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Juvenile Xanthogranuloma (Nevoxanthoendothelioma): Differential Diagnoses & Workup

Author: Caroline DS Piggott, MD, Resident, Department of Medicine, Division of Dermatology, University of California, San Diego
Coauthor(s): Michael L Smith, MD, Associate Professor of Medicine (Dermatology) and Pediatrics, Vanderbilt University School of Medicine; Consulting Staff, Vanderbilt University Hospital; Hunter H Sams, MD, Consulting Staff, Denver Dermatology Consultants, PC
Contributor Information and Disclosures

Updated: Oct 23, 2009

Differential Diagnoses

Dermatofibroma
Langerhans Cell Histiocytosis
Mastocytosis
Spitz Nevus
Xanthomas

Other Problems to Be Considered

Non–Langerhans cell histiocytosis
Benign cephalic histiocytosis14
Generalized eruptive histiocytoma
Self-healing reticulohistiocytoma
Tuberous xanthoma
Papular xanthoma
Xanthoma disseminatum
Mastocytosis (urticaria pigmentosa)

Workup

Procedures

A skin biopsy may be performed, both for diagnosis and cosmesis. The specimen usually consists of a complete excision of the papule or nodule.

Histologic Findings

Histological examination of juvenile xanthogranuloma (JXG) demonstrates a variety of findings. A time-dependent progression exists in the development of the characteristic histological features of JXG, which correlates with the age of the lesion. Early biopsy specimens reveal a dense monomorphous histiocytic infiltrate in the dermis. Extension into subcutaneous tissue, fascia, and muscle occurs in approximately one third of cases.15 Older lesions contain foam cells, Touton giant cells, and foreign body giant cells. A mixed cellular infiltrate of neutrophils, lymphocytes, eosinophils, and (rarely) mast cells may be noted. Old lesions demonstrate fibrosis. No histological difference is reported between cutaneous and systemic JXG. Because of the difficulty in diagnosing JXG and because of the transient presence of Touton giant cells in JXG lesions, these classic elements may not be present in every case.

The histiocytes contain pleomorphic nuclei, with few or absent mitotic figures, and irregular dense bodies. Clustered comma-shaped bodies occasionally are observed on electron microscopy but are not specific. Use of special stains is important to differentiate JXG from Langerhans and non–Langerhans cell histiocytoses. In JXG, histiocytes are positive to antibodies against factor XIIIa, HAM56, HHF35, KP1 (CD68), Ki-M1P, and Vimentin, and are generally negative to CD1a and S-100. New reports have also demonstrated a CD4 positivity, which has been used as evidence that plasmacytic monocytes may be the normal cell type of the major constituent of JXG, instead of the dermal dendrocyte.

More on Juvenile Xanthogranuloma (Nevoxanthoendothelioma)

Overview: Juvenile Xanthogranuloma (Nevoxanthoendothelioma)
Differential Diagnoses & Workup: Juvenile Xanthogranuloma (Nevoxanthoendothelioma)
Treatment & Medication: Juvenile Xanthogranuloma (Nevoxanthoendothelioma)
Follow-up: Juvenile Xanthogranuloma (Nevoxanthoendothelioma)
Multimedia: Juvenile Xanthogranuloma (Nevoxanthoendothelioma)
References
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References

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Further Reading

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Keywords

juvenile xanthogranulomas, nevoxanthoendothelioma, xanthoma multiplex, juvenile xanthoma, multiple xanthoma in infancy, congenital xanthoma tuberosum, xanthoma naviforme, juvenile giant cell granuloma

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Contributor Information and Disclosures

Author

Caroline DS Piggott, MD, Resident, Department of Medicine, Division of Dermatology, University of California, San Diego
Disclosure: Nothing to disclose.

Coauthor(s)

Michael L Smith, MD, Associate Professor of Medicine (Dermatology) and Pediatrics, Vanderbilt University School of Medicine; Consulting Staff, Vanderbilt University Hospital
Michael L Smith, MD is a member of the following medical societies: American Academy of Dermatology, American Academy of Pediatrics, and Society for Pediatric Dermatology
Disclosure: Nothing to disclose.

Hunter H Sams, MD, Consulting Staff, Denver Dermatology Consultants, PC
Hunter H Sams, MD is a member of the following medical societies: American Academy of Dermatology, American College of Mohs Micrographic Surgery and Cutaneous Oncology, and American Society for Dermatologic Surgery
Disclosure: Nothing to disclose.

Medical Editor

Jean Paul Ortonne, MD, Chair, Department of Dermatology, Professor, Hospital L'Archet, Nice University, France
Jean Paul Ortonne, MD is a member of the following medical societies: American Academy of Dermatology and American Dermatological Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA
Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Jeffrey Meffert, MD, Assistant Clinical Professor of Dermatology, University of Texas Health Science Center-San Antonio
Jeffrey Meffert, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Association of Military Dermatologists, and Texas Dermatological Society
Disclosure: Nothing to disclose.

CME Editor

Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania
Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology
Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds None; Genentech Consulting fee Consulting; Centocor Consulting fee Consulting; Centocor Grant/research funds None; Covance Consulting fee Consulting; Shire  Consulting

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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