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  In this article
    Abstract
    Introduction
    Case Report
    Discussion
    Acknowledgement
    References

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CASE REPORT
Year : 2001  |  Volume : 67  |  Issue : 1  |  Page : 33-34

Urticaria pigmentosa


Department of Pathology, Government Medical College, Chandigarh, India

Correspondence Address:
Department of Pathology, Government Medical College, Chandigarh, India

   Abstract 

A 2-year- old child with multiple brownish papules over trunk and limbs for one year having history of occasional blister formation is described. Histopathology revealed perivascular mast cell infiltration. Symptomatic treatment with topical corticosteroids and oral antihistaminics was effective.

How to cite this article:
Ritambhra, Mohan H, Tahlan A. Urticaria pigmentosa. Indian J Dermatol Venereol Leprol 2001;67:33-4


How to cite this URL:
Ritambhra, Mohan H, Tahlan A. Urticaria pigmentosa. Indian J Dermatol Venereol Leprol [serial online] 2001 [cited 2014 Apr 18];67:33-4. Available from: http://www.ijdvl.com/text.asp?2001/67/1/33/8131



   Introduction Top

Urticaria pigmentosa is the most common variant of cutaneous mastocytosis. The disease in­cludes urticaria pigmentosa, mastocytoma, telang­iectasia macularis eruptiva perstans, diffuse masto­cytosis and systemic mastocytosis. Mastocytosis is uncommon in any form.[1],[2] In about 70-75% of cases, lesions of mastocytosis are evident before the age of 2 years.[3] A typical case of this dermatosis is being presented.

   Case Report Top

A-2-year- old female child presented with generalized eruption of multiple brownish macular and papular lesions on the trunk and limbs of one year duration. There was history of generalised urti­carial flushing and bulla formation. There was no family history of similar disease and the general health, growth and development of the child was unaffected.
On examination, there were multiple hyper ­pigmented papular lesions on the trunk and limbs. However, there were no bullae at the time of exami­nation. On stroking the individual lesion, there was formation of wheal and erythema (Darier's sign- posi­tive). There was no hepatosplenomegaly or lymphad­ enopathy.
Histopathological examination of lesional skin showed infiltration of numerous mast cells in perivas­cular and periappendageal location in the upper der­mis. There were no nodular aggregates or sheets of mast cells. Mast cell granules were demonstrable on metachromatic stain as bluish purple granules [Figure - 1]. A few eosinophils were also present in the dermis. Overlying epidermis showed increased amount of melanin and many melanophages were scattered in the upper dermis. Features were consistent with the clinical diagnosis of urticaria pigmentosa. Complete blood count, liver and renal functions, urinanalysis and chest roentgenogram were normal. Lesions re­gressed with topical application of fluocinolone acetonide, 0.01% ointment applied twice daily along with administration of chlorpheniramine maleate, 2 mg thrice daily for four weeks. Necessary advice to parents was given regarding avoidance of excessive scrubbing and massage of the skin. At the end of 8 weeks the child had only residual pigmentation left at the lesional sites with no new lesion formation.

   Discussion Top

Urticaria pigmentosa is a rare disorder of mast cell infiltration. Primarily a disease of childhood, in over half the cases onset is before the age of 2 years and in 90% the disease is confined to the skin.[4] Lesions which manifest in childhood usually improve by puberty. Exact aetiology is not known. However, it is believed to be proliferation of mast cells medi­ated through cytokines. Interestingly the prognosis is best in childhood cases; bullae formation, how­ever extensive, is not a sign of poor prognosis.
Present case is a typical case of cutaneous mastocytosis without systemic involvement which responded well to symptomatic treatment with topi­cal corticosteroid and H1 antihistaminics.

   Acknowledgement Top

The contribution of Dr. G.P. Thami, Reader, Department of Derma­tology & Venereology is gratefully acknowledged. 

   References Top

1.Klaus S N, Winkelmann R K. Course of urticaria pigmentosa in children. Arch Dermatol 1962; 86: 68-71.  Back to cited text no. 1    
2.Harvard CWH, Scott R B. Urticaria with visceral and skeletal lesions. Q J Med 1959; 28: 459- 461.  Back to cited text no. 2    
3.Caplan RM. The natural history of urticaria pigmentosa. Analysis and follow up of 112 cases. Arch Dermatol 1963; 87: 146- 148.  Back to cited text no. 3    
4.Elisabeth H, Anthony DV (eds). Skin Disease in Childhood and Adolescence, Germany. Blackwell Scientific Publications; 1996: 225-256.  Back to cited text no. 4    

 

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