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Primary cutaneous endometriosis of umbilicus
284
1876
Persistent alopecia induced by vismodegib Salim Alkeraye, MD, Center Hospitalier Universitaire de Lille, Lille, France; Cyril Maire, MD, Center Hospitalier Universitaire de Lille, Lille, France; Carole Templier, MD, Center Hospitalier Universitaire de Lille, Lille, France; Eve Desmedt, MD, Center Hospitalier Universitaire de Lille, Lille, France; Laurent Mortier, MD, Center Hospitalier Universitaire de Lille, Lille, France
Pigmented squamous cell carcinoma presenting as melanonychia striata mimicking melanoma Claudia Hernandez, MD, University of Illinois at Chicago, Chicago, IL, United States; Entesar Elsaady, MD, University of Illinois at Chicago, Chicago, IL, United States; Stephanie Wang, MD, University of Illinois at Chicago, Chicago, IL, United States
Basal cell carcinoma accounts for 80% of nonmelanoma skin cancers. Although most basal cell carcinomas are readily treated by various surgical methods, these lesions occasionally, if not treated, progress to an advanced state that is no longer amenable to surgery or radiation. Vismodegib is a known inhibitor of the sonic hedgehog pathway (Shh) approved by the US FDA and the EMEA. It is the first systemic treatment for patients with locally advanced or metastatic basal cell carcinoma that is not amenable to surgery and radiation. We have treated 65 patients (between July 2011- March 2014) with vismodegib for locally advanced basal cell carcinomas. Among those, 4 patients presented with persistent alopecia (grade 2-3) even after several months (mean ¼ 15 months) of stopping the treatment. Common reversible adverse events with vismodegib include muscle spasms (68%), dysgeusia (51%), alopecia (63%), fatigue (36%), and weight loss (46%). Amenorrhea has been observed in clinical trials in females of reproductive potential. Shh pathway has an essential role during hair follicle morphogenesis, where it is required for normal advancement beyond the hair germ stage of development. It is speculated that Shh may regulate early folliculogenesis through its two general targets, Ptc1 and Gli1, that are normally expressed at highest levels in the dermal papilla. Data from multiple studies support a model in which the loss of dermal cilia results in arrested follicle development due to dysregulated Shh signaling pathway. Our experience suggests that vismodegib’s side effects are reversible after treatment cessation. However, we recognize that persistent alopecia has been reported in 4 out of 65 patients. In addition, recent data suggest that amenorrhea is a potential side effect of unknown reversibility.
Although subungual melanoma is the most concerning cause for melanonychia striata, there are a variety of other causes including fungal infections, trauma, benign melanocytic lesions, as well as other malignancies. We present a case of melanonychia striata due to pigmented SCC (pSCC) mimicking the clinical appearance of a subungual malignant melanoma in a renal transplant patient. A 62-year-old African American male presented for evaluation of a pigmented longitudinal streak located on his left third finger adjacent to the lateral nail fold. He complained of increasing tenderness of the nail and noted that the pigmented streak had been increasing in size. Nine years prior he had undergone renal transplantation due to end-stage renal disease secondary to glomerulonephritis. He was on a maintenance immunosuppressive regimen that included mycophenolate mofetil and cyclosporine. The patient underwent a nail avulsion and biopsy of the nail bed as well as the matrix. Histopathology was significant for malignant dyskeratosis with lack of nuclear maturation, occasional mitoses, multinucleation, and individual cell keratinization. Immunostaining for S100 was negative while staining for AE1/3 (cytokeratins) was positive consistent with pSCC. He underwent Mohs micrographic surgery where the SCC was found to be more extensive than originally suspected requiring en bloc excision of the nail repaired with a fullthickness skin graft from the left forearm. Subungual pigmented squamous cell carcinoma can present as melanonychia striata and should be considered in the differential diagnosis. It has been reported that approximately 10% of subungual pSCC cases present as melanonychia striata. Although cases of pSCC presenting as melanonychia striata have been previously reported our case occurred in the setting of a renal transplantation and immunosuppression. Our patient had many of the known risk factors for the development of pSCC, including gender, age, skin of color, history of renal transplantation, and immunosuppressive therapy. Although regular full body skin examinations are an accepted part of renal transplantation follow-ups due to the SCC risk in these patients, this case emphasizes the need to remain vigilant for atypical presentations among the immunosuppressed. The nail unit should not be overlooked during the clinical examination of these patients as demonstrated by our patient’s rare presentation of pSCC.
Commercial support: None identified.
Commercial support: None identified.
838
1985 Pigmented squamous cell carcinoma of the skin: Report of two cases with pathological correlation and review of the literature to define its dermoscopic findings Cristiano Horta, MD, Hospital Ipiranga, S~ao Paulo, Brazil; GabrielAribi Fiszbaum, MD, Hospital Ipiranga, S~ao Paulo, Brazil; Daniele De Mola Sponchiado, MD, Hospital Ipiranga, S~ao Paulo, Brazil; Camila Oliveira Alvarenga, MD, Hospital Ipiranga, S~ao Paulo, Brazil The pigmented squamous cell carcinoma is rare, with an incidence ranging from 0.01% to 7% of all squamous cell carcinomas. Differential diagnoses are nodular melanoma, blue nevus, and pigmented basal cell carcinoma. The dermatoscopic findings seem not to increase the clinical diagnostic accuracy. However, the authors highlight the most relevant dermatoscopic findings based on the presentation of two clinical cases with its dermatoscopic and pathologic correlation, besides the review of the literature. The dermatoscopy showed in both cases blueish lesions with structureless areas, skin plaques, and ulcerative areas. In one case, maple leaf structures and in the other blue-white veils were observed. These characteristics suggested the clinical diagnosis of pigmented basal cell carcinoma and melanoma. The diagnostic of pigmented squamous cell carcinoma was obtained after excision of the lesions and antomopathologic study. No evidence of recurrence or metastasis were found for 6 months after removal (at the time of submiting this report). The study of the literature and analysis of the clinical characteristics of the pigmented squamous cell carcinoma allowed the authors to identify the dermoscopic aspects that could assist clinical diagnosis believing that these lesions, although rare, should be included in the differential diagnosis of pigmented skin lesions. Commercial support: None identified.
MAY 2015
Primary cutaneous endometriosis of umbilicus Seung-Hee Loh, MD, Department of Dermatology, Kyung Hee University Hospital, Seoul, South Korea; Dong-Woo Suh, MD, Department of Dermatology, College of Medicine, Kyung Hee University, Seoul, South Korea; Bark-Lynn Lew, MD, PhD, Department of Dermatology, Kyung Hee University Hospital, Seoul, South Korea; Woo-Young Sim, MD, PhD, Department of Dermatology, Kyung Hee University Hospital, Seoul, South Korea Background: Primary cutaneous endometriosis of umbilicus, which is also called Villar nodule, is a rare manifestation of endometriosis, representing 0.5% to 1% of all patients with endometrial ectopia. Ectopic endometriosis could be found in nearly every tissue and it mostly occurs at surgical scar tissue secondarily to abdominal surgical procedures. The most widely accepted pathogenesis of secondary endometriosis is the iatrogenic implantation of endometrial cells as a result of surgery, commonly laparoscopic procedures. However, the pathogenesis of primary endometriosis is yet unknown. Patient and method: We report a case of a 38-year-old multigravida female with a 2.5 3 2.0 cm brownish to purplish colored painful nodule on the umbilicus since 2 years ago. The patient had no history of operation. The nodule became swollen with spontaneous frank bleeding during menstruation. The lesion was first diagnosed as a keloid at local clinic and has undergone steroid intralesional injection for several times but there was no improvement. Result: Umbilical ultrasonography and abdominal computed tomography showed an enhancing mass at the umbilicus without connection to abdominal organs. Histopathologic examination revealed dilated glandular structures surrounded by cellular endometrial type stroma and deep perivascular lymphocytic infiltration. Conclusion: According to these findings, the umbilical lesion was diagnosed as primary cutaneous endometriosis and it was removed by local excision without recurrence. Dermatologists should consider a Villar nodule when a nodule is clinically similar to a keloid with menstrually related, cyclic symptoms without any trauma history including operation. Commercial support: None identified.
J AM ACAD DERMATOL
AB189
1876
Persistent alopecia induced by vismodegib Salim Alkeraye, MD, Center Hospitalier Universitaire de Lille, Lille, France; Cyril Maire, MD, Center Hospitalier Universitaire de Lille, Lille, France; Carole Templier, MD, Center Hospitalier Universitaire de Lille, Lille, France; Eve Desmedt, MD, Center Hospitalier Universitaire de Lille, Lille, France; Laurent Mortier, MD, Center Hospitalier Universitaire de Lille, Lille, France
Pigmented squamous cell carcinoma presenting as melanonychia striata mimicking melanoma Claudia Hernandez, MD, University of Illinois at Chicago, Chicago, IL, United States; Entesar Elsaady, MD, University of Illinois at Chicago, Chicago, IL, United States; Stephanie Wang, MD, University of Illinois at Chicago, Chicago, IL, United States
Basal cell carcinoma accounts for 80% of nonmelanoma skin cancers. Although most basal cell carcinomas are readily treated by various surgical methods, these lesions occasionally, if not treated, progress to an advanced state that is no longer amenable to surgery or radiation. Vismodegib is a known inhibitor of the sonic hedgehog pathway (Shh) approved by the US FDA and the EMEA. It is the first systemic treatment for patients with locally advanced or metastatic basal cell carcinoma that is not amenable to surgery and radiation. We have treated 65 patients (between July 2011- March 2014) with vismodegib for locally advanced basal cell carcinomas. Among those, 4 patients presented with persistent alopecia (grade 2-3) even after several months (mean ¼ 15 months) of stopping the treatment. Common reversible adverse events with vismodegib include muscle spasms (68%), dysgeusia (51%), alopecia (63%), fatigue (36%), and weight loss (46%). Amenorrhea has been observed in clinical trials in females of reproductive potential. Shh pathway has an essential role during hair follicle morphogenesis, where it is required for normal advancement beyond the hair germ stage of development. It is speculated that Shh may regulate early folliculogenesis through its two general targets, Ptc1 and Gli1, that are normally expressed at highest levels in the dermal papilla. Data from multiple studies support a model in which the loss of dermal cilia results in arrested follicle development due to dysregulated Shh signaling pathway. Our experience suggests that vismodegib’s side effects are reversible after treatment cessation. However, we recognize that persistent alopecia has been reported in 4 out of 65 patients. In addition, recent data suggest that amenorrhea is a potential side effect of unknown reversibility.
Although subungual melanoma is the most concerning cause for melanonychia striata, there are a variety of other causes including fungal infections, trauma, benign melanocytic lesions, as well as other malignancies. We present a case of melanonychia striata due to pigmented SCC (pSCC) mimicking the clinical appearance of a subungual malignant melanoma in a renal transplant patient. A 62-year-old African American male presented for evaluation of a pigmented longitudinal streak located on his left third finger adjacent to the lateral nail fold. He complained of increasing tenderness of the nail and noted that the pigmented streak had been increasing in size. Nine years prior he had undergone renal transplantation due to end-stage renal disease secondary to glomerulonephritis. He was on a maintenance immunosuppressive regimen that included mycophenolate mofetil and cyclosporine. The patient underwent a nail avulsion and biopsy of the nail bed as well as the matrix. Histopathology was significant for malignant dyskeratosis with lack of nuclear maturation, occasional mitoses, multinucleation, and individual cell keratinization. Immunostaining for S100 was negative while staining for AE1/3 (cytokeratins) was positive consistent with pSCC. He underwent Mohs micrographic surgery where the SCC was found to be more extensive than originally suspected requiring en bloc excision of the nail repaired with a fullthickness skin graft from the left forearm. Subungual pigmented squamous cell carcinoma can present as melanonychia striata and should be considered in the differential diagnosis. It has been reported that approximately 10% of subungual pSCC cases present as melanonychia striata. Although cases of pSCC presenting as melanonychia striata have been previously reported our case occurred in the setting of a renal transplantation and immunosuppression. Our patient had many of the known risk factors for the development of pSCC, including gender, age, skin of color, history of renal transplantation, and immunosuppressive therapy. Although regular full body skin examinations are an accepted part of renal transplantation follow-ups due to the SCC risk in these patients, this case emphasizes the need to remain vigilant for atypical presentations among the immunosuppressed. The nail unit should not be overlooked during the clinical examination of these patients as demonstrated by our patient’s rare presentation of pSCC.
Commercial support: None identified.
Commercial support: None identified.
838
1985 Pigmented squamous cell carcinoma of the skin: Report of two cases with pathological correlation and review of the literature to define its dermoscopic findings Cristiano Horta, MD, Hospital Ipiranga, S~ao Paulo, Brazil; GabrielAribi Fiszbaum, MD, Hospital Ipiranga, S~ao Paulo, Brazil; Daniele De Mola Sponchiado, MD, Hospital Ipiranga, S~ao Paulo, Brazil; Camila Oliveira Alvarenga, MD, Hospital Ipiranga, S~ao Paulo, Brazil The pigmented squamous cell carcinoma is rare, with an incidence ranging from 0.01% to 7% of all squamous cell carcinomas. Differential diagnoses are nodular melanoma, blue nevus, and pigmented basal cell carcinoma. The dermatoscopic findings seem not to increase the clinical diagnostic accuracy. However, the authors highlight the most relevant dermatoscopic findings based on the presentation of two clinical cases with its dermatoscopic and pathologic correlation, besides the review of the literature. The dermatoscopy showed in both cases blueish lesions with structureless areas, skin plaques, and ulcerative areas. In one case, maple leaf structures and in the other blue-white veils were observed. These characteristics suggested the clinical diagnosis of pigmented basal cell carcinoma and melanoma. The diagnostic of pigmented squamous cell carcinoma was obtained after excision of the lesions and antomopathologic study. No evidence of recurrence or metastasis were found for 6 months after removal (at the time of submiting this report). The study of the literature and analysis of the clinical characteristics of the pigmented squamous cell carcinoma allowed the authors to identify the dermoscopic aspects that could assist clinical diagnosis believing that these lesions, although rare, should be included in the differential diagnosis of pigmented skin lesions. Commercial support: None identified.
MAY 2015
Primary cutaneous endometriosis of umbilicus Seung-Hee Loh, MD, Department of Dermatology, Kyung Hee University Hospital, Seoul, South Korea; Dong-Woo Suh, MD, Department of Dermatology, College of Medicine, Kyung Hee University, Seoul, South Korea; Bark-Lynn Lew, MD, PhD, Department of Dermatology, Kyung Hee University Hospital, Seoul, South Korea; Woo-Young Sim, MD, PhD, Department of Dermatology, Kyung Hee University Hospital, Seoul, South Korea Background: Primary cutaneous endometriosis of umbilicus, which is also called Villar nodule, is a rare manifestation of endometriosis, representing 0.5% to 1% of all patients with endometrial ectopia. Ectopic endometriosis could be found in nearly every tissue and it mostly occurs at surgical scar tissue secondarily to abdominal surgical procedures. The most widely accepted pathogenesis of secondary endometriosis is the iatrogenic implantation of endometrial cells as a result of surgery, commonly laparoscopic procedures. However, the pathogenesis of primary endometriosis is yet unknown. Patient and method: We report a case of a 38-year-old multigravida female with a 2.5 3 2.0 cm brownish to purplish colored painful nodule on the umbilicus since 2 years ago. The patient had no history of operation. The nodule became swollen with spontaneous frank bleeding during menstruation. The lesion was first diagnosed as a keloid at local clinic and has undergone steroid intralesional injection for several times but there was no improvement. Result: Umbilical ultrasonography and abdominal computed tomography showed an enhancing mass at the umbilicus without connection to abdominal organs. Histopathologic examination revealed dilated glandular structures surrounded by cellular endometrial type stroma and deep perivascular lymphocytic infiltration. Conclusion: According to these findings, the umbilical lesion was diagnosed as primary cutaneous endometriosis and it was removed by local excision without recurrence. Dermatologists should consider a Villar nodule when a nodule is clinically similar to a keloid with menstrually related, cyclic symptoms without any trauma history including operation. Commercial support: None identified.
J AM ACAD DERMATOL
AB189