Unusual aspects of juvenile xanthogranuloma

doi:10.1016/0190-9622(93)70259-V

Journal of the American Academy of Dermatology

Volume 29, Issue 5, Part 2, November 1993, Pages 868-870

https://doi.org/10.1016/0190-9622(93)70259-V Get rights and content

We describe three unusual features of juvenile xanthogranuloma that were observed in three different children. We also describe the mixed and clustered forms of juvenile xanthogranuloma and a giant juvenile xanthogranuloma of the nose.

References (15)

R Nomland
Nevoxantho-enodothelioma: a benign xanthomatous disease of infants and children
J Invest Dermatol
(1954)
F Gianotti et al.
Histiocytic syndromes: a review
J Am Acad Dermatol
(1985)
CJ Gallant et al.
Juvenile xanthogranulomas and xanthoma disseminatum-variations on a single theme [Letter]
J Am Acad Dermatol
(1986)
R Caputo
Juvenile xanthogranulomas and xanthoma disseminatum-variations on a single theme [Letter reply]
J Am Acad Dermatol
(1986)
FE Senear et al.
Nevoxantho-endothelioma of juvenile xanthoma
Arch Dermatol Syph
(1936)
EB Helwig et al.
Juvenile xanthogranuloma (nevoxantho-endothelioma) [Abstract]
Am J Pathol
(1954)
F Gianotti et al.
Xantho-granulomatoses juveniles

There are more references available in the full text version of this article.

Cited by (61)

Juvenile and adult xanthogranuloma: A 30-year single-center experience and review of the disorder and its relationship to other histiocytoses
2022, Annals of Diagnostic Pathology
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.
A retrospective review of all the cases with the pathologic diagnosis of “xanthogranuloma” was performed on our archives from 1989 to 2019.
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%).
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.
Juvenile Xanthogranuloma: An Entity With a Wide Clinical Spectrum
2020, Actas Dermo-Sifiliograficas
El xantogranuloma juvenil es un trastorno benigno poco frecuente, que pertenece al amplio grupo de las histiocitosis de células no Langerhans. Se presenta con uno o más nódulos eritematosos o amarillentos, ubicados preferentemente en la cabeza y el cuello. La mayoría de los casos se inician durante el primer año de vida, incluyendo lesiones congénitas. La afectación extracutánea es rara, sugiriéndose tradicionalmente en la literatura estudiar el compromiso ocular. El diagnóstico del xantogranuloma juvenil es fundamentalmente clínico, sin embargo, en ocasiones se requiere confirmarlo con biopsia de piel. Las lesiones cutáneas son autolimitadas, por lo que suelen no requerir tratamiento. En la presente revisión se describen los distintos aspectos clínicos y terapéuticos de esta enfermedad, resaltando la evidencia respecto al estudio diagnóstico del compromiso extracutáneo.
Juvenile xanthogranulomas (JXGs) are rare, benign lesions that belong to the large group of non-Langerhans cell histiocytoses. JXG presents with 1 or more erythematous or yellowish nodules that are usually located on the head or neck. Most JXG lesions are congenital or appear during the first year of life. Extracutaneous involvement is rare, but the literature traditionally suggests investigating the possibility of ocular compromise. JXG is mainly a clinical diagnosis, but a skin biopsy may sometimes be needed for confirmation. JXGs on the skin are self-limiting and usually do not require treatment. This review describes the clinical and therapeutic aspects of JXG, emphasizing available evidence and the diagnosis of extracutaneous involvement.
Skin and superficial soft tissue neoplasms with multinucleated giant cells: Clinical, histologic, phenotypic, and molecular differentiating features
2019, Annals of Diagnostic Pathology
Citation Excerpt :
JXG expresses factor XIIIa, CD68 (Fig. 6C), CD163, CD14, and fascin. S100 is rarely positive, while CD1a is only expressed in interspersed Langerhans cells [106,124-127]. The differential diagnosis of JXG includes Langerhans cell histiocytosis (positive for S100 and CD1a), papular xanthoma (very uncommon lesion, factor XIIIa negative, most cases located on the trunk in older individuals), dermatofibroma (dense collagenous stroma, storiform growth pattern, overlying pseudo-epitheliomatous hyperplasia), and Spitz nevus (positive for melanocytic markers such as S100, Melan-A, and tyrosinase) [128].
Multinucleated giant cells (MGC) are commonly seen in an array of neoplastic and non-neoplastic conditions, to include: granulomatous dermatitis, fibrohistiocytic lesions such as xanthogranulomas, and soft tissue tumors such as giant cell tumors of soft tissue. In addition, multinucleated giant cells are infrequently seen in melanoma, squamous cell carcinoma, and atypical fibroxanthoma. There are many different types of MGCs and their presence, cytologic, and immunohistochemical features within these pathologic entities vary. Thus, correct identification of the different types of MGCs can aid the practicing pathologist in making the correct diagnosis of the overall pathologic disease. The biologic diversity and variation of MGCs is currently best exemplified in cytologic appearance and immunohistochemical profiles. However, much remains unknown about the origination and evolution. In this review, we i) reflect on the various types of MGCs and the current understanding of their divergent development, ii) describe the histologic, immunohistochemical, and molecular (if previously reported) differentiating features of common skin and superficial soft tissue neoplasms that may present with multinucleated giant cells.
CD68-negative nonlipidized juvenile xanthogranuloma
2017, Dermatologica Sinica
Citation Excerpt :
The authors suggest that mononuclear cells in JXG might derive from plasmacytoid monocytes. CD68, a widely-used monocyte/macrophage lineage marker, was shown to be positive in all cases of JXG,16 and in 11 of 15 reported cases of NJXG.3,5,7–14 Of the four cases of CD68-negative NJXG, FXIIIa was positive in all four, weakly or focally Ham57- or Mac387-positive in three, and focally lysozyme-positive in one.
Juvenile xanthogranuloma (JXG) belongs to the group of non-Langerhans cell histiocytosis. JXG is typically factor XIIIa- and CD68-positive. Nonlipidized JXG (NJXG) is a rare, mononuclear variant of JXG that shows few or absence of foam cells or Touton giant cells in the histiocytic infiltrate. Herein, we describe an unusual case of NJXG in a 7-year-old boy presenting with a 1-month history of a flesh-colored nodule on the forehead. The lesion was factor XIIIa-positive and CD68-negative but CD163-positive. Our case illustrates that CD163, a more specific marker of macrophage and monocyte differentiation, is a valuable marker for the diagnosis of NJXG.
Juvenile xanthogranuloma in a 4-year-old child
2017, Archives de Pediatrie
Localized eruptive juvenile xanthogranuloma
2014, Annales de Dermatologie et de Venereologie
Le xanthogranulome juvénile (XGJ) est une histiocytose non langerhansienne se présentant classiquement sous la forme de nodules cutanés jaunâtres isolés ou multiples. Des formes cutanées atypiques, lichénoïdes, réticulées, maculopapuleuses, en plaque ou encore linéaires ont été décrites à partir d’observations isolées. Nous rapportons le cas d’un nourrisson ayant des XGJ éruptifs en plaque de la jambe gauche.
Une enfant de 13 mois avait depuis l’âge de 2 mois des lésions papuleuses rouge orangé asymptomatiques évoluant par poussées sur la face antérieure de la jambe gauche, sans placard sous-jacent. La recherche d’un signe de Darier était négative. L’examen histologique posait le diagnostic de XGJ.
Notre cas se rapproche des XGJ en plaque plutôt que d’une forme multiple, dont la topographie est diffuse. Seuls sept cas semblables ont été rapportés dans la littérature, tous survenus avant cinq mois de vie. La lésion correspondait le plus souvent à une plaque érythémateuse asymptomatique surmontée de petites lésions jaunâtres, de topographie variable et d’apparition parfois progressive. La guérison spontanée était la règle. Il n’y avait pas d’atteinte systémique. Nous présentons ici un cas unique de xanthogranulomes multiples localisés, sans plaque infiltrée sous-jacente évidente, évoluant par poussées.
Juvenile xanthogranuloma (JXG) is a non-Langerhans histiocytosis of young children characterized by solitary or multiple yellowish cutaneous nodules. Atypical skin lesions such as lichenoid eruptions, and pedunculated, maculopapular, plaque-like or linear lesions have been described. We report a case of eruptive XGJ en plaque in the left leg in an infant.
A 13-month-old child presented asymptomatic eruptive, yellowish papules of the leg measuring 5 to 10 mm since the age of 2 months. There was no cutaneous infiltration between the lesions. Darier's sign was negative. Histological examination confirmed the diagnosis of JXG. The course of the disease comprised a gradual decrease in the number of active lesions with slight residual pigmentation.
Our case was suggestive of JXG en plaque. Only 7 cases have been reported in the literature, all appearing before the age of 5 months. The lesions corresponded mostly to an asymptomatic erythematous plaque studded with small yellowish/red nodules of variable localisation. Spontaneous involvement was noted in all cases. No systemic involvement was found. Herein we present a unique case of localised multiple JXG without evident clinical infiltrating plaque progressing with self-resolving flares.

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Unusual aspects of juvenile xanthogranuloma

J Invest Dermatol

J Am Acad Dermatol

J Am Acad Dermatol

J Am Acad Dermatol

Nevoxantho-endothelioma of juvenile xanthoma

Arch Dermatol Syph

Juvenile xanthogranuloma (nevoxantho-endothelioma) [Abstract]

Am J Pathol

Xantho-granulomatoses juveniles