Dermatology: Practical and Conceptual Observation | Dermatol Pract Concept 2015;5(1):12 71 DERMATOLOGY PRACTICAL & CONCEPTUAL www.derm101.com Case presentation A 23-year-old female presented with an asymptomatic, ery- thematous, atrophic macule on the upper right side of her back that increased gradually in size within the last four years without any symptoms (Figure 1A). On physical examination the surface of the lesion was smooth and no induration was examined on palpation. There was no history of trauma. Der- matoscopic evaluation of the lesion using 3Gen DermLite-II Pro HR and documented with DermLite-Foto 3Gen (LLC, Dana Point, CA, USA) showed a homogenous pigment net- work on a purplish erythematous background (Figure 1B). A skin biopsy was taken with the preliminary diagnosis of morphea and cutaneous mastocytosis. Histopathological examination of H&E (hematoxylin and eosin) stained sec- tions revealed epidermal atrophy, increase in melanin in basal keratinocytes and heavy dermal cellular infiltrate composed of spindle-shaped cells arranged in a storiform pattern that extended into the subcutaneous tissue (Figure 2). Immunhis- tochemical staining for CD34 was positive, while factor XIIIa was negative (Figure 3A, B). The diagnosis of dermatofibro- sarcoma protuberans (DFSP) was made according to these findings. The patient was referred to Department of Plastic and Reconstructive Surgery for total excision. Excision was Dermatoscopic findings of atrophic dermatofibrosarcoma protuberans Bengu Nisa Akay1, Ezgi Unlu2, Cengizhan Erdem1, Aylin Okcu Heper3 1 Ankara University Faculty of Medicine, Department of Dermatology, Ankara, Turkey 2 Zekai Tahir Burak Women’s Health Education and Research Hospital, Department of Dermatology, Ankara, Turkey 3 Ankara University Faculty of Medicine, Department of Pathology, Ankara, Turkey Key words: atrophic variant, dermatofibrosarcoma protuberans, dermatoscopy, mesenchymal tumor Citation: Akay BN, Unlu E, Erdem C, Heper AO. Dermatoscopic findings of atrophic dermatofibroma protuberans. Dermatol Pract Concept 2015;5(1)12. doi: 10.5826/dpc.0501a12 Received: September 8, 2014; Accepted: October 5, 2014; Published: January 30, 2015 Copyright: ©2015 Akay et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Funding: None. Competing interests: The authors have no conflicts of interest to disclose. All authors have contributed significantly to this publication. Corresponding author: Dr. Ezgi Unlu, Zekai Tahir Burak Women’s Health Education and Research Hospital, Department of Dermatology, Samanpazarı-Ankara, Turkey. Tel. 90 (312) 3065000; Fax. 90 (312) 3124931. E-mail: drezgiyalcin@yahoo.com Dermatofibrosarcoma protuberans (DFSP) is an uncommon locally aggressive mesenchymal tumor with a high local recurrence rate. Atrophic DFSP is a rare variant of DFSP characterized by a non- protuberant lesion. We report on a 23-year-old female, who presented with an atrophic, asymptomatic macule on the right side of her back 2 cm in diameter. Dermatoscopic examination revealed homog- enous pigment network on a purplish erythematous background. The histopathological finding of the incisional biopsy material was consistent with DFSP. To our knowledge, this is the second case of atrophic DFSP discussing the dermatoscopic features of this relatively rare condition. ABSTRACT mailto:drezgiyalcin@yahoo.com 72 Observation | Dermatol Pract Concept 2015;5(1):12 tive for CD34 and negative for factor XIIIa, atrophic dermatofibroma cells are usually negative for CD34 and both of CD34 and factor XIIIa are positive in the medallion-like dermal dendrocyte hamartoma [3, 4]. Dermatoscopic studies of DFSP are very rare in the literature. In 2013, Ber- nard et al reported the first study of dermatoscopic analysis of DFSP in 15 cases. Delicate pigment network, vessels, structureless light brown areas, shiny white streaks, pink background color- ation and structureless hypopigmented or depigmented areas were defined as six main dermatoscopic features of DFSP [5]. In 2014, dermatoscopic fea- tures such as peripheral dilated vessels forming a mesh-like pattern, milky-red areas, whitish linear structures and fine pigment network were reported in a series of four cases of DFSP [6]. To our knowledge, only one case of atrophic DFSP was presented with dermato- scopic features in the literature [4]. The reported dermatoscopic findings of that case were branching vessels on a yel- lowish background without pigment network. The authors suggested that the dermatoscopic features might be the result of dermal atrophy and close approximation the subcutis to the epi- dermis. In contrast, our case showed made with 3 cm safe surgical margins. No recurrence has been observed for one year. Conclusion DFSP is a rare, slow growing, locally invasive cutaneous neoplasm of fibro- histiocytic origin with intermediate grade malignancy with an incidence between 0.8–5.0 cases per 1 million persons per year [1]. It often shows typi- cally protuberant morphology. The atro- phic presentation of DFSP is the rarest variant of this infrequent neoplasm, which was first described in 1985 [2]. The non-protuberant DFSP can be con- sidered the early clinical stage of DFSP before developing the typical protu- berant feature unless it remains as a non-protuberant tumor that is called atrophic DFSP. It occurs as an atro- phic, asymptomatic plaque that can be difficult to distinguish from morphea, morphea like basal cell carcinoma, scar, lipoatrophy and atrophic dermatofi- broma [3]. Although the clinical appear- ance of atrophic DFSP is different from common protuberant type, histopatho- logical features are similar. Atrophic presentation of the lesions may be due to the marked cellularity of the tumor cells in the dermis and infiltration to the subcutaneous fat [1]. Immunhistochemi- cal staining is important to distinguish DFSP from atrophic dermatofibroma and medallion-like dermal dendrocyte hamartoma. While DFSP cells are posi- Figure 1. (A) Erythematous, atrophic macule on the right side of upper aspect of the back; (B) dermatoscopic evaluation that shows homogenous pigment network on a purplish ery- thematous background. (Copyright: ©2015 Akay et al.) Figure 3. (A) Positive immunhistochemically staining for CD34 (X200); (B) negative immun- histochemically staining for factor XIIIa (X200). (Copyright: ©2015 Akay et al.) Figure 2. Heavy dermal cellular infiltrate composed of spindle shaped cells arranged in a storiform pattern that extended into the subcutaneous tissue (H&E, X25). (Copyright: ©2015 Akay et al.) Observation | Dermatol Pract Concept 2015;5(1):12 73 dermatoscopically regular brown lines reticular on a pur- plish erythematous background. Homogenous brown lines reticular seen in our case correspond to the accumulation of melanin in basal keratinocytes, while the erythematous back- ground is the result of dilated vessels in the dermal plexus. Here, the smooth surface reflects the stratum corneum being normal. The most frequent dermatoscopic pattern associated with dermatofibromas, a well-known and benign cousin of DFSP, is the central white scar-like patch and peripheral deli- cate pigment network [7]. The histopathologic correlation of white scar-like patch in dermatofibroma is pronounced fibro- sis within the papillary dermis. Contrary to dermatofibromas, fibroplasia in the upper half of the dermis is uncommonly observed in DFSP which may explain the absence of white scar-like areas in our case. In conclusion, we have presented the dermatoscopic findings in an unusual case of atrophic DFSP. Atrophic DFSP should be kept in the differential diagnosis for atrophic and depressed skin lesions, particularly those seen on the trunks of women. Dermatoscopy, a noninvasive method, may not only help to differentiate atrophic DFSP from other skin diseases but may also indicate the need for histopathological examina- tion since the disease has prognostic significance. References 1. Hanabusa M, Kamo R, Harada T, et al. Dermatofibrosarcoma protuberans with atrophic apperance at early stage of the tumor. J Dermatol 2007;34:336-39. 2. Lambert WC, Abramovits W, Gonzalez-Sevra A, et al. Dermatofi- brosarcoma nonprotuberans: description and report of five cases of a morpheaform variant of dermatofibrosarcoma. J Surg Oncol 1985;28:7-11. 3. Wu JK, Malik MM, Egan CA. Atrophic dermatofibrosarcoma pro- tuberans: an uncommon and misleading variant. Aus J Dermatol 2004;45:175-77. 4. Gungor S, Buyukbabani N, Buyuk M, et al. Atrophic dermatofibro- sarcoma protuberans: are there specific dermatoscopic features? J Dtsch Dermatol Ges 2014;12:425-27. 5. Bernard J, Poulalhon N, Argenziano G, et al. Dermoscopy of der- matofibrosarcoma protuberans: a study of 15 cases. Br J Dermatol 2013;169:85-90. 6. Avilés-Izquierdo JA, Conde-Montero E, Barchino-Ortiz L, et al. Dermoscopic features of dermatofibrosarcoma protuberans. Aus J Dermatol 2014;55:125-27. 7. Zalballos P, Puig S, Llambrich A, et al. Dermoscopy of dermato- fibromas: a prospective morphological study of 412 cases. Arch Dermatol 2008;144:75-83. http://www.ncbi.nlm.nih.gov/pubmed?term=Avil%C3%A9s-Izquierdo%20JA%5BAuthor%5D&cauthor=true&cauthor_uid=24720426