|Year : 2017 | Volume
| Issue : 4 | Page : 310-313
Giant nevus unius lateralis: Two cases
Ajay Govindrao Ovhal1, Shruthi Vasanth Kumar Deshkulakarani2, Viraj Jitendra Sadrani3
1 Department of Dermatology and Venereal Disease, Government Medical College, Latur, Maharashtra, India
2 Department of Histopathology, Hinduja Hospital, Mahim, Mumbai, Maharashtra, India
3 BDBA Hospital, Kandivali West, Mumbai, Maharashtra, India
|Date of Web Publication||29-Sep-2017|
Shruthi Vasanth Kumar Deshkulakarani
No. 6, Dhanvantri PG Hostel, Opposite Gandhi Chowk Police Station, Gandhi Chowk, Latur, Maharashtra - 413 512
Source of Support: None, Conflict of Interest: None
Verrucous epidermal nevus is a common type of keratinocyte hamartoma present at birth or occurring later in life. Widespread multiple epidermal nevi (EN) may reflect genetic or chromosomal mosaicism. Blaschko has documented characteristic linear, zosteriform, unilateral or systematized patterns with streaks and swirls. They are resistant to treatment with the risk of recurrence. Giant congenital nevus unius lateralis (NUL) is known for its benign course with good supportive treatment however one particular case had a fatal neonatal outcome due to associated complications. Two interesting rare cases will be reviewed here.
Keywords: Blaschko's lines, disembryoplasia, epidermal nevus, nevus unius lateralis
|How to cite this article:|
Ovhal AG, Deshkulakarani SV, Sadrani VJ. Giant nevus unius lateralis: Two cases. Indian J Paediatr Dermatol 2017;18:310-3
|How to cite this URL:|
Ovhal AG, Deshkulakarani SV, Sadrani VJ. Giant nevus unius lateralis: Two cases. Indian J Paediatr Dermatol [serial online] 2017 [cited 2020 Jun 1];18:310-3. Available from: http://www.ijpd.in/text.asp?2017/18/4/310/215797
| Introduction|| |
Verrucous epidermal nevus is seen at any site, but is less common on the head and neck, rarely seen on the face and very rarely involves the oral mucosa. Epidermal nevus (with or without other organ involvement) has an incidence of around 2 per 10,000 live births with the prevalence of nevus unius lateralis (NUL) estimated to be 1:1000 live births. Nevus unius lateralis (NUL) is a disembryoplasia or congenital verrucous epidermal nevus (VEN) having a unilateral distribution in linear streaks or bands, following the long axis of the limbs or extending transversely around the trunk following Blaschko's lines. The common sites are trunk and limbs, but the involvement of head and neck region is rare.
The intense supportive care given during the perinatal period is important in survival of the infant and to prevent the onset of septicaemia. The emphasis on the identification of syndromic association of nevi is important for therapeutic and morbidity purposes. This article also intends to provide some highlights on regression of nevus.
| Case Reports|| |
A 1-day-old male baby, born of nonconsanguineous marriage, full-term normal vaginal delivery at home, presented to dermatology outpatient department (OPD) with generalized, monomorphic, asymmetrical lesions in the form of cerebriform, cauliflower like greyish yellow verrucous plaques extending from the right nape of neck, extensor aspect of upper extremity, right half of trunk, genitals, up to dorsum and sole of foot of lower limb involving extensor and plantar aspect. There was sparing of creases of axillae and groin, head, face, and contralateral side. The greyish yellow verrucous lesions had a “half-armour like-appearance.” There was no history of bleeding from the lesion. The umbilical cord was swollen yellowish surrounded by erythematous skin. The baby showed refusal for feeds and was excessively crying. There was no history of convulsions or vomiting. No family history of similar disease. On palpation, the lesions were moist, rubbery and did not bleed on touch [Figure 1]a. The baby was febrile, had tachycardia and tachypnea. Systemic examination showed distended abdomen, moderate splenomegaly, dilated veins, and petechiae over the left side of abdomen. Blood investigations revealed anemia, elevated total leukocyte count and thrombocytopenia. The baby was admitted to neonatal intensive care unit but succumbed after 2 days. Parents denied consent for clinical autopsy hence cause of splenomegaly could not be elicited. However, retrospectively analyzing the case with available evidence by pediatricians, cause of death was mentioned to be septicemia due to the infected umbilical cord with multi-organ failure. After searching the literature, we could not find mortality in cases associated with NUL.
|Figure 1: (a) Case 1 - Extensive yellowish grey colored cauliflower like plaques over the right half of head, trunk, upper, and lower limb extending on the dorsum and sole of foot involving genitalia but sparing face. Admixed among the nevi were islands of normal skin. On palpation, the lesions were rubbery and did not bleed on touch. (b) Case 2 - Verrucous hyperpigmented plaques in zosteriform pattern over the left side of trunk up to middle of thigh. On palpation, the lesions were velvety and did not bleed on touch|
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A 6-month-old, female baby, born of nonconsanguineous marriage, full-term normal vaginal delivery presented to dermatology OPD with localized, asymmetrical, monomorphic lesion in the form of verrucous, warty, velvety hyperpigmented plaques in zosteriform pattern extending over left half of trunk up to middle of thigh involving labia majora giving “half bathing trunk appearance” [Figure 1]b. On examination, the plaques were velvety to touch, not fixed to the underlying structures and she did not cry on manipulating the lesion. Other cutaneous and systemic examination were unremarkable. There was no history of refusal to feeds, vomiting, or convulsions. No family history of similar disease.
Consent was obtained from parents for performing routine investigations and for obtaining skin punch biopsies of the lesions. Various routine investigations, X-ray of skull, computed tomography scan of brain, and ultrasonography (USG) abdomen were all normal. Skin biopsies sections at histopathology were stained with hematoxylin and eosin [Figure 2]a and [Figure 2]b. The differential diagnosis thought of were NUL, hyperkeratotic type of seborrheic keratosis and epidermolytic hyperkeratosis.
|Figure 2: (a) Case 1 and (b) Case 2 - histopathological findings suggestive of nevus unius lateralis-hyperplastic epidermis with hyperkeratosis, acanthosis, papillomatosis, and elongation of rete ridges. Adnexal structures appear normal (H and E, ×400)|
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Histopathological sections showed hyperplastic epidermis chiefly affecting stratum corneum and stratum malpighii with hyperkeratosis, acanthosis, papillomatosis, and moderate elongation of rete ridges with normal adnexal structures. No granular cell degeneration or proliferation of the capillaries in the dermis was noted. The diagnosis of NUL was confirmed.
The patient was reviewed at the age of 3 years. The plaques showed remarkable regression of lesions over abdomen. The lesions were noted below the umbilicus to the middle of thigh on the left side of the body still following the Blaschko's lines, and they appeared less verrucous [Figure 3]a and [Figure 3]b.
|Figure 3: (a) Anterior view and (b) lateral view – follow-up pictures of Case 2 showing receding pattern of the nevi following Blaschko's lines with abdominal distention|
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At present, the patient did not show any syndromic association. She had no developmental (facial)/mental or neurologic abnormalities, except that her upper and lower extremities (left half of body) measured an inch more, girth and length wise compared to that of right (considering it as asymmetrical growth of left half of body) along with abdominal distension. USG of abdomen and pelvis was unremarkable. This incidental observational finding was not significant enough to be considered as hemihypertrophy observed in Proteus syndrome. Hence, Proteus syndrome was ruled out. The genetic constitution leading to NUL and the immunohistochemical cause of regression could not be identified due to poor resources.
The treatment of EN is challenging. Multiple medical and surgical treatments have been attempted, but no ideal or universally acceptable treatment has emerged.
Corticosteroids applied under occlusion or by injection as well as tretinoin cream applied topically, may sometimes be partially effective. Oral retinoids may be beneficial for the treatment of widespread epidermal nevi (EN) but may require life-long therapy.
Systemic retinoids have been the treatment of choice for many disorders of keratinization. Isolated cases have documented partial or complete improvement of generalized EN from systemic retinoid with long term remission. Taşkapan et al. described a 20-year-old male with unilateral verrucous EN showing a response to 75 mg/day of acitretin in 3 weeks. The acute toxicities include mucocutaneous lesions (such as cheilitis and conjunctivitis) and hair loss, whereas chronic toxicities involve several organ systems, especially the skeletal (bony changes), hepatic and cardiovascular hepatic, and cardiovascular systems (atherosclerosis). The long-term use of oral acitretin on children, however, has been a major concern for most dermatologists. A retrospective study conducted by Lacour et al., reviewed all children inherited with disorders of keratinization receiving acitretin. Twenty-eight children with a cumulative dose of acitretin for more than 440 months of treatment had no major side effects.
The treatment of choice for small EN is surgical excision. Recent advances in laser technology for treating EN with CO2, long pulsed neodymium-doped yttrium aluminum garnet and 585 nm pulsed dye lasers have been developed. However, recurrences can occur months or years after removal of EN by any method. In view of the large surface area being involved by the nevi, our patient was started on retinoic acid derivatives cream and was advised for close follow-up.
| Discussion|| |
Epidermal nevus syndrome (ENS) is a term that refers to the association of EN with systemic anomalies. The neurologic, ophthalmologic, and skeletal systems are most commonly involved. ENS occurs in a sporadic fashion and many different mosaic phenotypes are expressed. It likely represents a lethal disorder that is rescued by mosaicism. EN are hamartomas of ectodermal origin that are classified according to their major constituent: keratinocytic, follicular, apocrine, eccrine, or sebaceous. The many names for ENS reflect the condition's heterogeneity. It has been known as Solomon syndrome, Jadassohn syndrome, Schimmelpenning- Feuerstein-Mims syndrome More Details, and nevus sebaceous syndrome. Dr. Gustav Schimmelpenning, a psychiatrist, was the first to recognize central nervous system defects with EN. Solomon and easterly proposed classifying the nevi based on the nevi's constituents and first coined the term “ENS” in 1968. Currently, four major different types of ENS are recognized: linear sebaceous nevus, linear nevus comedonicus, linear EN, and inflammatory linear VEN.
Linear EN clinically appears as verrucous papules and plaques distributed in a linear pattern following Blaschko's lines. Their extent varies from unilateral involvement (NUL) to extensive bilateral involvement (ichthyosis hystrix). Oral mucosal lesions have rarely been described. Lesions may be congenital or develop during early years of life and stabilizes during teenage years.
The gene mutations involved in most EN are unknown. NUL is caused by activation of an autosomal dominant lethal mutation that survives by somatic mosaicism.
The pattern of regression has been extensively studied as anin vivo model in Halo nevi for the immunologic mechanism responsible for regression in melanoma. Halo nevus is a phenomenon involving several-related conditions, including congenital melanocytic nevi, Spitz nevi, congenital giant nevocellular nevi, balloon cell nevi, and atypical nevi among others.
In a study of 4256 pediatric cases (below the age of 14 years) attending the dermatology OPD from August 2002 to 2004 were screened for nevi at JIPMER, Puducherry. Sixteen cases were found to have VEN, five of them were linear VEN. This indicates that NUL is an uncommon condition. Basal cell epithelioma and squamous cell carcinoma have been described rarely in lesions on the head.
Giant NUL with congenital onset may have a good prognosis when managed meticulously in infancy. The mechanism of regression of NUL is subject of research. Histopathology has an active role in diagnosis. The purpose of the article is to provide a rare presentation of giant NUL with favorable prognosis.
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Conflicts of interest
There are no conflicts of interest.
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