Images in paediatrics Arch Dis Child: first published as 10.1136/archdischild-2017-312704 on 26 May 2017. Downloaded from An infant with diffuse bullous lesions

Case history, and outcome A 7-month-old boy was admitted with generalised blisters to the paediatric emergency room. The lesions had first devel- oped on the head and neck, then rapidly spread to the trunk and extremities (figure 1). The patient was ill but not toxic or febrile. Initially bullous impetigo was suspected, and the patient was empirically treated with intravenous vancomycin. The parents subsequently reported that their infant has had pruritic macules and transient blisters on his scalp since 5 months of age. Also skin Figure 2 (A) Subepidermal bullae with a dense cellular infiltration in examination revealed discrete elevated rough surface macules the upper dermis (H&E ×10). (B) Dense mast cell infiltration with some with leathery appearance. Diffuse cutaneous mastocytosis was eosinophils in the upper dermis (H&E×20). (C) With Giemsa staining, then confirmed on skin biopsy (figure 2). cells showed metachromatic granules characteristic of mast cells ×40. The antibiotics were discontinued and oral prednisolone 1Skin Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran (0.5 mg/kg/day for 1 week and then tapered), oral hydroxyzine 2Department of Pathology, Shahid Beheshti University of Medical Sciences, Loghman- and topical betamethasone ointment were administered. The Hakim Hospital, Tehran, Iran patient’s symptoms improved within 1 week. Correspondence to Dr Nikoo Mozafari, Skin Research Center, Shohada e Tajrish hospital, Tajrish Square, Tehran, Iran; nikoo_​ ​md@yahoo.​ ​com Key learning points Competing interests None declared. Cutaneous mastocytosis has three major types: maculopapular Patient consent Obtained. type (urticaria pigmentosa), diffuse cutaneous mastocytosis Provenance and peer review Not commissioned; internally peer reviewed. 1 (DCM) and solitary mastocytoma. © Article author(s) (or their employer(s) unless otherwise stated in the text of the In DCM, skin infiltrations of mast cells produce thick and article) 2018. All rights reserved. No commercial use is permitted unless otherwise leathery skin (peau d’orange appearance), plaques and nodules.2 expressly granted. copyright. Blistering and bulla can be the first presenting symptoms of diffuse cutaneous mastocytosis, and the blisters may bleed or rupture, leaving erosions and crusts. Bullous eruptions may be 2 3 To cite Nasiri S, Bidari Zerehpoosh F, Dadkhahfar S, et al. Arch Dis Child caused by serine protease released from mast cells. 2018;103:335. Accepted 25 April 2017

Conclusions Published Online First 26 May 2017 http://adc.bmj.com/ Diffuse cutaneous mastocytosis in children is rare and underdi- Arch Dis Child 2018;103:335. doi:10.1136/archdischild-2017-312704 agnosed. When it presents with widespread bullae, DCM has a relatively poor prognosis and may be associated with life-threat- References ening complications (flushing, anaphylactic shock, abdominal 1 Castells M, Metcalfe DD, Escribano L. Diagnosis and treatment of cutaneous pain and gastrointestinal bleeding).1 Therefore, it is important mastocytosis in children: practical recommendations. Am J Clin Dermatol 2011;12:259–70. to differentiate from other bullous skin disorders observed in 2 Lange M, Nedoszytko B, Górska A, et al. Mastocytosis in children and adults: clinical

4 on 14 September 2018 by Anne Meneghetti. Protected infants (bullous impetigo, epidermolysis bullosa and contact disease heterogeneity. Arch Med Sci 2012;8:533–41. dermatitis) by skin biopsy. 3 Frieri M, Quershi M. Pediatric Mastocytosis: A Review of the Literature. Pediatr Allergy Immunol Pulmonol 2013;26:175–80. Soheila Nasiri,1 Farahnaz Bidari Zerehpoosh,2 Sahar Dadkhahfar,1 4 Salas-Alanis JC, Rosales-Mendoza CE, Ocampo-Candiani J. Bullous mastocytosis NafisehM ortazavi,2 Nikoo Mozafari1 mimicking congenital epidermolysis bullosa. Case Rep Dermatol 2014;6:129–33.

Figure 1 Multiple tense vesicles and haemorrhagic bullae developed on the peau d'orange-like skin on the face and scalp (A) and trunk (B).

Nasiri S, et al. Arch Dis Child April 2018 Vol 103 No 4 335