Letters to the Editor
as well as SSSS.5 Mesh-type silicone dressing in this case avoided excessive Nikolsky sign, and was also more useful for skin recovery without excessive residual bacterial infection. This supported the effect of antibacterial drugs.
REFERENCES 1 Kyriakos PK, Tadros A, Dimou A et al. Case detection rates of impetigo by gender and age. Infez Med 2012; 20: 105–107. 2 Pereira LB. Impetigo - review. An Bras Dermatol 2014; 89: 293– 299. 3 Amagai M, Stanley JR. Desmoglein as a target in skin disease and beyond. J Invest Dermatol 2012; 132: 776–784. 4 Bourayou R, Le Sache N, Kone-Puat I. Positive Nikolsky Sign due to Staphylococcal Scaleded Skin Syndome. J Pediatr 2011; 159: 868. 5 Handler MZ, Schwartz RA. Staphylococcal scalded skin syndrome: diagnosis and management in children and adults. J Eur Acad Dermatol Venereol 2014; 28: 1418–1423.
CONFLICT OF INTEREST: None. Junko SHIOHARA,1 Sonoe ONO,1 Toshie ENDO,2 Kazuhiko KANEKO2 Departments of 1Dermatology, and 2Palliative Care, Okaya City Hospital, Nagano, Japan doi: 10.1111/1346-8138.13003
Primary cutaneous rhabdomyosarcoma: Case report and review of published work However, the lesion grew in size during 1 month of follow up. The histological findings revealed a neoplastic infiltration consisting of small, round, blue cells with large, pleomorphic, hyperchromatic nuclei, separated by connective tissue trabeculae through the deep dermis (Fig. 1b–d). Immunohistochemical staining demonstrated that the tumor cells were positive for MyoD1, CD56, desmin and vimentin, and negative for pancytokeratin, myoglobin, CK20, neuron-specific enolase, synaptophysin and CD99 (Fig. 1e–g). The Ki-67 proliferative index of the tumor cells was 40%. Positron emission tomography computed tomography showed no evidence of metastatic origin. Based on these clinical and histological findings, the lesion was diagnosed as a solid variant of primary cutaneous alveolar RMS. She was referred to the pediatric
Dear Editor, Although rhabdomyosarcoma (RMS) is a common sarcoma in children, the primary cutaneous type is a rare entity.1 Herein, we report a case of primary cutaneous RMS that was initially misdiagnosed as an atrophic scar. A 2-year-old girl presented with a firm mass on the chin that was detected 2 months previously. She experienced a blunt trauma at the same site 3 months ago. Physical examination revealed a solitary 1-cm firm mass with an overlying atrophic erythematous plaque (Fig. 1a). On ultrasonography, an oval-shaped, hypoechoic lesion 1 cm in size with an irregular wall, echogenic septation and a nodular portion was noticed. Considering the history of trauma, the presumed diagnosis was an atrophic scar or traumatic panniculitis.
(a)
(d)
(b)
(e)
(c)
(f)
(g)
Figure 1. (a) Firm mass with overlying atrophic erythematous plaque on the chin. (b–d) The histological findings revealed a neoplastic infiltration consisting of small, round, blue cells with large, pleomorphic, hyperchromatic nuclei separated by connective tissue trabeculae through the deep dermis (hematoxylin–eosin, original magnifications: [b] 940; [c] 9200; [d] 9400). Immunohistochemical examination revealed that the tumor cells were positive for (e) MyoD1, (f) CD56 and (g) desmin (9300).
Correspondence: Young Min Park, M.D., Ph.D., Department of Dermatology, Seoul St Mary’s Hospital, College of Medicine, The Catholic University of Korea, 222 Banpo-daero, Seocho-gu, Seoul 137-701, Korea. Email: [email protected]
1014
© 2015 Japanese Dermatological Association
Letters to the Editor
oncology department of another hospital, where she received chemotherapy. Only 44 cases of primary cutaneous RMS have been reported in the published work, and they account for less than 1% of RMS.2 Primary cutaneous RMS presented bimodal age distribution like RMS, but without definite male predominance.2 To make a diagnosis of primary cutaneous RMS, the tumor should arise in the dermis or subcutaneous tissue with no evidence of deep soft tissue or metastatic origin.1,2 Of the 44 reported cases, the most common site is head and neck region (23 cases), and the most common histological subtype is alveolar (22 cases), followed by embryonal (10 cases) and pleomorphic (5 cases).2,3 Alveolar RMS is histologically characterized by the presence of small, round, neoplastic cells and alveolar-like spaces separated by connective tissue trabeculae.4 Muscle-specific actin and desmin have been reported as the most sensitive marker, whereas myoglobin, myogenin and MyoD1 are considered to have greater specificity.5 The differential diagnoses include general categories of tumors with small, round, blue cells. In our case, CD56 positivity made the differential diagnosis with Merkel cell carcinoma and malignant peripheral nerve sheath tumor difficult. However, CD56 expression has been demonstrated in RMS, and desmin and MyoD1 positivity with CK20 negativity facilitated the differential diagnosis.1 In cases of tumoral lesion following trauma, skeletal muscle regeneration (SMR) should also be included in the differential diagnosis. SMR is a pleomorphic proliferation with inflammatory and phagocytic reactions following trauma, and it may histologically simulate RMS. However, RMS lacks the peripheral maturation pattern observed in SMR.5 Our case is interesting in that the initial
presentation of primary cutaneous RMS clinically resembled an atrophic scar.
ACKNOWLEDGMENT:
All authors have contributed significantly and equally, and are in agreement with the content.
CONFLICT OF INTEREST:
None declared.
Yoon Seob KIM, Ji Hyun LEE, Jun Young LEE, Young Min PARK Department of Dermatology, Seoul St Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea doi: 10.1111/1346-8138.13011
REFERENCES 1 Sabater-Marco V. Zapater Latorre E, Martorell Cebollada M. Postradiation cutaneous pleomorphic rhabdomyosarcoma with extracellular collagen deposits reminiscent of so-called amianthoid fibers. J Cutan Pathol 2014; 41: 316–321. 2 Marburger TB, Gardner JM, Prieto VG, Billings SD. Primary cutaneous rhabdomyosarcoma: a clinicopathologic review of 11 cases. J Cutan Pathol 2012; 39: 987–995. 3 Scatena C, Massi D, Franchi A, De Paoli A, Canzonieri V. Rhabdomyosarcoma of the skin resembling carcinosarcoma: report of a case and literature review. Am J Dermatopathol 2012; 34: e1–e6. 4 Lee MW, Chung WK, Choi JH, Moon KC, Koh JK. A case of botryoid-type embryonal rhabdomyosarcoma. Clin Exp Dermatol 2009; 34: e737–e739. 5 Ghosn SH, Radfar A, Stefanato CM. Skeletal muscle regeneration: report of a case presenting as a cutaneous nodule following blunt trauma to the lip. J Cutan Pathol 2007; 34: 352–354.
Case of nevoid basal cell carcinoma syndrome with multiple cutaneous keratocysts Dear Editor, Nevoid basal cell carcinoma syndrome (NBCCS), also known as Gorlin syndrome, is an autosomal dominant disorder showing multiple basal cell carcinomas (BCC), palmoplantar pits, keratocystic odontogenic tumors (KCOT), calcification of the falx cerebri and skeletal abnormalities.1 Though cutaneous cysts, especially epidermal cysts, are also often observed in cases of NBCCS,1,2 cutaneous keratocysts are rare.3–5 We report a case of NBCCS with multiple cutaneous keratocysts. A 33-year-old Japanese man presented with a 4-year history of multiple, gradually enlarging, intracutaneous nodules. Physical examination showed 16 elastic, soft, subcutaneous, cystic nodules of varying size on his right upper eyelid, trunk and left
thigh (Fig. 1a–c). He also had 3 mm 9 3 mm and 8 mm 9 5 mm, dome-shaped, blackish-brown nodules on his cheeks (Fig. 1c). He had multiple milia, 2 mm in diameter, around his eyelid. Histological examination of the intracutaneous cystic nodule on the thigh revealed a corrugated wall surface lined by a thin layer of stratified squamous epithelium without a granular cell layer (Fig. 1d). Vellus and terminal hairs in the cavity were not observed, neither were sebaceous glands within or close to the cyst wall. Two cutaneous cysts on his right upper eyelid showed similar histological features, and were diagnosed as cutaneous keratocysts. Histological examination of the resected blackish-brown nodules confirmed solid-type BCC. He underwent operation for the jaw cysts, which were histologically diagnosed as KCOT. A computed tomographic scan of the head
Correspondence: Yoshiaki Kubo, M.D., Ph.D., Department of Dermatology, Institute of Biomedical Sciences, Tokushima University Graduate School, 3-15-18 Kuramoto-cho, Tokushima City, Tokushima 770-8503, Japan. Email: [email protected]
© 2015 Japanese Dermatological Association
1015
as well as SSSS.5 Mesh-type silicone dressing in this case avoided excessive Nikolsky sign, and was also more useful for skin recovery without excessive residual bacterial infection. This supported the effect of antibacterial drugs.
REFERENCES 1 Kyriakos PK, Tadros A, Dimou A et al. Case detection rates of impetigo by gender and age. Infez Med 2012; 20: 105–107. 2 Pereira LB. Impetigo - review. An Bras Dermatol 2014; 89: 293– 299. 3 Amagai M, Stanley JR. Desmoglein as a target in skin disease and beyond. J Invest Dermatol 2012; 132: 776–784. 4 Bourayou R, Le Sache N, Kone-Puat I. Positive Nikolsky Sign due to Staphylococcal Scaleded Skin Syndome. J Pediatr 2011; 159: 868. 5 Handler MZ, Schwartz RA. Staphylococcal scalded skin syndrome: diagnosis and management in children and adults. J Eur Acad Dermatol Venereol 2014; 28: 1418–1423.
CONFLICT OF INTEREST: None. Junko SHIOHARA,1 Sonoe ONO,1 Toshie ENDO,2 Kazuhiko KANEKO2 Departments of 1Dermatology, and 2Palliative Care, Okaya City Hospital, Nagano, Japan doi: 10.1111/1346-8138.13003
Primary cutaneous rhabdomyosarcoma: Case report and review of published work However, the lesion grew in size during 1 month of follow up. The histological findings revealed a neoplastic infiltration consisting of small, round, blue cells with large, pleomorphic, hyperchromatic nuclei, separated by connective tissue trabeculae through the deep dermis (Fig. 1b–d). Immunohistochemical staining demonstrated that the tumor cells were positive for MyoD1, CD56, desmin and vimentin, and negative for pancytokeratin, myoglobin, CK20, neuron-specific enolase, synaptophysin and CD99 (Fig. 1e–g). The Ki-67 proliferative index of the tumor cells was 40%. Positron emission tomography computed tomography showed no evidence of metastatic origin. Based on these clinical and histological findings, the lesion was diagnosed as a solid variant of primary cutaneous alveolar RMS. She was referred to the pediatric
Dear Editor, Although rhabdomyosarcoma (RMS) is a common sarcoma in children, the primary cutaneous type is a rare entity.1 Herein, we report a case of primary cutaneous RMS that was initially misdiagnosed as an atrophic scar. A 2-year-old girl presented with a firm mass on the chin that was detected 2 months previously. She experienced a blunt trauma at the same site 3 months ago. Physical examination revealed a solitary 1-cm firm mass with an overlying atrophic erythematous plaque (Fig. 1a). On ultrasonography, an oval-shaped, hypoechoic lesion 1 cm in size with an irregular wall, echogenic septation and a nodular portion was noticed. Considering the history of trauma, the presumed diagnosis was an atrophic scar or traumatic panniculitis.
(a)
(d)
(b)
(e)
(c)
(f)
(g)
Figure 1. (a) Firm mass with overlying atrophic erythematous plaque on the chin. (b–d) The histological findings revealed a neoplastic infiltration consisting of small, round, blue cells with large, pleomorphic, hyperchromatic nuclei separated by connective tissue trabeculae through the deep dermis (hematoxylin–eosin, original magnifications: [b] 940; [c] 9200; [d] 9400). Immunohistochemical examination revealed that the tumor cells were positive for (e) MyoD1, (f) CD56 and (g) desmin (9300).
Correspondence: Young Min Park, M.D., Ph.D., Department of Dermatology, Seoul St Mary’s Hospital, College of Medicine, The Catholic University of Korea, 222 Banpo-daero, Seocho-gu, Seoul 137-701, Korea. Email: [email protected]
1014
© 2015 Japanese Dermatological Association
Letters to the Editor
oncology department of another hospital, where she received chemotherapy. Only 44 cases of primary cutaneous RMS have been reported in the published work, and they account for less than 1% of RMS.2 Primary cutaneous RMS presented bimodal age distribution like RMS, but without definite male predominance.2 To make a diagnosis of primary cutaneous RMS, the tumor should arise in the dermis or subcutaneous tissue with no evidence of deep soft tissue or metastatic origin.1,2 Of the 44 reported cases, the most common site is head and neck region (23 cases), and the most common histological subtype is alveolar (22 cases), followed by embryonal (10 cases) and pleomorphic (5 cases).2,3 Alveolar RMS is histologically characterized by the presence of small, round, neoplastic cells and alveolar-like spaces separated by connective tissue trabeculae.4 Muscle-specific actin and desmin have been reported as the most sensitive marker, whereas myoglobin, myogenin and MyoD1 are considered to have greater specificity.5 The differential diagnoses include general categories of tumors with small, round, blue cells. In our case, CD56 positivity made the differential diagnosis with Merkel cell carcinoma and malignant peripheral nerve sheath tumor difficult. However, CD56 expression has been demonstrated in RMS, and desmin and MyoD1 positivity with CK20 negativity facilitated the differential diagnosis.1 In cases of tumoral lesion following trauma, skeletal muscle regeneration (SMR) should also be included in the differential diagnosis. SMR is a pleomorphic proliferation with inflammatory and phagocytic reactions following trauma, and it may histologically simulate RMS. However, RMS lacks the peripheral maturation pattern observed in SMR.5 Our case is interesting in that the initial
presentation of primary cutaneous RMS clinically resembled an atrophic scar.
ACKNOWLEDGMENT:
All authors have contributed significantly and equally, and are in agreement with the content.
CONFLICT OF INTEREST:
None declared.
Yoon Seob KIM, Ji Hyun LEE, Jun Young LEE, Young Min PARK Department of Dermatology, Seoul St Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, Korea doi: 10.1111/1346-8138.13011
REFERENCES 1 Sabater-Marco V. Zapater Latorre E, Martorell Cebollada M. Postradiation cutaneous pleomorphic rhabdomyosarcoma with extracellular collagen deposits reminiscent of so-called amianthoid fibers. J Cutan Pathol 2014; 41: 316–321. 2 Marburger TB, Gardner JM, Prieto VG, Billings SD. Primary cutaneous rhabdomyosarcoma: a clinicopathologic review of 11 cases. J Cutan Pathol 2012; 39: 987–995. 3 Scatena C, Massi D, Franchi A, De Paoli A, Canzonieri V. Rhabdomyosarcoma of the skin resembling carcinosarcoma: report of a case and literature review. Am J Dermatopathol 2012; 34: e1–e6. 4 Lee MW, Chung WK, Choi JH, Moon KC, Koh JK. A case of botryoid-type embryonal rhabdomyosarcoma. Clin Exp Dermatol 2009; 34: e737–e739. 5 Ghosn SH, Radfar A, Stefanato CM. Skeletal muscle regeneration: report of a case presenting as a cutaneous nodule following blunt trauma to the lip. J Cutan Pathol 2007; 34: 352–354.
Case of nevoid basal cell carcinoma syndrome with multiple cutaneous keratocysts Dear Editor, Nevoid basal cell carcinoma syndrome (NBCCS), also known as Gorlin syndrome, is an autosomal dominant disorder showing multiple basal cell carcinomas (BCC), palmoplantar pits, keratocystic odontogenic tumors (KCOT), calcification of the falx cerebri and skeletal abnormalities.1 Though cutaneous cysts, especially epidermal cysts, are also often observed in cases of NBCCS,1,2 cutaneous keratocysts are rare.3–5 We report a case of NBCCS with multiple cutaneous keratocysts. A 33-year-old Japanese man presented with a 4-year history of multiple, gradually enlarging, intracutaneous nodules. Physical examination showed 16 elastic, soft, subcutaneous, cystic nodules of varying size on his right upper eyelid, trunk and left
thigh (Fig. 1a–c). He also had 3 mm 9 3 mm and 8 mm 9 5 mm, dome-shaped, blackish-brown nodules on his cheeks (Fig. 1c). He had multiple milia, 2 mm in diameter, around his eyelid. Histological examination of the intracutaneous cystic nodule on the thigh revealed a corrugated wall surface lined by a thin layer of stratified squamous epithelium without a granular cell layer (Fig. 1d). Vellus and terminal hairs in the cavity were not observed, neither were sebaceous glands within or close to the cyst wall. Two cutaneous cysts on his right upper eyelid showed similar histological features, and were diagnosed as cutaneous keratocysts. Histological examination of the resected blackish-brown nodules confirmed solid-type BCC. He underwent operation for the jaw cysts, which were histologically diagnosed as KCOT. A computed tomographic scan of the head
Correspondence: Yoshiaki Kubo, M.D., Ph.D., Department of Dermatology, Institute of Biomedical Sciences, Tokushima University Graduate School, 3-15-18 Kuramoto-cho, Tokushima City, Tokushima 770-8503, Japan. Email: [email protected]
© 2015 Japanese Dermatological Association
1015
