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Arch Dermatol Res. Author manuscript; available in PMC 2022 Oct 1.
Published in final edited form as:
Arch Dermatol Res. 2021 Oct; 313(8): 633–640.
Published online 2020 Sep 25. doi:10.1007/s00403-020-02142-6
PMCID: PMC9396668
NIHMSID: NIHMS1825584
PMID: 32978676
Japbani K. Nanda, BS,1 Nadeem Marghoob, DO,2 Diana M. Forero Cuevas, MD,3 Katherine R. Lee, MD,4 Michelle Levy, MD,5 Ofer Reiter, MD,1,6 Klaus J. Busam, MD,7 and Ashfaq A. Marghoob, MD1
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The publisher's final edited version of this article is available at Arch Dermatol Res
Abstract
Fibroepithelioma of Pinkus (FEP) is a subtype of basal cell carcinoma (BCC) that can clinically resemble intradermal nevi (IDN) and fibromas. We performed a retrospective review of consecutively biopsied lesions confirmed to be FEP on histopathology diagnosed from January 1, 2008 to April 8, 2019. Clinical (n = 48), contact non-polarized dermoscopy (NPD) (n = 44), and contact polarized dermoscopy (PD) (n = 22) images from 36 patients were reviewed. Mean age was 64.5 years (SD 15.1 years, range 24–86 years) at diagnosis of first FEP lesion. Most lesions were located on the torso (n = 28, 58.3%), followed by the lower extremity (n = 9, 18.8%). The most common differential diagnoses at time of biopsy included BCC (n = 40) and nevus (other than IDN, n = 5). Clinically, FEP were pink (95.8%), scaly (66.7%) papules (77.1%) displaying disrupted skin markings (62.5%) and absence of hair follicles (87.5%). NPD revealed serpentine (97.7%), dotted (81.8%), or polymorphous vessels (86.4%), and hypopigmented to pink lines intersecting at acute angles (HPLA) (52.3%). PD demonstrated serpentine (95.5%), dotted (86.4%), or polymorphous vessels (81.8%), shiny white lines (50.0%), and HPLA (59.1%). Classic features of BCC such as arborizing vessels (n = 2), ulceration (n = 1), shiny white blotches and strands (n = 1), blue-gray ovoid nest (n = 1), and leaf-like areas (n = 1) were uncommon. FEP often presents as scaly, erythematous papules with disrupted skin markings and absence of hair follicles. Dermoscopy reveals polymorphous vessels with shiny white lines and HPLA.
Keywords: Fibroepithelioma of Pinkus, dermoscopy, basal cell carcinoma, polymorphous vessels, shiny white lines
Introduction
Fibroepithelioma of Pinkus (FEP) is a relatively indolent subtype of basal cell carcinoma (BCC) that was first described by Herman Pinkus in 1953.[12,6] While some debate exists as to whether FEP is a bona fide BCC or a fenestrated variant of trichoblastoma,[4,18,19,7] clinicians often manage the lesion as a BCC. Many cases of FEP (72%) are diagnosed in individuals who are 51 years of age or older[4] and FEP comprises less than 2% of all BCC subtypes.[2,15,3] FEP is often found on the torso with a lumbosacral predilection[4,3] as a flesh-colored to pink nodule, plaque, or papule,[12,16] but pigmented varieties have also been reported.[1,6] The differential diagnosis for tumors that prove to be FEP includes intradermal nevus, pedunculated fibroma, seborrheic keratosis, and melanoma.[1,16] Histologically, FEP is characterized by anastomosing epithelial strands traversing and separating a fibrous stroma.[12] This histologic pattern was postulated to be due to the spread of BCC along eccrine ducts and replacement of ducts with BCC strands.[20]
Dermoscopy may serve as a valuable tool to diagnose FEP and differentiate it from benign tumors, such as intradermal nevi and fibromas. Previous case reports and small case series have shown that FEP manifests a polymorphous vessel pattern than can include fine arborizing vessels, linear vessels, and dotted vessels.[22,24,21] In addition, FEP often reveals white streaks/striae/septal lines that are believed to correspond to fibrosis on histology.[22,24] Many of these same features have also been described in melanoma. However, limitations of previous studies are their lack of descriptors for the primary clinical morphology of FEP and their small sample sizes as highlighted by the fact that the largest previous case series that described dermoscopic features of FEP was limited to 10 cases.[22] We conducted this study to elucidate the clinical and dermoscopic morphology of FEP in a large case series of patients and to determine if they possess features that are uniquely different from the known features described for other lesions that may appear on the differential, including other subtypes of BCC, benign nevi, and melanoma.
Methods
Institutional Review Board approval was obtained for this retrospective study involving the review and study of existing data. We reviewed cases of histopathology-confirmed FEP diagnosed at our institution from January 1, 2008 to April 8, 2019 with pathology reports containing the terms “fibroepithel” or “pinkus.” The pathology reports were reviewed and only those with a diagnosis of FEP were included. We retrieved clinical, contact non-polarized dermoscopy (NPD), and contact polarized dermoscopy (PD) images of all cases. We excluded collision tumors and tumors without any images. In addition, poor quality clinical and dermoscopic images (overexposed, underexposed, or out of focus) were excluded. Clinical, contact NPD, and contact PD images were reviewed by at least three clinicians experienced in dermoscopy and the presence or absence of clinical and dermoscopic features was agreed upon via consensus. We distinguished between shiny white lines, which are short, thin white lines oriented parallel and orthogonal to each other, and shiny white blotches and strands, which are white structureless areas, ovals, or circular structures and poorly defined lines arranged in parallel or haphazardly, respectively. We also employed the phrase “hypopigmented to pink lines intersecting at acute angles” (HPLA) to describe lines surrounding pink vascular holes. The hypopigmented to pink lines themselves are devoid of any structures, including vessels. Based on prior experience, we created this new phrase because the HPLA noted in this study appear to represent a feature that is distinct from the previously described negative network and shiny white lines. HPLA is in contrast to the negative network that describes brown curvilinear, elongated structures that are surrounded by relative hypopigmentation which can appear as serpiginous lines.[11,13]
Manual chart review was performed to determine patient demographics, anatomic site, and differential diagnosis rendered at time of biopsy. Descriptive statistical analysis was performed for patient demographics while relative frequencies were calculated for anatomic site, clinical features, and dermoscopic features.
Results
A total of 48 lesions with associated clinical images met our inclusion criteria, of which 44 lesions had associated contact NPD images and 22 lesions had associated contact PD images. These 48 lesions were biopsied from 36 patients, 22 of whom were male (61.1%). The mean age was 64.5 years (SD 15.1 years, range 24 to 86 years) at the time of diagnosis of each patient’s first FEP lesion. Anatomic site (Table 1) and differential diagnosis at time of biopsy (Table 2) were noted. Representative FEP lesions with clinical, dermoscopic, and histopathologic images are depicted in Figures 1 and and22.
Fig. 1
Fibroepithelioma of Pinkus. This lesion is located on the lower extremity. a Clinical examination reveals a pink, scaly (black circle) papule with serous crust. b Contact non-polarized dermoscopy demonstrates a pink lesion with polymorphous vessels, including serpentine (long black arrow), dotted (white arrow), and looped vessels (short black arrow). In addition, hypopigmented to pink lines intersecting at acute angles are highlighted in the black circled area. c Contact polarized dermoscopy again highlights hypopigmented to pink lines intersecting at acute angles in the black circled area; however, superimposed shiny white lines are also appreciated (black arrows). d Histopathology revealed FEP with anastomosing epithelial tumor strands traversing between fibrotic stroma. Parakeratosis is also noted and may account for the clinically apparent scale
Fig. 2
Fibroepithelioma of Pinkus. This lesion is located on the lower back/buttock region. a Clinical examination reveals a pink papule. b Contact non-polarized dermoscopy demonstrates a pink lesion with polymorphous vessels, including serpentine (long black arrow), dotted (white arrow), and looped vessels (short black arrow). Hypopigmented to pink lines intersecting at acute angles are highlighted in the black circled area. c Contact polarized dermoscopy again highlights hypopigmented to pink lines intersecting at acute angles in the black circled area; however, superimposed shiny white lines are also appreciated (black arrows). d Histopathology revealed FEP with anastomosing epithelial tumor strands traversing between fibrotic stroma
Table 1
Anatomic Site of Fibroepithelioma of Pinkus
Anatomic Site | Frequency % (n) |
---|---|
Torso | 58.3 (28) |
Lower extremity | 18.8 (9) |
Groin/buttock | 12.5 (6) |
Head and neck | 6.3 (3) |
Upper extremity | 4.2 (2) |
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Table 2
Differential Diagnosis at Time of Biopsy
Differential Diagnosis | Frequency % (n) |
---|---|
Basal cell carcinoma | 83.3 (40) |
Nevus other than intradermal nevus | 10.4 (5) |
Intradermal nevus | 8.3 (4) |
Other melanocytic lesion | 6.3 (3) |
Bowen’s disease/squamous cell carcinoma in situ | 6.3 (3) |
Neurofibroma | 4.2 (2) |
Fibroepithelioma of Pinkus | 4.2 (2) |
Verruca | 4.2 (2) |
Seborrheic keratosis | 4.2 (2) |
Nonmelanoma skin cancer | 4.2 (2) |
Granuloma annulare | 2.1 (1) |
Large scar | 2.1 (1) |
Lichen planus-like keratosis | 2.1 (1) |
Epidermotropic metastatic melanoma | 2.1 (1) |
Other neoplasm | 2.1 (1) |
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Clinical features of Fibroepithelioma of Pinkus
FEP presented as pink (95.8%, n = 46), scaly (66.7%, n = 32) papules (77.1%, n = 37) with disrupted skin markings (62.5%, n = 30) and absent hair follicles (87.5%, n = 42). Other observed clinical features are presented in Table 3.
Table 3
Clinical Features of Fibroepithelioma of Pinkus
Clinical Feature | Frequency % (n) |
---|---|
Pink | 95.8 (46) |
Papule | 77.1 (37) |
Patch | 2.1 (1) |
Plaque | 20.8 (10) |
Sessile | 16.7 (8) |
Shiny | 33.3 (16) |
Pearly | 20.8 (10) |
Scaly | 66.7 (32) |
Crust | 33.3 (16) |
Serous crust | 25.0 (12) |
Erosion | 27.1 (13) |
Irregular Border | 10.4 (5) |
Rolled edges | 8.3 (4) |
Disrupted skin markings | 62.5 (30) |
Absent hair follicle | 87.5 (42) |
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Contact non-polarized and polarized dermoscopy features of Fibroepithelioma of Pinkus
With contact NPD, FEP demonstrated a polymorphous vessel pattern (86.4%, n = 38) with a high frequency of serpentine vessels (97.7%, n = 43) and dotted vessels (81.8%, n = 36). Less commonly, looped vessels (27.3%, n = 12), fine arborizing vessels (18.2%, n = 8), and arborizing vessels (4.5%, n = 2) were also observed. FEP often had a pink coloration (90.9%, n = 40). With regards to pigmentation, 18.2% (n = 8) of lesions were noted to have brown structureless pigment in the skin that was not associated with any specific dermoscopic structure, such as globules or leaf-like areas. The presence of hypopigmented to pink lines intersecting at acute angles (Figures 1 and and2)2) was noted in 52.3% (n = 23) of lesions. Other features observed with contact NPD are demonstrated in Table 4. Polarized dermoscopy revealed similar features as NPD (Table 4) with the addition of shiny white lines which were noted in 50.0% of lesions (n = 11).
Table 4
Dermoscopic Features of Fibroepithelioma of Pinkus
Dermoscopic Feature | Frequency % with Contact Non-Polarized Dermoscopy (n) | Frequency % with Contact Polarized Dermoscopy (n) |
---|---|---|
Pink coloration | 90.9 (40) | 90.9 (20) |
Erosion | 27.3 (12) | 36.4 (8) |
Ulceration | 2.3 (1) | 4.5 (1) |
Milia-like cyst | 9.1 (4) | 4.5 (1) |
Brown dots | 11.4 (5) | 9.1 (2) |
Blue-gray ovoid nest | 2.3 (1) | 4.5 (1) |
Blue-gray globule | 2.3 (1) | - |
Leaf-like area | 2.3 (1) | - |
Vasculature | ||
Serpentine vessels | 97.7 (43) | 95.5 (21) |
Dotted vessels | 81.8 (36) | 86.4 (19) |
Looped vessels | 27.3 (12) | 22.7 (5) |
Fine arborizing vessels | 18.2 (8) | 13.6 (3) |
Arborizing vessels | 4.5 (2) | 9.1 (2) |
Polymorphous vessels | 86.4 (38) | 81.8 (18) |
Shiny white structures | ||
Shiny white lines | N/A | 50.0 (11) |
Shiny white blotches and strands | N/A | 4.5 (1) |
Brown structureless pigmentation | 18.2 (8) | 22.7 (5) |
Hypopigmented to pink lines intersecting at acute angles (HPLA) | 52.3 (23) | 59.1 (13) |
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Discussion
We report the clinical and dermoscopic features of FEP in a large series of patients. Clinically, these lesions often presented as pink, scaly papules with disruption of skin markings and absence of hair follicles. A feature that has not been highlighted before in other studies was the presence of scale, which was found in 66.7% of our cases of FEP and differs from nodular basal cell carcinomas which often have a shiny, non-scaly surface. Another feature that differentiates FEP from nodular BCC is the conspicuous absence or near absence of arborizing vessels in the former. Instead, dermoscopy of FEP revealed a high frequency of polymorphous vessels with the most frequent vessel subtypes being serpentine vessels and dotted vessels. In addition, FEP often lacked shiny white blotches and strands which are another well-characterized feature of other BCC subtypes. Instead, shiny white lines were prominent in FEP. The presence of polymorphous vessels and shiny white lines in FEP can therefore result in a differential which includes melanoma.
Observations made in our study and experience of one of the authors (A.A.M.) raises an important point that is worthy of discussion regarding the dermoscopic terminology used to describe the so-called negative network. The classic negative network seen on both polarized and non-polarized dermoscopy describes brown curvilinear, elongated globular structures situated on background skin of lighter color compared to the globular structures (Figure 3A). If one focuses on the lighter background instead of the globular structures, the background can take on the appearance of serpiginous hypopigmented lines.[11,13] Other terms have been used synonymously with negative network including inverse network, white network, and reticular depigmentation and in the last terminology consensus meeting it was decided to group inverse network and reticular depigmentation under the term negative network.[8,23] However, it now appears highly probable that there are at least three different entities with different histopathology correlations that are grouped under the term negative network. Some have previously attempted to describe different subtypes of negative network with type A corresponding with globular structures and type B with dotted vessels in the holes of the network.[14] While these earlier observations did not gain traction, it has become increasingly apparent that there are indeed multiple different entities that are collectively grouped under the term negative network. Here we make a distinction between them and conclude that the term negative network should be exclusively used to describe the classic negative network elucidated above as a feature seen in melanocytic neoplasms, only (Figure 3A). The dermoscopic hypopigmented lines of the negative network correlate histopathologically with widened rete ridges and between these widened rete ridges there are multiple smaller, distorted rete ridges containing atypical melanocytes. These shorter distorted rete ridges correspond to the brown elongated globular structures seen with dermoscopy.[5]
Fig. 3
Melanomas with negative network, shiny white lines forming rectangles, and hypopigmented to pink lines intersecting at acute angles (HPLA). a This is a 0.65 mm melanoma. Dermoscopy of this pigmented lesion demonstrates the classic negative network (highlighted in the black oval) with brown curvilinear, elongated globular structures situated on lighter background skin that can take on the appearance of serpiginous hypopigmented lines. In addition, blue-white veil (black arrow) and atypical pigment network (white arrow) are also present. b This is a 0.4mm invasive melanoma on the upper extremity. Dermoscopy of this asymmetric lesion demonstrates patches of atypical pigment network (black arrows), dotted vessels (black star), and shiny white lines meeting at angles to form rectangles as highlighted in the black rectangle. c and d This is a 1.1mm invasive melanoma on the lower back. c Non-polarized dermoscopy demonstrates a pink lesion with HPLA surrounding pink vasculature structures as highlighted in the black circles and inverted black triangle. d Polarized dermoscopy of the same lesion demonstrates the same HPLA in the inverted black triangle with superimposed shiny white lines in some areas (black circles)
This classic negative network is distinctly different from a pattern of shiny white lines which unlike the classic negative network can only be seen with PD.[10] These shiny white lines can meet at acute angles to form rectangles, with squares being most common (Figure 3B). The histopathology of these shiny white lines corresponds with collagen[10] and may best be classified as shiny white network lines. We have noted these shiny white lines forming rectangles in various lesions, such as melanoma, dermatofibroma, and Spitz nevi. The second pattern that is distinctly different from the classic negative network is the pattern we see in FEP. It consists of hypopigmented to pink lines intersecting at acute angles (HPLA) as seen in Figures 1, ,2,2, ,3C,3C, ,3D.3D. This pattern may in fact correspond to what was called reticular depigmentation. The lines themselves are devoid of any structures or vessels within them and they surround pink vasculature structures. This pattern can be seen with both PD and NPD along with occasional overlap with shiny white lines on PD. We have observed this pattern in FEP, hypopigmented melanoma, and Spitz nevi. Close inspection will reveal that these two dermoscopic patterns are distinct from the classic negative network (Table 5).
Table 5
Spectrum of Hypopigmented or White Lines on Dermoscopy
Dermoscopic Feature | Negative network | Shiny white lines forming rectangles | Hypopigmented to pink lines intersecting at acute angles (HPLA) |
---|---|---|---|
Description of feature | Brown elongated and curvilinear globular structures surrounded by relative hypopigmentation which can appear as hypopigmented serpiginous lines.[11,13] | Shiny white lines meeting at acute angles to form rectangles, with squares being the most common. | Hypopigmented to light pink lines devoid of any structures or vasculature within them, meeting at acute angles and surrounding darker pink or vascular holes. |
Visualized with PD or NPD | PD and NPD | PD only | PD and NPD (may overlap with shiny white lines on PD) |
Histopathologic correlation | Hypopigmented lines correlate with widened rete ridges and brown elongated globular structures correspond to distorted rete ridges containing melanocytes.[5] | Collagen.[10] | Hypothesis when HPLA is noted in FEP: darker pink holes correlate with vessels within a fibrotic stroma and hypopigmented to light pink lines correspond to epithelial tumor strands. |
Exemplar figures | 3A3A | 3B3B | 1B,1B, ,1C,1C, ,2B,2B, ,2C,2C, ,3C,3C, and and3D3D |
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PD = polarized dermoscopy; NPD = non-polarized dermoscopy; FEP = Fibroepithelioma of Pinkus.
Based on review of the literature, we believe that hypopigmented to pink lines intersecting at acute angles (reticular depigmentation) in our study are similar to the negative network[21] and white network[9] that were previously reported on dermoscopic examination of FEP. In FEP, it is likely that the darker pink holes surrounded by HPLA correlate with vessels within a fibrotic stroma on histology. Meanwhile, the hypopigmented to light pink lines likely correspond to epithelial tumor strands that emanate from the epidermis in FEP, a pattern which can be seen in Figures 1D and and2D.2D. Future research should further investigate the histopathologic correlates of these hypopigmented to pink lines on dermoscopy.
Our study is limited by its retrospective design and the presence of few pigmented FEP lesions. The latter may account for the frequent lack of leaf-like areas and blue-gray ovoid nests in our series, which are features often associated with pigmented BCC.[17] In fact, a leaf-like area was only described in one of two FEPs that were pigmented as per pathology reports in our study. However, the lack of other common features of BCC, including arborizing vessels and shiny white blotches and strands,[17] suggests that the dermoscopic features of FEP are often distinct from those of other BCC subtypes.
In conclusion, Fibroepithelioma of Pinkus lesions are often clinically pink, scaly papules devoid of hair and can have a broad clinical differential diagnosis including basal cell carcinoma and nevus. On dermoscopy, there is a paradox in that these tumors tend not to manifest the known features of basal cell carcinoma such as arborizing vessels and shiny white blotches and strands. Instead, they tend to manifest features more commonly associated with melanoma, including polymorphous vessels and shiny white lines. A diagnosis of FEP should be strongly considered when one encounters raised, scaly, hairless lesions that lack common features of BCCs or intradermal nevi on dermoscopy and instead reveal polymorphous vessels, shiny white lines, and hypopigmented to pink lines intersecting at acute angles.
Funding: Funding/Support:
This work was funded in part through the National Institutes of Health/National Cancer Institute Cancer Center Support Grant P30 CA008748
Footnotes
Conflicts of interest/Competing interests: The authors declare that they have no conflict of interest.
Ethics approval: This retrospective study was performed in accordance with the 1964 Helsinki Declaration and later amendments. Institutional Review Board (IRB) approval was obtained for this study involving the review and study of existing data with a waiver for written informed consent from patients.
Availability of data and material: Not applicable
Code availability: Not applicable
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