22 September, 2018
The patient is a 2 yr and 10 month old girl with a few month history of a facial eruption. It began as hive-like plaques but has evolved over the past few weeks. Her paternal grandmother has Alpha-1 Antitrypsin disease and the child is a carrier. No family history of collagen vascular disease. She takes no medications save vitamins.
CBC, BUN, ANA: all normal or negative. ESR 7
Urine analysis normal
Pathology: The slides were read by Deon Wolpowitz and the photmicrographs were taken by Erin Tabata, both of Boston University Department of Skin Pathology.
Intermittent compact hyperkeratosis with parakeratosis, intermittent basal layer vacuolization with squamatization of the basal cell layer and occasional individually necrotic keratinocytes and mild lymphocytic exocytosis, papillary dermal edema that is focally prominent in a few papillae, ectatic blood vessels, and a moderate superficial and deep perivascular and interstitial, and focally periappendageal, lymphohistiocytic infiltrate with few extravasated erythrocytes.
Diagnosis: Polymorphous Light variant. This is not the common papulovesicular variant of PMLE.
Plan: Initially, she will use broad-spectrum sun-blocks containing zinc oxide or titanium dioxide and a large floppy hat whilst outdoors. As the season changes, and the light wanes, she should improve. Topical corticosteroids, calcineurin inhibitors, and hydroxychloroquine will be considered if her parents want further therapy. Phototesting at a centre is disruptive for this child, at this time.
What are your thoughts.
1.Alexis L. Dougherty, Cloyce L. Stetson, MD, Dr. Khachemoune. What are These Facial Plaques in a 4 year-old Child. The Dermatologist. 8.20.2013 Link