The face in congenital melanocytic nevus syndrome

2012 ◽  
Vol 158A (5) ◽  
pp. 1014-1019 ◽  
Author(s):  
Veronica Kinsler ◽  
Adam C. Shaw ◽  
Johannes H. Merks ◽  
Raoul C. Hennekam
Author(s):  
Fatima Bello Jiya ◽  
Maryam Amodu- Sanni ◽  
Nma Muhammed Jiya ◽  
Dada Muhammed Aquib ◽  
Muhammed Umar ◽  
...  

Aim: To present the first report of a large congenital melanocytic nevus with satellite nevi in an apparently healthy child from Sokoto, North-Western Nigeria. Presentation of Case: A three year old girl was brought to the paediatric out-patient clinic of Paediatrics department of Usmanu Danfodiyo University Teaching Hospital (UDUTH) Sokoto with complaints of darkened skin colour on the left side of the face and scalp, the left arm, lower back, buttocks, and thighs, and excessive hair growth over the same side of the face since birth. There were no neurological symptoms Physical examination findings revealed a well-nourished, not ill looking child. She had a hyper pigmented patch on the left side of the face extending from the lower jaw to the scalp, measuring 21 cm in its longest length, with hypertrichosis on the same site, and two distinct, firm, painless nodular lesions on the left temporal region measuring 3 mm and 4mm respectively. On the lower one-third of the left arm was a hairy, velvety area of hyperpigmentation measuring 2X3 cm in diameter. Other affected sites were the lower back, the gluteal region and the thighs. Her neurologic and other systemic examinations were normal. A diagnosis of large congenital facial melanocytic hairy nevus with multiple satellite nevi was made. Discussion: Congenital melanocytic nevi are benign proliferations of melanocytic cells said to be present at birth or in the first two years of life. Large lesions are rare, they measure 20 cm or more and are said to occur more commonly on the trunk and thighs. The exact pathogenesis of congenital melanocytic nevi is yet, unknown. It is thought to occur as a result of a morphological error in the neuroectoderm during embryogenesis. Treatment of patients with large congenital melanocytic nevus may include surgical or non-surgical procedures as well as psychological interventions. Large lesions, multiple satellite lesions or paravertebral and axial locations are sometimes associated with the risk of neurological complications and malignant transformation. Conclusion: Large congenital melanocytic nevi are uncommon skin lesions that can occur in apparently healthy children. Individualization of the patients with regards to treatment options and long term monitoring are imperative.


2015 ◽  
Vol 3 (2) ◽  
pp. 56-60 ◽  
Author(s):  
Margarita Sergeevna Tsyplakova ◽  
Anna Sergeevna Usoltseva ◽  
Yulia Vladimirovna Stepanova

This article describes a rare case of congenital anomalies: giant melanocytic nevus of the face. Errors in the choice of treatment strategy of children with this disease and their complications can lead to poor aesthetic and functional results. When choosing a method of plastic eliminate defects formed after removal of nevi, it is necessary to take into account anatomical features of the maxillofacial region. The department developed the scheme of complex treatment of these patients. Surgical treatment in combination with massage and myogymnastics, regular medical observation, provides a good aesthetic results. Integrated approach in the treatment of children with giant nevi of the face allows for an early medico-social rehabilitation of children with this pathology.


2015 ◽  
Vol 3 (4) ◽  
pp. 22-28
Author(s):  
Anna Sergeevna Usoltseva ◽  
Yulia Vladimirovna Stepanova ◽  
Ivan Nikolaevich Krasnogorskiy ◽  
Margarita Sergeevna Tsyplakova

Background. Congenital melanocytic nevus is a benign pigmented neoplasm composed of nevus cells that clinically manifest at birth. When choosing a treatment for nevi, the possibility of recurrence as well as the threat of tumor malignancy should be considered. The aim of this work is to justify the surgical removal of a large and giant nevi of the face as a method of treatment justified by the pathomorphological structure. Materials and Methods. In 40 children of different ages born with large and giant nevi of the face, we used various types of plastic surgery to eliminate any defects formed after the excised nevi. We accounted for the features of the maxillofacial area: local plasty, expander dermotension, and transplantation of free skin grafts. We performed a total of 68 surgical interventions. Sixteen patients underwent the surgery once and 24 patients underwent secondary surgery (from 2 to 4). We also developed a scheme of the staged surgical treatments and conservative methods. Results. All patients had stable positive results that were studied by comparing the outcomes of different surgical treatment options and accounting for various morphological characteristics of the removed nevi.


Dermatology ◽  
2007 ◽  
Vol 214 (3) ◽  
pp. 227-230 ◽  
Author(s):  
Vito Ingordo ◽  
Ciro Gentile ◽  
Silvia S. Iannazzone ◽  
Francesco Cusano ◽  
Luigi Naldi

2016 ◽  
Vol 20 ◽  
pp. 84-86
Author(s):  
Domagoj Eljuga ◽  
Ivan Milas ◽  
Iva Kirac ◽  
Mladen Stanec ◽  
Danko Velimir Vrdoljak

1993 ◽  
Vol 13 (6) ◽  
pp. 717-721 ◽  
Author(s):  
Eric Jauniaux ◽  
Marie-Christine de Meeus ◽  
G. Verellen ◽  
J. M. Lachapelle ◽  
Jean Hustin

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