hair of a child of same age

Click here to view


Histopathology of skin lesion [Figure 3] revealed papillated epidermal hyperplasia, with hypergranulosis and hyperkeratosis composed of compact ortho and parakeratosis consistent with epidermal nevus.
Figure 3: Histopathology (Hematoxylene and Eosin stain, ×40 magnification) showing papillated epidermal hyperplasia, with hypergranulosis and hyperkeratosis composed of compact ortho and parakeratosis

Click here to view



   Discussion Top


The term "woolly hair" has been derived due to coiling and crimping of hair similar to sheep wool. [2],[3] Woolly hair is a rare congenital structural abnormality of the scalp hair without increased fragility, characterized by tightly coiled hair involving part or the entire scalp occurring in a non-negroid individual. [1],[3] Woolly hair grows at a normal or slower rate as compared to normal hair and are different from curly hairs of Negros because the curls are separable in Negro hairs but not in woolly hairs. [3],[4] In 1974, Hutchinson et al. further classified woolly hair into a localized variant (woolly hair nevus) and two generalized variants, including autosomal dominant (AD) hereditary woolly hair and autosomal recessive (AR) familial woolly hair. Later on diffuse partial woolly hair has also been described as a separate entity. [1]

Epidermal nevi are nests of epidermal cells which are noted at birth or within the first year as a thin plaque or linear tan patch often along Blaschko lines. Extensive epidermal nevi, often in unilateral distribution, are called as systematized epidermal nevi. Histologically, epidermal nevi show hyperkeratosis and papillomatosis. Epidermal nevus can be an isolated finding or it can show syndromic/nonsyndromic association with other cutaneous/extracutaneous lesions. [5]

Localized variant of woolly hair (woolly hair nevus) is frequently found in association with a linear epidermal nevus of focal or systematized distribution. [6]

Generalized variants of woolly hair (involving the whole scalp) are also described in syndromic/nonsyndromic association with other lesions. [4] Notable syndromic association of woolly hair is found in autosomal recessive Nexos disease and Carvajal disease caused by mutation of desmosomal proteins plakoglobin and desmoplakin respectively. Both diseases are characterized by triad of generalized woolly hair, palmoplantar keratoderma, and cardiomyopathy (right ventricular in Naxos disease, left ventricular in Carvajal disease). [4] Therefore, with the diagnosis of generalized woolly hair, a thorough evaluation of all organ systems, especially of the heart, must be conducted.

The absence of family history of similar disorder, with diffuse scalp involvement, in our patient probably suggests a hereditary form of woolly hair resulting due to de novo mutation. Associated bilateral systematized epidermal nevus was also seen in our case. To the best of our knowledge, our case is the first reported case of such type of association.

 
   References Top

1.
Singh SK, Manchanda K, Kumar A, Verma A. Familial woolly hair: A rare entity. Int J Trichology 2012;4:288-9.  Back to cited text no. 1
    
2.
Thappa DM, Thadeus J, Garg BR. Woolly hair. Indian J Dermatol 1995;40:181-3.  Back to cited text no. 2
  Medknow Journal  
3.
Mirmirani P, Huang KP, Price VH. A practical, algorithmic approach to diagnosing hair shaft disorders. Int J Dermatol 2011;50:1-12.  Back to cited text no. 3
    
4.
Vasudevan B, Verma R, Pragasam V, Badad A. A rare case of woolly hair with unusual associations. Indian Dermatol Online J 2013;4:222-4.  Back to cited text no. 4
[PUBMED]  Medknow Journal  
5.
Brandling-Bennett HA, Morel KD. Epidermal naevi. Pediatr Clin North Am 2010;57:1177-98.  Back to cited text no. 5
    
6.
Wright S, lemoine NR, leigh IM. Woolly hair naevi with systematized linear epidermal naevus. Clin Exp Dermatol 1986;11:179-82.  Back to cited text no. 6
    

What is new?
Our case is of hereditary wooly hair in association with bilateral systematized epidermal nevus which is not described in literature previously.


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

Top
Print this article  Email this article
 
 
  Search
 
  
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Article in PDF (1,104 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  


    Abstract
   Introduction
   Case Report
   Discussion
    References
    Article Figures

 Article Access Statistics
    Viewed2662    
    Printed50    
    Emailed0    
    PDF Downloaded55    
    Comments [Add]    

Recommend this journal