An Unusual Presentation of Neurotized Congenital Giant Melanocytic Nevus and Type 1 Neurofibromatosis: A Diagnostic Challenge

@#Among the three subtypes of neurofibromatosis are type 1 and 2 neurofibromatosis and schwannomatosis, von Recklinghausen disease also known as type 1 neurofibromatosis has an autosomal dominant inheritance. It is the commonest form as and presents with numerous café-au-lait macules and neurofibromas. Giant congenital melanocytic nevus (CGMN) on the other hand is characterized by a melanocytic proliferation that present at birth. CGMN develops due to a defective embryonic pigment cell (melanocyte) precursors development and are often present at birth. Giant congenital melanocytic nevus (CGMN) and type 1 neurofibromatosis may occur together rarely. Clinicians should be aware of the rare presentation of both CGMN and type 1 neurofibromatosis in a patient.

Tumescent anesthesia combined with skin and soft tissue expansion for the repair of 41 cases of congenital giant melanocytic nevi / 中华皮肤科杂志

Objective:To investigate the efficacy of tumescent anesthesia combined with skin and soft tissue expansion for the repair of congenital giant melanocytic nevi.Methods:From July 2015 to December 2019, 41 patients with congenital giant melanocytic nevi, including 24 males and 17 females aged 7 - 45 years, were collected from the Department of Dermatology, Xijing Hospital, the Fourth Military Medical University. Skin lesions ranged from 5 cm × 12 cm to 12 cm × 18 cm in size, and were located on the scalp in 13 cases, on the face in 18 cases, as well as on the trunk in 10 cases. Before surgery, the composition of tumescent solution was adjusted according to the body weight, operation duration, skin lesion area, etc., and the total dose and peak plasma concentration of lidocaine should be below 35 mg/kg and 4 mg/L respectively. All the patients received tissue expander placement and second-stage flap transfer under tumescent anesthesia.Results:During surgery, satisfactory effect of tumescent anesthesia was achieved in all the 41 patients, the pain score assessed by a numerical rating scale was 1.82 ± 0.54. In addition, the surgical field and dissection levels were clear with little bleeding and no related complications. Follow-up of 3 - 36 months showed that the skin flaps matched the surrounding skin tissues well, with relatively concealed incision lines and soft flat scars.Conclusion:For the treatment of congenital giant melanocytic nevi, tumescent anesthesia is effective and safe, which combined with skin and soft tissue expansion can effectively reduce the incidence of postoperative complications, and this strategy is worthy of clinical promotion.

Two Cases of Congenital Giant Melanocytic Nevus (CGMN) with Vitiligo / 대한피부과학회지

Congenital giant melanocytic nevi (CGMN) are lesions of more than 20 cm in diameter, often in the distribution of a garment. Depigmented zones may appear around several types of acquired lesions such as dermal, junctional, and compound nevi, Spitz nevus, blue nevus, and malignant melanoma. The depigmentation may occur within the lesion, around it, or at a distant site. In contrast, congenital nevi rarely develop depigmentation. Vitiligo-like leukoderma distant from the nevus has developed in only three cases with a CGMN. We report two cases of CGMN with vitiligo which is located away from primary CGMN lesion.