Presentation of ichthyosis after substrate reduction therapy in Gaucher type 1.

We describe a case in which a type 1 Gaucher patient developed ichthyosis weeks after starting substrate reduction therapy (SRT) with eliglustat. There are no reports of ichthyosis in the literature in enzyme replacement or SRT for Gaucher disease. Ichthyosis is seen with type 2 and 3 Gaucher disease, but not type 1. This raises the question: Why would a patient develop ichthyosis after starting SRT?

Clinical and Visual Outcomes of Four Presbyopia Correcting Intraocular Lenses.

Purpose: To compare objective and subjective outcomes of the multifocal intraocular lenses ReSTOR SN6AD1 and Tecnis ZKB00, extended depth of focus IOL Symfony ZXR00, and trifocal IOL PanOptix TFNT00. Methods: This study included 262 patients (524 eyes) who had phacoemulsification with IOL implantation, 128 eyes with SN6AD1, 124 eyes with ZKB00, 136 eyes with ZXR00, and 136 eyes with TFNT00. Objective outcomes included one-month postoperative uncorrected (U) and corrected (C) distance (D) and near (N) visual acuities (VA). Subjective outcomes included photic phenomena, spectacle use, and spectacle-independent visual function. Results: Spectacle use (%) in the SN6AD1, ZKB00 ZXR00, and TFNT00 groups was 39, 64, 87, and 37 respectively (P < 0.0001). Presence of photic phenomena (%) for SN6AD1, ZKB00, ZXR00, and TFNT00 was 66, 61, and 67, and 73, respectively (P = 0.57). Spectacle-independent mean VF-14 score (%) for SN6AD1, ZKB00, ZXR00, and TFNT00 was 89.5, 87.2, 80.9, and 83.6, respectively (P < 0.01). Conclusion: All four IOLs provided excellent postoperative visual acuity and equally high rates of photic phenomena. SN6AD1 and TFNT00 provided the least spectacle use while ZXR00 had the highest spectacle use.

Zosteriform cutaneous metastases from urothelial carcinoma: Report of a rare case and literature review.

Zosteriform cutaneous metastases from urothelial carcinoma are rare. Here, we report a 50-year-old male with urothelial carcinoma who presented with multiple tender, erythematous papulonodules in an L1-L3 distribution approximately 6 years after primary tumor diagnosis. He had no history of prior herpes zoster infection. Histopathology showed lobules and small nests of atypical epithelioid cells positive for GATA3, CK20, CK7, and p40 throughout the dermis and within lymphatic vessels highlighted by D2-40, consistent with cutaneous metastases from urothelial carcinoma. No perineural invasion or viral cytopathic change was present. The patient died approximately 8 months after diagnosis of cutaneous metastases. Since its first report in 1986, there have been only six cases of zosteriform cutaneous metastases from urothelial carcinoma. We review the prior literature including hypotheses of the pathogenesis of zosteriform cutaneous metastases, which remain incompletely understood.

Dermatologic Sequelae Associated with Radiation Therapy.

Radiation therapy is a mainstay for the treatment of primary malignancies and metastatic disease and is associated with several dermatological adverse events that are underreported in the literature. The objective of this paper was to review the literature regarding cutaneous manifestations associated with radiation therapy in order to promote awareness of the cutaneous radiation therapy-associated adverse effects. This extensive literature review was performed using the Pubmed and Embase databases. Studies were reviewed for relevance and critical evidence pertaining to cutaneous manifestations of radiation therapy. The most commonly reported cutaneous sequelae associated with radiation therapy include radiation dermatitis, non-melanoma skin cancer, radiation-associated angiosarcoma, morphea, bullous pemphigoid, lymphangioma circumscriptum, and pseudosclerodermatous panniculitis. For many of these conditions, only case reports or case series exist and there is an absence of large-scale cohort studies. Despite these limitations, this review describes the wide range of dermatological adverse events associated with radiation therapy.