Case Report

Ichthyosis hystrix

Surajit Nayak, Basanti Acharjya, Prasenjit Mohanty

Department of Skin ABSTRACT and VD, MKCG Medical College and Hospital, The present report describes the condition in a three day old male child with bilateral ,linear, hyperpigmented and Berhampur, Orissa, India hyperkeratotic verrucous plaques and patchy alopecia over scalpe without any nail and skeletal abnormalities. It was suggestive of ichthyosis hystrix type of epidermal nevus,and is being reported in view of the rarity of this condition.

Key words: Icthyosis hystrix, epidermal nevus syndrome, etretinate

INTRODUCTION most part of the face. Nails were normal. In the lower limbs, in addition to the nevus, there were Ichthyosis hystrix the nomenclature comes unilateral hyperpigmented [Figure 1] macular from the Greek word and condition was first patches encircling right upper thigh and complete described in England in early 18th century. left thigh, sparing a band-like zone. The term ichthyosis hystrix is used to describe several rare skin disorders in the ichthyosis On physical examination, we could not observe family of skin disorders characterized by massive any defects, especially in skeletal or central hyperkeratosis with an appearance like spiny nervous systems. Routine laboratory examination scales. The term has also been employed to including complete blood count, urine analysis, describe localized and linear warty epidermal nevi liver function test and chest X-ray were all within sometimes associated with mental retardation, normal limits. The parents did not permit a biopsy. seizures or skeletal anomalies. Alopecia and hair and nail abnormalities as well as inner ear Based on the above constellation of clinical deafness were also seen in these patients. features (history, typical morphology, and generalized, roughly bilaterally symmetrical CASE REPORT involvement), a diagnosis of ichthyosis hystrix Access this article online type of epidermal nevus (systematized epidermal Website: www.idoj.in A 3-day-old full-term male, born by cesarean nevus) was made. DOI: 10.4103/2229-5178.105483 section out of non-consanguineous marriage, Quick Response Code: was referred to us from the Obstetrics and The name ichthyosis hystrix comes from the Gynaecology department with complaints of Greek words ichthyos meaning fish and hystrix extensive verrucous hyperpigmented lesions. meaning porcupine. It is a descriptive name for a heterogeneous group of keratinization A detailed general examination revealed an disorders sharing similar features of massive, extensive systematized epidermal nevus over the spiky or verrucous hyperkeratosis clinically and scalp, forehead, the entire trunk (both anteriorly epidermolytic hyperkeratosis histologically. and posteriorly) and both upper and lower limbs. Address for The condition was first described in the Lambert correspondence: The nevus was more prominent over the left Dr. Surajit Nayak, half of anterior trunk [Figure 1], extending along family in England in the early 18th century. This Department of Skin midline of genitalia. Posteriorly it was almost term is also used to refer to a type of epidermal and VD, MKCG Medical symmetrical [Figure 2]. In all sites, nevi followed nevus with extensive bilateral distribution.[1] College, Berhampur, the lines of Blaschko. Over the scalp [Figure 3], Orissa, India. it followed a fronto-occipital linear distribution and Since in 1902 Brocq[2] first reported ichthyosis E-mail: surajitnyk@ in the involved areas, there was complete loss of hystrix as a atypical form of congenital bullous yahoo.co.in hair. The palms and soles were spared, so also ichthyosiform erythroderma, many authors

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The term systematized epidermal nevus is used for lesions that are bilateral and excessive; similarity to the lesions of ichthyosis is emphasized in the term ichthyosis hystrix.[4]

Histological examination of the epidermis shows acanthosis, papillomatosis, and severe orthokeratotic hyperkeratosis, with frequent binucleate cells.[5] Study of three generations of the families of Curth and Macklin with ichthyosis identified a mutation in the variable tail domain (V2) of the keratin-1 gene.[6] Solomon and Esterly[7] provided a detailed account of the spectrum of epidermal nevi seen in the epidermal nevus syndrome. They described seven types of lesions. The majority of patients had nevus unius lateralis; 20% of patients had Figure 1: Hyperpigmented, verrucous patches on front following lines ichthyosis hystrix. of Blaschko In 1978, Basler et al.[8] first described a case of ichthyosis hystrix that was not associated with any other defects. Subsequently, a few authors reported similar cases of ichthyosis hystrix without involvement of other systems. Our case also did not have any systemic abnormality.

Clinical expression varies, even within families, from palmoplantar keratoderma to a severe, generalized involvement. There can be widespread, patchy, thick, gray-brown hyperkeratosis, most marked on extensor surface of the arms and legs. However, in contrast to epidermolytic hyperkeratosis, blistering does not occur.

A condition that closely resembles ichthyosis hystrix with a melanocytic nevus is phacomatosis pigmentokeratotica. It Figure 2: Similar lesions over back is characterized by the coexistence of an epidermal nevus following the Blaschko’s lines that usually shows sebaceous differentiation with a large speckled lentiginous nevus, typically arranged in a chequerboard pattern. This syndrome is an example of cutaneous mosaicism that is thought to be related to twin spotting phenomenon. It differs from ichthyosis hystrix by the absence of a systematized nevus.

There is no specific treatment for ichthyosis hystrix. Major treatment consists of symptomatic therapy for complications and cosmetic purposes. Topical retinoids, usually tretinoin, may be beneficial. A good result with etretinate is reported by many authors,[9,10] which can be planned once the baby attains the right age.

REFERENCES

Figure 3: Lesions over scalp, resulting in linear bands of alopecia 1. Duncan KO, Geisse JK, Leffel DJ. Benign epithelial tumours. In: Wolff K, Goldsmith LA, Katz S, Gilchrest BA, Paller AS, Leffell DJ, described it by different names including nevus verrucosus, editors. Epidermal and appendageal tumours. Fitzpatrick’s dermatology in general medicine. 7th ed. NY: McGraw-Hil Division; 2008. p. 1060-1. systematized epidermal nevi, and epidermolytic hyperkeratosis. 2. Brocq L. Erythrodermie congénitale ichthyosiforme avec In 1980, Gianotti[3] classified it as a localized form of congenital hyperepidermotrophie. Ann Dermatol Syphiligr 1902;4:1–31. [Quoted by: DiGiovanna JJ, Bale SJ. Clinical heterogeneity in epidermolytic epidermolytic hyperkeratosis. hyperkeratosis. Arch Dermatol 1994;130:102635.]

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3. Gianotti F. Congenital icthyosiform dermatoses in childhood. J Dermatol 7. Solomon LM, Esterly NB. Epidermal and other congenital organoid (Japan) 1980;7:1-9. nevi. Curr Probl Pediatr 1975;6:1-56. 4. Rogers M. Epidermal naevi/epidermal naevus syndromes. In: Textbook 8. Basler RS, Jacobs SI, Taylor WB. Icthyosis hystrix. Arch Dermatol of Pediatric Dermatology. 2nd edn. Eds. Harper J, Oranje A, Prose N. ed. 1978;114:1059. London, Blackwell Publishing Ltd; 2006.;1125. 9. Kang WH, Lee KH, Chin HS. Icthosis hystrix: A case treated with 5. Fleckman P, Digiovanna JJ. The Ichthyoses. In: Disorders of epidermal etretinate. Korean J Dermatol 1984;22:101. differentiation and keratinization. In: Fitzpatrick’s dermatology in 10. Kanerva L, Karvonen J, Oikarinen A, Lauharanta J, Ruokonen general medicine. 7th edn. Eds. Wolff K, Goldsmith LA, Katz S, A, Niemi KM. Ichthyosis hystrix (Curth-Macklin). Light and electron Gilchrest BA, Paller AS, Leffell DJ. McGraw-Hil Division, NY, microscopic studies performed before and after etretinate treatment. 2008: 414 Arch Dermatol. 1984 Sep;120(9):1218-23. 6. Sprecher E, Ishida-Yamamoto A, Becker OM, Marekov L, Miller CJ, Steinert PM, et al. Evidence for novel functions of the keratin tail Cite this article as: Nayak S, Acharjya B, Mohanty P. Ichthyosis hystrix. emerging from a mutation causing ichthyosis hystrix. J Invest Dermatol Indian Dermatol Online J 2013;4:47-9. 2001;1164:511-9. Source of Support: Nil, Conflict of Interest: None declared.

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