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Congenital Skin lesions
Question :
Mani Kant Kumar
Department of Pediatrics, Narayan Medical College and Hospital, Jamuhar, Sasaram, Bihar, India.


Address for Correspondence: Dr Mani Kant Kumar, Assistant Professor, Department of Pediatrics, Narayan Medical College and Hospital , Atand PO-Jamuhar, Sasaram, Dist- Rohtas, Bihar -821305, India. Email: manikant7@yahoo.com

A 4 year old boy presented with unilateral non progressive, non pruritic black discoloration of right arm, axilla and trunk since birth. There was no history of seizure or developmental delay. There was no family history of seizure disorder or similar lesions. Examination of the child revealed verrucous elevated papules and plaques over right arm, axilla and trunk dark blue in color with well defined margins {Fig.1}. Other systems were normal. Patient was started on topical vitamin D analogue Calcipotriol and oral isotretion {0.5 mg, kg, day} with advice of regular follow up.

What is the diagnosis_?
 
Answer :
Unilateral Verrucous Epidermal Nevus. Epidermal nevi are hamartomas that are characterized by hyperplasia of the epidermis and adnexal structures. These have been estimated to occur in 1: 1000 live births, affecting the sexes equally. They occur sporadically, however familial cases have been reported. An estimated one third of individuals with epidermal nevi have involvement of other organ systems` hence, this condition is considered to be an epidermal nevus syndrome {1}. Typically, epidermal nevi are present at birth or early infancy but have been described to appear in puberty {2}. Verrucous epidermal nevi occur in circumscribed patches or more often, in linear streaks or whorls following Blaschko`s lines {3}. The lesions typically occur on the trunk or extremities, but may also occur on the face and neck {1}. The lesions may vary from skin colored to brown. Histologically, keratinocytic, or verrucous epidermal nevi are characterized by acanthosis, orthohyperkeratosis, papillomatosis and an expanded papillary dermis which is sharply demarcated from the surrounding normal skin. No ideal treatment is yet available, the topical treatments such as combined therapy of retinoic acid and 5-fluorouracil, Vitamin D analogues {Calcipotriol}, dithranol, occlusive topical steroids, chemical peels and podophyllin may improve the keratotic surface however they are always associated with high rate of recurrence {4}. Other treatment includes laser ablation with variable results.

Acknowledgment: Author thanks Dr Amar Kant Jha, Associate Professor, Department of Dermatology, Patna Medical College, Patna for his help in managing the case.

Funding: None
Competing interests: None.

REFERENCES:
1. Losee JE, Serletti JM, Pennino RP. Epidermal nevus syndrome: a review and case report. Ann Plast Surg. 1999 Aug`43{2}:211-4
2. Rogers M, McCrossin I, Commens C. Epidermal nevi and the epidermal nevus syndrome. A review of 131 cases. J Am Acad Dermatol. 1989` 20: 476-488
3. Bolognia JL, Orlow SJ, Glick SA. Lines of Blaschko. J Am Acad Dermatol. 1994` 31{2 Pt 1}:157-190` quiz 190-192.
4. Fox BJ, Lapins NA. Comparison of treatment modalities for epidermal nevus: a case report and review. J Dermatol Surg Oncol. 1983` 9: 879-885

E-published: May 2011 Vol 8 Issue 5 Art # 33