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CASE REPORT
Year : 2019  |  Volume : 20  |  Issue : 2  |  Page : 177-179

Cutaneous horn, juvenile xanthogranuloma in the first decade of life: A clinico-dermatoscopic correlations


Private Practice, Tripoli-, Libya

Date of Web Publication29-Mar-2019

Correspondence Address:
Dr. Ebtisam Elghblawi
P.O.BOX 3232, Tripoli
Libya
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijpd.IJPD_44_17

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  Abstract 


Juvenile xanthogranuloma (JXG) is a rare disorder that is present at birth, in infancy, or in adults. It can be either solitary or multiple nodules of various sizes. It can be associated with internal organ involvements, especially eyes, lung, and liver. The diagnosis can be made with confidence by clinical examination alone and confirmed by histology in doubtful cases. Hereby, I report the useful benefit of dermoscopy in evaluating and diagnosing a skin lesion that is suggestive of JXG. A 1-year-old skin type I girl presented with gradual onset of a nodular lesion on her forearm that showed a surface projecting horn. A biopsy was planned and scheduled. Clinical features were in keeping with JXG. JXG is on the spectrum of histiocyte disarrays which should be examined thoroughly in children to exclude underlying malignancy. The purpose of this article is to describe the clinical dermatoscopic correlated features of JXG. This is an interesting case of JXG presenting in a young White Swedish female child.

Keywords: Child, dermatoscopy, dermoscopy, juvenile xanthogranuloma, nodule


How to cite this article:
Elghblawi E. Cutaneous horn, juvenile xanthogranuloma in the first decade of life: A clinico-dermatoscopic correlations. Indian J Paediatr Dermatol 2019;20:177-9

How to cite this URL:
Elghblawi E. Cutaneous horn, juvenile xanthogranuloma in the first decade of life: A clinico-dermatoscopic correlations. Indian J Paediatr Dermatol [serial online] 2019 [cited 2019 Apr 19];20:177-9. Available from: http://www.ijpd.in/text.asp?2019/20/2/177/255204




  Introduction Top


Juvenile xanthogranuloma (JXG) is a rare benign, non-Langerhans histiocytosis, with macrophages loaded with lipids, self-healing, and a skin lesion that is predominantly affecting infants and children. It can be either solitary or multiple yellowish or red-brown, asymptomatic firm papules or nodules affecting trunk or limbs without a preceding trauma and can resolve spontaneously within several years with Skin type V male predominance; however, this remains arguable.[1] The main differentials can be molluscum contagiosum (MC) or viral warts, mastocytomas, Spitz nevi, leishmaniasis, tuberculosis, and Langerhans cell histiocytosis, reticulohistiocytoma, and sometimes pyogenic granuloma.

Histology usually reveals hyperkeratosis and parakeratosis in the epidermis and collection of histiocytes with infiltration in the dermis with foamy and multinucleate Touton giant cells.[1] The diagnosis is mainly clinical; however, sometimes, a biopsy reading is necessary.

JXG is the variant of non-Langerhans cell histiocytosis. The orange-yellow background coloration with cloud of pale yellow globules is the most distinctive dermoscopic finding of fully developed and regressed JXG stages, with erythematous margin, faint pigment network, and white linear streak.


  Case Report Top


A healthy 1-year-old Swedish female child with onset in the 8th month of life presented with a pimple-like skin lesion on the right mid-flexor forearm at the clinic of dermatology [Figure 1]. She was born without any morbidity. The parents reported that in the past 4 months, the lesion was growing steadily and had developed a surface crust without ulceration that enlarged and expanded gradually into an upward-projecting horn (2 × 1). Medical history was unremarkable [Figure 2].
Figure 1: Right mid-flexor forearm showing hard conical-shaped lesion with brownish-red hue. Image courtesy Dr. Jan Lapins

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Figure 2: Upward-projecting cutaneous horn. Image courtesy Dr. Jan Lapins

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Examination revealed a firm conical-shaped lesion with brownish orange-red hue, 5 mm in diameter and 3 mm high. The child was otherwise healthy and feeding well. No other skin lesion was found.

Dermatoscopy was conducted by Heine Delta 20 nonpolarized contact and it revealed a well-circumscribed skin lesion with yellowish-white clods, and linear and branched vessels were predominantly at the periphery of the lesion at one pole on an orange-yellow background [Figure 3].
Figure 3: Dermatoscopy showing a well-circumscribed skin lesion with yellowish-white clods, and linear and branched vessels were predominantly at the periphery of the lesion at one pole on an orange-yellow background. Image courtesy Dr. Jan Lapins

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A surgical planned excision biopsy was given; however, total spontaneous involution had happened insidiously, and thus, histology diagnosis was not verified. Thus, histopathology correlation is lacking though the clinical presentation with its involution supporting the clinical provisional diagnosis.


  Discussion Top


JXG is the most common non-Langerhans cell histiocytosis of childhood.[1],[2]

These lesions are normally asymptomatic and disappear spontaneously within 3–6 years. They are a type of granulomatous process, and the actual cause is unknown; however, there is some postulation of an underlying injury or infection that causes macrophage response to be disorderly.[3] It has been stated that children younger than two and with multiple nodules are at risk for ocular involvement.[3] Any organ can be involved with JXG; however, the most common in order are eyes, lungs, and liver consecutively.

JXG was first coined in 1905 by Adamson.[3] Dermoscopic appearances of JXG were first described by Palmer and Bowling.[3] Since then, few cases have emphasized the clinical usefulness of dermoscopy.[4]

Cutaneous horn is characteristic in such cases which can be similar to an animal horn. Horns can be seen in other similar conditions where it could need a thorough workup to distinguish between them. Horns can be seen in premalignant cases such as actinic keratosis and Bowen disease, MC, and xanthomatous dermatofibroma and in seborrheic keratosis and filiform warts and malignant cases such as keratoacanthoma base and squamous cell carcinoma. Thus, finding out the underlying disease is paramount to send for histology sign out.

JXG is not a true pathological condition, however, a reactive one with keratinization.[4]

JXG has three distinctive stages: (1) early evolutionary stage, (2) fully developed stage, and (3) late regressive stage.[4] Dermoscopic features constitute some main features. The setting-sun appearance which can be seen mostly in the first two stages of the JXG, then in the later regression stage, it has been found that the surrounding erythema declines with the development of the white streaks. Added to that the vacuolated cells transform across the stages to xanthomatized cells that become yellowish and can be distinguished across the three stages. However, the branched and linear vessels are constantly appreciated in the three stages as well.[4],[5]

The main observed dermatoscopic feature of JXG is the setting-sun appearance which can be appreciated in the three stages.[4] Moreover, clouds of pale yellowish globules can be seen and represent lipid-laden histiocytes in the upper dermis, with subtle pigment network and white streak which implies fibrosis process.[4]

JXG constitutes a buildup of histiocytes that are devoid of Birbeck granules (non-Langerhans cells) that can be made distinctive by special stains.[6]

Dermoscopy is valuable to differentiate between different stages of JXG and other similar conditions of yellowish papules and nodules.[5]

Cutaneous horns are seldom reported in children, and histopathologic examination remains the only way to establish the diagnosis of JXG. There are, however, a few report cases of JXG in the existing literature of cutaneous horns in children.

Histology is mandatory in most cases to establish a confirmed diagnosis of dermatoscopic histologic correlation; nonetheless, this cannot be possible in all cases, especially when the lesion follows the natural call for regression and dissolution.[7]

A careful dermatoscopic examination in the hand with the clinical picture should be exercised to avoid unnecessary surgical intervention in infants and young children. Following up with a dermatoscope will expand the physician's knowledge to the different stages of the JXG evolution. Thus, the extended application of dermatoscope in pediatric age group will be paramount to aid in diagnosis and avoid unnecessary painful surgical excision.

To the best of my knowledge, visceral lesions have not been described in this young girl.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Yoon YH, Ju HJ, Lee KH, Park CJ. A cutaneous horn-like form of juvenile xanthogranuloma. Ann Dermatol 2016;28:783-4.  Back to cited text no. 1
    
2.
Caputo R, Grimalt R, Gelmetti C, Cottoni F. Unusual aspects of juvenile xanthogranuloma. J Am Acad Dermatol 1993;29:868-70.  Back to cited text no. 2
    
3.
Pajaziti L, Hapçiu SR, Pajaziti A. Juvenile xanthogranuloma: A case report and review of the literature. BMC Res Notes 2014;7:174.  Back to cited text no. 3
    
4.
Song M, Kim SH, Jung DS, Ko HC, Kwon KS, Kim MB, et al. Structural correlations between dermoscopic and histopathological features of juvenile xanthogranuloma. J Eur Acad Dermatol Venereol 2011;25:259-63.  Back to cited text no. 4
    
5.
Rubegni P, Mandato F, Fimiani M. Juvenile xanthogranuloma: Dermoscopic pattern. Dermatology 2009;218:380.  Back to cited text no. 5
    
6.
Hernandez-Martin A, Baselga E, Drolet BA, Esterly NB. Juvenile xanthogranuloma. J Am Acad Dermatol 1997;36:355-67.  Back to cited text no. 6
    
7.
Palmer A, Bowling J. Dermoscopic appearance of juvenile xanthogranuloma. Dermatology 2007;215:256-9.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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