A woman in her 70s presented with a new 4-cm left upper lobe lung mass with hilar lymphadenopathy. Biopsy results demonstrated a squamous cell carcinoma (SCC) of the lung, and chemoradiotherapy with carboplatin and paclitaxel for stage 3A disease was initiated. An allergic reaction prompted a change to cisplatin-etoposide, which continued as adjuvant chemotherapy after the radiation was complete. At first restaging, a recurrence of disease in the lung was detected, which was treated with cisplatin and gemcitabine for 4 months before she enrolled in a clinical trial (NCT02009449) in which she received combination therapy with pembrolizumab, 2 mg/kg intravenously for 21 days, and an investigational agent, AM0010 (pegylated recombinant human interleukin-10), 10 μg/kg subcutaneously on days 1 through 14 of the 21-day cycle. Three months into treatment, she developed multiple thick, mildly pruritic, yellow hyperkeratotic plaques on her lower extremities (Figure , A). A referral to a dermatology expert was initiated. Clinical image A Histopathologic image B Histopathologic image C Figure. A, Clinical image showing representative plaque on left leg of patient on initial presentation. B, Shave biopsy of skin (hematoxylin-eosin; original magnification ×10). C, Deeper biopsy demonstrating bandlike lymphocytic infiltration (hematoxylin-eosin; original magnification ×20). WHAT IS YOUR DIAGNOSIS? A. Squamous cell carcinoma B. Plaque psoriasis C. Hypertrophic lichen planus D. Keratoacanthoma Clinical Review & Education
Stevens-Johnson syndrome and toxic epidermal necrolysis comprise a spectrum of severe mucocutaneous hypersensitivity reactions. A paucity of data limits current understanding of the etiology, treatment options, and prognosis of this entity in the infantile population compared to that in the adult and pediatric literature. We describe the case of an 8-week-old male with toxic epidermal necrolysis treated successfully with intravenous immunoglobulin and amniotic membrane transplant. This patient is the youngest surviving infant with toxic epidermal necrolysis to be reported.
Pemphigus and pemphigus‐like reactions can be triggered by a variety of medications including topical therapies, such as imiquimod. While the association between imiquimod and pemphigus‐like reactions has been reported in adults, this is the first report of a generalized reaction beyond the site of imiquimod application in a child. The mechanism by which this occurs may be through a unique pathway, separate from the classic antibody‐mediated pathway. Our patient had a full recovery without recurrence after cessation of the inciting drug.
Adams-Oliver syndrome (AOS) is a multiple congenital anomaly syndrome characterized by aplasia cutis congenita (ACC) and transverse terminal limb defects (TTLDs). We present a case of type 2 autosomal recessive AOS associated with heterozygous mutations in the dedicator of cytokinesis 6 (DOCK6) gene, with characteristic findings of ACC, TTLD, intracerebral periventricular calcifications, and polymicrogyria.
Author Contributions: Drs Shields and Rosenbach had full access to all of the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis. Concept and design: Shields, Gelfand, Rosenbach.
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