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March 2015 Dermatology Clinic
A mother brought her son, aged 2-1/2 years, into the clinic concerned about a growth on his cheek. The mother noted that the lesion appeared approximately 1 year prior. It slowly enlarged over the course of the first few months following its initial appearance, but it has recently been stable in size. The growth did not seem to bother the child. The patient had no known medical conditions and was not on any medications. Physical exam revealed a fleshy, yellowish, slightly firm 5-mm papule on the left cheek. The papule had no pigment network on dermoscopy. A full skin examination did not reveal any other skin lesions.
This The Clinical Advisor CME activity consists of 3 articles. To obtain credit, read Progressive, diffuse scalp hair loss in a woman and Annular lesions with dark centers. Then take the post-test here.
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This The Clinical Advisor CME activity consists of 3 articles. To obtain credit, read Progressive, diffuse scalp hair loss in a woman and Annular lesions with dark centers. Then take the post-test here.
Cutaneous juvenile xanthogranuloma (JXG), also known as juvenile xanthoma, is a relatively common and benign condition that presents with single or multiple nodules. The lesion represents a proliferation of cells derived from dermal dendrocytes. JXG is the most common condition in the group of non-Langerhans cell histiocytoses.
The typical clinical appearance of a JXG is a well-demarcated, firm, dome-shaped papule or papulonodule, ranging in size from 5 mm to 2 cm. In some cases, the lesion may be ulcerated or crusted. Initially, the color is usually erythematous or orange, but with time, it may evolve to a yellow, brown, tan, or flesh color. Most cutaneous JXGs present as a solitary lesion. Typical sites of presentation are the head and neck, although it may appear on the trunk or extremities.
Less commonly, multiple cutaneous lesions may develop and these may have systemic implications. In rare cases, JXGs may appear primarily in extracutaneous sites, including the subcutaneous soft tissue, central nervous system, liver, spleen, and lungs.1Ocular involvement has also been documented; therefore, when a patient presents with multiple JXGs, clinicians should ask about ocular symptoms and consider referral to an ophthalmologist.
The true incidence of JXG is unknown, as many cases are undiagnosed or misdiagnosed. However, a review from the Kiel Pediatric Tumor Registry identified 129 cases of JXG, representing an incidence of 0.52%.2Most JXGs appear in the first year of life, with an average age of onset of 5 months to 1 year;35% to 17% of JXGs are present at birth.4The condition rarely presents in adulthood, although there are a few such documented cases.5Earlier age of onset is associated with the appearance of a greater number of lesions. Male children are affected more often than female children, whereas in adulthood, both sexes are affected equally.
Although its cause is unknown, JXG is thought to be a granulomatous reaction of histiocytes to an unknown cause. The prefix “xantho” is a misnomer, as JXG is not associated with any lipid abnormalities. Genetic factors have been suggested, although familial cases have not been described. In addition, there have been associations reported between JXG, neurofibromatosis, and juvenile chronic myelogenous leukemia. Therefore, patients with JXG should be screened for other skin lesions, and if markers of neurofibromatosis are present, they should be screened for juvenile chronic myelogenous leukemia.
Solitary JXG can mimic other single tumors of childhood, including Spitz nevus, mastocytoma, neurofibroma, and molluscum.
Although the diagnosis of JXG is often made clinically, biopsy is sometimes necessary in questionable cases. Immunohistochemical staining with macrophage markers are often necessary. Histologic examination reveals a dense infiltrate of foamy histiocytes, foreign body cells, and often Touton giant cells,6usually in the upper and mid-reticular dermis. It should be noted that the histopathologic appearance of JXG lesions varies depending on their stage of evolution.
For solitary lesions, reassurance and observation for spontaneous resolution is often all that is necessary. In childhood, most lesions resolve within three to six years, usually without scarring. Infrequently, however, atrophy or pigmentary changes may result. In adulthood, however, JXGs often persist indefinitely. Surgical removal will likely result in a permanent scar, and therefore, it is often not recommended for single cutaneous lesions in children.
Extracutaneous and systemic JXG carry significant morbidity and should be treated. Treatment for intraocular lesions includes corticosteroids. In rare cases, low-dose radiation therapy is used to prevent vision loss. Systemic JXG may require chemotherapy, applying regimens similar to those used to treat Langerhans cell histiocytosis.
The mother of the young patient in this case was counseled regarding the diagnosis and the benign nature of the condition. She was instructed to be aware for the development of other skin lesions. At four years after the initial onset of the lesion, the patient’s mother reported that the lesion had fully resolved with no evidence of a remaining scar.
Esther Stern, NP-C, is a family nurse practitioner at Advanced Dermatology & Skin Surgery, P.C., in Lakewood, N.J.
This The Clinical Advisor CME activity consists of 3 articles. To obtain credit, read Progressive, diffuse scalp hair loss in a woman and Annular lesions with dark centers. Then take the post-test here.
References
- Freyer DR, Kennedy R, Bostrom BC, et al. Juvenile xanthogranuloma: Forms of systemic disease and their clinical implications. J Pediatr . 1996;129:227-237.
- Janssen D and Harms D. Juvenile xanthogranuloma in childhood and adolescence: A clinicopathologic study of 129 patients from the Kiel Pediatric Tumor Registry. Am J Surg Pathol. 2005;29(1):21-28.
- Dehner LP. Juvenile xanthogranulomas in the first two decades of life: A clinicopathologic study of 174 cases with cutaneous and extracutaneous manifestations. Am J Surg Pathol . 2003;27(5):579-593.
- Macrophage/monocyte disorders. In: James WD, Berger TG, Elston DM. Andrews’ Diseases of the Skin: Clinical Dermatology. 10th ed. Philadelphia, Pa.: Saunders Elsevier; 2006:715-716.
- Ranasinghe A, Todd P, Bardsley V. Juvenile xanthogranuloma in an adult male. J Am Acad Derm. 2013;68(4 Suppl 1):AB53.
- Cypel TK and Zuker RM. Juvenile xanthogranuloma: Case report and review of the literature. Can J Plast Surg. 2008;16(3):175-177. Available at ncbi.nlm.nih.gov/pmc/articles/PMC2691016
All electronic documents accessed on February 27, 2015.
This The Clinical Advisor CME activity consists of 3 articles. To obtain credit, read Progressive, diffuse scalp hair loss in a woman and Annular lesions with dark centers. Then take the post-test here.
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