Brief Report
744 Ann Dermatol
Received September 15, 2017, Revised January 2, 2018, Accepted for publication January 10, 2018
Corresponding author: Kwang Hyun Cho, Department of Dermatology, Seoul National University Hospital, 101 Daehak-ro, Jongno-gu, Seoul 03080, Korea.
Tel: 82-2-2072-2412, Fax: 82-2-742-7344, E-mail: khcho@snu.ac.kr ORCID: https://orcid.org/0000-0001-9581-0265
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/
licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Copyright © The Korean Dermatological Association and The Korean Society for Investigative Dermatology
nail is a distinguishing trait of onychopapilloma.
In conclusion, we report two rare cases of onychopapillo- ma, which occurred in Asia. Dermoscopic assessment of subtle changes of the nail and its margins facilitates the recognition of onychopapilloma in the clinical setting.
SUPPLEMENTARY MATERIALS
Supplementary data can be found via http://anndermatol.
org/src/sm/ad-30-742-s001.pdf.
CONFLICTS OF INTEREST
The authors have nothing to disclose.
REFERENCES
1. Baran R, Perrin C. Longitudinal erythronychia with distal subungual keratosis: onychopapilloma of the nail bed and Bowen's disease. Br J Dermatol 2000;143:132-135.
2. Tosti A, Schneider SL, Ramirez-Quizon MN, Zaiac M, Miteva M. Clinical, dermoscopic, and pathologic features of onychopapilloma: a review of 47 cases. J Am Acad Dermatol 2016;74:521-526.
3. Kim M, Sun EY, Jung HY, Cho BK, Park HJ. Onychopapil- loma: a report of three cases presenting with various longitudinal chromonychia. Ann Dermatol 2016;28:655-657.
4. Min J, Nam JH, Chae SW, Lee GY, Kim W. Case of onychopapilloma presenting as longitudinal melanonychia.
Dermatologica Sinica 2017;35:102-103.
5. Baran R, Perrin C. Localized multinucleate distal subungual keratosis. Br J Dermatol 1995;133:77-82.
https://doi.org/10.5021/ad.2018.30.6.744
A Case of Indeterminate Dendritic Cell Tumor:
A Rare Neoplasm with Langerhans Cell Lineage
Jungyoon Moon, Ji Hoon Yang, Jaewon Lee, Jong Seo Park, Kwang Hyun Cho
Department of Dermatology, Seoul National University College of Medicine, Seoul, Korea
Dear Editor:
Indeterminate dendritic cell tumor (IDCT) is an extremely rare neoplasm derived from indeterminate cells consid- ered to differentiate into Langerhans cells incompletely1. A 61-year-old man presented with 2-year history of multi- focal ulcerative nodules on his back and chest (Fig. 1). We received the patient’s consent form about publishing all photographic materials. Each lesion started with a red pap- ule and turned into a necrotic nodule. He stated that he
had tried to be treated by surgery in another hospital be- cause the number of the lesions was small at the beginning of the symptom. However, their recurrence in new areas despite repetitive excision allowed him to visit our hospi- tal for a secondary opinion. There was no previous or fam- ily history of skin cancers. He denied any subjective symptoms including pruritus and pain.
Histopathology revealed heavy infiltrates of round to oval tumor cells, filling up the entire dermis with epidermal
Brief Report
Vol. 30, No. 6, 2018 745 Fig. 2. (A) Aggregates of tumor cells resembling Langerhans cells throughout dermis (H&E, ×40). (B) Tumors cells exhibiting varied shaped nuclei and abundant cytoplasm (H&E, ×400). (C) Positive immunohistochemical staining for CD1a (×400), (D) CD68 (×400), and (E) S100 proteins (×400). (F) Negative immunohistochemical staining for langerin (×400).
Fig. 1. Multiple reddish brown necrotic and crusted nodules on the back and chest.
Brief Report
746 Ann Dermatol
sparing (Fig. 2A). Each cell was composed of abundant cy- toplasm and various shaped nuclei with fine chromatin (Fig. 2B). Some cells displayed atypical chromatin-rich patterns. Several lymphocytes surrounding the tumor cells but not in the epidermis were found, but eosinophils were obviously absent. The tumor cells represented strong im- munoreactivity for CD1a (Fig. 2C), CD68 (Fig. 2D), and S100 proteins (Fig. 2E), but were negative for Langerhans cell markers and follicular dendritic cell markers such as langerin (Fig. 2F) and CD21, respectively. The Ki-67 pro- liferation index was 70%. Based on the above findings, he was finally diagnosed with IDCT. The patient was trans- ferred to the hemato-oncologic division for further evalua- tion, and no definite evidence of internal organ involve- ment was detected. His condition began to improve spon- taneously, so he has been on regular follow-up for 3 months without treatment now.
According to the updated version of 2011 on the 2008 World Health Organization classification, IDCT is classi- fied into a group of histiocytic and dendritic cell neo- plasms. Due to the rarity of IDCT with only about 80 cas- es demonstrated before, its etiology has not been well elu- cidated yet. IDCT can arise de novo, or in conjunction with several diseases including B-cell lymphoma, an- gioimmunoblastic T-cell lymphoma, and mosquito bites1,2. IDCT is usually confined to the skin, and occurs in adults without sex predilection3. The diagnosis is very challeng- ing for clinicians and pathologists because of its un- familiarity, and it is essential to be distinguished from oth- er Langerhans cell lineage tumors such as Langerhans cell histiocytosis (LCH). Tumors cells in IDCT exhibit similar morphology to Langerhans cells with expression of CD1a and S100 proteins1. However, Birbeck granules and lan- gerin are not expressed in IDCT unlike in LCH3. Also, there are lack of apparent epidermotropism and significant eosinophilic infiltration in IDCT, which are characteristic
features of LCH3. From this perspective, ultrastructural analysis of Birbeck granules or immunostaining for langer- in is regarded to be valuable for accurate diagnosis of IDCT instead of LCH. Chemotherapy, phototherapy or electron beam therapy effectively results in favorable re- sponse, but relapse is common after cessation of the treat- ment4. Spontaneous regression without recurrence over 17 months to 4 years of follow-up has also been reported in some cases, like our patient2,5.
Herein, we introduce a case of IDCT, an uncommon neo- plasm composed of indeterminate cells resembling Langerhans cells characterized by unique clinicopatho- logical features.
CONFLICTS OF INTEREST
The authors have nothing to disclose.
REFERENCES
1. Rezk SA, Spagnolo DV, Brynes RK, Weiss LM. Indeterminate cell tumor: a rare dendritic neoplasm. Am J Surg Pathol 2008;32:1868-1876.
2. Mo X, Guo W, Ye H. Primary indeterminate dendritic cell tumor of skin correlated to mosquito bite. Medicine (Baltimore) 2015;94:e1443.
3. Roh J, Kim SW, Park CS. Indeterminate dendritic cell tumor:
a case report of a rare langerhans cell lineage disease. J Pathol Transl Med 2016;50:78-81.
4. Cheuk W, Cheung FY, Lee KC, Chan JK. Cutaneous indeterminate dendritic cell tumor with a protracted relapsing clinical course. Am J Surg Pathol 2009;33:1261- 1263.
5. Contreras F, Fonseca E, Gamallo C, Burgos E. Multiple self-healing indeterminate cell lesions of the skin in an adult.
Am J Dermatopathol 1990;12:396-401.
