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British Journal of Oral and Maxillofacial Surgery 53 (2015) 86–88
Short communication
Juvenile xanthogranuloma: unusual intraoral finding L. Collins a,∗ , R. Banks a , M. Robinson b a b
Department of Oral and Maxillofacial Surgery, Sunderland Royal Hospital, Kayll Road, Sunderland SR4 7TP, United Kingdom Centre for Oral Health Research, Newcastle University, Framlington Place, Newcastle Upon Tyne NE2 4BW, United Kingdom
Accepted 17 September 2014 Available online 7 October 2014
Abstract Juvenile xanthogranuloma is a rare non-Langerhans cell histiocytosis that usually presents as a self-limiting dermatological condition in young children. Rarely, extracutaneous sites may also be involved. We report a case in a 3-year-old girl that presented intraorally as a solitary, well-defined, soft, purple palatal swelling. Patients with these rare intraoral lesions may present to dentists and subsequently to oral and maxillofacial surgeons. Diagnosis requires histopathological analysis and immunohistochemical staining. Further investigation from other specialties is required to rule out involvement of other organ systems. © 2014 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
Keywords: Xanthogranuloma; Intraoral; Palatal swelling; Non-Langerhans cell histiocytosis
Introduction Juvenile xanthogranuloma is a rare non-Langerhans cell histiocytosis that usually presents as a dermatological disorder in infants.1 The lesions, which commonly present as single or multiple yellow-brown papules a few millimetres in diameter, most often occur on the head and neck, but are also seen on the trunk and extremities.2,3 Extracutaneous sites may also be affected. The uveal tract is the most common of these, but other organs include the central nervous system, liver, spleen, lungs, gonads, bone, kidneys, and adrenal glands.4 The disease is a histiocytic disorder but the exact pathogenesis is not known. It is thought to involve the proliferation of reactive monocytes in response to a non-specific physical or infectious agent.5 Typically, infants are affected and up to 10% of cases present at birth. Usually there is a single lesion on the skin but children younger than 6 months of age are more likely to have many.6
∗
Corresponding author. Fax: +44 191 5699231. E-mail address: [email protected] (L. Collins).
Cutaneous lesions are usually self-limiting and asymptomatic. Ocular lesions usually involve the iris and may present as heterochromia, uveitis, spontaneous hyphema, or secondary glaucoma. Abdominal masses or involvement of the central nervous system may respond to excision with or without radiation or chemotherapy.7 However, when there is visceral involvement, the condition can potentially be fatal. Intraoral lesions are rare and we know of only 31 documented cases published in the English language.
Case report A 3-year-old girl had a routine dental examination after her mother had noticed a small palatal swelling. It had gradually increased in size but did not seem to cause any pain or functional problems. The patient’s past medical history was unremarkable. On clinical examination there was a purple, soft swelling attached to the right hard palate that measured 0.5 cm in diameter. The overlying mucosa was intact. Based on the clinical appearance the differential diagnoses included fibroepithelial polyp, mucocoele, or vascular lesion.
http://dx.doi.org/10.1016/j.bjoms.2014.09.014 0266-4356/© 2014 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
L. Collins et al. / British Journal of Oral and Maxillofacial Surgery 53 (2015) 86–88
87
Fig. 1. Photomicrograph showing nodule of oral mucosa. There are sheets of histiocytes, extravasated erythrocytes, and haemosiderin deposits (haematoxylin and eosin, original magnification ×200).
Excisional biopsy examination under general anaesthesia showed no involvement of the underlying periosteum or bone. The excised sample was sent for histopathological examination. Macroscopically, it consisted of firm, cream, polypoidal tissue measuring 8 mm × 5 mm × 3 mm. Sectioning showed a uniform tan-coloured cut surface. Microscopically, the lamina propria and submucosa were stretched by a cellular lesion that comprised numerous histiocytes and occasional multinucleate giant cells embedded in a fibrovascular matrix. There was haemosiderin deposition, and some histiocytes had foamy cytoplasm. Immunohistochemical testing confirmed that the lesion contained no Langherans cells. Cytoplasmic immunoreaction to CD68 confirmed the type of histiocytosis, and a diagnosis of juvenile xanthogranuloma was made (Fig. 1). After review at the clinic, further questioning showed that the patient had a history of multiple skin lesions, particularly on her back. Examination revealed multiple asymptomatic red-yellow papules (Fig. 2), which, until then, had been diagnosed as molluscum contagiosum. After diagnosis of extracutaneous juvenile xanthogranuloma and dermatological findings of suspected juvenile xanthogranuloma, the patient was referred for assessment by a paediatrician and ophthalmologist. Unfortunately, she failed to attend these appointments and has not presented again.
Fig. 2. Photograph of multiple red-yellow papules on the patient’s back previously diagnosed as molloscum contagiosum before being identified as intraoral juvenile xanthogranuloma (published with the parent’s consent).
established with histopathological analysis and immunohistochemical staining. Whilst the index lesions are usually cutaneous, our case shows that the oral cavity can occasionally be involved, and the dentist may be the first to see it. Appropriate referral with subsequent investigation by relevant specialists is important to establish diagnosis and, where indicated, treat lesions to prevent potentially serious consequences. Conflict of interest We have no conflict of interest. Ethics statement/confirmation of patient permission
Discussion Consent was obtained for the publication of clinical images. Intraoral juvenile xanthogranuloma is extremely rare and we know of only 6 previous reports of palatal lesions. Given their rarity, identification and diagnosis may be difficult. Differential diagnoses (depending on site) may include dental abscess, fibroepithelial polyp, mucocoele, vascular malformation, lipoma, gingival cyst, peripheral giant cell granuloma, foreign body granuloma, pyogenic granuloma, and mucoepidermoid carcinoma.8 Definitive diagnosis is
References 1. Satow SJ, Zee S, Dawson KH, et al. Juvenile xanthogranuloma of the tongue. J Am Acad Dermatol 1995;33:376–9. 2. Flaitz C, Allen C, Neville B, et al. Juvenile xanthogranuloma of the oral cavity in children: a clinicopathologic study. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2002;94:345–52.
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L. Collins et al. / British Journal of Oral and Maxillofacial Surgery 53 (2015) 86–88
3. Kjaerkeim A, Stokke T. Juvenile xanthogranuloma of the oral cavity. An electron microscopic study. Oral Surg Oral Med Oral Pathol 1974;38:414–25. 4. Webster SB, Reister HC, Harman Jr LE. Juvenile xanthogranuloma with extracutaneous lesions. A case report and review of the literature. Arch Dermatol 1966;93:71–6. 5. Favara BE, Feller AC, Pauli M, et al. Contemporary classification of histiocytic disorders. The WHO Committee on Histiocytic/Reticulum Cell
Proliferations. Reclassification Working Group of the Histiocyte Society. Med Pediatr Oncol 1997;29:157–66. 6. Cypel TK, Zuker RM. Juvenile xanthogranuloma: case report and review of the literature. Can J Plast Surg 2008;16:175–7. 7. Stover DG, Alapati S, Regueira O, et al. Treatment of juvenile xanthogranuloma. Pediatr Blood Cancer 2008;51:130–3. 8. Villa A, Mariani U, Villa F. Lingual juvenile xanthogranuloma in a woman: a case report. J Med Case Rep 2011;5:30.
British Journal of Oral and Maxillofacial Surgery 53 (2015) 86–88
Short communication
Juvenile xanthogranuloma: unusual intraoral finding L. Collins a,∗ , R. Banks a , M. Robinson b a b
Department of Oral and Maxillofacial Surgery, Sunderland Royal Hospital, Kayll Road, Sunderland SR4 7TP, United Kingdom Centre for Oral Health Research, Newcastle University, Framlington Place, Newcastle Upon Tyne NE2 4BW, United Kingdom
Accepted 17 September 2014 Available online 7 October 2014
Abstract Juvenile xanthogranuloma is a rare non-Langerhans cell histiocytosis that usually presents as a self-limiting dermatological condition in young children. Rarely, extracutaneous sites may also be involved. We report a case in a 3-year-old girl that presented intraorally as a solitary, well-defined, soft, purple palatal swelling. Patients with these rare intraoral lesions may present to dentists and subsequently to oral and maxillofacial surgeons. Diagnosis requires histopathological analysis and immunohistochemical staining. Further investigation from other specialties is required to rule out involvement of other organ systems. © 2014 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
Keywords: Xanthogranuloma; Intraoral; Palatal swelling; Non-Langerhans cell histiocytosis
Introduction Juvenile xanthogranuloma is a rare non-Langerhans cell histiocytosis that usually presents as a dermatological disorder in infants.1 The lesions, which commonly present as single or multiple yellow-brown papules a few millimetres in diameter, most often occur on the head and neck, but are also seen on the trunk and extremities.2,3 Extracutaneous sites may also be affected. The uveal tract is the most common of these, but other organs include the central nervous system, liver, spleen, lungs, gonads, bone, kidneys, and adrenal glands.4 The disease is a histiocytic disorder but the exact pathogenesis is not known. It is thought to involve the proliferation of reactive monocytes in response to a non-specific physical or infectious agent.5 Typically, infants are affected and up to 10% of cases present at birth. Usually there is a single lesion on the skin but children younger than 6 months of age are more likely to have many.6
∗
Corresponding author. Fax: +44 191 5699231. E-mail address: [email protected] (L. Collins).
Cutaneous lesions are usually self-limiting and asymptomatic. Ocular lesions usually involve the iris and may present as heterochromia, uveitis, spontaneous hyphema, or secondary glaucoma. Abdominal masses or involvement of the central nervous system may respond to excision with or without radiation or chemotherapy.7 However, when there is visceral involvement, the condition can potentially be fatal. Intraoral lesions are rare and we know of only 31 documented cases published in the English language.
Case report A 3-year-old girl had a routine dental examination after her mother had noticed a small palatal swelling. It had gradually increased in size but did not seem to cause any pain or functional problems. The patient’s past medical history was unremarkable. On clinical examination there was a purple, soft swelling attached to the right hard palate that measured 0.5 cm in diameter. The overlying mucosa was intact. Based on the clinical appearance the differential diagnoses included fibroepithelial polyp, mucocoele, or vascular lesion.
http://dx.doi.org/10.1016/j.bjoms.2014.09.014 0266-4356/© 2014 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
L. Collins et al. / British Journal of Oral and Maxillofacial Surgery 53 (2015) 86–88
87
Fig. 1. Photomicrograph showing nodule of oral mucosa. There are sheets of histiocytes, extravasated erythrocytes, and haemosiderin deposits (haematoxylin and eosin, original magnification ×200).
Excisional biopsy examination under general anaesthesia showed no involvement of the underlying periosteum or bone. The excised sample was sent for histopathological examination. Macroscopically, it consisted of firm, cream, polypoidal tissue measuring 8 mm × 5 mm × 3 mm. Sectioning showed a uniform tan-coloured cut surface. Microscopically, the lamina propria and submucosa were stretched by a cellular lesion that comprised numerous histiocytes and occasional multinucleate giant cells embedded in a fibrovascular matrix. There was haemosiderin deposition, and some histiocytes had foamy cytoplasm. Immunohistochemical testing confirmed that the lesion contained no Langherans cells. Cytoplasmic immunoreaction to CD68 confirmed the type of histiocytosis, and a diagnosis of juvenile xanthogranuloma was made (Fig. 1). After review at the clinic, further questioning showed that the patient had a history of multiple skin lesions, particularly on her back. Examination revealed multiple asymptomatic red-yellow papules (Fig. 2), which, until then, had been diagnosed as molluscum contagiosum. After diagnosis of extracutaneous juvenile xanthogranuloma and dermatological findings of suspected juvenile xanthogranuloma, the patient was referred for assessment by a paediatrician and ophthalmologist. Unfortunately, she failed to attend these appointments and has not presented again.
Fig. 2. Photograph of multiple red-yellow papules on the patient’s back previously diagnosed as molloscum contagiosum before being identified as intraoral juvenile xanthogranuloma (published with the parent’s consent).
established with histopathological analysis and immunohistochemical staining. Whilst the index lesions are usually cutaneous, our case shows that the oral cavity can occasionally be involved, and the dentist may be the first to see it. Appropriate referral with subsequent investigation by relevant specialists is important to establish diagnosis and, where indicated, treat lesions to prevent potentially serious consequences. Conflict of interest We have no conflict of interest. Ethics statement/confirmation of patient permission
Discussion Consent was obtained for the publication of clinical images. Intraoral juvenile xanthogranuloma is extremely rare and we know of only 6 previous reports of palatal lesions. Given their rarity, identification and diagnosis may be difficult. Differential diagnoses (depending on site) may include dental abscess, fibroepithelial polyp, mucocoele, vascular malformation, lipoma, gingival cyst, peripheral giant cell granuloma, foreign body granuloma, pyogenic granuloma, and mucoepidermoid carcinoma.8 Definitive diagnosis is
References 1. Satow SJ, Zee S, Dawson KH, et al. Juvenile xanthogranuloma of the tongue. J Am Acad Dermatol 1995;33:376–9. 2. Flaitz C, Allen C, Neville B, et al. Juvenile xanthogranuloma of the oral cavity in children: a clinicopathologic study. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2002;94:345–52.
88
L. Collins et al. / British Journal of Oral and Maxillofacial Surgery 53 (2015) 86–88
3. Kjaerkeim A, Stokke T. Juvenile xanthogranuloma of the oral cavity. An electron microscopic study. Oral Surg Oral Med Oral Pathol 1974;38:414–25. 4. Webster SB, Reister HC, Harman Jr LE. Juvenile xanthogranuloma with extracutaneous lesions. A case report and review of the literature. Arch Dermatol 1966;93:71–6. 5. Favara BE, Feller AC, Pauli M, et al. Contemporary classification of histiocytic disorders. The WHO Committee on Histiocytic/Reticulum Cell
Proliferations. Reclassification Working Group of the Histiocyte Society. Med Pediatr Oncol 1997;29:157–66. 6. Cypel TK, Zuker RM. Juvenile xanthogranuloma: case report and review of the literature. Can J Plast Surg 2008;16:175–7. 7. Stover DG, Alapati S, Regueira O, et al. Treatment of juvenile xanthogranuloma. Pediatr Blood Cancer 2008;51:130–3. 8. Villa A, Mariani U, Villa F. Lingual juvenile xanthogranuloma in a woman: a case report. J Med Case Rep 2011;5:30.
