Sharon A. Glick

Lines of Blaschko.

The lines of Blaschko represent a pattern followed by many skin disorders. We review the clinical and histologic features of X-linked, congenital/nevoid, and acquired skin diseases that follow these lines. We also include cutaneous disorders that have a linear distribution but do not follow Blaschkos lines. Finally, we differentiate Blaschkos lines from other patterns on the skin such as dermatomes and Langers lines.

Hypopigmented mycosis fungoides in childhood and adolescence: a long-term retrospective study.

Patients with hypopigmented mycosis fungoides (HMF) present at a younger age than those with classic MF. Our goal was to describe the clinical presentation, histopathologic features and long‐term outcome in patients who developed HMF before the age of 21. It was observed that among 69 pediatric patients diagnosed with MF between 1992 and 2010, 50 had HMF. Thirty‐five patients had clinical follow‐up. There were 37 males and 32 females with a mean age of 13.6 years. Most patients were African American or Hispanic and presented with multiple hypopigmented patches. All biopsies showed epidermotropism of T‐lymphocytes, whereas fibroplasia and lichenoid infiltrate were variable. All specimens tested were CD8+. Treatment modalities included topical steroids, narrow band ultraviolet B and psoralen and ultraviolet A. HMF patients were followed for <1–12 years. Most children responded to treatment, but recurrence rates were high. One patient progressed to plaque/tumor stage. Others did not progress; however, many were lost to follow‐up. We present a large cohort of children with HMF and report on the features of disease and progression. A major difference in histology of HMF was lack of fibroplasia and lichenoid infiltrate, probably because of presentation in the early patch stage. Most patients have a waxing‐and‐waning course and relapse after discontinuation of therapy, requiring repetitive treatment.

Childhood acne: evaluation and management

ABSTRACT:  Acne is a disease that can be seen in the first year of life, early childhood, prepubertal age, and puberty. The purpose of this article is to review the clinical presentation and pathogenesis of the various forms of prepubertal acne and to propose guidelines regarding its evaluation and treatment. The early clinical recognition of the disease and prompt initiation of therapy in these age groups will help prevent the sequelae of emotional distress and severe scarring in both the child and parents.

Sirtuins in dermatology: applications for future research and therapeutics.

Sirtuins are a family of seven proteins in humans (SIRT1–SIRT7) that are involved in multiple cellular processes relevant to dermatology. The role of sirtuins in other organ systems is established. However, the importance of these proteins in dermatology is less defined. Recently, sirtuins gained international attention because of their role as “longevity proteins” that may extend and enhance human life. Sirtuins function in the cell via histone deacetylase and/or adenosine diphosphate ribosyltransferase enzymatic activity that target histone and non-histone substrates, including transcription regulators, tumor suppressors, structural proteins, DNA repair proteins, cell signaling proteins, transport proteins, and enzymes. Sirtuins are involved in cellular pathways related to skin structure and function, including aging, ultraviolet-induced photoaging, inflammation, epigenetics, cancer, and a variety of cellular functions including cell cycle, DNA repair and proliferation. This review highlights sirtuin-related cellular pathways, therapeutics and pharmacological targets in atopic dermatitis, bullous dermatoses, collagen vascular disorders, psoriasis, systemic lupus erythematosus, hypertrophic and keloid scars, cutaneous infections, and non-melanoma and melanoma skin cancer. Also discussed is the role of sirtuins in the following genodermatoses: ataxia telangiectasia, Cowden’s syndrome, dyskeratosis congenita, Rubenstein–Taybi, Werner syndrome, and xeroderma pigmentosum. The pathophysiology of these inherited diseases is not well understood, and sirtuin-related processes represent potential therapeutic targets for diseases lacking suitable alternative treatments. The goal of this review is to bring attention to the dermatology community, physicians, and scientists, the importance of sirtuins in dermatology and provide a foundation and impetus for future discussion, research and pharmacologic discovery.

Prenatal diagnosis of genodermatoses: current scope and future capabilities

The genodermatoses encompass a range of inheritable skin diseases that may be associated with significant mortality and long‐term morbidity. In the past, options for prenatal diagnosis of these diseases were limited to fetal skin biopsy. As a result of recent leaps made in genetics and molecular biology, DNA‐based prenatal diagnosis is now available for an increasing number of genodermatoses, and newer non‐invasive methods are being developed that have the potential for tremendous future impact in dermatology. Dermatologists caring for patients with genodermatoses should be aware of the options for screening and prenatal testing and partake in a multi‐disciplinary approach to patient care.

Procedures and drugs in pediatric dermatology: Iatrogenic risks and situations of concern

Over the past several decades, improved technologies used in the care of hospitalized and outpatient pediatric populations have resulted in a decreased but still significant number of iatrogenic injuries. Children at the highest risk for cutaneous injury include those with the most immature skin barriers, such as neonates younger than 32 weeks of gestational age. Additional risk factors include low birth weight, increased length of hospital stay, and indwelling instrumentation. Also at risk are older children with compromised skin barriers owing to infectious disease (staphylococcal scalded skin syndrome), inflammatory disease (atopic dermatitis), drug eruptions, and inherited or acquired blistering disorders. This review highlights the presentation, course, and management of iatrogenic skin injury events in children.

In-Utero Presentation of Aggressive Systemic Mastocytosis in a Neonate.

Mastocytosis is a clinically heterogenous disease characterized by mast cell hyperplasia in skin, bone marrow and/or visceral organs. Cutaneous mastocytosis is more frequently observed in children, whereas indolent systemic mastocytosis is more commonly observed in adults. Aggressive systemic presentation, particularly of the neonate, is exceptionally rare. We present a rare case of congenital aggressive systemic mastocytosis. The patient was a 37‐week‐old male, born by caesarean section owing to hepatosplenomegaly and ascites diagnosed in utero, who exhibited extensive cutaneous and systemic manifestations of mastocytosis at birth. Mutation analysis of c‐KIT identified D816V mutation in exon 17. Although initial bilateral bone marrow aspirates demonstrated no mast‐cell infiltrates or haematological neoplasm, subsequent bone‐marrow biopsies postmortem exhibited multifocal mast‐cell aggregates. Clinical course was complicated by bacteraemia and cardiorespiratory failure, leading to death at 10 weeks.

Genetic susceptibility to cutaneous radiation injury

The use of ionizing radiation is critical to cancer treatment and fluoroscopic procedures. However, despite efforts to minimize total radiation dose, many patients experience toxic cutaneous side-effects of ionizing radiation, ranging from mild erythema to subcutaneous fibrosis, telangiectasia formation, and ulceration. Extent of injury is highly variable among patients. Studying the genetic determinants of radiation injury can help develop protocols to reduce radiation toxicity, as well as drive research into effective modulators of the genes and gene products associated with radiation injury. Many studies in the past two decades have identified single-nucleotide polymorphisms that may be associated with susceptibility to cutaneous radiation injury, such as those in genes related to the following cellular responses to ionizing radiation: inflammation, DNA repair, oxidation and stress response, and cell-cycle and apoptosis. This review summarizes the current literature on potential major genes and polymorphisms, in the previously described damage response pathways, that are involved in susceptibility to cutaneous radiation injury. Potential pitfalls of current research and further avenues of discovery will be explored.

Lack of evidence that bedbugs transmit pathogens to humans

To the Editor: The global population of bedbugs that feed on humans (Cimex lectularius, Cimex hemipterus, and Leptocimex boueti) has undergone a significant resurgence since the late 1990s. Due to increased international travel and pesticide resistance, bedbugs once thought to be native to certain geographic locations have been found in other parts of the world. Bedbugs present a socioeconomic burden because they are costly to eradicate and infestations often recur. According to the US Environmental Protection Agency, bedbugs are ‘‘a pest of significant health importance,’’ and upwards of 45 disease pathogens have been reported in bedbugs. It stands to reason to ask if bedbugs might transmit human pathogens. We performed a literature review on August 6, 2015, and searched the computerizedmedical bibliographic databases PubMed, EMBASE, CINAHL, and Web of Science with the search terms: ‘‘bedbug’’ OR ‘‘cimex lectularius.’’ A total of 1790 articles were returned, and 12 articles were suitable for inclusion (clinical and laboratory published studies [1990 to 2015] investigating bedbugs as potential vectors of infectious disease) after screening of titles, abstracts, and/or full-text articles. These articles demonstrated that although bedbugs may carry pathogens such as methicillin-resistant Staphylococcus aureus, vancomycin-resistant Enterococcus faecium, and hepatitis B virus, and may be competent vectors of Bartonella quintana and Trypanosoma cruzi, there were no confirmed cases of human disease transmission. Previous reports suggest that, although a number of different disease pathogen species have been detected in or on bedbugs, there is a lack of definitive evidence that bedbugs transmit human pathogens. An important challenge for scientists is to determine the reason for this interesting finding.

Cutaneous Langerhans cell histiocytosis presenting with hypopigmented lesions: Report of two cases and review of literature

Langerhans cell histiocytosis is a rare group of disorders that results from the abnormal proliferation and accumulation of dendritic‐derived cells in various organs of the body, such as the skin and bones. Hypopigmented macules are a rare cutaneous presentation of Langerhans cell histiocytosis that may pose a diagnostic dilemma when no other findings of Langerhans cell histiocytosis are present at the time of examination. We present 2 cases of the hypopigmented variant of Langerhans cell histiocytosis, including a case with histopathologic features of regression, and a review of the literature. These cases highlight the importance of including Langerhans cell histiocytosis in the differential diagnosis of an infant with hypopigmented macules and papules.