Case Report

Received: November 6, 2006

Accepted: April 9, 2007

Dermatology 2007;215:256259
DOI: 10.1159/000106586

Dermoscopic Appearance of Juvenile

Xanthogranuloma
Antony Palmer Jonathan Bowling
Department of Dermatology, Churchill Hospital, Oxford, UK

Abstract
Juvenile xanthogranuloma (JXG) is the commonest form of non-Langerhans cell histiocytosis and manifests as asymptomatic yellow-red papulonodules that usually occur in
childhood and spontaneously regress within a year of formation. The diagnosis may be
made by clinical examination alone and confirmed by histology in cases of diagnostic
doubt. Here we report the use of dermoscopy in evaluating skin lesions suggestive of
JXG. Three patients diagnosed as having JXG
in our dermatology department over the
last year showed a characteristic orange-yellow setting sun appearance on dermoscopy. We therefore feel that the use of dermoscopy can be extended to include the
examination of non-pigmented skin lesions,
particularly in paediatric patients.
Copyright 2007 S. Karger AG, Basel

Introduction

Juvenile xanthogranuloma (JXG) is the

commonest form of non-Langerhans cell
histiocytosis and manifests as asymptomatic yellow-red papulonodules. Clinically,

the list of differential diagnoses is large.

We present 3 cases that attended our department, with lesions exhibiting a similar
orange-yellow setting sun colouration
when viewed with a dermatoscope.
Case Reports

Case 1
A 32-year-old lady presented with a 6month history of a red nodule on her left
scapula, approximately 1 cm in diameter
(fig. 1). Clinical features were in keeping
with xanthogranuloma. She declined excision biopsy.
Case 2
A 7-month-old boy presented with a 4month history of a solitary slowly enlarging soft red nodule located on his left lower back (fig. 2). Clinical features were in
keeping with JXG, and conservative management was adopted.
Case 3
A 32-year-old lady attended her routine
melanoma follow-up appointment. On examination an asymptomatic orange-yellow firm papule was noticed on her right
shin, approximately 4 mm in diameter
(fig. 3). It is not known how long the lesion
had been present. A skin biopsy confirmed
a spindle cell variant of JXG (fig. 4).

2007 S. Karger AG, Basel

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Histology Report

Macroscopic Report
An ellipse of skin measuring 13 ! 5 !
2 mm with a yellow papule measuring
4 mm in diameter.
Microscopic Report
Skin containing a dermal histiocytic
tumour positive for CD68 and negative for
S100 and melan A. CD1A is negative and
the features are within the spectrum of
a non-Langerhans cell histiocytosis. Although the features are not entirely classical, it fits best with a spindle cell variant of
JXG.
Discussion

JXG is a common form of non-Langerhans cell histiocytosis and manifests as

asymptomatic yellow-red papulonodules
that usually occur in childhood and spontaneously regress within a year of formation. Lesions may be solitary or multiple,
and although they are most often found in
the skin, they can also develop within other organs [1]. As the name suggests, 70%
develop in the first year of life [2]; however,
they are not confined to childhood and
may be found in patients of any age, when
they are sometimes denoted adult xanthogranuloma [3]. JXG lesions are benign and

Antony Palmer
Dermatology Department
Churchill Hospital
Oxford OX3 7LJ (UK)
Tel. +44 1865 228 205, Fax +44 1865 228 260, E-Mail antonypalmer@doctors.org.uk

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Key Words
Dermatoscopy  Dermoscopy 
Juvenile xanthogranuloma

Fig. 1. Case 1. Macroscopic appearance of the lesion (a) and dermoscopy showing orange-yellow background
colouration with clouds of xanthomatous deposits (b). Heine Delta 20. Original magnification !10.

Fig. 2. Case 2. Macroscopic appearance of the lesion (a) and dermoscopy showing orange-yellow background
colouration with clouds of xanthomatous deposits (b). Heine Delta 20. Original magnification !10.

Dermoscopic Appearance of Juvenile

Xanthogranuloma

cases [5], a conservative approach may be

adopted.
Dermoscopy is a non-invasive technique that allows better visualisation of
structures within the epidermis, dermalepidermal junction and superficial dermis. The dermoscopic features of a large
number of non-melanocytic lesions have
been reported which helps to reduce the
differential diagnosis of solitary lesions

[6]. The infiltrate of JXG occurs in the superficial dermis, an area where structures
can be seen with dermoscopy. On dermoscopy each lesion showed a characteristic
orange-yellow background colouration,
with clouds of paler yellow deposits consistent with a xanthogranulomatous dermal infiltrate. The clouds of paler yellow
deposits are similar to those seen in sebaceous hyperplasia [7]. To our knowledge

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comprise a diffuse infiltrate of histiocytes

between the epidermis and reticular dermis [4]. Excision for solitary lesions should
be considered as the differential diagnoses include: xanthoma, dermatofibroma,
Spitzs naevus, molluscum contagiosum,
neurofibroma, mastocytosis, Langerhans
cell histiocytosis, mycobacterial infection.
However, in view of the spontaneous
regression occurring in the majority of

Fig. 3. Case 3. Macroscopic appearance of the lesion (a) and dermoscopy showing orange-yellow background
colouration with central linear blood vessels (b). Heine Delta 20. Original magnification !10.

Fig. 4. Microscopic appearance of the lesion in figure 3 at !10 magnification (a) and !100 magnification (b).

Microscope: Olympus BX40. Camera: Nikon Coolpix 950.

258

taneous resolution occurs in the majority

of cases, any case that persists should be
considered for excision biopsy. Patients
should be made aware that atrophy or hyperpigmentation of the skin may occur
following spontaneous resolution and remain in up to half of the patients [5].
In conclusion, dermoscopy may add
additional information to the clinical examination in xanthogranulomatous lesions. However, we would recommend

Dermatology 2007;215:256259

further studies demonstrating dermoscopic features with histopathological correlation, in patients with JXG and other
xanthogranulomatous disorders, to confirm this dermoscopic sign.
Acknowledgement

Many thanks are due to Dr. Olivia Espinosa for her histological diagnosis.

Palmer/Bowling

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this dermoscopic feature has not been described in xanthogranulomatous lesions.

A xanthogranulomatous lesion was confirmed histologically in case 3. We appreciate that histology is needed in all cases
for more accurate dermoscopic/histopathological correlation; however, this may be
difficult, particularly in lesions where the
natural history is for spontaneous resolution and because the majority of cases will
occur in early childhood. Although spon-

Dermoscopic Appearance of Juvenile

Xanthogranuloma

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3 Saad N, Skowron F, Dalle S, et al: Multiple
adult xanthogranuloma: case report and literature review. Dermatology 2006; 212: 73
76.

4 Lazova R, Shapiro PE: Juvenile xanthogranuloma versus Langerhans cell histiocytosis.

Semin Cutan Med Surg 1999;18:7177.
5 Sonoda T, Hashimoto H, Enjoji M: Juvenile
xanthogranuloma: clinicopathologic analysis and immunohistochemical study of 57
patients. Cancer 1985;56:22802286.
6 Zalaudek I, Argenziano G, Di Stefani A, et al:
Dermoscopy in general dermatology. Dermatology 2006; 212:718.
7 Zaballos P, Ara M, Puig S, Malvahy J: Dermoscopy of sebaceous hyperplasia. Arch
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References