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E-IJD® - CORRESPONDENCE
Year : 2022  |  Volume : 67  |  Issue : 4  |  Page : 482
Infantile urticaria pigmentosa with eosinophilic infiltration: A case report


From the Department of Dermatology, Fukushima Medical University, Fukushima, Japan

Date of Web Publication2-Nov-2022

Correspondence Address:
Masato Ishikawa
From the Department of Dermatology, Fukushima Medical University, Fukushima
Japan
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.ijd_887_20

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How to cite this article:
Ishikawa M, Hanami Y, Yamamoto T. Infantile urticaria pigmentosa with eosinophilic infiltration: A case report. Indian J Dermatol 2022;67:482

How to cite this URL:
Ishikawa M, Hanami Y, Yamamoto T. Infantile urticaria pigmentosa with eosinophilic infiltration: A case report. Indian J Dermatol [serial online] 2022 [cited 2022 Nov 26];67:482. Available from: https://www.e-ijd.org/text.asp?2022/67/4/482/360361




Sir,

A 7-month-old girl was referred to our department for pigmented lesions that had appeared one month after birth. The patient and her mother did not have any significant medical history. Physical examination revealed multiple brownish pigmented macules on the right head, face, chest, back and left lower limb [Figure 1]. Darier's sign was negative, and a biopsy was taken from the left lower limb. Histological examination of the biopsy showed pigmentation of the basal layer of the epidermis and dense mononuclear cell infiltration in the papillary dermis [Figure 2]a. Toluidine blue staining revealed a number of mast cell infiltration [Figure 2]b, and Dylon staining revealed an increased number of eosinophils [Figure 2]c. In addition, immunohistochemistry results revealed IL-5 positive cells within the upper dermis [Figure 2]d, and laboratory data showed peripheral blood eosinophilia (960 cells/mm3: normal <590). We diagnosed the eruptions as urticaria pigmentosa and followed up for three months without any treatment until the patient moved to another prefecture. Although the eruptions remained unchanged, the patient did not show any other symptoms during our follow-up period.
Figure 1: A 7-month-old girl showed multiple brownish pigmented macules on the right head, face (a) (arrowhead), chest (b) and left lower limb (c)

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Figure 2: (a) Histological examination of the biopsy showed pigmentation of the basal layer of the epidermis and dense mononuclear cell infiltration in the papillary dermis (hematoxylin-eosin staining, original magnification × 200). (b) Toluidine blue staining revealed many mast cells within the infiltrating cells (original magnification × 200). (c) Dylon staining revealed an increased number of eosinophils (original magnification × 200). (d) Immunohistochemistry results revealed interleukin 5-positive cells within the upper dermis (original magnification × 400)

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In this report, we showed a rare case of urticaria pigmentosa with eosinophilic infiltration in an infant. Urticaria pigmentosa, also known as maculo-papular cutaneous mastocytosis, usually shows generalized reddish-brown macules or papules.[1] A typical histopathologic finding is perivascular and interstitial infiltration of mast cells in the upper dermis. However, there are rare cases of urticaria pigmentosa accompanied by eosinophilic infiltration. To date, a few reports demonstrated that cutaneous mastocytosis was rarely accompanied by eosinophilic infiltration.[2],[3] The mechanism of eosinophilic infiltration in patients with urticaria pigmentosa has not yet been elucidated. A possible mechanism is that mechanical stress induces eosinophilic infiltration, as well as urticaria (Darier's sign).[2] Mast cells contain a variety of cytokines and growth factors, and mechanical stress induces mast cell degranulation. Degranulated mast cells that release prostaglandin D2, platelet-activating factor, and interleukin 5 (IL-5) can promote eosinophilic infiltration in skin lesions.[4] Our case is unique because the patient also showed peripheral blood eosinophilia. To our knowledge, cases of urticaria pigmentosa accompanied by eosinophilic infiltration do not show peripheral blood eosinophilia. A previous study demonstrated that inhalation of IL-5 in patients of asthma leads to the development of peripheral blood eosinophilia.[5] In addition, T helper 2 cells that release IL-5 are activated by mast cells via the interleukin 4 pathway.[4] In the present case, we examined whether IL-5-positive cells increased in the skin lesions of urticaria pigmentosa associated with eosinophilic infiltration, and there were IL-5 positive cells within the upper dermis. Thus, IL-5 released from mast cells and/or T helper 2 cells can cause eosinophilic infiltration in urticaria pigmentosa. In conclusion, we demonstrated a unique case of urticaria pigmentosa, which showed eosinophilic infiltration in the skin lesions and peripheral blood eosinophilia.

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.
Hartmann K, Escribano L, Grattan C, Brockow K, Carter MC, Alvarez-Twose I, et al. Cutaneous manifestations in patients with mastocytosis: Consensus report of the European competence network on mastocytosis; the American academy of allergy, asthma & immunology; and the European academy of allergology and clinical immunology. J Allergy Clin Immunol 2016;137:35-45.  Back to cited text no. 1
    
2.
Maehara E, Kido-Nakahara M, Ito T, Uchi H, Furue M. Darier's sign in urticaria pigmentosa–significance of perivascular eosinophilic infiltration. Allergol Int 2018;67:532-4.  Back to cited text no. 2
    
3.
Ishida M, Iwai M, Kagotani A, Iwamoto N, Okabe H. Cutaneous mastocytosis with abundant eosinophilic infiltration: A case report with review of the literature. Int J Clin Exp Pathol 2014;7:2695-7.  Back to cited text no. 3
    
4.
Kovalszki A, Weller PF. Eosinophilia in mast cell disease. Immunol Allergy Clin North Am 2014;34:357-64.  Back to cited text no. 4
    
5.
Adamjee J, Suh YJ, Park HS, Choi JH, Penrose J, Lam B, et al. Expression of 5-lipoxygenase and cyclooxygenase pathway enzymes in nasal polyps of patients with aspirin-intolerant asthma. J Pathol 2006;209:392-9.  Back to cited text no. 5
    


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