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Year : 2015  |  Volume : 6  |  Issue : 6  |  Page : 464-465  

Urticaria pigmentosa

1 Department of Dermatology, KJ Somaiya Medical College and Research Centre, Mumbai, Maharashtra, India
2 Department of Dermatology, Government Medical College, Aurangabad, Maharashtra, India

Date of Web Publication17-Nov-2015

Correspondence Address:
Resham J Vasani
C-1 22, Karmakshetra, Near Shanmukhananda Hall, Sion, Mumbai, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2229-5178.169727

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How to cite this article:
Vasani RJ, Medhekar SV. Urticaria pigmentosa. Indian Dermatol Online J 2015;6:464-5

How to cite this URL:
Vasani RJ, Medhekar SV. Urticaria pigmentosa. Indian Dermatol Online J [serial online] 2015 [cited 2021 Jun 22];6:464-5. Available from: https://www.idoj.in/text.asp?2015/6/6/464/169727

A 6-year-old female, presented with multiple dark-colored lesions, which started over the scalp and further progressed to involve the face and trunk since past six months. She gave a history of elevation, redness, and itching on the lesions on application of pressure. There was no associated flushing, vomiting, diarrhoea, or wheeze. The personal and family history was not contributory.

On examination, there were multiple hyperpigmented macules over the scalp, forehead, face, and neck in addition to minimally elevated hyperpigmented plaques over the chest and the back [Figure 1]a,[Figure 1]b,[Figure 1]c. Gentle rubbing of the lesions elicited urtication and itching within 2 min and it resolved within 15-20 minutes, suggestive of the Darier's sign [Figure 1]d.
Figure 1: (a) Multiple ill-defined hyperpigmented macules on the forehead, nose, and the upper lip, (b) multiple ill-defined hyperpigmented macules over the scalp, (c) minimally elevated hyperpigmented plaques on the chest, and (d) positive lesional Darier's sign

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A complete blood count, liver and renal functions, urine analysis, and chest roentgenogram were normal. The histopathology of a minimally elevated lesion from the back demonstrated increased melanization of the basal layer with sheets of oval mast cells infiltrating the upper and lower dermis [Figure 2]a,[Figure 2]b,[Figure 2]c. The metachromatic granules of the mast cells were visualized using the toulidine blue stain [Figure 2]d. The diagnosis was thus established as urticaria pigmentosa.
Figure 2: (a) 4×—Scanner view: Sheets of mast cells seen occupying the upper mid and the lower dermis, (b) 10×—Basal layer hyperpigmentation with oval mast cells seen infiltrating the upper and mid-dermis without any evidence of epidermotropism, (c) 40×—Sheets of oval monomorphic mast cells, (d) Oil immersion-toulidine blue stain demonstrating the metachromatic granules of the mast cells

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Urticaria pigmentosa is the most common form of mastocytosis with onset generally below two years of age. The lesions are rose colored, pruritic, urticarial, slightly pigmented macules, papules, or nodules. The nonevanescent urticated lesions persist and gradually become pigmented. The increased number of the functionally normal mast cells in the dermis degranulate in response to stroking with the release of histamine, which is responsible for the local itching, erythema, and weal formation or the Darier's sign.[1] The lesions usually disappear within a few years, usually before puberty, although in a few cases the lesions may persist into adult life. Although systemic involvement is possible, malignant transformation is exceedingly rare.

The multiplicity of lesions on the scalp is an uncommon finding in the case discussed.[2]

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.

   References Top

Surjushe A, Jindal S, Gote P, Saple DG. Darier's sign. Indian J Dermatol Venereol Leprol 2007;73:363-4.  Back to cited text no. 1
[PUBMED]  Medknow Journal  
Gratlan CE, Kobza Black A. Urticaria and mastocytosis. In: Burns T, Breathnach S, Cox N, Griffiths C, editors. Rook's Textbook of Dermatology. 8th ed. Singapore: Wiley-Blackwell; 2010. p. 22.1-22.36.  Back to cited text no. 2


  [Figure 1], [Figure 2]


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