Is Neural Crest Malignancy On The Same Side As The Skin Lesions Of Segmental Neurofibromatosis

Instance Report (ISSN: 2643-6760)

Segmental Neurofibromatosis: Two Moroccan Cases Volume one - Consequence 4

Department of Dermatology-Venereology, Morocco

Received: November 22, 2018; Published: November 28, 2018

Respective author: El Anzi Ouiam, Department of Dermatology-Venereology, Morocco

DOI: x.32474/SCSOAJ.2018.01.000119

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Abstruse

Segmental neurofibromatosis (SN) or blazon Five Neurofibromatosis is a genodermatos almost often characterized by the presence of neurofibromas, more rarely café au lait spots, and sometimes lentigines express to a limited trunk region. We report ii Moroccan cases of truthful Segmental Neurofibromatosis with simply pigmented lesions.

Keywords: Segmental Neurofibromatosis; Type V Neurofibromatosis; Café au lait spots; Neurofibromas; Lentigines

Introduction

Segmental neurofibromatosis (SN) or type Five Neurofibromatosis is a genodermatos about often characterized by the presence of neurofibromas, more rarely café au lait spots, and sometimes lentigines limited to a limited body region, or more rarely on several bilateral segments. It is infrequent, with an estimated prevalence between 0.0014-0.002%. Nosotros written report 2 Moroccan cases of SN.

Clinical case

Observation N ane: A 10-year-old boy, with no family history of Neurofibromatosis (NF), who has had numerous café-au-lait spots and lentigines at the left lumbar region since the age of 2. No ophthalmologic and neurological symptoms of neurofibromatosis were diagnosed. The diagnosis of segmental neurofibromatosis (NF-5) has therefore been established.

Observation N2: A 35-twelvemonth-erstwhile adult female with no concept of consanguinity, followed in neurology for a multiple sclerosis nether treatment presented with a 25-year history of coffee-milk spots and lentigines at the correct lumbar region.

Comments: The term segmental neurofibromatosis was introduced by Miller and Sparkes in 1977 [i-ii]. Segmental NF1 is caused by somatic mosaicism due to a postzygotic mutation in the NF1 cistron. This results in some cells containing two normal NF1 genes and other cells having a mutation in one copy of the NF1 gene [iii]. Due to the multiplicity of clinical manifestations of NF, Riccardi ranked NF in eight categories in 1982. Type V was reserved for the NS [4-two]. In 1987, [5] Have classified the NS in iv variants: truthful segmental, Localized with deep development, hereditary and bilateral. Both of our patients had a true NS.

The most common clinical manifestation of NS is: neurofibromas, and less frequently, coffee-au-lait spots.Clinically, patients can be divided into four groups: NS with only pigmented lesions, NS with merely neurofibromas, NS with pigmented lesions and neurofibromas, and NS with isolated plexiform neurofibromas [1,2,four]. Our patients have only pigmented lesions. Systemic involvement in NS is rare, except in patients with plexiform neurofibromas. NF I and NS may be associated with malignant tumors such equally peripheral nerve sheath tumor, melanoma, chest cancer, colon cancer, stomach cancer, lung cancer and Hodgkin lymphoma [six] (Figures ane & 2).

Effigy one: Patient 1 : Multiple lentigines and café au lait spots on the left lumbar region.

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Figure ii: Patient 2: Multiple lentigines and café au lait spots on the correct lumbar region.

Conclusion

We present the first two cases of Moroccan SN. The run a risk of association with malignant tumors or with systemic involvement is not confirmed, and so patients presenting SN must have a long-term medical follow-up.

References

Miller RM, Sparkes RS (1977) Segmental neurofibromatosis. Arch Dermatol 123: 837-838.
Michał Sobjanek, Dobosz Kawałko K, Michajłowski I, Pęksa R, Nowicki R (2014) Segmental neurofibromatosis. Postep Derm Alergol 31(half dozen): 410- 412.
He D, Li Y, Yu Y, Cai Thousand, Ouyang F, et al. (2010) Segmental neurofibromatosis type 1 complicated with multiple intracranial arteriovenous fistulas: a case study, Clinical Neurology and Neurosurgery 168: 108-111.
Riccardi VM (1982) Neurofibromatosis: clinical heterogeneity. Curr Probl Cancer 7: 1-34.
Roth RR, Martines R, James WD (1987) Segmental neurofibromatosis. Arch Dermatol 123: 917-920.
Dang JD, Cohen PR (2010) Segmental neurofibromatosis and malignancy. Skinmed 8(iii): 156-159.