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Key Words lands. Conclusion: The ivory-white back- Patients with the second variant of TE
Familial trichoepithelioma Desmoplastic ground throughout the lesion seen on der- present with multiple lesions, with each le-
trichoepithelioma Reflectance confocal moscopy may be helpful in distinguishing sion exhibiting similar clinical character-
microscopy Dermoscopy desmoplastic TE from BCC. The RCM find- istics as seen in the solitary variant. This
ings in TE of keratin-filled cysts in tumor is- variant is often transmitted as an autoso-
lands and attachment of the tumor to follic- mal dominant trait. Multiple TE first ap-
Abstract ular structures have not been previously pears during adolescence as small papules
Background: Trichoepitheliomas (TE) are observed in BCC, and thus may also be diag- on the central face, with predilection for
benign neoplasms of follicular differentia- nostically helpful. Further study is necessary the skin near the nasolabial folds, fore-
tion. Solitary lesions are often confused with for validation of these findings. head, chin and periauricular area [6]. The
basal cell carcinoma (BCC). Reflectance con- Copyright 2007 S. Karger AG, Basel combination of multiple TE with cylindro-
focal microscopy (RCM) and dermoscopy are mas and/or spiradenomas can be seen in
imaging tools for in vivo, noninvasive evalu- the Brooke-Spiegler syndrome [7, 8].
ation of skin lesions. To date, there has been Introduction Desmoplastic TE, the third variant, of-
no description of their findings in the evalu- ten presents as a firm, annular, asymptom-
ation of TE. Objective: Our aim is to describe Trichoepitheliomas (TE) are benign atic, white to yellow papule or plaque vary-
the dermoscopic and RCM findings of histo- cutaneous neoplasms that reveal features ing in size from 3 to 8 mm. However, it is
pathologically confirmed TE. Methods: Four of follicular differentiation on histopa- not uncommon to encounter desmoplastic
TE were evaluated, 2 each of the desmoplas- thology. In fact, the cells in TE have similar TE that are larger than 12 cm. The center
tic and nondesmoplastic variants. RCM was immunohistochemical staining patterns of a desmoplastic TE is frequently de-
performed on 1 of the desmoplastic and to that of cells in the outer root sheath [1]. pressed; however, ulceration is not en-
both of the nondesmoplastic lesions. Re- Three distinct subtypes of TE are rec- countered [9]. The borders are frequently
sults: Dermoscopically, all of the lesions ognized, namely solitary, multiple and raised and can exhibit a rolled appear-
showed arborizing telangiectasias. The des- desmoplastic TE [2]. The solitary variant, ance, a feature characteristically associat-
moplastic lesions also had an ivory-white seen in adults, usually presents as a skin- ed with basal cell carcinoma (BCC). How-
background throughout. RCM showed oval, colored papule 58 mm in diameter, locat- ever, unlike BCC, desmoplastic TE tend to
darker-appearing tumor islands that con- ed on the central face with a predilection enlarge very slowly over many years to de-
tained brightly refractile material, consis- for the perinasal area [3]. Infrequently, sol- cades, and the lesions tend not to develop
tent with keratin horn cysts at the center, as itary TE can obtain a diameter greater ulcerations.
well as parallel bundles of highly refractile than 1 cm or can be located on the neck, Histologically, solitary and multiple
dermal collagen surrounding the tumor is- scalp or trunk [4, 5]. TE are seen as relatively symmetric and
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sharply circumscribed epithelial prolifera- scopic and reflectance confocal microsco- (fig. 2). At higher-magnification RCM the
tions characterized by a predominance of py (RCM) attributes of a small case series tumor islands showed morphology similar
follicular germinative cells arrayed as of TE, as a first step toward being able to to that seen in BCC (fig. 3). In the dermis,
small clusters or as reticulate and cribri- better discriminate it from BCC. RCM examination revealed brightly re-
form cords of basaloid cells. The tumor fractile collagen, arranged in parallel
cell aggregates are enveloped by a fibrotic, bundles, encasing the tumor islands. In ad-
conspicuous stroma that typically main- Case Report dition, round black spaces filled with
tains tight adherent contact to the neoplas- brightly refractile material were seen in
tic cells [2, 10]. Brownstein and Shapiro [9] Case 1 the center of several tumor islands, cor-
provided histopathological criteria for A 60-year-old white woman with a pre- responding to early horn cysts seen on
desmoplastic TE. They attributed three vious history of multiple BCC on the face histology. The lesion was surgically re-
features to desmoplastic TE: (1) narrow presented for routine dermatological fol- moved, and histopathological examination
strands of tumor cells, (2) keratinaceous low-up. She denied any familial syndromes. revealed the presence of basaloid tumor is-
cysts and (3) desmoplastic stroma. Fur- On clinical examination she had multiple lands composed of branching and budding
thermore, they also listed features that skin-colored papular lesions, ranging in nests, recapitulating follicular germs and
help differentiate desmoplastic TE from size from 2 to 4 mm, located predomi- producing horn cysts, wrapped in a fi-
conventional TE, syringomas, morphei- nately on the forehead and central face. A brotic stroma.
form BCC and metastatic breast cancer 3-mm dome-shaped lesion on her fore-
[9]. Another differential diagnosis that has head, representative of her other lesions, Case 2
to be considered is the microcystic adnex- was selected for clinical, dermoscopic and A 68-year-old woman with a previous
al carcinoma [11]. RCM analysis and biopsy. The selected le- diagnosis of multiple familial TE was seen
TE can closely mimic BCC, clinically sion consisted of a skin-colored, translu- for routine follow-up. She presented with
and dermoscopically. In this paper we de- cent and well-defined papule (fig. 1a). By multiple, dome-shaped, whitish to skin-
scribe cases of TE which in clinical and dermoscopy, small and thin arborizing tel- colored papules, ranging from 2 to 6 mm
dermoscopic examinations were sugges- angiectasias were observed on a shiny white in diameter located on her forehead,
tive of BCC; however, the patients reported background (fig. 1b). RCM examination cheeks, chin, and in the perinasal, perocu-
long-standing, completely unchanged le- revealed the presence of a sharply demar- lar and zygomatic areas. After clinical
sions, the final histopathological diagnosis cated lesion composed of tumor islands lo- evaluation, she underwent dermoscopic
being indeed TE. We describe the dermo- cated in a highly refractile (bright) stroma and RCM examination of 2 papular le-
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sions, a 4-mm lesion located on the right the examination, a 14-mm atrophic plaque on the remainder of the lesion. RCM mo-
cheek and a 5-mm lesion on the chin. In was noted on his right chin. The lesion had saic revealed the presence of dark, small,
both lesions, dermoscopy disclosed the a scar-like appearance, but its edge re- round and elongated tumor nests in a
presence of well-defined lesions with fine vealed a rolled border (fig. 4a). The patient brightly refractile stroma arranged in par-
arborizing telangiectasias and numerous stated that this lesion was a scar that re- allel bundles (fig. 5). The stroma tightly
milia-like structures. RCM examination sulted after attempts to remove a skin cyst wrapped around the tumor islands. In
revealed a normal epidermal architecture; 60 years ago. He was also convinced that some foci, tumor nests showed a small,
however, in the dermis one could identify the scar had not changed in appearance round, dark central structure containing
islands and cords of tumor cells. These tu- over time. On dermoscopy, the lesion brightly refractile material, corresponding
mor structures were embedded in a stroma showed an ivory-white color, reflecting on histopathology to horn cysts (fig. 6).
of brightly refractile collagen arranged in an underlying fibrotic process, as well
parallel bundles. Numerous dilated blood as prominent arborizing telangiectasias Case 4
vessels were evident throughout the stro- (fig. 4b) that were significantly different as A 77-year-old man with a history of
ma. In some areas, the tumor cords were compared to the vessels in the surround- multiple actinic keratoses was seen for
connected to follicular structures. At the ing actinically damaged skin. A shave bi- skin cancer screening. On his left medial
center of several tumor islands, round, opsy of a portion of this lesion was per- cheek, he had a 2 ! 1 cm sharply demar-
black areas filled with brightly refractile formed, and histopathology revealed mul- cated ivory-colored firm plaque, which he
material with RCM features of keratin ma- tiple, narrow strands of basaloid cells stated had been present for over 50 years.
terial were seen corresponding on histopa- without atypia or mitotic figures. The The lesion was slightly raised at the pe-
thology to pseudo-horn cysts. strands were surrounded by a desmoplas- ripheral margin. Dermoscopically, there
tic stroma, and there was no clefting be- were prominent and sharply focused ar-
Case 3 tween the strands and the stroma. Multi- borizing telangiectasias visible within the
An 83-year-old male with no history of ple keratinaceous cysts were also present. central white area, and the lesion had
skin cancer presented for routine derma- The diagnosis of desmoplastic TE was ren- sharply defined borders. The arborizing
tological screening examination. During dered. RCM examination was performed vessels were markedly different from the
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vessels in the surrounding actinically ited to the evaluation of pigmented lesions shaped, white lesions with superficial ar-
damaged skin. A shave biopsy of a portion [13]. borizing telangiectasias. These lesions also
of the lesion was performed, and the ini- Thus, dermoscopy has the potential to had similar arborizing telangiectasias and
tial pathological diagnosis favored that be useful in the diagnosis of TE, especially a shiny white color like BCC. However, un-
of a morpheiform BCC. However, based the desmoplastic variant, which is chal- like desmoplastic TE, the lesions did not
on the presence of multiple strands of lenging to distinguish from BCC. Arboriz- have the ivory-white background through-
basaloid cells without surrounding cleft- ing telangiectasias and focal shiny-white out the lesion, rendering them dermoscop-
ing, atypia or mitotic figures, and due to areas are features commonly seen in BCC, ically indistinguishable from BCC.
the presence of multiple keratinaceous and we observed similar findings in des- RCM allows for the visualization of mi-
cysts and focal calcification, the diagno- moplastic TE. However, the distinguish- croscopic features in vivo [15]. Gaining in-
sis was subsequently amended to a des- ing feature in desmoplastic TE was the sight into the RCM appearance of lesions
moplastic TE. presence of a pearl-white to ivory-white may help us to improve our bedside clini-
background color throughout the entire cal diagnostic accuracy and reduce unnec-
lesion with prominent, large arborizing essary excisions of benign lesions [16, 17].
Discussion vessels. These lesions lack any other der- In all 4 TE lesions analyzed with RCM, we
moscopic feature of BCC, such as leaf-like observed features that were previously de-
The clinical diagnosis of familial mul- structures and ovoid nests. Ivory-white scribed for BCC [18, 19] such as the pres-
tiple TE is generally straightforward. How- color seen on dermoscopy is usually indic- ence of islands or cords of tumor cells com-
ever, solitary and desmoplastic TE vari- ative of a sclerotic stroma [14], and this posed of basaloid epithelial cells with elon-
ants may be difficult to differentiate from correlated well with the desmoplastic stro- gated nuclei. However, some features of
BCC [12]. The addition of dermoscopy to ma seen under histology. TE, not previously described for BCC,
the unaided eye examination may aid in In addition to the 2 desmoplastic TE were noted on RCM. In familial TE, der-
reaching the correct diagnosis. It has be- cases, we also evaluated 2 patients with mal tumor proliferations were shown to
come evident that dermoscopy is not lim- classical TE, presenting with dome- be connected with follicular structures;
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