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Congenital skin lesion
Question :
Rajendra P Karambelkar, Suresh Pethe
Consultant Pediatrician, Aditya Birla Memorial Hospital, Chinchwad, Pune, Consultant Dermatologist, Nigdi, Pune, India.
Address for Correspondence: Dr. Rajendra P Karambelkar, Consultant Pediatrician, Department of Pediatrics and Neonatology, Aditya Birla Memorial Hospital, Chinchwad Pune 411033, India. Email: rjkarambelkar@yahoo.co.in
A six-year boy was brought with skin lesion involving entire trunk and smaller lesions on rest of the body since birth and increasing proportional to the body size. On examination, he had uniformly hyperpigmented, raised, hairy, jacket like plaque over the entire trunk covering More than 40 percent of body surface, with sharp borders, velvety surface and multiple small and medium sized nevi on forehead, lips, and extremities {Fig.1}.

What is the diagnosis_?
 
Answer :
Giant Congenital Melanocytic Nevus {GCMN} with multiple small and medium melanocytic nevi. GCMN is More than 20cm in diameter {adult size} or involve More than 5 percent of body surface area. {1} Lesions commonly involve trunk and are often associated with multiple small, medium size congenital nevi. Nevus has sharp borders, variable pigmentation and surface may be smooth, rugose, velvety and verrucous at places. {2} Hair may be present from birth or appear later. Lesions grow proportional to body over time. GCMN may co-exist with leptomeningeal nevi and neurofibromatosis. {3} Diagnosis is usually clinical. Lesions like mongolian spots, smooth muscle hamartoma, café-au-lait spots or epidermal nevi may be mistaken for GCMN especially if lesion is non-hairy. GCMN have a very high predisposition to develop potentially fatal malignant melanoma at a median age of 7 years. Increase in size of nevus, change in color {1}, development of nodules in deeper dermis {4}, itching, pain, ulcerative lesions and bleeding {3} are the early clinical clues to development of melanoma. Periodic examination is recommended to watch for development of melanoma. Prophylactic surgical excision to reduce the risk of malignant melanoma is generally undertaken after the age of 6 months after neural involvement is ruled out. However, surgery may not avoid possible complication of melanoma {5}. Sunscreens with More than 15 spf, sun protective clothing, patient education and self examination are the recommended measures for prevention and monitoring of malignant melanoma.
Funding: None
Competing Interests: None

References:
1. Morelli JG. Cutaneous Nevi. In: Kleigman, Stanton, St. Geme, Schor, Behrman, {eds}. Nelson Textbook of Pediatrics, 19 th Ed. Elsevier` 2011. p.2231-36.
2. Tronnier M. Melaonotic Spots and Melanocytic Nevi. In: Burgdorf WHC, Plewig G, Wolff HH, Landthaler M. {Eds}. Braun-Falco’s Dermatology, 3rd Ed. Springer` 2010. p.1409-13.
3. Grichnik JM, Rhodes AR, Sober AJ. Benign Neoplasias and Hyperplasias of Melanocytes. In: Wolff K, Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ {Eds}. Fitzpatrick’s Dermatology in General Medicine, 7th edition. McGraw Hill` 2008. p. 1099-1122.
4. Chiller KG, Washington C, Sober AJ, Koh HK. Cancers of the skin. In: Kasper, Braunwald, Fauci, Hauser, Longo, Jameson {Eds}. Harrison’s Principles of Internal Medicine, 16 th Ed. McGraw Hill` 2005. p. 497-503.
5. Ruiz-Maldonado R, Orozco-Covarrubias ML. Malignant melanoma in children: A review. Arch Dermatology 1997`133:363-71.

E-published: April 2013 Vol 10 Issue 4 Art # 23

DOI No. : 10.7199/ped.oncall.2013.23