Urticaria Pigmentosa in Children: A Case Report

Syafria Zidni

, Elin Herlina

, Retno Indar Widayati

, Diah Adriani Malik

Meira Dewi Kusuma Astuti

Department of Dermatology and Venereology, Diponegoro University/Dr. Kariadi Hospital

Departement of Pathology and Anatomical, Diponegoro University/Dr. Kariadi Hospital

*Corresponding author

Keywords: Urticaria pigmentosa, mast cell, ketotifen.

Abstract: Mastocytosis is a disorder of abnormal mast cell proliferation presented as an abnormal accumulation of

mast cells in various tissues. Urticaria pigmentosa (UP) is the most common form of this disorder in

children, particularly in the first year of life. Darier’s sign is pathognomonic for UP. Management for UP

consists of patient education, triggering factors exposure prevention and symptomatic treatment to reduce

the release of mast cells mediator.A5-year-old girl presented with brown spots and itch in various area of

the body with dry skin. Physical examination found multiple hyperpigmented macules on her body, arms,

legs and face. Darier’s sign was positive. Histopathology examination demonstrated mast cell granules in

the superficial layer of dermis with Giemsa staining, consistent with the diagnosis of UP. There is no

systemic involvement. Patient was treated with ketotifen syrup, 10% urea cream, and betamethasone

valerate 0,1% cream for the erythema lesions.Ketotifen is a mast cell stabilizer that was given to relieve the

symptoms of UP. Urea was given to reduce dry skin and enhance the absorption of betamethasone.

Betamethasone was given to reduce the lesions of UP. After 12 weeks, patient showed some improvement

in her pruritus and skin dryness. The prognosis was quo ad vitam, quo ad sanam and quo ad cosmeticam

dubia ad bonam.

1 INTRODUCTION

Mastocytosis is a disorder of abnormal mast cell

proliferation presented as an abnormal accumulation

of mast cells in various tissues, include the skin

and/or bone marrow. There are two types of this

disease, cutaneous mastocytosis and systemic

mastocytosis (Wagner et al, 2017; Tharp, 2012).

Urticaria pigmentosa is the most common form of

cutaneous mastocytosis in children with onset in the

first year of life, generally below two years of age

(Tharp, 2012; Vasani et al, 2015).

Mastocytosis is rare. The incidence of 2 cases

per 300.000 people per year is estimated and may be

observed in all ethnic group. The disease is higher

predominant in children and in adults between the

third and fourth decade of life. Children are more

affected before the age of six months in 50% of the

cases. There is no gender predilection (Wagner et al,

2017; Pires et al, 2018).

In patients with urticaria pigmentosa, there is an

abnormality in stem cell factor (SCF) metabolism

that affects in cell mast proliferation and

differentiation. SCF which is bound with membrane

was released, causing an increase of dissolved SCF,

and when accumulated it will stimulates melanocyte

to produces melanin (Tharp, 2012; James et al,

2011). In the other side, mast cells contain histamine

to be released which can cause redness on the skin,

swollen, and itchy (Tharp, 2012).

Urticaria pigmentosa lesions appear as multiple,

fixed, reddish-brown, hyperpigmented macules,

papules, and nodules that have a tendency to

coalesce into plaques and often exhibit increased

skin markings (Prose et al, 2008). In children, these

lesions may be present at birth or arise during

infancy. They frequently appear on the trunk and

often spare the central face, scalp, palms, and sole.

Rubbing or trauma of the affected skin results in a

weal with flare (Darier sign) in more than 90% of

patients. The diagnosis may be confirmed by skin

biopsy and histopathology examination that shows

mast cell infiltratesindicated with metachromatic

granules in cytoplasm using special stains, such as

366

Zidni, S., Herlina, E., Widayati, R., Malik, D. and Astuti, M.

Urticaria Pigmentosa in Children: A Case Report.

DOI: 10.5220/0009989103660370

In Proceedings of the 2nd International Conference on Tropical Medicine and Infectious Disease (ICTROMI 2019), pages 366-370

ISBN: 978-989-758-469-5

Toluidine or Giemsa (Tharp, 2012; Vasani et al,

2015; Gysel et al, 2011; Rapini, 2012).

The management of patients with urticaria

pigmentosa consists of patient education, triggering

factors exposure prevention and symptomatic

treatment to reduce the release of mast cells

mediator (Tharp, 2012; Gysel et al, 2011; Grattan et

al, 2010).

Pediatric-onset urticaria pigmentosa has a

favorable prognosis because the lesions usually

disappear within a few years, usually before puberty,

although in a few cases the lesions may persist into

adult life (Tharp, 2012; Vasani et al, 2015; Prose et

al, 2008).

This case report was made with the aim of better

understanding about diagnosis and management of

urticaria pigmentosa in children which is relatively

rare.

2 CASE

A girl who was taken to the Dr Kariadi General

Hospital Medical Center by her parents presented

with brown patches on some parts of her body.

Based on her parent’s recollection, the spots first

manifested when the patient was 4 months old. The

lesion started as red patches on the patient’s back,

which sometimes was accompanied by itching

especially when she was sweating, causing her to

habitually scratches her body. Eventually the patches

expanded to her face, neck, arms, trunk, and legs.

The color of the patches gradually became darker

and brown, accompanied by dry skin. There was no

family history a similar disease.

Physical examination showed that the

patient was in an excellent health condition, has

normal general health status, with a weight of 17 kg.

The dermatologic examination found erythematous

and hyperpigmented macular eruption, with fine

scales on the face, neck, trunk, arms and legs.

Positive Darier’s sign.

Figure 1: A,B,C. Generalized hyperpigmented macules before treatment. 2A,B,C. After 12 weeks treatment.

Urticaria Pigmentosa in Children: A Case Report

367

Laboratory tests found no abnormalities.

Histopathological examination by the use of special

Giemsa stain has found granulated mast cells in the

superficial dermal layer, consistent with the

diagnosis of mastocytosis. There were no systemic

abnormalities.

The patient was treated with Ketotifen syrup 1

mg bid, Urea 10% cream and Betamethasone cream.

The parents were also educated to avoid some

known triggering factors. Itching and dry skin has

showed some improvements after 12 weeks of

treatment. The patient was expected to have

spontaneous resolution at puberty.

Figure 2. A. Lymphocyte infiltration, histiocyte, and mast cell around skin adnexa. B &C.Mast cell granule (Giemsa, 400x)

3 DISCUSSION

UP was diagnosed based on the medical history,

clinical examination and histopathological

examination.

Based on the medical history, a 5-year-old girl

was taken to the Dr Kariadi General Hospital

Medical Center by her parents with brown patches

on some parts of her body. The spots first

manifested when the patient was 4 months old. The

lesion started as red patches on the patient’s back,

which sometimes was accompanied by itching

especially when she was sweating, causing her to

habitually scratches her body. Eventually the patches

expanded to her face, neck, arms, trunk, and legs.

The color of the patches gradually became darker

and brown, accompanied by dry skin. There was no

family history a similar disease. The literature

specified that UP is the most common variant of

pediatric mastocytosis, with onset in the first year of

life. The lesion usually manifested before the second

year of life, and the disease is usually confined to the

skin. There were no difference in prevalence

between male and female, all race can be affected.

(Tharp, 2012; Vasani et al, 2015; Pires et al, 2018)

The physical examination found erythematous

macular lesion, hyperpigmented macular lesion, and

fine scales on the face, neck, trunk, arms and legs.

Positive Darier’s sign. The literature mentioned that

UP usually manifested as reddish-brown patches,

papules, nodules, or plaques, with positive Darier’s

sign, where rubbing or scratching may cause the

formation of urticarial lesions surrounded with areas

of erythema. The lesion is usually distributed on the

trunk, but it may manifested on other body parts, or

even widespread (generalized). The lesion is usually

accompanied by itching, but there are no signs and

ICTROMI 2019 - The 2nd International Conference on Tropical Medicine and Infectious Disease

368

symptoms of systemic mastocytosis, such as

gastrointestinal problems, lymphedema, or skeletal

abnormalities. Pediatric consult found no systemic

involvements. This result corresponded with the

literature that specified that pediatric UP is mostly

confined to the skin, and rarely shows systemic

involvements. (Tharp, 2012; James et al, 2011).

Blood laboratory examination found no

abnormalities. Histopathological examination by the

use of special Giemsa stain has found purplish

stained granulated mast cells in the superficial

dermal layer. The literature mentioned that UP can

be diagnosed based on the result of skin biopsy and

histopathological examination by the use of special

stain to clearly examine the mast cells, such as

Giemsa or Toluidine Blue stain. . (Tharp, 2012;

Vasani et al, 2015;Gysel et al,2011;Rapini,2012).

Differential diagnosis of juvenile

xanthogranuloma can be excluded because the

lesions are mostly soft, well-defined papules or

nodules. The lesions are red-orange or red-brown in

color, and then it may change into yellowish color.

The lesion are mostly found at the upper body, and

in children, the lesion will spread quickly and then

spontaneously regress in approximately a

year.(Gelmetti,2012;Burgdof et al,2010)

Differential diagnosis of Spitz nevus can be

excluded because the lesions are mostly red to dark

brown, flat, smooth, hairless, hard, well-defined

papules or nodules, with the distribution in the head

and neck region.(Grichnik at al,2012;James et

al,2011)

The current case was treated with Ketotifen

syrup 1 mg bid, Urea 10% cream and

Betamethasonevalerate 0,1% cream. The parents

were also educated to avoid some known triggering

factors, such as temperature change, physical

activity, food, and nonsteroidal anti-inflammatory

drugs, and to avoid scratching or trauma to the skin.

The literature mentioned that the treatments are

usually symptomatic. Ketotifen is a mast-cell

stabilizer that has been shown to be effective in

reducing urtica and pruritus in patients with UP.Urea

10% cream helps to moisturize the skin and prevent

dryness, and to improve the absorption of

Betamethasonevalerate 0,1% cream. Very potent

topical corticosteroid applied with occlusion for 8-

12 weeks may reduce the number lesions in UP.The

current case was treated with betamethasonevalerate

0,1% cream, a medium potency topical

corticosteroid, that was applied to the erythematous

lesions onlyto alleviate the skin lesions.After 12

weeks of treatment, there were no new lesions,

itching was reduced, and there was no skin dryness.

(Wagner et al, 2017; Tharp, 2012). The prognosis of

the current patient was, quo ad vitam and quo ad

sanam dubia ad bonam because there were no

systemic involvement and the patient was expected

to have spontaneous resolution before puberty, quo

ad cosmeticamdubia ad bonam because of the

remaining hyperpigmented macules. (Wagner et al,

2017; Tharp, 2012;Prose et al,2008).

4 CONCLUSION

A case of UrticariaPigmentosa treated with

ketotifen, urea, and betamethasone has been reported

that can reduce the complaint and lesions.

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