RESUMO
BACKGROUND: The "C group" of the histiocytic disorders is characterized by non-Langerhans-cell histiocytic lesions in the skin, mucosal surfaces, or both, out of which Juvenile xanthogranuloma (JXG) is the most common typically affecting the skin. The eye is the most common extra-cutaneous site of JXG., we aim at providing our clinical and histopathological experience with this group of diseases including the adult-onset xanthogranuloma (AXG). METHODS: This is a retrospective cohort study of all patients with the tissue diagnosis of ocular and periocular cutaneous and mucocutaneous non-LCH disorders who presented to us over a period of 25 years (January 1993 to December 2018). RESULTS: Twenty patients were diagnosed as "Group C" disease with an age range of 2 months-60.9 years. Eleven patients were females (55%) and nine were males (45%). The involvement was mostly unilateral in 80.9%. All cases fell into the xanthogranuloma family with 11 JXG patients, 8 AXG patients of skin and ocular surface, and one patient with solitary reticulohistiocytoma (SRH). The clinical site of involvement in JXG was primarily in the eyelid in 5 patients (45%), ocular surface lesions in 2 (18%), iris in 2 (18%), choroidal and bilateral orbital lesions in 1 patient each (9%). The group of AXG, presented equally with eyelid lesions in 4/8 and ocular surface lesions in 4/8. The non-Langerhans' histiocytic infiltrate showed supportive immunohistochemical staining properties (reactive to CD68 marker and negative to S-100 and langerin markers). CONCLUSION: Among the rare histiocytic disorders, xanthogranulomatosis is the commonest and has wide clinical manifestations. Accurate diagnosis needs to be supported by typical histopathological findings. JXG was the commonest in our study with relatively older mean age at presentation and frequent eyelid rather than iris involvement. AXG is often confused with xanthelasma when involving the eyelids with corneal limbal involvement is relatively frequent.
Assuntos
Neoplasias Cutâneas , Xantogranuloma Juvenil , Masculino , Adulto , Feminino , Humanos , Lactente , Estudos Retrospectivos , Xantogranuloma Juvenil/diagnóstico , Xantogranuloma Juvenil/metabolismo , Xantogranuloma Juvenil/patologia , Face , IrisRESUMO
Juvenile xanthogranuloma is the most frequent form of non-Langerhans cell histiocytosis in children. Clinically, it presents as well defined, yellowish papules that are typically located on the head, neck, upper trunk, and proximal region of the extremities. Although solitary lesions are the most common presentation, few cases of multiple juvenile xanthogranuloma have been described, more frequently associated with extracutaneous involvement. We report a 2-month-old girl with 22 cutaneous papules, clinically and histologically compatible with juvenile xanthogranulomas. Screening of visceral involvement was performed with no evidence of systemic disease. Identifying high-risk factors of systemic disease in patients with multiple juvenile xanthogranuloma is essential to perform an appropriate management of this entity.
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Xantogranuloma Juvenil , Humanos , Xantogranuloma Juvenil/patologia , Xantogranuloma Juvenil/diagnóstico , Feminino , LactenteRESUMO
Juvenile xanthogranuloma (JXG) is the most common non-Langerhans cell histiocytic disorder. It can rarely be associated with systemic involvement. There is a paucity of literature on JXG in Asian children. We aim to describe the epidemiology, clinical features, systemic associations, histological features and outcome of a cohort of Asian children with JXG, and review the literature on the condition. We retrospectively reviewed the demographic, clinical and histological data of patients less than 16 years of age, diagnosed with JXG at our tertiary pediatric hospital between January 2002 and April 2019. A total of 147 children with JXG were identified, with a slight male preponderance of 53.1%. The median age of the onset was 15.5 months, with 69.4% presenting before 2 years of age. There was no racial predilection. The most frequently involved site was the head and neck region (44.2%). The majority of patients (76.2%) presented with a solitary lesion. Spontaneous resolution was documented in 57.7% of our patients with mean duration to resolution of 18.8 months. The proportion and speed of resolution did not differ in children with single or multiple lesions. No ophthalmologic complications were detected in our study cohort. JXG in children is generally limited to the skin and is rarely associated with systemic involvement, including the eye. Unless clinically indicated, the results from our study does not support routine screening for juvenile myelomonocytic leukemia, eye or systemic complications, even in the setting of multiple cutaneous JXGs.
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Xantogranuloma Juvenil , Povo Asiático , Criança , Histiócitos/patologia , Humanos , Lactente , Masculino , Estudos Retrospectivos , Pele/patologia , Xantogranuloma Juvenil/complicações , Xantogranuloma Juvenil/diagnóstico , Xantogranuloma Juvenil/epidemiologiaRESUMO
Juvenile xanthogranuloma (JXG) is a rare, non-Langerhans cell histiocytosis. It is usually a benign and self-limiting condition. The most common sites are skin and soft tissue. Pancreatic involvement is extremely rare. We present an unusual case of a 13-month-old female child with JXG of the pancreas and elevated cancer antigen 19-9. JXG should always be considered as a differential diagnosis for pediatric patients presenting with a pancreatic mass, solid and/or cystic in nature. Therefore, avoiding unnecessary invasive diagnostic procedures.
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Neoplasias Pancreáticas , Xantogranuloma Juvenil , Criança , Diagnóstico Diferencial , Feminino , Humanos , Lactente , Pâncreas , Neoplasias Pancreáticas/diagnóstico , Pele , Xantogranuloma Juvenil/diagnósticoRESUMO
PURPOSE: Solitary eyelid juvenile xanthogranuloma (JXG) is extremely rare, and there is limited literature on its clinical features and treatment outcomes. Here, we present a case series and comprehensive review of the literature on patients with isolated eyelid JXG. METHODS: We systematically extracted data from our institution's records of isolated eyelid JXG cases and conducted a search for additional cases from the literature utilising the PubMed, Wanfang, and Chinese National Knowledge Infrastructure (CNKI) databases. Patients with JXG were analysed with respect to age, sex, clinical presentation, therapy, and outcome. Group comparisons were performed. RESULTS: Thirty-two patients (including 13 at our institution and 19 from prior publications) were identified. The median age at first presentation was higher in current patients than in the patients from the published cases (median 9 years, range 1.2 to 47.0 years; median 2 years, range 0.5 months to 46.0 years, respectively, P = 0.014). Of the patients who had known characteristics, no significant differences were observed between the two groups in terms of sex, affected eye, eyelid site, type of cutaneous involvement, or duration of symptoms (each P > 0.05). Seventeen (54.8%) patients were male. The most common lesion location was the upper eyelid (n = 10, 62.5%). Twenty-four (75.0%) cutaneous lesions had full-thickness skin involvement; 8 (25.0%) subcutaneous masses had a chalazion-like appearance. Histologically, the JXG masses were characterised by Touton giant cells with inflammatory cells. Additionally, there was no significant difference in treatment modalities between the two groups (P = 0.072), and 24 (75.0%) patients underwent surgical excision. The overall recurrence-free survival was 3.6 to 52.8 (median 27.0) months in the current patients. For published cases with available follow-up information, there was no recurrence in 10 cases and improvement in 1 case, with a median follow-up of 9.5 months. CONCLUSION: Solitary eyelid JXG is a rare clinical entity and should be included in the differential diagnosis of eyelid mass lesions in patients of all age groups. Surgical excision is often selected for efficient treatment and to obtain an excisional biopsy.
Assuntos
Xantogranuloma Juvenil , Biópsia , Pré-Escolar , Diagnóstico Diferencial , Pálpebras/patologia , Pálpebras/cirurgia , Feminino , Humanos , Lactente , Masculino , Resultado do Tratamento , Xantogranuloma Juvenil/diagnóstico , Xantogranuloma Juvenil/patologia , Xantogranuloma Juvenil/cirurgiaRESUMO
Non-Langerhans cell histiocytosis is a collective term encompassing a vast group of benign proliferative disorders of histiocytes, macrophages and dendritic cells that do not meet the criteria of Langerhans cell histiocytosis. We describe a case of juvenile xanthogranuloma with an unusual clustered distribution.
Assuntos
Xantogranuloma Juvenil/patologia , Dermoscopia , Diagnóstico Diferencial , Feminino , Humanos , Lactente , Tórax/patologia , Xantogranuloma Juvenil/diagnósticoRESUMO
BACKGROUND: Juvenile xanthogranuloma (JXG) is the most common type of non-Langerhans cell histiocytosis whose cell of origin, etiology and pathogenesis are not fully understood. We aimed to provide an update on histopathologic and immunophenotypic profile of this well-characterized entity whose relationship to the other histiocytoses has received renewed attention in light of recent molecular genetic studies. MATERIALS AND METHODS: A retrospective review of all the cases with the pathologic diagnosis of "xanthogranuloma" was performed on our archives from 1989 to 2019. RESULTS: A total of 525 patients with 547 lesions diagnosed as JXG were identified with the median age of 4.5 years, a male predominance (M:F ratio 1.3:1) and a predilection for the head and neck region (40.8%). Cutaneous lesions comprised 76.8% cases and another 15.7% presented within soft tissues. The most common non-soft tissue, extracutaneous lesions included the brain (2.6%), and lungs (1.8%). Three basic histopathologic patterns were identified: early classic (EJXG) (14.2%), classic (CJXG) (45.3%), and transitional JXG (TJXG) (40.5%). Multinucleated giant cells, either Touton or non-Touton, were most frequently present in CJXG followed by TJXG. Mitosis was rare (<1/10 high-power field) among different patterns. There was an association among the patterns and lymphocytic infiltrates (P = 0.036), and presence of Touton or non-Touton giant cells (P < 0.001 for both) but not for mitotic count (P = 0.105) or eosinophilic infiltrates (P = 0.465). Additionally, there was a correlation between age groups and presence of non-Touton giant cells (P = 0.012) but not for Touton cells (P = 0.127). We have demonstrated that immunophenotypic expression of the lesion was not associated with age at diagnosis nor morphologic pattern: factor XIIIa 192/204 (94.1%), CD11c 75/77 (97.4%), CD4 82/84 (97.6%), CD68 200/201 (99.5%), CD163 15/15 (100%), CD1a 1/110 (0.9%), S-100 48/152 (31.6%), CD31 15/21 (71.4%), and vimentin 104/105 (99.0%). CONCLUSION: We have documented in a substantial series of cases of JXG that there is a correlation between one of the three basic histopathologic patterns with age at diagnosis, but with a consistent immunophenotype among the three patterns. Considering sensitivity and specificity rates, we suggest that a combination of CD11c, CD4, CD1a and either CD163 (preferred) or CD68 stains provides more specific diagnostic yield in the differentiation of JXG from other histiocytic disorders. JXG is also discussed in terms of its relationship and distinction from other similar histiocytic disorders in the context of MAPK/ERK pathway mutations.
Assuntos
Neoplasias Hematológicas , Neoplasias Cutâneas , Xantogranuloma Juvenil , Adulto , Pré-Escolar , Feminino , Histiócitos/patologia , Humanos , Masculino , Estudos Retrospectivos , Proteínas S100 , Xantogranuloma Juvenil/complicações , Xantogranuloma Juvenil/diagnóstico , Xantogranuloma Juvenil/patologiaRESUMO
OBJECTIVE: To explore the genetic basis for a child with café-au-lait macules and juvenile xanthogranuloma. METHODS: Clinical data and peripheral blood samples of the patient and her family members were collected and subjected to targeted capture and high-throughput sequencing. Candidate variant was verified by Sanger sequencing. RESULTS: A deletional variant in exon 23 of the NF1 gene was detected in the proband. Sanger sequencing has verified it as a de novo variant, which was highly correlated with the clinical manifestations of the patient and her mother. The diagnosis of neurofibromatosis 1 (NF1) was established. The variant was unreported previously. CONCLUSION: Targeted capture and next-generation sequencing combined with Sanger sequencing can facilitate early diagnosis of NF1 and provide a basis for the clinical treatment, genetic counseling and prenatal diagnosis.
Assuntos
Neurofibromatose 1 , Xantogranuloma Juvenil , Criança , Feminino , Humanos , Manchas Café com Leite/diagnóstico , Manchas Café com Leite/genética , Genes da Neurofibromatose 1 , Neurofibromatose 1/diagnóstico , Neurofibromatose 1/genética , Xantogranuloma Juvenil/diagnóstico , Xantogranuloma Juvenil/genéticaRESUMO
BACKGROUND: Juvenile Xanthogranuloma (JXG) is a non-hereditary, self-limiting disease which is usually presented in infancy or early childhood and in males over females. CASE PRESENTATION: We report a rare case of oral Juvenile Xanthogranuloma with recurrent progressive gingival hyperplasia and concomitant presentation of osteolysis in a 21-year-old adult male with no significant medical history. Patient presented with generalized gingival hyperplasia, osteolysis of the maxilla and mandible, and a round, firm, nodular mass with clear circumference on the left shoulder. Results of gingival tissue biopsy, karyotype, bone marrow biopsy and immunohistochemistry were suggestive of a diagnosis of Juvenile Xanthogranuloma with no association to hematologic malignancy. Unfortunately, patient declined treatment and elected to be transferred back to local hospital for future evaluation. CONCLUSIONS: Juvenile Xanthogranuloma in adults can have atypical manifestations including generalized gingival hyperplasia and osteolysis of the maxilla and mandible. It should be differentiated between Langerhans cell histiocytosis, Papillon-Lefevre Syndrome, and Pyogenic Granulomas. Despite uncommon incidence, it should be included in differential diagnoses in cases of similar clinical presentations.
Assuntos
Hiperplasia Gengival , Histiocitose de Células de Langerhans , Osteólise , Xantogranuloma Juvenil , Feminino , Humanos , Adulto , Masculino , Pré-Escolar , Adulto Jovem , Xantogranuloma Juvenil/diagnóstico , Xantogranuloma Juvenil/patologia , Osteólise/etiologia , Hiperplasia Gengival/diagnóstico , Hiperplasia Gengival/etiologia , Histiocitose de Células de Langerhans/diagnóstico , Imuno-HistoquímicaRESUMO
A 4-month-old boy was admitted to the hospital after his parents noticed fog in his right eye for 27 days. Physical examination revealed multiple light brown macules on the skin. The intraocular pressure (IOP) of the right eye was 34.4 mmHg (1 mmHg=0.133kPa) and the corneal was enlarged and edema while the aqueous humor was cloudy with blood in the right eye. Yellow peripheral anterior synechia was seen on the temporal and inferior iris. Ultrasound biomicroscopy (UBM) showed extensive adhesion closure of the anterior chamber angle and the local thicken iris with the nodule. No obvious abnormality was observed in the left eye. It was confirmed as juvenile xanthogranuloma by skin lesion biopsy. IOP was normal after systemic and local therapy with corticosteroids and local IOP lowering medication. Follow up 35 months, IOP is normal without medication.
Assuntos
Glaucoma de Ângulo Fechado , Glaucoma , Xantogranuloma Juvenil , Criança , Glaucoma/complicações , Glaucoma de Ângulo Fechado/diagnóstico , Humanos , Lactente , Pressão Intraocular , Iris , Masculino , Tonometria Ocular , Xantogranuloma Juvenil/complicações , Xantogranuloma Juvenil/diagnósticoRESUMO
The Shapiro xanthogranuloma is a histopathologic form of xanthogranuloma that shows closely packed monomorphous cells, which can extend into the subcutaneous fat; it usually lacks routine diagnostic features of xanthogranuloma. Herein we describe two cases of Shapiro xanthogranuloma occurring in a neonate and in an infant, which were initially thought to be hematologic malignancies. One patient's presentation as a "blueberry muffin baby" added to the diagnostic confusion. Pediatric dermatologists, dermatologists, and dermatopathologists need to be aware of the Shapiro xanthogranuloma and its clinicopathologic features to avoid misdiagnosis of a hematopoietic malignancy in neonates and infants.
Assuntos
Granuloma/diagnóstico , Neoplasias Hematológicas/diagnóstico , Leucemia/diagnóstico , Dermatopatias/patologia , Xantogranuloma Juvenil/diagnóstico , Xantomatose/diagnóstico , Conscientização , Dermatologistas , Erros de Diagnóstico , Feminino , Granuloma/patologia , Neoplasias Hematológicas/patologia , Humanos , Lactente , Recém-Nascido , Leucemia/patologia , Masculino , Neurofibromatoses/complicações , Síndrome de Noonan/complicações , Patologistas , Xantogranuloma Juvenil/patologia , Xantomatose/patologiaRESUMO
BACKGROUND: Dermoscopy is useful for the evaluation of juvenile xanthogranuloma (JXG). The classical "setting sun" pattern is characteristic of JXG, but its sensibility appears to be limited. An extensive description of other dermoscopic findings is not available in the literature. OBJECTIVES: The aim of this study was to valuate and describe the clinical and dermoscopic characteristics of a series of JXG cases. METHODS: This is a retrospective descriptive study, including cases with histopathologic diagnosis of JXG, and the availability of clinical and dermoscopic images, assessed for the presence of dermoscopic features based on the available literature. RESULTS: A total of 17 lesions were analyzed. 70.6% showed global symmetry, 35.3% presented the typical "setting sun" pattern. All lesions showed yellow-orange and/or pink-red structureless color. Other dermoscopic features were yellow globules (35.3%), shiny white streaks (23.5%), brown globules (17.6%), pale-brown network (11.8%), negative network (11.8%), erosion/ulceration (11.8%), rosettes (5.9%), and hemorrhage (5.9%). Scales were seen in 64.7% of patients. Vascular structures were observed in all the lesions, mostly in an irregular distribution (76.5%). The observed vessel types were dotted (52.9%), linear (52.9%), branching-arboriform (29.4%), comma-like (23.5%), hairpin-like (17.6%), globular (17.6%), coiled (11.8%), and milky-red globules (5.9%). CONCLUSIONS: Symmetry, yellow/orange-pink/red color, yellow globules, shiny white streaks, and irregularly distributed different types of vascular structures are the main dermoscopic features of JXG. This is the largest dermoscopic registry of JXG published to date.
Assuntos
Dermoscopia , Xantogranuloma Juvenil/diagnóstico , Adolescente , Adulto , Fatores Etários , Criança , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto JovemRESUMO
BACKGROUND: Systemic juvenile xanthogranuloma is a very rare disease typically presents as skin lesions with yellow papules or nodules and is sometimes fatal. We report a case of congenital neonatal systemic juvenile xanthogranuloma with atypical skin appearance that made the diagnosis difficult. CASE PRESENTATION: A preterm Japanese female neonate with prenatally diagnosed fetal hydrops in-utero was born with purpuric lesions involving the trunk and face. Since birth, she had hypoxemic respiratory failure, splenomegaly, anemia, thrombocytopenia, coagulopathy, and was transfusion dependent for red blood cells, fresh frozen plasma, and platelets. Multiple cystic lesions in her liver, part of them with vascular, were detected by ultrasound. A liver biopsy was inconclusive. A skin lesion on her face similar to purpura gradually changed to a firm and solid enlarged non-yellow nodule. Technically, the typical finding on skin biopsy would have been histiocytic infiltration (without Touton Giant cells) and immunohistochemistry results which then would be consistent with a diagnosis of systemic juvenile xanthogranuloma, and chemotherapy improved her general condition. CONCLUSIONS: This case report shows that skin biopsies are necessary to detect neonatal systemic juvenile xanthogranuloma when there are organ symptoms and skin eruption, even if the skin lesion does not have a typical appearance of yellow papules or nodules.
Assuntos
Púrpura , Xantogranuloma Juvenil , Biópsia , Edema , Feminino , Humanos , Recém-Nascido , Pele , Xantogranuloma Juvenil/complicações , Xantogranuloma Juvenil/diagnósticoRESUMO
Juvenile xanthogranuloma (JXG) is a common form of non-Langerhans cell histiocytosis, which usually presents with spontaneously regressing skin lesions. Systemic involvement is rare and mostly seen in patients with multiple skin nodules. It can spontaneously regress, but sometimes systemic involvement can cause life-threatening symptoms and can be fatal. Herein, we report a case of congenital systemic JXG with multiple skin nodules, soft tissue and pulmonary involvement. She was successfully treated with chemotherapy according to Langerhans cell histiocytosis treatment protocol IV of the Histiocyte Society (LCH-IV).
Assuntos
Histiocitose de Células de Langerhans , Histiocitose de Células não Langerhans , Neoplasias Cutâneas , Xantogranuloma Juvenil , Feminino , Histiocitose de Células de Langerhans/diagnóstico , Histiocitose de Células de Langerhans/tratamento farmacológico , Humanos , Xantogranuloma Juvenil/diagnóstico , Xantogranuloma Juvenil/tratamento farmacológicoRESUMO
INTRODUCTION: Xanthogranuloma of the sellar region is a rare benign lesion, and there are few cases reported in children. Its histogenesis is controversial, and it is difficult to strictly differentiate it from craniopharyngioma (CP), Rathke's cleft cyst, or pituitary adenoma. CASE PRESENTATION: A 16-year-old boy presented with a rare xanthogranuloma of the sellar region after complaining of retardation of growth 5 years previously. The ophthalmologic evaluation revealed no visual field disturbance. Endocrinological examination revealed hypopituitarism. Magnetic resonance imaging showed an intrasellar mass extending into the suprasellar region and compressing the optic chiasma, which appeared mixed signals on T1-weighted images. Endonasal transsphenoidal resection of the tumor was performed. Histological analysis of the tumor sections demonstrated granulomatous tissue with cholesterol clefts, hemosiderin deposits, fibrous tissues, multinucleated giant cells, and lymphocyte. Thus, the tumor was pathologically diagnosed as xanthogranuloma of the sellar region, which is different from adamantinomatous CP. There was no epithelial tissue in any part of the tumor including tumor capsule but have focal necrosis and calcification. His endocrinological dysfunction did not recover, so a hormonal replacement was continuously required. CONCLUSION: Xanthogranuloma of the sellar region is a rare entity but must be considered in the differential diagnosis of lesions of the sellar region, even in pediatric population. We should think about this disease when dealing with children with stunted growth accompanied by a long medical history. Our case demonstrates the natural progression of the disease, suggesting that xanthogranuloma of the sellar region without epithelial components may be an independent disease.
Assuntos
Craniofaringioma , Hipopituitarismo , Neoplasias Hipofisárias , Xantogranuloma Juvenil , Adolescente , Criança , Craniofaringioma/diagnóstico por imagem , Craniofaringioma/cirurgia , Humanos , Imageamento por Ressonância Magnética , Masculino , Neoplasias Hipofisárias/diagnóstico por imagem , Neoplasias Hipofisárias/cirurgia , Sela Túrcica/diagnóstico por imagem , Sela Túrcica/cirurgia , Xantogranuloma Juvenil/diagnóstico , Xantogranuloma Juvenil/cirurgiaRESUMO
BACKGROUND: Juvenile xanthogranuloma (JXG) is a non-Langerhans cell histiocytosis characterized by yellowish papules in the skin. JXGs most often occur in infancy or early childhood and are typically solitary and asymptomatic, often regressing after several years. While JXGs predominantly occur on the skin, extracutaneous JXGs also exist. AIMS: In this paper, we review the literature on single, multiple, and visceral JXGs and provide recommendations on monitoring and work-up. MATERIALS & METHODS: A literature review was conducted with the PubMed database using selective search terms for single, multiple, ocular, and visceral lesions as well as NF1/JMML. RESULTS / DISCUSSION: JXG is typically a self-limited disorder if lesions are cutaneous and singular. While rare, JXGs may manifest as multiple and extracutaneous lesions. Further screening and referral to specialists may be warranted in these cases based on age and extent of involvement. CONCLUSION: Our review demonstrates common presentations of single, multiple, and extracutaneous lesions in addition to those that occur with NF1 and JMML. We suggest patients be evaluated on a case-by-case basis by a dermatologist and referred to specialists as appropriate.
Assuntos
Histiocitose de Células não Langerhans , Xantogranuloma Juvenil , Pré-Escolar , Humanos , Lactente , Pele , Xantogranuloma Juvenil/diagnósticoRESUMO
Juvenile xanthogranuloma (JXG) and cutaneous mastocytosis (CM) are two distinct conditions that have rarely been reported in association. We report a child with CM and disseminated JXG, who showed a significant decrease in serum tryptase levels and regression of JXG lesions over time. Due to the paucity of reports, a true association between these two conditions has not been validated, although a potential induction of histiocytic lesions by mast cell degranulation has been proposed.
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Mastocitose Cutânea , Xantogranuloma Juvenil , Criança , Família , Histiócitos , Humanos , Mastocitose Cutânea/complicações , Mastocitose Cutânea/diagnóstico , Xantogranuloma Juvenil/complicações , Xantogranuloma Juvenil/diagnósticoRESUMO
We report a case of cutaneous cryptococcosis due to Cryptococcus neoformans in a pediatric patient with hyper IgM syndrome with scalp lesions that resembled tinea capitis on gross examination and mimicked juvenile xanthogranuloma on histologic examination. This case highlights the importance of considering cutaneous cryptococcosis in patients with hyper IgM syndrome.