Generic selectors
Exact matches only
Search in title
Search in content
Search in posts
Search in pages
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstract
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Addendum
Announcement
Art & Psychiatry
Article
Articles
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Commentary
Conference Oration
Conference Summary
Continuing Medical Education
Correspondence
Corrigendum
Cosmetic Dermatology
Cosmetology
Current Best Evidence
Current View
Derma Quest
Dermato Surgery
Dermatopathology
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
e-IJDVL
Editor Speaks
Editorial
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Errata
Erratum
Focus
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
General
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
History
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL AWARDS 2015
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
Index
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
LETTER TO THE EDITOR - LETTERS IN RESPONSE TO PUBLISHED ARTICLES
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters in Response to Previous Publication
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Medicolegal Window
Messages
Miscellaneous Letter
Musings
Net Case
Net case report
Net Image
Net Letter
Net Quiz
Net Study
New Preparations
News
News & Views
Obituary
Observation Letter
Observation Letters
Oration
Original Article
ORIGINAL CONTRIBUTION
Original Contributions
Pattern of Skin Diseases
Pearls
Pediatric Dermatology
Pediatric Rounds
Perspective
Presedential Address
Presidential Address
Presidents Remarks
Quiz
Recommendations
Regret
Report
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Retraction
Review
Review Article
Review Articles
Revision Corner
Self Assessment Programme
SEMINAR
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Special Article
Specialty Interface
Studies
Study Letter
Supplement-Photoprotection
Supplement-Psoriasis
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
SYMPOSIUM - VITILIGO
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Systematic Reviews and Meta-analysis
Tables
Technology
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapeutics
Therapy
Therapy Letter
View Point
Viewpoint
What’s new in Dermatology
View/Download PDF

Translate this page into:

Observation Letters
87 (
5
); 693-695
doi:
10.25259/IJDVL_713_20
pmid:
34379942

Atrophic pigmented dermatofibrosarcoma protuberans misdiagnosed as hyperpigmentation

Department of Dermatology and Venereology, Peking University First Hospital, Xicheng, Beijing, China
Corresponding author: Dr. Hang Li, Department of Dermatology and Venereology, Peking University First Hospital, Xicheng, Beijing, China. drlihang@126.com
Licence
This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

How to cite this article: Lin P, Yang Z, Tu P, Li H. Atrophic pigmented dermatofibrosarcoma protuberans misdiagnosed as hyperpigmentation. Indian J Dermatol Venereol Leprol 2021;87:693-5.

Sir,

Dermatofibrosarcoma protuberans is a rare cutaneous fibroblastic sarcoma of intermediate malignancy characterized histologically by spindle cells arranged in a storiform pattern.1 Although, several uncommon subtypes of dermatofibrosarcoma protuberans including pigmented or atrophic variants have been described, atrophic pigmented dermatofibrosarcoma protuberans is extremely rare, making it much more difficult to identify clinically. Here, we describe a case of atrophic pigmented dermatofibrosarcoma protuberans misdiagnosed as hyperpigmentation.

A 33-year-old female had an asymptomatic, bluish and slightly depressed lesion on her left upper back for ten years which was previously diagnosed as postinflammatory hyperpigmentation and did not receive any treatment. Three years ago after her last child birth, she noticed that the lesion began to gradually enlarge and developed peripheral erythema. No history of trauma could be identified. She was otherwise healthy and denied systemic symptoms or relevant family history. On dermatological examination, there was a firm, 1.6 × 1.3 cm, erythematous-to-bluish ill-defined, slightly depressed plaque on the left upper back [Figure 1a]. Nonpolarized dermoscopic evaluation with DERMOSCOPY-II (Dermat, Beijing) at 20x magnification revealed linear vessels within yellowish background with bluish structureless areas and shiny white streaks. No pigment network was observed [Figure 1b]. Histopathological analysis on low-power view revealed fascicles of densely packed spindle cells extending around fat tissue with a reduced dermal thickness [Figure 2a]. Cytologically, the monomorphic spindle cells had elongated darkly staining nuclei and bland cytoplasm with minimal mitotic figures [Figure 2b]. Scattered dendritic cells abundant in melanin were noticed [Figure 2b]. Immunohistochemical staining of spindle cells was positive for CD34 [Figure 2c] and vimentin, while negative for factor XIIIa and S-100. Melanin-laden dendritic cells were positive for S-100 [Figure 2d]. Modified Mohs micrographic surgery was performed to excise the lesion followed by secondary healing. No recurrence was observed at one-year follow-up.

Figure 1a:: A firm, 1.6 × 1.3 cm, erythematous-to-bluish ill-defined, slightly depressed plaque seen on the left upper back
Figure 1b:: Non-polarized dermoscopy with DERMOSCOPY-II (Dermat) at 20x magnification revealed linear vessels (arrowhead) within yellowish background with blue structureless areas and shiny white streaks (arrows)
Figure 2a:: Histopathologically, fascicles of densely packed spindle cells extending around fat tissue with reduced dermal thickness by about half on low-power view (Hematoxylin-eosin; ×15)
Figure 2b:: Monomorphic spindle cells display elongated darkly staining nuclei and bland cytoplasm with minimal mitotic figures. Scattered dendritic cells abundant in melanin also seen (Hematoxylin-eosin; ×200)
Figure 2c:: Immunohistochemistry showed CD34 + cells infiltrating into subcutis and forming honeycomb-like pattern (anti-CD34; ×200)
Figure 2d:: Immunohistochemistry showed spindle cells were negative for S-100, in contrast, melanin-bearing dendritic cells were positive for S-100 (anti-S-100; ×200)

Based on the clinicopathological features, more than ten variants of dermatofibrosarcoma protuberans including atrophic and pigmented variants have been described.2 Atrophic dermatofibrosarcoma protuberans was first described by Lambert in 1985 characterized by a slow-growing depressed plaque which revealed decreased dermal thickness by >50% compared with the surrounding dermis histologically.1,3 According to a single-center experience, atrophic variant accounts for only 1.7% (16/937) of all dermatofibrosarcoma protuberans cases.4 Pigmented dermatofibrosarcoma protuberans, also known as Bednar tumor, usually presents as a bluish plaque and is characterized by melanin-laden cells histologically.1 It predominantly occurs in Black population and accounts for approximately 1–5% of all dermatofibrosarcoma protuberans cases.1 With both characteristic histological features seen in our case, the diagnosis of atrophic pigmented dermatofibrosarcoma protuberans was established. A typical dermatofibrosarcoma protuberans lesion appears as an indurated protuberant plaque or nodule which may mimic dermatofibroma, neurofibroma, leiomyoma, keloid and desmoid tumors and other soft-tissue sarcomas such as Kaposi sarcoma and fibrosarcoma.5 The differential diagnosis of atrophic dermatofibrosarcoma protuberans, however, consists of another group of disorders including morphea, anetoderma, morphea-like or sclerosing basal cell carcinoma, atrophic dermatofibroma, atrophic scar, lipoatrophy, lymphocytoma etc.6 When it presents with bluish pigmentation, differential diagnoses of bruise, blue nevi, melanocytic nevi and malignant melanoma should also be taken into consideration.

So far, seven cases of atrophic pigmented dermatofibrosarcoma protuberans in addition to ours have been reported in PubMed, all presenting with a depressed pigmented plaque, three of which developed a nodule within the plaque [Table 1].4,7-10 Due to their atypical presentations, various incorrect diagnoses were considered, including lipoatrophy, hemangioma and neurofibroma.4,9 In our case, misdiagnosis of hyperpigmentation was made at initial visiting and only observation was recommended. It was gradual enlargement of the lesion that made us reconsider the diagnosis. Therefore, any solitary hyperpigmented lesion that does not resolve spontaneously or evolves on follow up should be biopsied to exclude malignancy even if it is not suspected clinically.

Table 1:: Cases of atrophic pigmented dermatofibrosarcoma protuberans reported in PubMed
Age Sex Location Size (mm) Clinical presentations Clinical differential diagnosis Reference
24 F Infraorbital area NA Well-demarcated bluish depressed lesion NA Chuan et al., 19977
16 F Thigh 12×15 Multiple papules, plaques and nodules superimposed over several atrophic or indurated plaques NA Sathyanarayana et al., 200410
34 F Buttock 11×12 Pigmented plaque NA Taura et al., 20168
7 M Wrist 20×40 Hard hemispherical nodule in a red-brown irregular atrophic patch Lipoatrophy Zhang et al., 20189
8 F Forearm 10×10 Nodule over a bluish-black atrophic plaque Hemangioma Zhang et al., 20189
7 M Forearm 5×5 NA NA Xu et al., 20194
44 M Back 25×25 NA Neurofibroma Xu et al., 20194

F: Female, M: Male, NA: Not available

Dermoscopy is a valuable non-invasive tool to differentiate a variety of cutaneous pigmented or non-pigmented tumors. However, it is not widely employed in the diagnosis of dermatofibrosarcoma protuberans. Bernard et al.11 summarized six dermoscopic features based on 15 classical cases of dermatofibrosarcoma protuberans: delicate pigment network, vessels, structureless light brown areas, shiny white streaks, pink background color and structureless hypo- to depigmented areas which are presented in a multicomponent pattern, suggesting the diagnosis of malignancy. Another recent study summarized dermoscopic features of 32 dermatofibrosarcoma protuberans cases as follows: features of vessels, pigment network and pinkish background presented in 26 (81.3%), 25 (78.1%) and 21 (65.6%) patients, respectively.12 On a literature search, we could not come across any specific dermoscopic features of atrophic pigmented dermatofibrosarcoma protuberans. Vessels and white streaks seen in our case were similar to the description of classic types. Structureless yellowish background may be a distinctive finding in atrophic type which might be the result of dermal atrophy and approximation of the subcutis to the epidermis. Bluish pigmentation likely corresponded to the distribution of melanin-laden cells in the dermis in pigmented dermatofibrosarcoma protuberans.

Modified Mohs micrographic surgery was performed to achieve complete resection with minimal sacrifice of normal tissue. No differences in terms of treatment options and prognosis between classical and atrophic or pigmented dermatofibrosarcoma protuberans have been delineated. However, atypical presentations often lead to misdiagnosis, delayed surgery as well as inadequate resection margins. Knowledge of the clinical characteristics dermoscopic features of this variant may aid in early diagnosis of atypical dermatofibrosarcoma protuberans.

Declaration of patient consent

The patient's consent is not required as the patient's identity is not disclosed or compromised.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

  1. , , , , . Dermatofibrosarcoma protuberans: A comprehensive review and update on diagnosis and management. Semin Diagn Pathol. 2013;30:13-28.
    [CrossRef] [Google Scholar]
  2. , , , , , , et al. Outcome after surgical treatment of dermatofibrosarcoma protuberans: Is clinical follow-up always indicated? Cancer. 2019;125:735-41.
    [CrossRef] [Google Scholar]
  3. , , , , , . Dermatofibrosarcoma non-protuberans: Description and report of five cases of a morpheaform variant of dermatofibrosarcoma. J Surg Oncol. 1985;28:7-11.
    [CrossRef] [Google Scholar]
  4. , , . Atrophic dermatofibrosarcoma protuberans: A clinicopathological study of 16 cases. Pathology. 2019;51:615-20.
    [CrossRef] [Google Scholar]
  5. , . Dermatofibrosarcoma protuberans. Curr Treat Options Oncol. 2017;18:56.
    [CrossRef] [Google Scholar]
  6. , . Atrophic dermatofibrosarcoma protuberans: Case report, review, and proposed molecular mechanisms. J Am Acad Dermatol. 2003;49:761-4.
    [CrossRef] [Google Scholar]
  7. , , , . Atrophic pigmented dermatofibrosarcoma presenting as infraorbital hyperpigmentation. Dermatology. 1997;194:65-7.
    [CrossRef] [Google Scholar]
  8. , , , , , , et al. Case of pigmented dermatofibrosarcoma protuberans with atrophic change. J Dermatol. 2016;43:1231-2.
    [CrossRef] [Google Scholar]
  9. , , . Two childhood cases of pigmented dermatofibrosarcoma protuberans with atrophic change. Eur J Dermatol. 2018;28:225-6.
    [CrossRef] [Google Scholar]
  10. . Childhood onset dermatofibrosarcoma protuberans. Indian J Dermatol Venereol Leprol. 2004;70:310-2.
    [Google Scholar]
  11. , , , , , . Dermoscopy of dermatofibrosarcoma protuberans: A study of 15 cases. Br J Dermatol. 2013;169:85-90.
    [CrossRef] [Google Scholar]
  12. , , , , . Dermoscopy of dermatofibrosarcoma protuberans: What do we know? Dermatol Pract Concept. 2019;9:139-45.
    [CrossRef] [Google Scholar]

Fulltext Views
3,504

PDF downloads
193
View/Download PDF
Download Citations
BibTeX
RIS
Show Sections