Bathing suit ichthyosis: Two Burmese siblings and a review of the literature.

Bathing suit ichthyosis (BSI) is a subtype of autosomal recessive congenital ichthyosis (ARCI) characterized by the development of large platelike scales mainly limited to the trunk. It is caused by temperature sensitive variants in transglutaminase 1, encoded by the gene TGM1. We describe a rare case of intrafamilial variation in phenotypic expressivity in two Burmese siblings with BSI that demonstrates the heterogeneity of the disorder within the same family and even in the same individual across time. We also present a concise review of the genotypic spectrum of BSI from 54 cases reported in the literature as evidence that both environmental and additional genetic factors can significantly alter the clinical phenotype.

Atopic dermatitis made easy: The Schachner Ladder.

The vast majority of atopic dermatitis follows a mild, chronic relapsing course. In this article, we highlight the art and practice of treating atopic dermatitis based upon a foundation of maintenance care and a ladder of therapy that can teach patients and their families how to best tailor their pharmaceutical options to optimize the management of their disease.

Drug reaction with eosinophilia and systemic symptoms: Pediatric case series and literature review.

BACKGROUND: Pediatric Drug reaction with eosinophilia and systemic symptoms (DRESS) is an uncommon disease that can be difficult to diagnose. This case series and literature review highlights the clinical features of pediatric DRESS and underscores the differential diagnoses, culprit medications, and need for clinical follow-up to detect associated autoimmune sequelae. OBJECTIVE: To describe the clinical and laboratory features of pediatric DRESS, identify associated culprit medications, and discuss the natural history of disease. METHODS: Ten cases of pediatric DRESS were identified in the electronic medical record by searching the inpatient dermatology consultation list at Indiana University between 2013 and 2018. Clinical and laboratory data were collected including demographics, differential diagnoses, culprit medications, resolution of disease, and autoimmune sequelae. RESULTS: Pediatric patients with DRESS presented at a mean age of 11.5 years and demonstrated a mean time from drug initiation to onset of symptoms of 4 weeks. The most common inciting drugs included antibiotics (62.5%) followed by antiepileptics (37.5%). Rash and transaminitis resolved by 3 weeks, and 20% of patients, all female, developed autoimmune sequelae including Hashimoto's thyroiditis and an undifferentiated connective tissue disorder and occurred at an average of 14.5 weeks after diagnosis. LIMITATIONS: This was a small retrospective study of an uncommon clinical diagnosis at a single institution. CONCLUSIONS: Pediatric DRESS was most commonly caused by antibiotics which are being increasingly recognized in the literature as the predominant culprit medications. The development of autoimmune sequelae is a notable consequence that can present weeks after illness and may preferentially affect female patients.

Short anagen syndrome: Case series and literature review.

BACKGROUND/OBJECTIVES: Short anagen syndrome is a hair cycle disorder usually diagnosed in early childhood and characterized by short hair length due to short duration of the anagen phase. The objective was to review the presentation and demographic characteristics of short anagen syndrome and compare them with the most common differential diagnosis, loose anagen syndrome. METHODS: A retrospective review of eight children with short anagen syndrome was performed at the University of Miami Outpatient Dermatology Clinic. RESULTS: The diagnosis of short anagen syndrome was confirmed according to clinical findings and characteristic short telogen hairs with pointed tips on the hair pull test. CONCLUSION: This is the largest reported clinical series of short anagen syndrome thus far in the literature.

Phaeohyphomycosis due to Exophiala infections in solid organ transplant recipients: Case report and literature review.

This case report and literature review underscores the cutaneous presentations of phaeohyphomycosis in the solid organ transplant population. Increased cognizance with prompt identification is critical. The therapy and clinical outcomes of phaeohyphomycosis, caused by the Exophiala genus, in the solid organ transplant population, is analyzed to examine optimal care. This review highlights the inherent difficulties in providing the appropriate duration of antifungal therapy to avoid relapsing infections in immunosuppressed patients.

Pediatric pearls from the AAD Annual Meeting.

This article summarizes novel pediatric dermatology clinical pearls and emerging literature highlights compiled from the 2017 Annual Meeting of the American Academy of Dermatology in Orlando, Florida.

Vesiculobullous and pustular diseases in newborns.

This article exhibits the most common presentations of vesiculobullous diseases in newborns and reviews the clinical characteristics unique to each diagnosis. Furthermore, a schematic for the workup of neonatal vesicular disorders is presented to empower dermatology residents to execute accurate diagnoses and maximize patient care.

Pediatric leg ulcers: going out on a limb for the diagnosis.

This article exhibits the most common differential diagnoses for pediatric leg ulcers and contrasts the etiologies with the adult population. The diagnoses are further categorized into hematologic, infectious, genodermatoses, and autoimmune etiologies to help the dermatologist deduce the accurate diagnosis in this unique patient population.

Five steps for delivering an effective and educational lecture.

This article highlights 5 essential components of creating and effectively delivering an educational lecture in the medical arena. Optimizing the transmission of knowledge is ideal for fostering an atmosphere that is conducive to all learners and helps physicians pursue their goal of lifelong learning.

Expanding uses of propranolol in dermatology.

Propranolol as a dermatologic therapeutic tool was first described in 2008. Since then, propranolol has had a pivotal role in the dermatology arena for a myriad of cutaneous disorders. This article highlights the timeline of the incorporation of propranolol as a treatment option for a number of vascular lesions.

Pediatric nail diseases: clinical pearls.

This article highlights pearls shared during a unique and enlightening lecture by Antonella Tosti, MD, a professor at the University of Miami Health System, Florida, on the presentation and management of common pediatric nail diseases. These clinical pearls are shared to help deliver utmost care to our pediatric patients presenting with nail pathology and may help shed light on the management of pediatric nail diseases.

Ichthyosiform Pityriasis Rubra Pilaris-Like Eruption Secondary to Ponatinib Therapy: Case Report and Literature Review.

Tyrosine kinase inhibitors have revolutionized the chemotherapy arena as targeted therapies for a multitude of malignancies. They are more selective than conventional chemotherapy, and often elicit fewer systemic adverse events, however toxicities still exist. Cutaneous toxicities are common and their management presents a novel challenge to physicians and patients. Ponatinib is a third-generation tyrosine kinase inhibitor increasingly reported to cause cutaneous eruption. A 50-year-old woman with a history of chronic myelogenous leukemia presented with a 4-month history of worsening atrophic and ichthyosiform pink plaques involving the axillae, thighs and abdomen; red patches were also observed on the cheeks and forehead. She was started on the third-generation, ponatinib, 5 months earlier because of disease refractory to previous therapies including interferon, imatinib, dasatinib and bosutinib. A skin biopsy revealed perifollicular fibrosis, alternating orthokeratosis and parakeratosis, and a sparse perivascular lymphocytic infiltrate consistent with a pityriasis rubra pilaris-like reaction. Topical tretinoin 0.025% cream was initiated, resulting in resolution within 3 weeks without discontinuation of ponatinib. A review of previous reports identified significant similarities among the ponatinib-induced drug reactions. Here, we highlight not only that cutaneous eruptions occur on ponatinib therapy, but that the dermatologic manifestations are characteristic and unique, and benefit from retinoid therapy, without requiring interruption of vital chemotherapy.