10.5826/dpc.1302a91
text/xml
2160-9381
2684
en
Vito Di Lernia
https://creativecommons.org/licenses/by-nc/4.0
Dermatology Practical & Conceptual
Congenital Multiple Nevoid Hypertrichosis
Text.Serial.Journal
https://dpcj.org/index.php/dpc
Image Letter
2023-04-29
2022-07-04
2023-04-29
2023-06-05
e2023091
Dermatol Pract Concept
Vito Di Lernia
2023/04/29
10.5826/dpc.1302a91
e2023091
Dermatology Unit, Arcispedale Santa Maria Nuova, Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy
2160-9381
en
e2023091
Gupta L, Gautam RK, Bharadwaj M. Nevoid hypertrichosis: case report with review of the literature. Int J Trichology 2011; 3: 115-7.
Congenital Multiple Nevoid Hypertrichosis
https://dpcj.org/index.php/dpc/article/download/2684/2087
            Congenital Multiple Nevoid Hypertrichosis
                     Nicola Lippolis1, Alex Curti2, Caterina Longo1,2, Vito Di Lernia3


1 Centro Oncologico ad Alta Tecnologia Diagnostica-Dermatologia, Azienda Unità Sanitaria Locale - IRCCS di Reggio Emilia,
  Reggio Emilia, Italy
2 Dermatology Department, University of Modena & Reggio Emilia, Modena, Italy
3 Dermatology Unit, Arcispedale Santa Maria Nuova, Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy



Citation: Lippolis N, Curti A, Longo A, Di Lernia V. Congenital Multiple Nevoid Hypertrichosis. Dermatol Pract Concept.
2023;13(2):e2023091. DOI: https://doi.org/10.5826/dpc.1302a91
Accepted: August 17, 2022; Published: April 2023
Copyright: ©2023 Lippolis et al. This is an open-access article distributed under the terms of the Creative Commons
Attribution-NonCommercial 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: Vito Di Lernia, Dermatology Unit, Arcispedale Santa Maria Nuova, Azienda USL-IRCCS di Reggio Emilia,
Reggio Emilia, Italy. E-mail: vito.dilernia@ausl.re.it




Case Presentation                                                        Teaching Point
A 3-month-old male infant of Georgian origin was brought                 Nevoid hypertrichosis is a rare disorder consisting of a
for evaluation of three congenital, well-demarcated patches              solitary, circumscribed area characterized by terminal hair
characterized by dark, terminal hairs on the sacral-lumbar               growth on otherwise appearing skin [1]. Multiple patches
region and right lower limb (Figure 1, A and B). On phys-                may be rarely observed. It is predominant in females and
ical examination, the patches had coarse hair of abnormal                arises at birth or shortly after. Hypopigmentation of the un-
length. No changes in the underlying skin were found. Der-               derlying skin has rarely been reported. Histopathologically,
moscopy did not show brown pigmentation or pigment net-                  hair follicles are increased in number, but structurally nor-
work. The pseudo-Darier sign was not detected on palpation.              mal, without characteristic or diagnostic features.
There was no relevant family history. The child had followed                  Nevoid hypertrichosis, in particular when it occurs with
normal developmental milestones. A multidisciplinary pedi-               multiple patches, may be associated with a series of ocular,
atric consultation did not disclose abnormalities. Histology             cerebral, mental, musculoskeletal, neurological, gastroin-
performed on the patient’s back showed only terminal hair                testinal, pulmonary, cardiac abnormalities and with facial
without additional relevant epidermal and dermal changes.                dysmorphia [1,2]. Due to heterogeneity in extracutaneous
A diagnosis of nevoid hypertrichosis was made. A magnetic                manifestations, specific diagnostic procedures should follow
resonance imaging was considered but postponed in absence                a clinical suspicion.
of clinical issues. At eighteen-month follow-up no additional                 The differential diagnosis includes hypertrichosis
clinical problems were discovered.                                       cubiti [1]. Patches of unusually long pigmented hair or focal




Image Letter | Dermatol Pract Concept. 2023;13(2):e2023091                                                                          1
                                                                       hypertrichosis may be observed in individuals with Gorlin
                                                                       syndrome and Aicardi syndrome, suggesting a role of the
                                                                       PTCH protein, a negative regulator of pathways involved
                                                                       in hair follicle formation and growth cycle and subjects with
                                                                       Proteus syndrome, due to a possible role of the AKT1 kinase
                                                                       in the hair follicle development [1]. Smooth muscle hamar-
                                                                       toma, Becker nevus, and melanocytic nevi may present an
                                                                       overlying focal hypertrichosis, but epidermal changes are
                                                                       helpful for the diagnosis.


                                                                       References
                                                                        1. Sotiriadis D, Patsatsi A, Lazaridou E, Sotiriou E,
                                                                           Devliotou-Panagiotidou D. Multiple nevoid ­hypertrichosis as an iso-
                                                                           lated developmental defect. Pediatr Dermatol. 2009;26(4):436-438.
                                                                           DOI: 10.1111/j.1525-1470.2009.00948.x. PMID: 19689520.
                                                                        2. Gupta L, Gautam RK, Bharadwaj M. Nevoid hypertrichosis: case
                                                                           report with review of the literature. Int J ­Trichology. 2011;3(2):
                                                                           115-117. DOI: 10.4103/0974-7753.90829.PMID: 22223975.
                                                                           PMCID:PMC3250008.




Figure 1. Coarse, black hair on the lumbosacral region (A) and right
lower limb (B) (lateral aspect of the right thigh and right ankle).




2                                                                             Image Letter | Dermatol Pract Concept. 2023;13(2):e2023091
