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An infant boy, born to a nonconsanguineously wed couple, presented to the dermatology outpatient clinic with recurrent skin blisters since age 2 months. The lesions initially started over the trunk and subsequently progressed to involve the face, hands, and feet. The episodes of skin blisters were accompanied by pruritus and redness all over the body. There was no history of oral erosions or difficulty in feeding. Bowel and bladder movements were normal. The child had an older sibling who had no skin ailments, and none of the other family members were affected.
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C. Bullous mastocytosis
Darier sign, urtication, and erythematous halo produced in response to rubbing and scratching was diffusely positive over the trunk. The skin biopsy from the vesicle revealed clefting at the dermo-epidermal junction containing fibrin. Upper dermis showed bandlike inflammatory infiltrate comprising mast cells that had ovoid- to spindle-shaped nuclei. Some scattered eosinophils were also noted (Figure, C and D). The mast cells stained positive for CD117 and toluidine blue. Results of immunofluorescence studies from perilesional skin were negative. The diagnosis of bullous mastocytosis was thus confirmed.
Complete blood cell count and liver function test results were found to be normal. Ultrasonography did not reveal any organomegaly. Owing to lack of any evidence of systemic involvement, bone marrow studies were not done. The child was prescribed hydroxyzine syrup, 0.5 mg/kg, per day with topical fluticasone propionate cream, 0.05%, to be applied twice daily. The episodes were well controlled while the child took antihistamines with mild flare-up during episodes of upper respiratory tract infections. The parents were told the necessary precautionary measures such as avoidance of extreme temperatures, rubbing of the skin, insect bites, and physical trauma and were also provided with a detailed safe drug list that avoided the mast cell–degranulating agents. The possible perioperative complications and instructions were also provided to them.
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Corresponding Author: Keshavamurthy Vinay, MD, Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Sector 12, Chandigarh, India (firstname.lastname@example.org).
Published Online: January 2, 2020. doi:10.1001/jamadermatol.2019.4038
Conflict of Interest Disclosures: None reported.
Additional Contributions: We thank the patient’s father for granting permission to publish this information.
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