10.5826/dpc.1202a87
text/xml
2160-9381
1957
en
Jie Liu
https://creativecommons.org/licenses/by-nc/4.0
Dermatology Practical & Conceptual
high-frequency ultrasound
Syringocystadenoma Papilliferum and Eccrine Poroma Arising in Verrucous Epidermal Nevus: A Case Report and Multidimensional Skin Imaging Evaluation
Text.Serial.Journal
https://dpcj.org/index.php/dpc
Research Letter
2022-05-02
2021-08-18
2022-05-02
2022-05-24
e2022087
Dermatol Pract Concept
Jie Liu
2022/05/02
10.5826/dpc.1202a87
e2022087
Department of Dermatology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic Diseases  , Beijing, China 
2160-9381
high-frequency ultrasound
en
e2022087
Syringocystadenoma Papilliferum and Eccrine Poroma Arising in Verrucous Epidermal Nevus: A Case Report and Multidimensional Skin Imaging Evaluation
https://dpcj.org/index.php/dpc/article/download/1957/1580
                   Dermatology Practical & Conceptual


       Syringocystadenoma Papilliferum and Eccrine
       Poroma Arising in Verrucous Epidermal Nevus:
         A Case Report and Multidimensional Skin
                    Imaging Evaluation
                                                      Shan Zhang1, Jie Liu1


1 Department of Dermatology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical College Hospital, Chinese
  Academy of Medical Science and Peking Union Medical College, National Clinical Research Center for Dermatologic and Immunologic
  Diseases, Beijing, China



Key words: verrucous epidermal nevus, syringocystadenoma papilliferum, eccrine poroma, dermoscopy, high-frequency ultrasound
Citation: Zhang S, Liu J. Syringocystadenoma Papilliferum and Eccrine Poroma Arising in Verrucous Epidermal Nevus: A Case Report and
Multidimensional Skin Imaging Evaluation. Dermatol Pract Concept. 2022;12(2):e2022087. DOI: https://doi.org/10.5826/dpc.1202a87
Accepted: October 4, 2021; Published: April 2022
Copyright: ©2022 Zhang 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: This work is supported by grants from National Natural Science Foundation of China (No. 82173449) and the Non-profit
Central Research Institute Fund of Chinese Academy of Medical Sciences (No. 2019XK320024).
Competing interests: None.
Authorship: Both authors have contributed significantly to this publication.
Corresponding author: Jie Liu, Department of Dermatology, State Key Laboratory of Complex Severe and Rare Diseases, Peking Union Medical
College Hospital, Chinese Academy of Medical Science and Peking Union Medical College, National Clinical Research Center for Dermatologic
and Immunologic Diseases, No.1 Shuaifuyuan Wangfujing Dongcheng District, Beijing, China 100730. E-mail: Liujie04672@pumch.cn




Introduction                                                            Four years ago, a hemispherical nodule, with erosion and
                                                                        bleeding recurrently, and multiple pink to violaceous papules
Verrucous epidermal nevus (VEN) is hamartoma charac-                    gradually developed on the preexisting lesion.
terized by hyperplasia of keratinocytes. Some cases of sec-                    Clinical examination revealed a band-like, light-brown
ondary tumors developing in VEN have been reported, but                 plaque, extending from the middle of the right thigh to the
adnexal tumors are infrequent, especially coexistence of                dorsum of the right foot. On the popliteal fossa, there was
more than one type of adnexal tumors. We report a case of               a black-brown verrucous hyperplastic plaque of 10 cm in
syringocystadenoma papilliferum (SCAP) and multiple ec-                 length, without erosion or exudation (Figure 1A). Skin bi-
crine poromas (EP) arising in a VEN, and the dermoscopic                opsy revealed VEN.
and ultrasonic features of these lesions.                                      On the right medial malleolus, there was a red nodule
                                                                        of 1.5 cm in diameter with erosions and yellowish crusts
                                                                        (Figure 1B). Dermoscopy revealed pinkish-white ulcerated
Case Presentation
                                                                        areas, polymorphic vessels and yellow crusts (Figure 1C).
A 28-year-old woman had the band-like, light-brown verru-               Ultrasound showed a superficial dermal lesion with regular
cous plaque on her right leg since birth, with no symptoms.             shape, well-defined margin and heterogeneous internal echo,



Research Letter | Dermatol Pract Concept. 2022;12(2):e2022087                                                                          1
Figure 1. (A) Clinical presentation: light-brown plaques extending from the middle of the right thigh to the right foot. (B) Clinical presenta-
tion: a red nodule of 1.5 cm in diameter on the right medial malleolus with erosions and yellowish crusts. (C) Dermoscopically, polymorphic
vessels were seen within the pinkish-white ulcerated areas, with yellow crusts. (D) High-frequency ultrasound showed a superficial dermal
lesion with regular shape, well-defined margin and heterogeneous internal echo, and superficial hyperechoic focus (white circle) (50 MHz).
(E) Histopathology showed papillomatosis of the epidermis. Endophytic tumor extended from the epidermis with intraluminal papillary
fronds (H&E staining; original magnification, ×40). (F) The fronds are lined by double-layered epithelium, with basal cuboidal cells and api-
cal columnar apocrine cells. Dense infiltration of lymphocytes and plasma cells in the cores of fronds and decapitation secretion were noted
(H&E staining; original magnification, ×100).


and superficial hyperechoic focus (Figure 1D). Histopatho-               and most of them are epithelial tumors [1,2]. However,
logic examination revealed endophytic tumor extended from                SCAP and EP are both benign adnexal neoplasms, which are
the epidermis with intraluminal papillary fronds, which                  quite rare in VEN. To the best of our knowledge, the case of
were lined by a bilayer. Dense infiltrate of lymphocytes                 SCAP and EP successively developed in a VEN has not been
and plasma cells and decapitation secretion could be seen                documented previously.
(Figure 1, E and F). It was consistent with SCAP.                            We applied non-invasive skin imaging techniques in the
    On the medial of the right lower leg and knee, there were            diagnosis. We observed the dermoscopic feature of polymor-
3 pink to violaceous papules (Figure 2, A and B). Under                  phous vascular pattern, a sign of malignancy. It indicated
dermoscopy, white streaks, short linear, coiled and looped               a biopsy should be performed. The ultrasound showed the
vessels with yellow background were observed (Figure 2C).                lesions were in the superficial dermal with well-defined mar-
Ultrasound showed well-defined, oval-shaped dermal lesions               gin, which indicated that they tended to be benign conditions
with heterogeneous internal echo and hyperechoic spots                   and helped assess the excision extension.
(Figure 2D). These papules had a similar appearance under
microscopy. Well-circumscribed dermal neoplasms contin-
                                                                         Conclusions
uous with the epidermis. Ductal differentiation was noted.
(Figure 2, E and F). The diagnosis of EP was made.                       We reported a quite rare case of SCAP and EP arising in
    The tumors were resected and the patient was still under             a VEN. We applied dermoscopy and high-frequency ultra-
follow-up.                                                               sound in the evaluation, and demonstrated the multidimen-
                                                                         sional skin imaging features of SCAP and EP.

Discussion
VEN derive from hyperplasia of keratinocytes, unlike organ-
oid epidermal nevi, secondary tumor is relatively infrequent,



2                                                                             Research Letter | Dermatol Pract Concept. 2022;12(2):e2022087
Figure 2. (A,B) Clinical presentation: 2 violaceous papules on the medial of the right knee, and one pink papule on the lower leg (black
arrows). (C) Dermoscopy showed white streaks, short linear, coiled and looped vessels, with yellow background. (D) Ultrasound revealed a
well-defined, oval-shaped dermal lesion with heterogeneous internal echo and hyperechoic spots (50 MHz). (E) Epitheliomatous hyperplasia
of the epidermis, well-circumscribed dermal neoplasms continuous with the epidermis. Inflammatory cells infiltrated in the superficial dermis
(H&E staining; original magnification, ×40). (F) Ductal differentiation was noted (H&E staining; original magnification, ×100).



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Research Letter | Dermatol Pract Concept. 2022;12(2):e2022087                                                                              3
