NA
text/xml
2160-9381
2200
en
Meera Thomas
https://creativecommons.org/licenses/by-nc/4.0
Dermatology Practical & Conceptual
Blue Naevi
 Dermoscopy of cutaneous neurocristic hamartoma and report of its rare clinical presentation: Cutaneous Neurocristic hamartoma
Text.Serial.Journal
https://dpcj.org/index.php/dpc
Research Letter
2022-07-28
2021-11-25
2022-07-28
2022-09-15
e2022138
Dermatol Pract Concept
NA
Meera Thomas
2022/07/28
e2022138
Department of Pathology, Christian Medical College, Vellore, Tamil Nadu, India
2160-9381
Blue Naevi
en
e2022138
 Dermoscopy of cutaneous neurocristic hamartoma and report of its rare clinical presentation: Cutaneous Neurocristic hamartoma
https://dpcj.org/index.php/dpc/article/download/2200/1693
Dermoscopy of Cutaneous Neurocristic Hamartoma
   and Report of its Rare Clinical Presentation
                   Dincy Peter1, Priya Kuryan1, Ashish Kumar Gupta2, Meera Thomas3


1 Department of Dermatology, Venereology and Leprosy, Christian Medical College, Vellore, Tamil Nadu, India
2 Department of Plastic Surgery, Christian Medical College, Vellore, Tamil Nadu, India
3 Department of Pathology, Christian Medical College, Vellore, Tamil Nadu, India



Keywords: dermoscopy, cuticis verticis gyrata, neurocristic hamartoma, Blue Naevi
Citation: Peter D, Kuryan P, Gupta AK, Thomas M. Dermoscopy of cutaneous neurocristic hamartoma and report of its rare clinical
presentation. Dermatol Pract Concept. 2022;12(3):e2022138. DOI: https://doi.org/10.5826/dpc.1203a138
Accepted: December 27, 2021; Published: July 2022
Copyright: ©2022 Peter et al. 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: Dincy Peter, Department of Dermatology, Venereology and Leprosy, Christian Medical College, Vellore – 632004,
Tamil Nadu, India. Telephone: 091-04162282054 Fax: +91-4162232035 E-mail: dincy.peter@cmcvellore.ac.in




Introduction                                                             colors from blue, gray, white and brown color. The dermos-
                                                                         copy of nodules showed blue clods over a gray background
Cutaneous neurocristic hamartoma (NCH) is a rare ham-                    with peripheral brown pigment network (Figure 1C), blue
artomatous proliferation of melanocytic, neuroid, and mes-               gray clods on a white background (Figure 1D) blue clods
enchymal tissues first described by Tuthill et al in 1982 [1].           on a white background with brown dots (Figure 1E). The
                                                                         scalp nodule showed blue gray clods, white featureless areas,

Case Presentation                                                        brown dots and streaks, multiple clustered dotted and linear
                                                                         vessels (Figure 2A). The clinical differential diagnoses con-
A 33-year-old man presented with swelling of the scalp and               sidered were blue nevus, cerebriform intradermal nevi, ne-
multiple bluish swellings on the body since birth. He had a              vus sebaceous and cylindroma. An excision biopsy and split
surgical excision of a scalp lesion at four years of age after           thickness skin grafting of the scalp lesion was done. Histopa-
which the swelling had recurred. There was a gradual in-                 thology from the scalp and trunk nodule showed proliferated
crease in size of the swelling since the past four years. On             spindled to epithelioid nevus cells in dermis and intervening
examination, he had a cerebriform swelling with alopecia,                stroma showing a proliferation of bland spindle shaped cells,
akin to cuticis verticis gyrata with areas of bluish color and           few of which had wavy nuclear contour (Figure 2, B and C).
depigmentation seen over the occipital scalp and extending               On immunohistochemistry, the nevus cells were positive for
onto the neck. (Figure 1A). Multiple bluish firm nodules                 S100, HMB 45 and Melan A and stromal spindle cells for
were seen over the trunk (Figure 1B). The skin lesions on                CD 34 (Figure 2, D and E). The MIB index was less than 1%.
dermoscopy (FotoFinder Systems GmbH) showed varying                      A diagnosis of benign NCH was made.



Research Letter | Dermatol Pract Concept. 2022;12(3):e2022138                                                                          1
Figure 1. (A) Cerebriform swelling with alopecia, akin to cuticis verticis gyrata with areas of bluish color and depigmentation seen over
the occipital scalp. (B) Multiple bluish firm nodules over the back. (C) Dermoscopy of skin nodule showing blue clods (white arrow) over
a gray background with peripheral brown pigment network (white arrow head, (20x, non-polarized, fotofinder GmBH). (D) Dermoscopy
of skin nodule showing blue gray clods (white arrow) on a white back ground (20X, non-polarized, fotofinder, GmBH). (E) Dermoscopy
of nodule over soles showing blue clods (black arrow) on a white background with brown dots (black arrowhead, (20x, non-polarized,
fotofinder, GmBH).




    Cutaneous neurocristic hamartoma is a rare hamartoma-             histopathologically, and even dermoscopically NCH can re-
tous proliferation of melanocytic, neuroid, and mesenchymal           semble a blue nevus. Dermoscopy of blue nevi showed a ho-
tissues. They show variable differentiation including melano-         mogeneous, structureless pigment pattern, with either blue,
cytic (nevoid, spindle cell, and dendritic), neurosustentacular       white–blue, black, brown, and polychromatic [4]. The white-
(Schwann cell and perineural cells), and mesenchymal fibro-           blue color was attributed to the oval/spindle cells the deeper
genic elements [2]. Congenital or acquired lesions present            location of the lesions within the dermis and the presence of
as pigmented macules, papules or nodules with or without              a thicker subepidermal grenz zone and was seen more with
alopecia. There is a predilection for the scalp. Benign NCH           hypochromic blue nevi [4]. Our patient had polychromatic
presenting as cutis verticis gyrata is very rare but has been         lesions but predominantly blue gray and white clods. On his-
reported [3].                                                         topathology had epithelioid/spindle cells with deep reticular
    Neurocristic hamartoma is a dermal dendritic melano-              dermis extension. The course of NCH is generally indolent,
cytic proliferation under borderline category [4] and shares          however, longstanding NCH has been reported to give rise
histological features with blue and congenital nevi. Clinically,      to cutaneous malignant melanocytic neurocristic tumors [5].




2                                                                          Research Letter | Dermatol Pract Concept. 2022;12(3):e2022138
                                                                    Figure 2. (A) Dermoscopy of scalp lesion showing blue gray clods
                                                                    (black arrow), white featureless areas, brown dots and streaks
                                                                    (black arrow head), multiple clustered dotted (white arrow) and
                                                                    linear vessels (20x, nonpolarized, fotofinder, GmBH). (B) Dendritic
                                                                    melanocyte (black arrow) with nests of nevus cells close to a nerve
                                                                    (H&E 40x). (C) Dermis showing spindle shaped cells with perineu-
                                                                    ral distribution (white arrow) and cells seen involving the interven-
                                                                    ing stroma, with wavy nuclear contours (black arrow,(H&E 40x).
                                                                    (D) Immunohistochemistry stain showing S 100 positive neural
                                                                    cells (S 100 40x). (E) Immunohistochemistry stain showing mela-
                                                                    nocytes stained with Melan A (black arrow, Melan A 40x).




Conclusions                                                            Clinical Correlations. 2nd ed. Philadelphia, PA: Elsevier Mosby;
                                                                       2005:1241–1308.
Cutaneous NCH presenting as cuticis verticis gyrata is rare.        3. Saha D, Kini UA, Kini H. Cutaneous Neurocristic Hamartoma
                                                                       Presenting as Cutis Verticis Gyrata. Am J Dermatopathol. 2014;
We have described the dermoscopic findings seen in our
                                                                       36(3):e66–69. DOI: 10.1097/DAD.0b013e3182919c76. PMID:
patient.                                                               23812020.
                                                                    4. Ferrara G, Soyer HP, Malvehy J, et al. The many faces of blue nevus.
References                                                             A clinicopathologic study. J Cutan Pathol. 2007:34(7): 543– 551.
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    Arch Dermatol. 1982;118(8):592–596. DOI: 10.1001/arch-             melanoma and malignant blue nevus. Am J Surg Pathol. 2011;
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Research Letter | Dermatol Pract Concept. 2022;12(3):e2022138                                                                           3
