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Revista Odontológica Mexicana Facultad de Odontología

Vol. 16, No. 2 April-June 2012 pp 123-130 CASE REPORT

Oral manifestations of Langerhans cell (LHC): Review of scientifi c literature and case report Manifestaciones orales de la histiocitosis de células de Langerhans (HCL): Revisión de literatura y reporte de un caso

Martínez DSM,* Villagrán UJ,§ Ajqui RR,II Cervantes CK¶

ABSTRACT RESUMEN

Langerhans cell histiocytosis (LCH) is a dendritic cell disease. It is La histiocitosis de células de Langerhans (HCL) es una enfermedad classifi ed into two variations: localized or disseminated. Frequency de las células dendríticas. Se clasifi ca en formas localizadas o dise- per year is 1 in 200,000 children under 15 years of age. Aggres- minadas. La frecuencia por año es de 1 en 200,000 niños menores sive forms of the disease are mainly found in younger children. This de 15 años de edad. Las formas agresivas ocurren sobre todo en disease presents oral manifestations, which can sometimes be the niños pequeños. La enfermedad cursa con manifestaciones orales, fi rst expression of the condition. With relative frequency, jaws can pudiendo ser éstas, la primera manifestación de la enfermedad. result affected. Radiographic study of the condition shows well cir- Los maxilares pueden ser afectados con relativa frecuencia. En el cumscribed, osteolytic lesions imparting a pathognomomic «fl oating estudio radiográfi co se observan lesiones osteolíticas con bordes teeth» image. Patients can be affl icted with lymphadenopathy, fever, bien defi nidos dando una imagen de «dientes fl otantes», lo cual es irritability, anorexia, pallor, middle ear otitis and anemia. el signo patognomónico. Pueden aparecer linfadenopatías, fi ebre, The present article conducts a literature review and presents the irritabilidad, anorexia, palidez, otitis media y anemia. Se realiza una case of an 11 month old male infant . This case presents histiocy- revisión de literatura y además se presenta el caso de un lactante tosis oral expressions, incidence, clinical characteristics, diagnosis varón de once meses de edad, con el propósito de dar a conocer and treatment. las manifestaciones orales por las que cursa la histiocitosis, su inci- dencia, características clínicas, diagnóstico y tratamiento.

Key words: Langerhans cell histiocytosis, cervical lymphadenopathy, fl oating teeth, premature exfoliation. Palabras clave: Histiocitosis de células de Langerhans, linfoadenopatías cervicales, dientes fl otantes, exfoliación prematura.

INTRODUCTION Class III: Histiocytic malignancies.1,3,4 Nevertheless, Langerhans cell Histiocytosis is the Histiocytosis is the general term used to indicate currently preferred term. It is classifi ed into localized or some reticulo-endothelial system diseases. These disseminated form. conditions are the product of accumulation or primary proliferation of the mononuclear phagocytic system (MPS). Histiocytosis encompasses two types of im- mune cells: * DDS, Candidate to Pediatrics- Stomatology Specialty, Tamaulipas 1) Children´s Hospital (Hospital Infantil de Tamaulipas), Tamaulipas, 2) Dendritic cells1 www.medigraphic.org.mxMexico. § DDS. Maxillofacial Surgeon, Veterinarian, Public Health teacher, candidate to Master´s Degree in Medical Sciences, Attached HISTIOCYTOSIS CLASSIFICATION to and Head of the Stomatological Department, Tamaulipas Children`s Hospital, Tamaulipas, Mexico. II Histiocytosis, according to Society2, is Surgeon and accoucheur, specialized in general anatomy pa- thology and pediatric pathology. traditionally classifi ed into three main groups: ¶ DDS specialized in Stomatological Pediatrics, attached to the Class I: (Langerhans cell histiocytosis), formerly Stomatological Department of the Tamaulipas Children´s Hospi- called X histiocytosis tal, Tamaulipas, Mexico. Class II: Histiocytosis of other mononuclear phago- This article can be read in its full version in the following page: cytes different from Langerhans cells http://www.medigraphic.com/facultadodontologiaunam 124 Martínez DSM et al. Oral manifestations of Langerhans cell histiocytosis (LHC)

1. Localized form. Skin or self-involution expression, syndromes which encompass any abnormal increase bone affectation only, () nor- in the number of certain immune cells called histio- mally found in long bones.5 cytes.9 These cells include monocytes, dendritic cells 2. Disseminated form: Normally found in infants or and macrophages. It is a rare condition characterized children under 3 years of age, presenting a gen- by the accumulation of Langerhans dendritic cells in erally compromised physical condition. In these granulomatous lesions more frequently located in cases, the following characteristics are frequently bone and skin, but which can also be found in many found: lytic bone lesions compromising neighboring other organs.1 soft tissue areas, lung damage as well as repeated infections. This form has predilection for seborrheic PATHOLOGICAL ETIOLOGY areas (scalp and skinfolds).2 LCH etiology is as yet unknown. Recent research Recently, a new classifi cation has been proposed has established which particular cell generates this according to the producing cell: Langerhans cell his- disease. Ultra-structural and immune-histo-chemical tiocytosis is now part of the dendritic cells diseases similarities have been found between the proliferative group.1,2 cell of this disease and Langerhans cell, found nor- mally in epidermis and mucosa.4 LCH three «traditional» forms are: PATHOLOGICAL ANATOMY • Eosinophilic granuloma: It encompasses only pa- tients with isolated or multiple4 bone lesions, lacking The specifi c pathological fi nding is the presence of other expressions of the disease.1,6 It is a lytic lesion a dendritic cell (DC) within the components of the le- more frequently found in the skull, lower jaw, femur sion.1 or vertebrated bodies. It bears good diagnosis.1,7 Microscopically, the lesion appears as a yellowish • Hand-Shuller-Christian disease: A disseminated, granuloma. Cells similar to normal LC cells can be ob- chronic variation of this condition. It consists of mul- served. These are mononuclear cells, 15 to 25 μm in tiple bone lesions associated to diameter, with moderate cytoplasm, pale eosinophil, as well as exophthalmus (through retro-orbital gran- a centered or somewhat off-center nucleus, kidney uloma). Many patients can also present lymphade- shape, with a nuclear membrane cleft or fold appear- nopathy, dermatitis, splenomegaly or hepatomega- ing as a «coffee bean»,10 sometimes erythrophagocy- ly.1,4 Lesions found in the mouth are the following: tosis can equally be found11 (Figure 1). ulcers, edema, hyperplasia and gingival necrosis, When observed under electron microscope, Birbeck lesions of the maxillary bones and tooth loss, taste granules1,8,12,13 or «racket» bodies can be observed.10 disorders, halitosis, protracted wound healing. It affects children between 3 and 6 years of age. It INCIDENCE bears positive diagnosis. • Letterer-Siwe disease. This is an acute and dis- The National Pediatric Institute (Instituto Nacional seminated form of the disease, common in children de Pediatria) reports a total 224 patients treated in the under two years of age. It is characterized by fever, period 1970 - 1999. Out of this number, gingival le- rash, lymphadenopathy, hepatomegaly and sple- sions were identifi ed in 21 patients; 16 of these pa- nomegaly, osteolytic lesions, general skin eruption tients presented dental lesions.14 (petechiae, scaly papules, nodules and vesicles). Frequency found for this disease is 1 in 200,000 From a stomatological point of view, the following children under 15 years of age.1,10 It can appear at any traits can be found: ecchymosis,www.medigraphic.org.mx ulcers, gingivitis, age. Incidence peak is found to be between 1 and 3 periodontitis, bone disease and tooth loss. It bears years of age. Patient mean age is from 5 to 6 years. A severe prognosis.1,6,7 predilection for males has been observed. Aggressive forms occur mainly in young children.1 BACKGROUND CLINICAL PICTURE LANGERHANS CELL HISTIOCYTOSIS (LCH) Clinical picture depends on the disease and com- Encompasses disorders traditionally called X histio- promised tissue or organ.12 It appears in varied forms, cytosis.8 This is a general name allotted to a group of since any organ or system can be compromised. Nev- Revista Odontológica Mexicana 2012;16 (2): 123-130 125

zone,8,16 causing erosion of the lamina dura. This ero- sion can elicit a mandibular ramus height decrease.8 There is pain and gum inflammation (swelling). Upon palpation, a tumefaction is found, which corre- a sponds to Langerhans cell accumulation. This elicits oral ulceration with the possibility of tooth loss.16 Radiographically, teeth are observed as «fl oating teeth».1,3,6,8,11,16 A typical lesion presents lytic shape, as well as well circumscribed borders since, especially in b the early stages of the disease, they are surrounded by a radiolucid1,8,16 granulomatous material which dis- places forming teeth buds.1,16 Oral lesions can be the fi rst and only expression of the disease; This is the case in 35% of all cases.16 It has been reported that 30% of LCH cases with oral or peri- oral compromise, present cervical lymphadenopathies.6,8 Premature loss of deciduous teeth associated with bone loss is a clear sign of histiocytosis. Anterior teeth Figure 1. Left inguinal lymphatic ganglion imprint showing involvement is infrequent, and indicative of a negative «coffee bean» appearance (a) as well as erythrophagocy- prognosis.16 tosis (b) harvested from present case patient. Sample dyed In some cases, there is loss of deciduous teeth with with Hematoxylin and Eosin. precocious replacement and early eruption of permanent teeth. This is frequently the consequence of prepubes- cent periodontal disease associated to Actinobacillus ac- ertheless, there are preferential locations.1 In dissemi- tinomycetencomitans and is conductive to tooth mobility nated disease instances, many organs are compro- and loss when the patient is about three years old.8 mised, and several general expressions of the disease Skin lesions: Skin lesions are observed in over one can be present, such as fever, anorexia, weight loss, third of children affl icted with LCH. They can be the fi rst anemia, hemorrhagic manifestations (petechiae on the and sometimes only symptom as is the case in oral trunk) asthenia and irritability.12 lesions. Typical lesions appear diffuse, papular and ex- foliative, similar to seborrheic dermatitis lesions. They Bone lesions: Bone lesions are present in most are located in the scalp, folds, and perineal region. patients with localized disease form and are very fre- Lymph nodes and thymus: The cervical region is quent in patients affl icted with the disseminated dis- the most frequent location. ease variation.12 Single bone eosinophilic granuloma Liver: In cases of disseminated disease hepato- is the most frequent and benign clinical expression. megaly is frequent.1 Hematopoietic active bones are involved, mainly those Spleen: In cases of disseminated disease spleno- pertaining to the skull, followed by femur, lower jaw, megaly is present in 5% of cases. pelvis and vertebrae. Lung: 40 to 50% of the children affl icted with dissem- According to their location they give rise to typical inated disease present pulmonary affection, although in clinical pictures of varied degree.1 It is common to fi nd the localized variety it is rarely found in patients under lytic bone lesions with involvement of neighboring soft 15 years of age.1 These cases are associated with fe- tissue, which give rise to a geographical skull; in skull ver, dyspnoea and weight loss. From the evolutionary base bones they give rise to exophtalmoswww.medigraphic.org.mx and diabe- standpoint, sometimes cysts or bullae appear which, tes insipidus; in mastoids to exudation and otorrhea, upon rupture, produce pneumothorax. In the final in vertebrae to vertebral crush. In cases where lysis phase, emphysema and fi brosis can be observed. occurs in the jaw, there will be a «fl oating teeth» radio- Bone marrow: Pancytopenia is common in the dis- graphic image.1,4,7,8,12,15,16 seminated variety of the disease. Patients with severe hematologic affectation bear worse diagnosis, espe- Lesions in the mouth: In nearly half the cases cially in cases where thrombocytopenia is present. there are lesions in gums and teeth.3 The disease nor- Central nervous system: Few patients present mally initiates in the periapical region of the tooth. The alteration of this system. Some signs are intracranial posterior area of the lower jaw is the most affected hypertension, trembling, dysarthria, hyperreflexia, 126 Martínez DSM et al. Oral manifestations of Langerhans cell histiocytosis (LHC)

hemiplegia, quadriplegia, dysphagia with or without 2. Number of affected teeth intellectual defi cit.12 3. Organic dysfunction degree6 The characteristic disorder in this condition is dia- betes insipidus due to hypothalamus-pituitary affecta- Children under two years of age at the moment tion.1 when diagnosis is emitted present a higher mortality rate than those older than this age. DIAGNOSIS The development of untreated disease is extremely variable. LHC can spontaneously resolve, or it can dis- In cases when LCH is not borne in mind, variety of seminate, compromising visceral organs, with a result- symptoms present and its rare occurrence might ren- ing fatal outcome.1 der diagnosis diffi cult. Clinical criteria might suggest When deciding upon disease extension and sever- presence of the disease, but histological confi rmation ity, it must be borne in mind that the important fact is is required in all cases.1 Final diagnosis is attained not the organ` s lesion in itself, but rather the presence with the help of lesion biopsy,16 (incisional, excisional or absence of dysfunction in that organ. etc). It is compulsory to observe with electron micro- When viscera are compromised, the prognosis is scope in search of intracellular presence of Birbeck dimmer.8 There is linear correlation between mortality granules1,4,8,12 (rod shaped cytoplasm structures) or and the amount of affected organs. In cases when 1 ascertain positivity in the immune-histochemical test of or 2 organs are compromised, mortality reaches 10%. CD1 markers (antigen-presenting immunoglobulins) in When three or more organs are compromised, mortal- the membranes of cells found in the lesions.1,2 In cases ity reaches 90% of cases.12 when the whole system is compromised, it is recom- Patients with bone located disease bear excellent mended to perform simple radiological study, comput- prognosis. In these cases mortality is almost nonex- erized axial tomography (CAT) or magnetic resonance istent.12 (MR) to confi rm presence of bone and lung lesions.2 As a consequence of bone affectation and depend- ing on the lesion location, orthopedic and aesthetic al- DIFFERENTIAL DIAGNOSIS terations can be present.1,5

Bone lesions can present radiologic image similar TREATMENT to that of bone tumors and osteomyelitis. Skin lesions are many times mistaken for seborrheic dermatitis The International Hystiocyte Society internationally cases. When the whole system is compromised, with classifi es treatment into: Low, Intermediate or High ac- presence of hepatomegaly and splenectomegaly, cas- cording to risk of patients. es are often mistaken for and lymphoma.1 The following methods are included: The classical sign of LCH found in the jaws is the premature loss of teeth. In these situations, differential 1. Surgical intervention diagnosis must include juvenile or diabetic periodonti- 2. Radiotherapy tis, hypophosphatasia, cyclic , agranulocy- 3. Chemotherapy tosis and primary or metastatic malignant tumors. Vi- tality in the affected teeth pulp precludes the possibility There is general consensus stating that surgical of apical granuloma or cysts. curettage is the treatment of choice for isolated bone Central and solitary radiolucent lesions in the jaws lesions, and radio- and chemotherapy are reserved for must be differentiated from odontogenic cysts and tu- lesions that cannot be accessed through surgery. It is mors. If a certain number of lesions are found, they of utmost importance to carefully select affected teeth can suggest a case of multiplewww.medigraphic.org.mx myeloma, neverthe- to be extracted, since not all affected teeth must be less, this condition is normally found in older patients.4 treated in such an aggressive fashion. Only teeth pre- In all cases, diagnosis will be established through his- senting severe mobility and extensive lytic lesions are tological evidence. candidates for extraction.8

PROGNOSIS CASE REPORT

Prognosis is contingent upon three factors: 11 month old male patient (V.M.K.A.) referred to the emergency clinic of the Tamaulipas Children`s Hos- 1. Age of patient when condition begins pital (Hospital Infantil de Tamaulipas) due to a sus- Revista Odontológica Mexicana 2012;16 (2): 123-130 127

pected peri-tonsillar abscess. The patient presented a lesion in the mouth which warranted consultation with the Pediatrics- Stomatological Department. The patient came from San Fernando, Tamaulipas, where his mother took him to the community health center. Medical reports of the aforementioned health center inform that the patient began showing symp- toms one month before consultation. The patient presented hyaline rhinorrhea, non-assessed fever, volume increase in the submandibular and bilateral parotid region. The lesion was indurated and pain- ful to palpation, emitted foul odor, there was a puru- lent blood discharge from the right ear, with epistaxis equally present. The mother informed of having ad- ministered several analgesic and antibiotic drugs to treat her son`s condition. Upon arriving at the ER of the Health Center, the patient was found to be irritable, with 38ºC body tem- perature. CBC was taken which showed the follow- ing: Hemoglobin value of 8 mg/dL, leukocytes: 17,800 mmc, neutrophils :68.7%, lymphocytes 24.5% plate- lets: 297,000 mmc. The patient `s history was as follows: 24 year old mother affl icted with hypothyroidism, 6 year old male sibling suffering from asthmatic bronchitis, maternal grandmother afflicted with type II diabetes mellitus. The mother informed that in the family there had been three deaths of maternal grand-uncles, during child- hood, due to neck lymphadenopathy.

CLINICAL EXAMINATION Figure 2. Upper photograph shows scalp erythematous, The patient`s apparent age matched chronological scaly lesions. Lower photographs shows cervical lymphade- age. nopathy. Physical exploration showed erythematous, scaly skin lesions in the scalp, 3 cm3 lymphadenopathy in parotid and cervical regions. Lesion was indurated and ground oral pouch and lingual portion. The patient also painful to palpation;. Clinical observation showed ab- presented halitosis (Figure 3). sence of heat or blush, bloody and purulent discharge off the right ear, epistaxis and generalized foul smell of RADIOLOGICAL EXAMINATION the patient (Figure 2). Extraorally, lips appeared dehydrated. Intraorally no A lower occlusal x-ray was taken. In it, a well cir- pathological clinical manifestations were observed in cumscribed radiolucent area was observed at the site the tongue, fl oor of the mouth,www.medigraphic.org.mx hard and soft palate, of teeth number 7.2 and 7.3. There was no evidence anterior pillars, mucosa and retromolar area in cheeks. of trabecular bone or root formation in the aforemen- The following was equally observed: hypertrophic ton- tioned teeth, which gave them a «fl oating teeth» ap- sils, active eruption of teeth number 5.2, 5.1, 6.1, 6.2, pearance (Figure 4). 7.2, 7.1, 8.1, 8.2. Teeth number 7.1 and 7.2 respec- Due to the general systemic endangerment of the tively presented 1st and 3rd degree mobility. patient, he was admitted so as to perform biopsies and At tooth 7.2 location where tooth 7.3 was to erupt, initiate treatment. a 3 cm3 purplish color volume increase was observed. Clinicians at the Stomatological Department recom- In that region, the mass presented necrotic areas and mended cleansing with bicarbonate mixed in lukewarm was of a friable nature, where it extended to the back- water after meals at the site of the lesion, as well as 128 Martínez DSM et al. Oral manifestations of Langerhans cell histiocytosis (LHC)

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Figure 4. Occlusal x-ray showing lytic lesion at teeth 7.2 and 7.3 level.

In the operating theater, resection was made of the left inguinal ganglion (lymph). An incisional biopsy was taken of the oral mucosa lesion to perform hystopatho- logical analysis. The diagnosis emitted was Langer- hans cell histiocytosis with inguinal lymph and oral mucosa compromise (Figure 5). Once the diagnosis was established, chemotherapy treatment was initiated with 3.4 mg IV (6.5 mg 2 twice a day and 26.5 mg IV (50 mg twice a day), during 2 cycles. The patient experienced a general health improve- ment. The following was observed: volume decrease Figure 3. Upper picture shows lesion´s clinical appearance in cervical lymphadenopathy, lesser ear and nose dis- on exploration day. Lower picture shows lesion appearance charge, in the mouth the lesion changed color: from after 17 days. purplish red it turned pink, extending still to the inner- most location oral pouch and lingual portion. Necrotic areas had disappeared (Figure 6). in the whole mouth as temporary treatment until fi nal Tooth # 7.3 was actively erupting. This tooth pre- diagnosis was emitted. sented 3rd degree mobility. Even teeth # 7.1 and 7.2 During the patient´s stay at the hospital, labora- presented mobility. tory analyses were undertaken. The following values During the period when chemotherapy was admin- were observed: severe anemia values HB 7 mg/dL, istered, the patient was affl icted with Oral Candidiasis. Hematocrit 21.1%, Leukocytes 12,700 mmc, Lympho- This condition could be observed in the cheeks muco- cytes 42%, Neutrophils 55%, eosinophils 3%, plate- sa and hard palate. Lesions had a whitish appearance lets 40,000 mmc, Prothrombin time: 14.5 sec. Tissue which, when rubbed, came loose leaving an erythe- Thromboplastin Partial Time : 24.8 sec. matosus area. In this case, Daktarin gel® was admin- A treble antibiotic schemewww.medigraphic.org.mx was initiated with the istered as treatment. During this period the patient following drugs: Ceftrixone, 365 mg, IV; Amikacin, contracted chickenpox, which in the mouth manifested 75 mg IV; Metronidazole 100 mg IV. The following itself as ulcers in the pouch mucosa, at the level of drugs were also administered: diclofenac drops, tooth # 8.4. This situation was treated with Kaolin and 100 mg every 8 hours, ranitidine, 10 mg IV and so- Pectin, as well as Benadryl® mixed in equal parts, to dium metamizole (sodium suphonate of aminopyri- be applied in ulcerated areas. dine)180 mg IV. The patient was later afflicted with the following: After consultation with the genetics department, he- abdominal distension, tachycardia and respiratory dif- reditary connection with the present state of the pa- ficulties. Three times he required transfusions. The tient was precluded. patient s clinical picture became more severe up to his Revista Odontológica Mexicana 2012;16 (2): 123-130 129

Figure 6. Clinical changes observed after chemotherapy ad- Figure 5. Sample harvesting and oral mucosa incisional bi- ministration. opsy cut with Pass Tincture. 40x

demise after 41 days in hospital. The causes of death were skin (scalp) cervical and inguinal lymph nodes as were deemed to be congestive cardiac insuffi ciency, well as the oral mucosa. pancytopenia, Langerhan s cell histiocytosis, probable INP study states that the most frequent diagnostic intracranial hemorrhage and chickenpox. anatomical site was bone, followed by (ganglion), and skin assuming the third place. In the DISCUSSION present case, diagnosis was conducted in the inguinal www.medigraphic.org.mxganglion and oral mucosa. Clinical characteristics of this patient were com- Treatment of the present case concurred with treat- pared with results of a study conducted in 2002 by the ment reported in the INP study. National Institute of Pediatrics (INP), (Instituto Nacio- Hepatic and pulmonary dysfunction are associated nal de Pediatria).14 The following facts were observed: with high mortality. The patient in our study presented The study mentions this condition affl icts children those characteristics before his demise. under one year of age. This was confi rmed in the pres- The aforementioned INP study states that all pa- ent case. tients died in a time span shorter than 20 months, and The present and the aforementioned study concur this was directly associated to the condition. 100% of in the manner of disease presentation: affected organs patients were under two years of age.14 Our case con- 130 Martínez DSM et al. Oral manifestations of Langerhans cell histiocytosis (LHC)

curs in age of patient at time of death and evolution 3. Kanadani CM, Borato VM, Oliveira V, Dias RD, Resende SC. time of the disease, but differs in the sense that our Langehans cell histiocytosis: a 16-years experience. J. de Pedia- tria. Sociedade Brasileira de Pediatria 2007; 83 (1): 79-86. patient additionally contracted smallpox. 4. Regezi JA, Sciubba JJ. Tumores benignos odontológicos. En: Another relevant clinical manifestation when com- Stewart JC, editor. Patología Bucal. 2a ed. México: McGraw-Hill paring with the INP article, is the fact that the patient Interamericana; 1995: 413-535. presented a lesion in the mouth, in which the following 5. Gómez LS, Padrón SN, González HF. Histiocitosis de células de Langerhans en un lactante menor: reporte de un caso. Rev clinical characteristics were observed: abnormal mo- Hosp. Jua Mex 2005; 72 (3): 131-4. bility of affected teeth, premature eruption of tooth # 6. Pinkham JR, Casamassimo P, Fields H, McTigue D, Nowak A. 7.3, volume increase in this area, which presented an Enfermedades y manifestaciones bucales de padecimientos unclean, friable appearance with necrotic areas. Ac- sistémicos. En: Sonis A, editor. Odontología pediátrica. 3a ed. México: McGraw-Hill interamericana; 2001: 71-90. cording to statistics presented in the INP article, this 7. Laskaris G. Enfermedades metabólicas. Patología de la cavidad type of lesion is infrequent. bucal en niños y adolescentes. 1a ed. Colombia: América; 2001: 218-233. CONCLUSIONS 8. Hernández JM, Boj QJ, Gallego MS. Manifestaciones orales de la histiocitosis de células de Langerhans. A propósito del caso de un niño de dos años. Med Oral [serial online] 2003 Dic-May; Langerhan`s cells Histiocytosis is a condition which 8:19-25 [7 pantallas]. Disponible en: URL: http://www.medicina- can present different clinical pictures, according to the oral.com/medoralfree/v8i1/medoralv8i1p19.pdf. extension and severity of organic endangerment . This 9. Weiss G. Cáncer pediátrico. En: Parmley R, editores. Oncología clínica. 1a ed. México: Manual Moderno;1997.p.383-401. renders diagnosis diffi cult. 10. Asociación Española contra la histiocitosis (ACHE). Enfermedad The patient` s case here described presented the Histiocitosis (HCL). España: Asociación; 2008. Disponible en: same clinical manifestations as those described in INP URL: http://www.histiocitosis.org study. 11. Sierra SY, Sulbaran J, Rosas UA. Histiocitosis de células de Langerhans (Histiocitosis X): Características clínicas y Patológi- It is of paramount importance for the dentist to be cas en 12 pacientes. Rev Latinoamericana Patología Patología more cognizant on LCH, since oral lesions can well 1989; 27 (1): 59-67. be the fi rst manifestation of the disease, and in many 12. Svarch E, Arteaga R, Pavón MV, González OA. Las histioci- cases, the oral cavity can be the only affected site. tosis. Rev Cubana Hematol Inmunol Hemoter [serial online] 2001 Sep-Dic [citado 7 feb 2008];17(3): [15 antallas]. Dis- Due to the scarcity of cases, this condition is often ponible en: URL: http://scielo,sld.cu/scielo.php?pid=S0864- ill-treated. Accurate diagnosis is based upon histologi- 02892001000300001&script=sci_arttext. cal, radiographic and clinical examination. Final diag- 13. Orkin M, Maiboch H, Dahl MV. Linfoma cutáneo, leucemia y nosis will be based upon histological evidence. enfermedades relacionadas. En: Zackheim H, editor. Derma- tología. 1a ed. México: Manual Moderno;1994: 651-671. When devising a treatment plan, it is of the utmost 14. Zapata TM, Leal LC, Rodríguez JR, Rivera LR. Histiocitosis de importance to take into account the patient´s general células de Langerhans: experiencia clínica y terapéutica en ni- state of health, since it could prove to be a limiting ños en el Instituto Nacional de Pediatría en 30 años. Bol Med factor. Hosp Infant Mex 2003; 60 (1): 70-8. 15. Nazco RC, González DM, Bello RR, Torrens NR. Granulomato- The quality of life for Langerhans cells histiocytosis sis de células de Langerhans. Presentación de un caso. Rev Cu- patients depends on the onset of the condition, gen- bana Estomatol [serial online] 1998 Sep-Dic [citado 7 feb 2008]; eral damage incurred, and administered treatment. 35(3):[4 pantallas]. Disponible en URL: http://scielo.sld.cu/scielo. This case is an example of the disease aggressive- php?pid=S0034-75071998000300010&script=sci_arttext. 16. Boj JR, García BC, Mendoza A. Odontología en pacientes ness and of the importance of early diagnosis to pro- pediátricos con necesidades especiales (I): patología sistémica. vide adequate treatment. En: Bolaños C, Manrique M, Briones L, editores. Odontopedi- atría. 2a ed. España: Masson; 2005: 455-465. REFERENCES

1. Madero LL, Muñoz VA. Histiocitosis.www.medigraphic.org.mx En: Sastre UA, Cervera BA, editores. Hematología y Oncológica Pediátrica, 2a ed. Barce- lona: Ergon; 2005: 693-712. 2. Ferrando BJ, Cruz MO. Histiocitosis. Asociación Española de Mailing Address: Pediatría [serial online] 2007. Disponible en: URL: www.aeped. Martinez DSM es/protocolos/dermatologia/dos/1. E-mail: [email protected]