10.5826/dpc.1201a40
text/xml
2160-9381
1810
en
Ayse Akbas
https://creativecommons.org/licenses/by-nc/4.0
Dermatology Practical & Conceptual
developmental abnormalities
Giant Congenital Melanocytic Nevi and Poliosis in a 3-Day-Old Boy: Is it Just a Coincidence?
Text.Serial.Journal
https://dpcj.org/index.php/dpc
Research Letter
2022-02-02
2021-05-27
2022-02-02
2022-02-09
e2022040
Dermatol Pract Concept
Ayse Akbas
2022/02/02
10.5826/dpc.1201a40
e2022040
Ankara Bilkent City Hospital, Department of Dermatology, Pediatric Dermatology Unit, Ankara, Turkey
2160-9381
developmental abnormalities
en
e2022040
Giant Congenital Melanocytic Nevi and Poliosis in a 3-Day-Old Boy: Is it Just a Coincidence?
https://dpcj.org/index.php/dpc/article/download/1810/1486
                    Dermatology Practical & Conceptual


Giant Congenital Melanocytic Nevi and Poliosis in a
      3-day-old Boy: Is It Just a Coincidence?
                                                 Ahu Yorulmaz1, Ayse Akbas1


1 Ankara Bilkent City Hospital, Department of Dermatology, Pediatric Dermatology Unit, Ankara, Turkey



Key words: congenital, melanocytic nevi, giant, poliosis, developmental abnormalities
Citation: Yorulmaz A, Akbas A. Giant congenital melanocytic nevi and poliosis in a 3-day-old boy: is it just a coincidence?
Dermatol Pract Concept. 2022;12(1):e2022040. DOI: https://doi.org/10.5826/dpc.1201a40
Accepted: July 10, 2021; Published: January 2022
Copyright: ©2022 Yorulmaz and Akbas. This is an open-access article distributed under the terms of the Creative Commons
Attribution License BY-NC-4.0, https://creativecommons.org/licenses/by-nc/4.0/, which permits unrestricted noncommercial use,
distribution, and reproduction in any medium, provided the original authors and source are credited.
Funding: None.
Competing interests: None.
Authorship: All authors have contributed significantly to this publication.
Corresponding author: Ahu Yorulmaz, Ankara Bilkent City Hospital, Department of Dermatology, Universiteler Ankara.
E-mail: ahuyor@gmail.com




Introduction                                                             Conclusions
Giant congenital melanocytic nevi (GCMN) are rare benign                 This is the third report in the literature describing the coex-
nevomelanocytic proliferations that are expected to reach a              istence of GCMN and poliosis [1,2]. In 1999 Yosipovitch et
diameter of at least 20 cm in adulthood. Here, we present a              al presented a patient with GCMN with poliosis [1] and in
case of GCMN associated with poliosis.                                   2013 Lee et al reported a patient with GCMN of the scalp
                                                                         with cranial defect, poliosis and hair loss [2]. GCMN have

Case Presentation                                                        been associated with several developmental abnormalities,
                                                                         including cutis verticis gyrata, limb atrophy/hypertrophy
A 3-day-old baby boy, born of a non-consanguineous mar-                  and hamartomas. Postzygotic mutations affecting somatic
riage, was examined at our pediatric dermatology outpatient              cells may provide one explanation of the occurrence of these
clinic for a black nevus on his back. He was otherwise healthy           associations. Mutations in NRAS, BRAF, and Tp53 have
and his family history was unremarkable. We observed a                   been specified in congenital melanocytic nevi (CMN) sam-
black patch measuring approximately 10 cm 3 15 cm in size                ples. If the mutation develops early enough, it can affect a
on the left upper back of the patient. A localized patch of              multipotent progenitor cell, leading not only to CMN in the
white hair on the occiput and satellite nevi on the face, trunk          cutaneous tissue, but also abnormalities in the other organs.
and extremities of the patient were also noticed (Figure 1). A           Given the accompanying cranial defect and alopecia, Lee et
thorough neurological examination and magnetic resonance                 al proposed that the destruction of cranium, hair follicles and
imaging of the brain and spine didn’t reveal any pathology.              bulb melanocytes might have been caused by an inflammatory
Diagnoses of GCMN and poliosis were made and the patient                 or autoimmune response, or even the destructive potential of
was taken under follow-up.                                               the nevus cells per se [2].




Research Letter | Dermatol Pract Concept. 2022;12(1):e2022040                                                                         1
                                                                      fetal melanocytes into the hair bulbs. It is well-known that
                                                                      melanoblasts originate from pluripotent precursor neural
                                                                      crest cells, migrate along defined pathways and differentiate
                                                                      into melanocytes.
                                                                         The fact that poliosis may arise embryogenetically strongly
                                                                      implies the role of genes in the pathogenesis of poliosis asso-
                                                                      ciated with GCMN. However, the main question still remains
                                                                      unclear: is poliosis an associated feature of melanocytic lesions
                                                                      or is the coexistence of poliosis and GCMN in the same
                                                                      individual a coincidental finding? If this is a real association,
                                                                      what’s the exact underlying mechanism in the pathogenesis?
                                                                      Further case reports will allow the pathogenesis of the asso-
                                                                      ciation between GCMN and poliosis to be better understood.
Figure 1. A bolero type giant congenital melanocytic nevi measuring      Informed consent: Written informed consent for publica-
approximately 10 cm 3 15 cm in size on the left upper back of the     tion of clinical details and clinical images was obtained from
patient. Note the poliosis on the occiput.                            the patient’s parents.


    Poliosis, which is defined as a localized patch of white hair
                                                                      References
within a group of hair follicles, has been described in associ-
ation with different genetic or acquired conditions, including         1. Yosipovitch G, Feinmesser M, Mutalik S. Poliosis associated with
                                                                          a giant congenital nevus. Arch Dermatol. 1999;135(7):859–861.
benign and malignant melanocytic neoplasms. A perifollicu-
                                                                          DOI: 10.1001/archderm.135.7.859. PMID: 10411174.
lar lymphohistiocytic infiltration suggests an inflammatory/
                                                                       2. Lee WJ, Lee SM, Won CH, et al. Coexistence of congenital giant
autoimmune response as the causative mechanism in the                     melanocytic nevus of the scalp with cranial defect, poliosis,
pathogenesis of poliosis in patients with melanocytic lesions.            and hair loss. Pediatr Dermatol. 2013;30(6):e226-e227. DOI:
Another suggested theory involves the migration arrest of                 10.1111/pde.12101. PMID: 23437823.




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