Am J Dermatopathol Volume 36, Number 9, September 2014
Letters to the Editor tumor associated with human herpesvirus type 8? J Am Acad Dermatol. 2000;42:290–292. 5. Ioannidou D, Panayiotides J, Krasagakis K, et al. Retiform hemangioendothelioma presenting as bruise-like plaque in an adult woman. Int J Dermatol. 2006;45:53–55. 6. Tan D, Kraybill W, Cheney RT, et al. Retiform hemangioendothelioma: a case report and review of the literature. J Cutan Pathol. 2005;32: 634–647. 7. Sanz-Trelles A, Rodrigo-Fernández I, AyalaCarbonero A, et al. Retiform hemangioendothelioma. A new case in a child with diffuse endovascular papillary endothelial proliferation. J Cutan Pathol. 1997;24:440–444. 8. Zhang GY, Lu QJ, Yin H, et al. A case of retiform-hemangioendothelioma with unusual presentation and aggressive clinical features. Int J Clin Exp Pathol. 2010;3:528–533. 9. Bhutoria B, Konar A, Chakrabarti S, et al. Retiform hemangioendothelioma with lymph node metastasis: a rare entity. Indian J Dermatol Venereol Leprol. 2009;75:60–62. 10. Ulrich D, Hrynyschyn K. Fast growing multifocal retiform hemangioendothelioma: a case report. Eur J Plast Surg. 2002;25:47–49. 11. Duke D, Dvorak AM, Harris TJ, et al. Multiple retiform hemangioendotheliomas. A low-grade angiosarcoma. Am J Dermatopathol. 1996;18: 606–610. 12. Keiler SA, Honda K, Bordeaux JS. Retiform hemangioendothelioma treated with Mohs micrographic surgery. J Am Acad Dermatol. 2011;65: 233–235. 13. Fukunaga M, Endo Y, Masui F, et al. Retiform haemangioendothelioma. Virchows Arch. 1996;428:301–304. 14. Yarmel D, Dormans JP, Pawel BR, et al. Recurrent pedal hobnail (Dabska-retiform) hemangioendothelioma with forefoot reconstructive surgery using a digital fillet flap. J Foot Ankle Surg. 2008;47:487–493.
FIGURE 3. Immunohistochemical staining of tumor tissue. A and B, Tumor cells reacted with endothelial markers CD31 diffusely but CD34 focally (·200). C, This tissue is positive for D2-40 (·200). D, Ki-67 showed a lower proliferation index (·100).
papillary endothelial projections and lack of arborizing rete testis–like architecture.6 Surgery with tumor-free margins has been considered the treatment of choice.10,12,14 Our patient experienced tumor recurrence 6 months after surgery. It illustrates a recurrent example of RH, which should prompt an accurate diagnosis and early intervention. Li-qiang Zheng, MM Xiang-Chun Han, MM† Yong Huang, MM‡ Jing-Yuan Fan, BS Departments of *Dermatology and ‡Pathology, the 251st Hospital of Chinese PLA, Zhangjiakou, China 2013 Lippincott Williams & Wilkins
†Department of Pathology, the First Affiliated Hospital to Hebei North University, Zhangjiakou, China
Intralymphatic Histiocytosis Mimicking Oral Lymphangioma Circumscriptum
REFERENCES 1. Calonje E, Fletcher CD, Wilson-Jones E, et al. Retiform hemangioendothelioma. A distinctive form of low-grade angiosarcoma delineated in a series of 15 cases. Am J Surg Pathol. 1994;18: 115–125. 2. Parsons A, Sheehan DJ, Sangueza OP. Retiform hemangioendotheliomas usually do not express D2-40 and VEGFR-3. Am J Dermatopathol. 2008;30:31–33. 3. Emberger M, Laimer M, Steiner H, et al. Retiform hemangioendothelioma: presentation of a case expressing D2-40. J Cutan Pathol. 2009;36:987–990. 4. Schommer M, Herbst RA, Brodersen JP, et al. Retiform hemangioendothelioma: another
To the Editor: Intralymphatic histiocytosis (ILH) is a rare disorder characterized by proliferation of histiocytes in lymphatic vessels.1 It is thought to be a reactive condition frequently associated with rheumatoid arthritis or certain stimuli such as orthopedic metal implants.1,2 We present a case of ILH occurring in the oral cavity with clinical and histopathologic resemblance to The authors declare no conflicts of interest.
www.amjdermatopathology.com |
759
Letters to the Editor
Am J Dermatopathol Volume 36, Number 9, September 2014
FIGURE 1. Clinical appearance. A, Whitish papules on right buccal mucosa. B, Partial improvement of skin lesion on 1-month follow-up.
that of oral lymphangioma circumscriptum (LC). A 35-year-old Korean woman presented with a 1-year history of asymptomatic bilateral mucosal lesions of oral cavity. It showed a fluctuating course of size and had caused only mild discomfort when chewing foods but had never completely disappeared. Physical examination showed bilateral multiple whitish papules on buccal mucosa (Fig. 1A). Skin biopsy was performed from the papule and histopathological findings consisted of dilated and thrombosed vessels throughout the dermis. Within these vessels, aggregates of mononuclear cells were found, but there was no evident nuclear atypia within the cells (Fig. 2A). We performed immunohistochemical staining to investigate the nature of the cells. Most mononuclear cells in the intraluminal aggregate were positive for CD68 (Fig. 2B). But they were only minimally positive for CD3 and negative for langerin and CD20. Staining with D2-40 indicated that the vessels were of a lymphatic nature (Fig. 2C). Some of the endothelial cells lining the vessels but not the mononuclear cells themselves were positive for CD31 and CD34. The patient was referred to the Department of Otolaryngology, where she was evaluated for additional lesions; however, no other lesions were found. On 1-month follow-up visit after treatments for oral corticosteroids and streptokinases, in an attempt to reduce the lesions, partial improvement of the lesions were observed (Fig. 1B). Based on the clinical and histopathological findings, the diagnosis of ILH was made.
760
| www.amjdermatopathology.com
The most interesting fact about our case was that it took place in oral mucosa, for mucosal involvement of ILH or ILH occurring in the oral cavity has never been reported. In the reported cases, the most common sites affected were the upper extremities, followed by lower extremities.3 Clinical appearance of ILH seems to vary from papules, vesicles, nodules, plaques, and even sometimes to livedo reticularis–like lesions.3 These manifestations follow a chronic but benign course. In some cases, lesions tend to be recurring and refractory to treatment. Unfortunately, there is no specific or consistent treatment proven to be efficient.4 The pathogenesis of ILH is still uncertain. Some authors suggested that ILH may be an early stage of reactive angioendotheliomatosis because of their microscopic similarities.5 However, as ILH exclusively affects lymphatic vessels, it seems natural to focus on change of the lymphatic vessels, not blood vessels. All reported cases of ILH, including our case, are characterized by lymphangiectasis with intraluminal aggregates of histiocytes. Thus, any cause of damage to lymphatic vessels that results in lymphagiectasis, such as infection, trauma, surgery, and persistent inflammation is likely to play a part in the process.4 The relatively frequent association of ILH occurring proximal to the site of rheumatoid arthritis or metal implant surgery also suggests that secondary lymph stasis because of chronic inflammation or traumatic irritation might be a cause.4 It seems noteworthy to mention that lesions in our case happened to arise from buccal
mucosa, which is known for the most common site of trauma and irritation, usually by cheek biting.6 In our case, differential diagnosis with lymphangioma was necessary, as the first line of treatment of lymphangioma is surgical excision, whereas excision may not be the most recommended treatment to ILH because of its benign course and possible recurrence. The difficulty in diagnosing the disease came from the clinical resemblance of the lesion to that of LC. But the low incidence of oral LCs on buccal mucosa and late and relatively abrupt onset of disease allowed us to suspect disease entity other than LC.7 The bilaterality of the lesion, the absence of sanguinous fluid containing vesicles and size change after medication were also contradicting clinical data to that of lymphangioma.8 On microscopy, marked perivascular inflammatory cell infiltration of entire dermis and relatively scattered distribution of the vessels were the histopathologic clues that suggested lymphatic dilatation as a secondary change instead of a tumor. Thus, we were able to confirm the diagnosis as ILH rather than LC. In conclusion, this report documents the first case of ILH arising from mucosa and also oral cavity. The clinical appearance of the intralympahtic histiocytosis of mucosa may differ from preceding reported cases and resemble LC. Young Joon Park, MD Ji Eun Kwon, MD† Jae Ho Han, MD, PhD† Chul Ho Kim, MD, PhD‡ Hee Young Kang, MD, PhD Departments of *Dermatology, †Pathology, and ‡Otolaryngology, Ajou University School of Medicine, Suwon, Korea
REFERENCES 1. Okazaki A, Asada H, Niizeki H, et al. Intravascular histiocytosis associated with rheumatoid arthritis: report of a case with lymphatic endothelial proliferation. Br J Dermatol. 2005;152:1385–1387. 2. Watanabe T, Yamada N, Yoshida Y, et al. Intralymphatic histiocytosis with granuloma formation associated with orthopaedic metal implants. Br J Dermatol. 2008;158:402–404. 3. Washio K, Nakata K, Nakamura A, et al. Pressure bandage as an effective treatment for intralymphatic histiocytosis associated with rheumatoid arthritis. Dermatology. 2011;223:20–24.
2013 Lippincott Williams & Wilkins
Am J Dermatopathol Volume 36, Number 9, September 2014
Letters to the Editor 4. Requena L, El-Shabrawi-Caelen L, Walsh SN, et al. Intralymphatic histiocytosis. A clinicopathologic study of 16 cases. Am J Dermatopathol. 2009;31:140–151. 5. Rieger E, Soyer HP, Leboit PE, et al. Reactive angioendotheliomatosis or intravascular histiocytosis? An immunohistochemical and ultrastructural study in two cases of intravascular histiocytic cell proliferation. Br J Dermatol. 1999;140:497–504. 6. Fowler CB. Benign and malignant neoplasms of the periodontium. Periodontol 2000. 1999;21: 33–83. 7. Poh CF, Priddy RW. Acquired oral lymphangioma circumscriptum mimicking verrucous carcinoma. Oral Oncol Extra. 2005;41:277–280. 8. Stanescu L, Georgescu EF, Simionescu C, et al. Lymphangioma of the oral cavity. Rom J Morphol Embryol. 2006;47:373–377.
FIGURE 2. Immunohistopathologic studies. A, Aggregation of mononuclear cells was seen within dilated vessels in the upper dermis (haematoxylin and eosin stain, original magnification, ·50). B, The predominant cells of the aggregates were CD68 positive histiocytes (original magnification, ·400). C, Staining with D2-40 demonstrated that the dilated vessels were of lymphatic nature (original magnification, ·400).
2013 Lippincott Williams & Wilkins
www.amjdermatopathology.com |
761
Letters to the Editor tumor associated with human herpesvirus type 8? J Am Acad Dermatol. 2000;42:290–292. 5. Ioannidou D, Panayiotides J, Krasagakis K, et al. Retiform hemangioendothelioma presenting as bruise-like plaque in an adult woman. Int J Dermatol. 2006;45:53–55. 6. Tan D, Kraybill W, Cheney RT, et al. Retiform hemangioendothelioma: a case report and review of the literature. J Cutan Pathol. 2005;32: 634–647. 7. Sanz-Trelles A, Rodrigo-Fernández I, AyalaCarbonero A, et al. Retiform hemangioendothelioma. A new case in a child with diffuse endovascular papillary endothelial proliferation. J Cutan Pathol. 1997;24:440–444. 8. Zhang GY, Lu QJ, Yin H, et al. A case of retiform-hemangioendothelioma with unusual presentation and aggressive clinical features. Int J Clin Exp Pathol. 2010;3:528–533. 9. Bhutoria B, Konar A, Chakrabarti S, et al. Retiform hemangioendothelioma with lymph node metastasis: a rare entity. Indian J Dermatol Venereol Leprol. 2009;75:60–62. 10. Ulrich D, Hrynyschyn K. Fast growing multifocal retiform hemangioendothelioma: a case report. Eur J Plast Surg. 2002;25:47–49. 11. Duke D, Dvorak AM, Harris TJ, et al. Multiple retiform hemangioendotheliomas. A low-grade angiosarcoma. Am J Dermatopathol. 1996;18: 606–610. 12. Keiler SA, Honda K, Bordeaux JS. Retiform hemangioendothelioma treated with Mohs micrographic surgery. J Am Acad Dermatol. 2011;65: 233–235. 13. Fukunaga M, Endo Y, Masui F, et al. Retiform haemangioendothelioma. Virchows Arch. 1996;428:301–304. 14. Yarmel D, Dormans JP, Pawel BR, et al. Recurrent pedal hobnail (Dabska-retiform) hemangioendothelioma with forefoot reconstructive surgery using a digital fillet flap. J Foot Ankle Surg. 2008;47:487–493.
FIGURE 3. Immunohistochemical staining of tumor tissue. A and B, Tumor cells reacted with endothelial markers CD31 diffusely but CD34 focally (·200). C, This tissue is positive for D2-40 (·200). D, Ki-67 showed a lower proliferation index (·100).
papillary endothelial projections and lack of arborizing rete testis–like architecture.6 Surgery with tumor-free margins has been considered the treatment of choice.10,12,14 Our patient experienced tumor recurrence 6 months after surgery. It illustrates a recurrent example of RH, which should prompt an accurate diagnosis and early intervention. Li-qiang Zheng, MM Xiang-Chun Han, MM† Yong Huang, MM‡ Jing-Yuan Fan, BS Departments of *Dermatology and ‡Pathology, the 251st Hospital of Chinese PLA, Zhangjiakou, China 2013 Lippincott Williams & Wilkins
†Department of Pathology, the First Affiliated Hospital to Hebei North University, Zhangjiakou, China
Intralymphatic Histiocytosis Mimicking Oral Lymphangioma Circumscriptum
REFERENCES 1. Calonje E, Fletcher CD, Wilson-Jones E, et al. Retiform hemangioendothelioma. A distinctive form of low-grade angiosarcoma delineated in a series of 15 cases. Am J Surg Pathol. 1994;18: 115–125. 2. Parsons A, Sheehan DJ, Sangueza OP. Retiform hemangioendotheliomas usually do not express D2-40 and VEGFR-3. Am J Dermatopathol. 2008;30:31–33. 3. Emberger M, Laimer M, Steiner H, et al. Retiform hemangioendothelioma: presentation of a case expressing D2-40. J Cutan Pathol. 2009;36:987–990. 4. Schommer M, Herbst RA, Brodersen JP, et al. Retiform hemangioendothelioma: another
To the Editor: Intralymphatic histiocytosis (ILH) is a rare disorder characterized by proliferation of histiocytes in lymphatic vessels.1 It is thought to be a reactive condition frequently associated with rheumatoid arthritis or certain stimuli such as orthopedic metal implants.1,2 We present a case of ILH occurring in the oral cavity with clinical and histopathologic resemblance to The authors declare no conflicts of interest.
www.amjdermatopathology.com |
759
Letters to the Editor
Am J Dermatopathol Volume 36, Number 9, September 2014
FIGURE 1. Clinical appearance. A, Whitish papules on right buccal mucosa. B, Partial improvement of skin lesion on 1-month follow-up.
that of oral lymphangioma circumscriptum (LC). A 35-year-old Korean woman presented with a 1-year history of asymptomatic bilateral mucosal lesions of oral cavity. It showed a fluctuating course of size and had caused only mild discomfort when chewing foods but had never completely disappeared. Physical examination showed bilateral multiple whitish papules on buccal mucosa (Fig. 1A). Skin biopsy was performed from the papule and histopathological findings consisted of dilated and thrombosed vessels throughout the dermis. Within these vessels, aggregates of mononuclear cells were found, but there was no evident nuclear atypia within the cells (Fig. 2A). We performed immunohistochemical staining to investigate the nature of the cells. Most mononuclear cells in the intraluminal aggregate were positive for CD68 (Fig. 2B). But they were only minimally positive for CD3 and negative for langerin and CD20. Staining with D2-40 indicated that the vessels were of a lymphatic nature (Fig. 2C). Some of the endothelial cells lining the vessels but not the mononuclear cells themselves were positive for CD31 and CD34. The patient was referred to the Department of Otolaryngology, where she was evaluated for additional lesions; however, no other lesions were found. On 1-month follow-up visit after treatments for oral corticosteroids and streptokinases, in an attempt to reduce the lesions, partial improvement of the lesions were observed (Fig. 1B). Based on the clinical and histopathological findings, the diagnosis of ILH was made.
760
| www.amjdermatopathology.com
The most interesting fact about our case was that it took place in oral mucosa, for mucosal involvement of ILH or ILH occurring in the oral cavity has never been reported. In the reported cases, the most common sites affected were the upper extremities, followed by lower extremities.3 Clinical appearance of ILH seems to vary from papules, vesicles, nodules, plaques, and even sometimes to livedo reticularis–like lesions.3 These manifestations follow a chronic but benign course. In some cases, lesions tend to be recurring and refractory to treatment. Unfortunately, there is no specific or consistent treatment proven to be efficient.4 The pathogenesis of ILH is still uncertain. Some authors suggested that ILH may be an early stage of reactive angioendotheliomatosis because of their microscopic similarities.5 However, as ILH exclusively affects lymphatic vessels, it seems natural to focus on change of the lymphatic vessels, not blood vessels. All reported cases of ILH, including our case, are characterized by lymphangiectasis with intraluminal aggregates of histiocytes. Thus, any cause of damage to lymphatic vessels that results in lymphagiectasis, such as infection, trauma, surgery, and persistent inflammation is likely to play a part in the process.4 The relatively frequent association of ILH occurring proximal to the site of rheumatoid arthritis or metal implant surgery also suggests that secondary lymph stasis because of chronic inflammation or traumatic irritation might be a cause.4 It seems noteworthy to mention that lesions in our case happened to arise from buccal
mucosa, which is known for the most common site of trauma and irritation, usually by cheek biting.6 In our case, differential diagnosis with lymphangioma was necessary, as the first line of treatment of lymphangioma is surgical excision, whereas excision may not be the most recommended treatment to ILH because of its benign course and possible recurrence. The difficulty in diagnosing the disease came from the clinical resemblance of the lesion to that of LC. But the low incidence of oral LCs on buccal mucosa and late and relatively abrupt onset of disease allowed us to suspect disease entity other than LC.7 The bilaterality of the lesion, the absence of sanguinous fluid containing vesicles and size change after medication were also contradicting clinical data to that of lymphangioma.8 On microscopy, marked perivascular inflammatory cell infiltration of entire dermis and relatively scattered distribution of the vessels were the histopathologic clues that suggested lymphatic dilatation as a secondary change instead of a tumor. Thus, we were able to confirm the diagnosis as ILH rather than LC. In conclusion, this report documents the first case of ILH arising from mucosa and also oral cavity. The clinical appearance of the intralympahtic histiocytosis of mucosa may differ from preceding reported cases and resemble LC. Young Joon Park, MD Ji Eun Kwon, MD† Jae Ho Han, MD, PhD† Chul Ho Kim, MD, PhD‡ Hee Young Kang, MD, PhD Departments of *Dermatology, †Pathology, and ‡Otolaryngology, Ajou University School of Medicine, Suwon, Korea
REFERENCES 1. Okazaki A, Asada H, Niizeki H, et al. Intravascular histiocytosis associated with rheumatoid arthritis: report of a case with lymphatic endothelial proliferation. Br J Dermatol. 2005;152:1385–1387. 2. Watanabe T, Yamada N, Yoshida Y, et al. Intralymphatic histiocytosis with granuloma formation associated with orthopaedic metal implants. Br J Dermatol. 2008;158:402–404. 3. Washio K, Nakata K, Nakamura A, et al. Pressure bandage as an effective treatment for intralymphatic histiocytosis associated with rheumatoid arthritis. Dermatology. 2011;223:20–24.
2013 Lippincott Williams & Wilkins
Am J Dermatopathol Volume 36, Number 9, September 2014
Letters to the Editor 4. Requena L, El-Shabrawi-Caelen L, Walsh SN, et al. Intralymphatic histiocytosis. A clinicopathologic study of 16 cases. Am J Dermatopathol. 2009;31:140–151. 5. Rieger E, Soyer HP, Leboit PE, et al. Reactive angioendotheliomatosis or intravascular histiocytosis? An immunohistochemical and ultrastructural study in two cases of intravascular histiocytic cell proliferation. Br J Dermatol. 1999;140:497–504. 6. Fowler CB. Benign and malignant neoplasms of the periodontium. Periodontol 2000. 1999;21: 33–83. 7. Poh CF, Priddy RW. Acquired oral lymphangioma circumscriptum mimicking verrucous carcinoma. Oral Oncol Extra. 2005;41:277–280. 8. Stanescu L, Georgescu EF, Simionescu C, et al. Lymphangioma of the oral cavity. Rom J Morphol Embryol. 2006;47:373–377.
FIGURE 2. Immunohistopathologic studies. A, Aggregation of mononuclear cells was seen within dilated vessels in the upper dermis (haematoxylin and eosin stain, original magnification, ·50). B, The predominant cells of the aggregates were CD68 positive histiocytes (original magnification, ·400). C, Staining with D2-40 demonstrated that the dilated vessels were of lymphatic nature (original magnification, ·400).
2013 Lippincott Williams & Wilkins
www.amjdermatopathology.com |
761
![[Lymphangioma circumscriptum].](/assets/img/hold.png)
