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CASE REPORT
Year : 2015  |  Volume : 16  |  Issue : 2  |  Page : 78-80

Papular xanthoma in a child: Report of a rare entity


1 Department of Dermatology, SNMC, Bagalkote, Karnataka, India
2 Department of Dermatology, AIIMS, Bhopal, Madhya Pradesh, India
3 Department of Dermatology, IPGMER and SSKM Hospital, Kolkata, West Bengal, India
4 Department of Dermatology, RG Kar Hospital, Kolkata, West Bengal, India
5 Department of Health and Family Welfare, Swasthya Bhavan, Government of West Bengal, Kolkata, West Bengal, India

Date of Web Publication9-Apr-2015

Correspondence Address:
Nidhi Choudhary
H No. H3/16, Arvind Vihar Colony, Bhopal, Madhya Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2319-7250.152139

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  Abstract 

Papular xanthoma (PX) is a form of Non-Langerhan's cell histiocytosis, which is rare and primarily affects adults. Rarely cases have been reported in children as well though the presentation in both the age groups vary considerably. We present a case of PX in a 4½-year-old male child who presented with multiple asymptomatic skin-colored papular lesions on the body.

Keywords: childhood, non langerhan cell histiocytosis, papular xanthoma


How to cite this article:
Jadhav S, Choudhary N, Ahar R, Ghosh A, Singha J, Saha A. Papular xanthoma in a child: Report of a rare entity . Indian J Paediatr Dermatol 2015;16:78-80

How to cite this URL:
Jadhav S, Choudhary N, Ahar R, Ghosh A, Singha J, Saha A. Papular xanthoma in a child: Report of a rare entity . Indian J Paediatr Dermatol [serial online] 2015 [cited 2020 Nov 25];16:78-80. Available from: https://www.ijpd.in/text.asp?2015/16/2/78/152139


  Introduction Top


Papular xanthoma (PX) is a rare form of Non-Langerhan cell histiocytosis, which primarily affects adults. It is characterized by development of yellow to reddish-yellow papules and nodules of 2-15 mm diameter occurring chiefly over back and neck. It was first reported in adults, but subsequently PX in children also came into view. [1],[2] There is a marked difference between presentations of PX in adults and children, as in children no involvement of mucous membranes has been reported. The disease is self-resolving in children, while in adults progressive disease has been reported. [3] We report a case of PX in child for its rarity.


  Case Report Top


A 4 ½-year-old male child presented with asymptomatic skin-colored papular lesions on face, trunk, back, upper arms, axillae and groin [Figure 1]a and b] since 6 months. The disease began as 2-3 reddish papules over abdomen and soon progressed to the current state. None of the family members including his three younger siblings had similar lesions. The child was otherwise normal except for complaints of occasional low-grade fever. On examination, numerous close set xanthomatous lesions chiefly comprising of flat-topped papules of various sizes, the largest being 7 mm in diameter were seen on face, trunk, back, upper arms, axillae and groin creases. Eyelids were spared, so was the scalp. Few lesions showed central umbilication. The papules were discrete with no tendency to coalesce. There was a line of demarcation just above the buttocks, below which the lesions were conspicuously absent (except a few in popliteal fossae). The papules were firm on palpation and were nontender. Hair, nails, oral and genital mucosa were normal. General examination revealed no abnormality. There was no lymphadenopathy. A differential diagnosis of benign cephalic histiocytosis (BCH) and xanthoma disseminatum were considered. All hematological investigations including the serum lipid profile were normal. Ultrasonography abdomen-pelvis and chest X-ray revealed no abnormalities. Histopathological examination of one of the papules showed a thin epidermis, dense histiocytic infiltrate in the upper and mid dermis with numerous foam cells and a moderate numbers of foamy multinucleate Touton giant cells [Figure 2]a]. The lower epidermis showed mild infiltrate with hyalinization of collagen. Immunohistochemistry showed positive staining for CD68 and was negative for CD1a [Figure 2]b]. With the constellation of clinical, histopathological and immunohistochemical findings, a diagnosis of PX was made. The child is under follow-up for expectant spontaneous regression of lesions.
Figure 1: (a) Skin colored papular lesions on face, trunk, upper arms, axillae and groin, (b) Skin colored papular lesions on back

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Figure 2: (a) Histopathology showing dense histiocytic infiltrate in the upper and mid dermis with numerous foam cells and Touton giant cells (b) Immunohistochemical study showing CD1a negative and CD68 positive

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  Discussion Top


Many of the histiocytic disorders originating from the cells of the dermal dendrocyte lineage affect children, particularly juvenile xanthogranuloma (JXG), BCH and PX.

Papular xanthoma is a normolipaemic nonLangerhans cell histiocytosis and a rare disorder. Apart from children it is reported in adults as well. [1],[2] It is characterized by yellowish-brown papules and nodules over skin of the face, trunk, and limbs. In adults, mucous membrane is also involved, but not in children. In children the disease resolves spontaneously leaving anetoderma like scarring, [4] but in adults the disease may be progressive. [3] Systemic involvement does not occur. Serum lipid levels are always within normal limits [5] although qualitative abnormalities in lipoproteins may be present. [6]

Histologically, it is characterized by a dense distribution of foamy macrophages interspersed with numerous Touton giant cells.

The clinical, histologic and immunohistochemical findings are typical for PX and help to differentiate it from other xanthomatous lesions. In PX, histiocytes are positive for CD68 and factor XIIIa and negative for S100 and CD1a. [2],[4] A series noted positivity for macrophage markers such as KiM1p, CD68, and HAM-56. [7]

There are some case reports of PX occurring in association with systemic disorders. Darwin et al. have reported a case of generalized PX in a patient with Sezary syndrome. [8] Lima et al. reported a patient with cutaneous PX who later developed a CD3 (/+dim)/CD4(+) T-cell lymphoma. [9]

Though there is a widespread involvement of body by papules, a case of PX with localized involvement of knees has been reported. [10]

Treatment is not needed in children with PX as the condition is self-limiting in them. In adults, the disease is progressive, and no treatment has been reported to be effective. Though there is a case report of an adult PX being resolved satisfactorily with doxycycline. [11]

Juvenile xanthogranuloma is characterised by presentation in infancy, single or few lesions, involvement of mucous membranes, presence of telengiectasia and involvement of visceral organs such as lung, liver, spleen, testes, pericardium, gastrointestinal tract, kidney, deeper soft tissues and central nervous system. Histopathology (HPE) reveals mixed cellular dermal infiltrate comprising of histiocytes, lymphocytes, eosinophils, occasional neutrophils, plasma cells and extending into the subcutaneous fat.

Benign cephalic histiocytosis (considered by some to be a variant of JXG), also presents in early childhood. Papules, nodules or plaques first develop on the cheek and spread to the forehead, earlobes, and neck.

Xanthoma disseminatum presents with multiple papules and nodules, symmetrically distributed over the trunk, face, and extremities. These lesions become confluent to form plaques. Mucous membranes are involved in 30% of cases. Meningeal involvement is common and occur in the form of diabetes insipidus (40% of cases), seizures and growth retardation. HPE shows scalloped macrophages.

 
  References Top

1.
Winkelmann RK. Adult histiocytic skin diseases. G Ital Dermatol Venereol 1980;15:67-76.  Back to cited text no. 1
    
2.
Caputo R, Gianni E, Imondi D, Carminati G, Gianotti R. Papular xanthoma in children. J Am Acad Dermatol 1990;22:1052-6.  Back to cited text no. 2
    
3.
Beurey J, Lamaze B, Welere M. Xanthoma disseminatum (syndrome de Montgomery). Ann Dermatol Vénéréol 1979;106:353-9.  Back to cited text no. 3
    
4.
Fonseca E, Contreras F, Cuevas J. Papular xanthoma in children: Report and immunohistochemical study. Pediatr Dermatol 1993;10:139-41.  Back to cited text no. 4
    
5.
Winkelmann RK. Cutaneous syndromes of non-X histiocytosis. A review of the macrophage-histiocyte diseases of the skin. Arch Dermatol 1981;117:667-72.  Back to cited text no. 5
    
6.
Thomas RH, Miller NE, Payne CM, Black MM, Crow KD. Papular xanthoma associated with primary dysbetalipoproteinaemia. J R Soc Med 1982;75:906-8.  Back to cited text no. 6
    
7.
Breier F, Zelger B, Reiter H, Gschnait F, Zelger BW. Papular xanthoma: A clinicopathological study of 10 cases. J Cutan Pathol 2002;29:200-6.  Back to cited text no. 7
    
8.
Darwin BS, Herzberg AJ, Murray JC, Olsen EA. Generalized papular xanthomatosis in mycosis fungoides. J Am Acad Dermatol 1992;26:828-32.  Back to cited text no. 8
    
9.
Lima M, Velho G, Alves R, Cunha M, Teixeira Mdos A, Canelhas A, et al. Atopic dermatitis-like non-erythrodermic leukemic variant of CD3(-/+dim) CD4(+) cutaneous T-cell lymphoma preceded by cutaneous papular xanthomatosis. Leuk Lymphoma 2004;45:597-603.  Back to cited text no. 9
    
10.
Choi HJ, Chang SE, Lee MW, Choi JH, Moon KC, Koh JK. Primary papular xanthoma limited to the knees. J Dermatol 2004;31:773-5.  Back to cited text no. 10
    
11.
Bastida J, Camacho-Galán R, Armesto-Fernández MA, Díaz-Cascajo C. Adult disseminated primary papular xanthoma treated with doxycycline. Arch Dermatol 2007;143:667-9.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2]


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