2. Trevisan G, Padovan C, Scaini MT, Cinco M, Floris R, Bonin S. Anetoderma Associated with Lyme Disease : A case report. Acta Derm Venereol 2008; 88: 536-8. 3. Eisendle K, Grabner T, Zelger B. Morphea : a manifestation of infection with Borrelia species? Br J Dermatol 2007; 157: 1189-98. 4. Özkan S, Fetil E, Izler F, Pabucc¸uoglu U, Yalc¸u N, Günes AT. Anetoderma secondary to generalized granula annulare. J Am Acad Dermatol 2000; 42: 335-8. 5. Schuttelaar M, Laeijendecker R, Heinhuis R, Van Joost T. Erythema multiforme and persistent erythema as early cutaneous manifestations of Lyme disease. J Am Acad Dermatol 1997; 37: 873-5. 6. Strunk T, Hetzer S, Homey B, Reifenberger J. Anetoderma with positive Borrelia serology. Hautarzt 2011; 62: 720-2. doi:10.1684/ejd.2015.2536
Multiple pigmented trichoblastomas, sebaceous carcinoma and syringocystadenoma papilliferum arising within a nevus sebaceous We present a 43-year-old man with a 3-year history of slowly-enlarging pigmented nodules on his right face. The patient reported a yellowish verrucous plaque on the right side of his face since birth. At the age of 10 years, cryotherapy was applied to remove the plaque, leaving a residual scar-like patch. Three years ago, the patient noted several pigmented nodules within the patch. A new nodule appeared in another area of the patch one year ago and grew rapidly. The patient denied any personal or family history of cutaneous or internal malignancies. Systemic examination was unremarkable. Physical examination revealed an irregular, reddish, scar-like patch, measuring 8 cm at its widest, on the right side of the face. Four pigmented dome-shaped nodules, ranging from 0.2 to 0.5 cm in diameter, were found within the patch. At the periphery of the patch, a 2.5 cm × 2.0 cm tumor with an ulcerated surface and a hemorrhagic crust was observed. A 0.6 cm × 0.6 cm, pinkish, firm nodule was seen in front of the right ear (figure 1A). Histopathological examination of the pigmented nodules revealed a wellcircumscribed aggregation of follicular germinative cells containing abundant melanin and melanophages located in the dermis without connection to the epidermis (figure 1B). Peripheral palisading was observed. The aggregations were surrounded by a highly cellular fibrous stroma and a follicular germ-like structure and papillary mesenchymal bodies were seen. Neither high-grade cytologic atypia nor necrosis was seen (figure 1C). These histologic features were consistent with the diagnosis of pigmented trichoblastoma. Scanning magnification of the ulcerated neoplasm revealed an asymmetric, poorly-circumscribed silhouette composed of basaloid and vacuolated cells (figure 1D). The basaloid cells contained large, crowded, and pleomorphic nuclei with prominent nucleoli and frequent mitoses. Vacuolated cells showed bubbly cytoplasm and a scalloped nucleus (figure 1E). The histopathologic diagnosis was sebaceous carcinoma. The pinkish nodule was syringocystadenoma papilliferum (SCAP), which was
EJD, vol. 25, n◦ 3, May-June 2015
A
B
E
C
F
D
G
Figure 1. A) Nevus sebaceus with a red ulcerated nodule (sebaceous carcinoma) in the peripheral area of the patch (down arrow), four pigmented nodules (trichoblastoma) (up arrows), and a pinkish nodule (SCAP) in the front of the right ear (right arrow). B) Low power view shows nodular pigmented tumor nests involving the dermis. C) High-power view reveals palisading basaloid tumor cells lacking highgrade cytologic atypia within a fibrocellular stroma. D) The neoplasm shows an asymmetric, poorly circumscribed silhouette. E) The neoplastic aggregations are composed of basaloid germinative cells and vacuolated cells. F) Many irregular cystic spaces and duct-like structures, some of which connect to the overlying epidermis. G) Duct-like structures and cystic spaces covered by double layers of epithelium with stroma containing plasma cells. (haematoxylin-eosin stain; original magnification, B×20, C×100, D×20, E×200, F×40, G×200).
composed of duct-like structures and cystic spaces covered by a double-layer epithelium with stroma containing lymphocytes and numerous plasma cells (figures 1F, G). The epidermis showed acanthosis and papillomatosis. The number of closely set lobules and malformed ducts in the dermis increased. These features were consistent with the diagnosis of nevus sebaceus (NS). The final diagnosis was multiple pigmented trichoblastomas, sebaceous carcinoma, and SCAP arising within a NS. A large excision of the entire lesion with a free margin of 15 mm was performed, followed by a free full-thickness skin graft. The patient was clinically free of disease at his follow-up one year after treatment. Although many benign or malignant tumors have been reported to arise in long-standing NS, most commonly trichoblastoma and SCAP [1], the coexistence of 3 or more tumors, including sebaceous carcinoma, as in our case, is rare. Manonukul et al. [2] reported a case of NS coexisting with trichoblastoma, trichilemmoma, sebaceous adenoma, follicular infundibulum tumor, SCAP and mucoepidermoid or adenosquamous carcinoma. Our case is interesting because of the extraordinary clinical presentation of multiple pigmented trichoblastomas and the simultaneous occurrence of sebaceous carcinoma and SCAP arising within the NS. Sebaceous carcinoma arising from NS is rare, with fewer than 30 cases reported in the literature [3]. No recurrences, metastases, or tumor-related deaths in cases of sebaceous carcinoma arising from NS have been reported to date; thus, this disease behaves more like a low-grade malignancy than its de novo counterpart [4]. Given that the majority of neoplasms arising from NS are benign, close observation and the excision of suspicious lesions is the preferred management [3].
279
Disclosure. Financial support: none. Conflict of interest: none. 1 Institute of Dermatology, Chinese Academy of Medical Sciences, Nanjing 210042, China 2 Ackerman Academy of Dermatopathology, New York, NY 10016, USA
a These authors contributed equally to this work.
280
Hao SONG1,a Qiang WANG1,a Ying GUO2 Xiu-Lian XU1 Jian-Fang SUN1
1. Gonzalez Guemes M, Gonzalez Hermosa R, Calderon Gutierrez MJ, et al. Development of multiple tumors arising in a nevus sebaceous of Jadassohn. J Eur Acad Dermatol Venereol 2005; 19: 658-9. 2. Manonukul J, Omeapinyan P, Vongjirad A. Mucoepidermoid (adenosquamous) carcinoma, trichoblastoma, trichilemmoma, sebaceous adenoma, tumor of follicular infundibulum and syringocystadenoma papilliferum arising within 2 persistent lesions of nevus sebaceous: report of a case. Am J Dermatopathol 2009; 31: 658-63. 3. Wang E, Lee JS, Kazakov DV. A rare combination of sebaceoma with carcinomatous change (sebaceous carcinoma), trichoblastoma, and poroma arising from a nevus sebaceus. J Cutan Pathol 2013; 40: 676-82. 4. Izumi M, Tang X, Chiu CS, et al. Ten cases of sebaceous carcinoma arising in nevus sebaceus. J Dermatol 2008; 35: 704-11. doi:10.1684/ejd.2015.2535
EJD, vol. 25, n◦ 3, May-June 2015
Multiple pigmented trichoblastomas, sebaceous carcinoma and syringocystadenoma papilliferum arising within a nevus sebaceous We present a 43-year-old man with a 3-year history of slowly-enlarging pigmented nodules on his right face. The patient reported a yellowish verrucous plaque on the right side of his face since birth. At the age of 10 years, cryotherapy was applied to remove the plaque, leaving a residual scar-like patch. Three years ago, the patient noted several pigmented nodules within the patch. A new nodule appeared in another area of the patch one year ago and grew rapidly. The patient denied any personal or family history of cutaneous or internal malignancies. Systemic examination was unremarkable. Physical examination revealed an irregular, reddish, scar-like patch, measuring 8 cm at its widest, on the right side of the face. Four pigmented dome-shaped nodules, ranging from 0.2 to 0.5 cm in diameter, were found within the patch. At the periphery of the patch, a 2.5 cm × 2.0 cm tumor with an ulcerated surface and a hemorrhagic crust was observed. A 0.6 cm × 0.6 cm, pinkish, firm nodule was seen in front of the right ear (figure 1A). Histopathological examination of the pigmented nodules revealed a wellcircumscribed aggregation of follicular germinative cells containing abundant melanin and melanophages located in the dermis without connection to the epidermis (figure 1B). Peripheral palisading was observed. The aggregations were surrounded by a highly cellular fibrous stroma and a follicular germ-like structure and papillary mesenchymal bodies were seen. Neither high-grade cytologic atypia nor necrosis was seen (figure 1C). These histologic features were consistent with the diagnosis of pigmented trichoblastoma. Scanning magnification of the ulcerated neoplasm revealed an asymmetric, poorly-circumscribed silhouette composed of basaloid and vacuolated cells (figure 1D). The basaloid cells contained large, crowded, and pleomorphic nuclei with prominent nucleoli and frequent mitoses. Vacuolated cells showed bubbly cytoplasm and a scalloped nucleus (figure 1E). The histopathologic diagnosis was sebaceous carcinoma. The pinkish nodule was syringocystadenoma papilliferum (SCAP), which was
EJD, vol. 25, n◦ 3, May-June 2015
A
B
E
C
F
D
G
Figure 1. A) Nevus sebaceus with a red ulcerated nodule (sebaceous carcinoma) in the peripheral area of the patch (down arrow), four pigmented nodules (trichoblastoma) (up arrows), and a pinkish nodule (SCAP) in the front of the right ear (right arrow). B) Low power view shows nodular pigmented tumor nests involving the dermis. C) High-power view reveals palisading basaloid tumor cells lacking highgrade cytologic atypia within a fibrocellular stroma. D) The neoplasm shows an asymmetric, poorly circumscribed silhouette. E) The neoplastic aggregations are composed of basaloid germinative cells and vacuolated cells. F) Many irregular cystic spaces and duct-like structures, some of which connect to the overlying epidermis. G) Duct-like structures and cystic spaces covered by double layers of epithelium with stroma containing plasma cells. (haematoxylin-eosin stain; original magnification, B×20, C×100, D×20, E×200, F×40, G×200).
composed of duct-like structures and cystic spaces covered by a double-layer epithelium with stroma containing lymphocytes and numerous plasma cells (figures 1F, G). The epidermis showed acanthosis and papillomatosis. The number of closely set lobules and malformed ducts in the dermis increased. These features were consistent with the diagnosis of nevus sebaceus (NS). The final diagnosis was multiple pigmented trichoblastomas, sebaceous carcinoma, and SCAP arising within a NS. A large excision of the entire lesion with a free margin of 15 mm was performed, followed by a free full-thickness skin graft. The patient was clinically free of disease at his follow-up one year after treatment. Although many benign or malignant tumors have been reported to arise in long-standing NS, most commonly trichoblastoma and SCAP [1], the coexistence of 3 or more tumors, including sebaceous carcinoma, as in our case, is rare. Manonukul et al. [2] reported a case of NS coexisting with trichoblastoma, trichilemmoma, sebaceous adenoma, follicular infundibulum tumor, SCAP and mucoepidermoid or adenosquamous carcinoma. Our case is interesting because of the extraordinary clinical presentation of multiple pigmented trichoblastomas and the simultaneous occurrence of sebaceous carcinoma and SCAP arising within the NS. Sebaceous carcinoma arising from NS is rare, with fewer than 30 cases reported in the literature [3]. No recurrences, metastases, or tumor-related deaths in cases of sebaceous carcinoma arising from NS have been reported to date; thus, this disease behaves more like a low-grade malignancy than its de novo counterpart [4]. Given that the majority of neoplasms arising from NS are benign, close observation and the excision of suspicious lesions is the preferred management [3].
279
Disclosure. Financial support: none. Conflict of interest: none. 1 Institute of Dermatology, Chinese Academy of Medical Sciences, Nanjing 210042, China 2 Ackerman Academy of Dermatopathology, New York, NY 10016, USA
a These authors contributed equally to this work.
280
Hao SONG1,a Qiang WANG1,a Ying GUO2 Xiu-Lian XU1 Jian-Fang SUN1
1. Gonzalez Guemes M, Gonzalez Hermosa R, Calderon Gutierrez MJ, et al. Development of multiple tumors arising in a nevus sebaceous of Jadassohn. J Eur Acad Dermatol Venereol 2005; 19: 658-9. 2. Manonukul J, Omeapinyan P, Vongjirad A. Mucoepidermoid (adenosquamous) carcinoma, trichoblastoma, trichilemmoma, sebaceous adenoma, tumor of follicular infundibulum and syringocystadenoma papilliferum arising within 2 persistent lesions of nevus sebaceous: report of a case. Am J Dermatopathol 2009; 31: 658-63. 3. Wang E, Lee JS, Kazakov DV. A rare combination of sebaceoma with carcinomatous change (sebaceous carcinoma), trichoblastoma, and poroma arising from a nevus sebaceus. J Cutan Pathol 2013; 40: 676-82. 4. Izumi M, Tang X, Chiu CS, et al. Ten cases of sebaceous carcinoma arising in nevus sebaceus. J Dermatol 2008; 35: 704-11. doi:10.1684/ejd.2015.2535
EJD, vol. 25, n◦ 3, May-June 2015
