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Year : 1996  |  Volume : 62  |  Issue : 6  |  Page : 379-380

Ichthyosis linearis circumflexa

Correspondence Address:
Subrata Malakar

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Source of Support: None, Conflict of Interest: None

PMID: 20948134

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A case of ichthyosis linearis circumflexa in a 9-year old boy is reported here for its rarity.

Keywords: Ichthyosis linearis circumflexa, Comel - Netherton′s syndrome

How to cite this article:
Malakar S, Lahiri K, Sengupta S R. Ichthyosis linearis circumflexa. Indian J Dermatol Venereol Leprol 1996;62:379-80

How to cite this URL:
Malakar S, Lahiri K, Sengupta S R. Ichthyosis linearis circumflexa. Indian J Dermatol Venereol Leprol [serial online] 1996 [cited 2020 Apr 6];62:379-80. Available from: http://www.ijdvl.com/text.asp?1996/62/6/379/4471

  Introduction Top

This rare and distinctive entity was recognised by clinicians for many years but confusion existed due to a lack of proper descriptive terminology. It was Comel in 1949, who first coined the term ichthyosis linearis circumflexa giving it a clearcuit individuality.[1] Netherton in 1958 reported an association of bamboo hair (trichorrhexis invaginata) and ichthyosiform erythroderma.[2] Atopic diathesis was also reported in some patients. Since the initial description of this disorder by Comel in 1949, less than 70 cases were reported by 1987.[3]

  Case Report Top

A 9-year-old boy, the second issue of a non-consanguineous couple reported to us with generalised scaly lesions since his infancy. On examination the child was found to be of average built with wide spread erythematous, serpiginous, annular or polycyclic, scaly eruptions with double edged scales at the periphery of the lesions [Figure:l]. The lesions are continuously changing their patterns and shape with a bizarre migratory nature, involuting in a week or two after reaching their maximum size leaving no scarring, atrophy or pigmentary changes. The lesions are distributed mainly over the trunk [Figure - 1] with hyperkeratotic flexures in the extremities. Hair, nail, genitalia and mucous membrane was not involved. The child was not atopic. Routine physical examination, X-ray chest, Mantoux test, complete haematological check up did not reveal any abnormality. Histopathological examination from the periphery of the lesion showed hyperkeratosis, papillomatosis with upward projection into the epidermis and suprapapillary thinning [Figure - 2]. Collection of homogenous material in the horny layer was also present [Figure - 3]. These features coroborated well with our clinical diagnosis.[4]

  Discussion Top

ILC is a very rare autosomal dominant disorder. Generalised erythema and scaling are seen at birth or shortly thereafter. Subsequently polymorphic, serpiginous, erythematous, slowly changing lesions with double-edged scaly border appear on the trunk and proximal extremities. Lichenification of the popliteal and cubital fossae and red, scaly face and eyelids are also seen. Till date only 4 cases are reported in Indian journals. In 1985 Chandwala et al reported a brother and sister from Pune, both in their teens, with this rare disease.[5] The other two were from Libya. Kanwar et al in 1987 reported a 6-year-old boy from Benghazi and in 1989 Khatri et al reported another male patient of same age from Tripoli. [6,7]

Clinically and histologically ours was a classical case of ILC but associations like hair abnormalities, atopy, palmoplantar hyperkeratosis and hyperhydrosis, pruritus, stunted growth and mental retardation where absent in our case.

To the best of our knowledge, except for the cases reported by Chandwala, ours is the only other Indian case of ILC and the very first one reported from eastern India.

  References Top

1.Comel M. Ichthyosis linearis circumflexa. Dermatologica 1949;93:133-5.  Back to cited text no. 1    
2.Netherton EW. A unique case of trichorrhexis nodosa-"bamboo hairs". Arch Dermatol 1958,78:483.  Back to cited text no. 2  [PUBMED]  
3.Manabe M, Yoshiike T, Negi M, et al. Successful therapy of ichthyosis linearis circumflexa with PUVA. J Am Acad Dermatol 1983;8:905-7.  Back to cited text no. 3  [PUBMED]  
4.Lever WR, Lever GS. Congenital diseases (genodermatoses). In: Histopathology of the skin. Philadelphia : JB Lippincott, 1990:65-102.  Back to cited text no. 4    
5.Chandwala KR, Gharpuray MB, Shah NV. Ichthyosis linearsis circumflexa. Ind J Dermatol Venereol Leprol 1985:51:353.  Back to cited text no. 5    
6.Kanwar AJ, Singh M, Bhanja SC, et al. Ichthyosis linearis circumflexa. Ind J Dermatol Venereol Leprol 1987;53:300-1.  Back to cited text no. 6    
7.Khatri ML, Shafi M. Netherton's syndrome. Ind J Dermatol Venereol Leprol 1989;55:135-6.  Back to cited text no. 7    


[Figure - 1], [Figure - 2], [Figure - 3]


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