Search

BACKGROUND Accurate diagnosis of epidermolysis bullosa (EB) has significant implications for prognosis, management, and genetic counseling. OBJECTIVE To describe diagnostic testing patterns and assess diagnostic concordance of transmission electron microscopy (TEM), immunofluorescence mapping (IFM), and genetic analysis for EB. METHODS A retrospective cohort included patients enrolled in the Epidermolysis Bullosa Clinical Characterization and Outcomes Database from January 1, 2004, to July 8, 2019. Tests concluding the same EB type (EB simplex, junctional EB, dominant dystrophic EB, and recessive dystrophic EB) were considered concordant; those concluding different EB types were considered discordant; and those with nonspecific/nondefinitive results were equivocal. RESULTS A total of 970 diagnostic tests were conducted from 1984 to 2018 in 771 patients. Genetic analyses were performed chronologically later than IFM or TEM (P

Background/objective Herpes simplex virus (HSV) infection acquired in utero may present with non-vesicular dermatologic findings in affected newborns, which may pose a diagnostic dilemma. We aimed to describe and assess the range of non-vesiculobullous skin lesions that neonates with intrauterine HSV infection may manifest at birth. Methods We collected a multicenter case series and conducted a literature review of neonates with intrauterine HSV infection presenting with non-vesiculobullous cutaneous lesions. Results Twenty-two cases were reviewed, including six managed clinically by members of our team and 16 identified in the literature. Four (18%) were associated with twin pregnancies, and thirteen (59%) cases occurred in premature infants. Only four (18%) mothers had a documented history of HSV infection. Twelve (55%) cases resulted in poor outcomes, including long-term neurologic sequelae or death. Cutaneous manifestations included erosions, ulcerations, crusted papules or plaques, calcinosis cutis, excoriations, macules (erythematous, hypopigmented, or hyperpigmented), cutaneous atrophy, contractures, and bruising. About one-third of neonates developed new-onset vesicular lesions within a week of birth; in each of these cases, accurate diagnosis and therapy were delayed until appearance of vesicles. Conclusions The range of dermatologic findings associated with intrauterine HSV is extremely broad, and the various morphologies present at birth likely reflect different stages of the ongoing evolution of an HSV infection that began in utero. Clinicians should have a low threshold for HSV testing in premature neonates born with atypical cutaneous lesions, since early detection and treatment of HSV may reduce morbidity and mortality from systemic complications.

Multisystem inflammatory syndrome in children (MIS-C) is a syndrome associated with coronavirus disease 2019. Various phenotypes of MIS-C have been described including Kawasaki disease (KD). Although perineal desquamation is a known early sign of KD, to our knowledge, this rash has not yet been described in the KD phenotype of MIS-C. In this article, we report two patients in whom perineal desquamation was an early clue for the KD phenotype of MIS-C.

Background/objectives Epidermolysis bullosa (EB) comprises a group of inherited skin blistering diseases. There is currently no cure, and management includes skin protection and prevention of infection. To date, there has been no systematic investigation of home skin care practices among EB patients on a multicenter scale. Methods This cross-sectional, observational study included data collected from patients with EB enrolled in the Epidermolysis Bullosa Characterization and Clinical Outcomes Database (EBCCOD) who provided answers to a patient-directed questionnaire between January 1, 2017, and December 31, 2017. Results Of 202 respondents, 130 (64.4%) had dystrophic EB, 51 (25.2%) had EB simplex, 21 (7.4%) had junctional EB, 3 (1.5%) had Kindler syndrome, and 3 (1.5%) had an unspecified subtype. Seventy-eight patients reported cleansing in plain water only (39%). Of those who used an additive in their cleansing water, 75 (57%) added salt, 71 (54%) added bleach, 36 (27%) added vinegar, and 34 (26%) endorsed the use of an "other" additive (multiple additives possible). Reported concentrations of additives ranged widely from 0.002% sodium hypochlorite and 0.002% acetic acid solutions, which are thought to have negligible effects on microbes, to 0.09% sodium hypochlorite and 0.156% acetic acid, concentrations shown to be cytotoxic. One hundred eighty-eight patients answered questions regarding topical product use (93%). Of those, 131 reported topical antimicrobial use (70%). Mupirocin and bacitracin were the most commonly reported topical antibiotics (59, 58 [31.4%, 30.9%], respectively). Conclusions These findings highlight the variety of skin care routines and frequent use of topical antimicrobials among EB patients and have potential implications for antibiotic resistance. The reported range of bleach and vinegar additives to cleansing water, including cytotoxic concentrations, emphasizes the need for clear and optimized skin cleansing recommendations.

Proper mask usage is a cornerstone of the prevention of COVID-19 transmission. Hospitals, in particular, are important settings for proper mask compliance due to the risk for viral exposure. Despite the presence of health care personnel and financial resources to ensure proper compliance, mask usage is variable in health care settings. The impact of mask compliance is particularly important in New York City (NYC) because of the burden of COVID-19 and at-risk demographics. We conducted a prospective observational study in 4 NYC hospitals assessing rates of proper mask compliance among adult patients entering the hospital. Six hundred unique individuals were observed for proper mask fit, exposure of the nose and mouth, and the presence of nontraditional face coverings in lieu of a mask at 4 NYC hospitals. Proper mask usage is a large health education gap that must be addressed by health care administrations and governmental agencies, as mask usage continues to be an effective form of COVID-19 prevention.

There has been a recent focus on the association between human microbiomes and disease development, disease resistance, and therapy response. Fecal transplants for inflammatory bowel disease and resistant Clostridium difficile infection have demonstrated that manipulating the gut microbiome can be beneficial in treating disease. Microbiomes are important in dermatology, where response to immune checkpoint inhibitors for melanoma therapy can be affected by differences in gut microbial composition. Bleach baths, which alter the skin microbiome, are known to be beneficial in atopic dermatitis. Gut dysbiosis, or disturbance in the gut microbiome in early life, can influence the development of systemic sclerosis and atopic dermatitis. Metagenomic sequencing can therefore be a useful addition to personalized medicine to identify therapy responders versus non-responders, patients at risk of serious side-effects from biologics and immune checkpoint inhibitors, and prebiotic supplements that aid in improving therapy response.

Reactive infectious mucocutaneous eruption (RIME) was recently proposed to replace the term Mycoplasma pneumoniae (MP)-induced rash and mucositis to account for the fact that non-MP pathogens may also cause rash and mucositis. In this report, we describe a unique case of recurrent RIME featuring a total of three episodes. As two of the episodes demonstrated contemporaneous infection with MP and group A streptococcus or influenza B, this case lends further support to use of the term RIME. In addition, although RIME typically involves at least two mucous membranes, this case shows that recurrent episodes may fall into the rare exception in which mucositis is limited to one site.

Staphylococcal scalded skin syndrome (SSSS) is a disease caused by certain toxigenic strains of Staphylococcus aureus. While the classic severe phenotype is widely recognized in children, SSSS in fact exists on a spectrum with mild and moderate variants. Misunderstanding the phenotypic spectrum of SSSS may result in misdiagnosis of an otherwise treatable condition. To increase awareness of the heterogeneity of SSSS, we report four cases that together represent a range of clinical presentations.

Neonatal lupus erythematosus (NLE) is an autoimmune disease characterized by a periorbital erythematous rash. Although post-inflammatory hypopigmentation and telangiectasias are known possible sequelae, these features may be particularly noticeable in skin of color. Herein, we describe two infants with skin of color in whom periorbital hypopigmentation and telangiectasias were clues to the diagnosis of NLE.

Background/objectives Patients with epidermolysis bullosa (EB) require care of wounds that are colonized or infected with bacteria. A subset of EB patients are at risk for squamous cell carcinoma, and bacterial-host interactions have been considered in this risk. The EB Clinical Characterization and Outcomes Database serves as a repository of information from EB patients at multiple centers in the United States and Canada. Access to this resource enabled broad-scale analysis of wound cultures. Methods A retrospective analysis of 739 wound cultures from 158 patients from 13 centers between 2001 and 2018. Results Of 152 patients with a positive culture, Staphylococcus aureus (SA) was recovered from 131 patients (86%), Pseudomonas aeruginosa (PA) from 56 (37%), and Streptococcus pyogenes (GAS) from 34 (22%). Sixty-eight percent of patients had cultures positive for methicillin-sensitive SA, and 47%, methicillin-resistant SA (18 patients had cultures that grew both methicillin-susceptible and methicillin-resistant SA at different points in time). Of 15 patients with SA-positive cultures with recorded mupirocin susceptibility testing, 11 had mupirocin-susceptible SA and 6 patients mupirocin-resistant SA (2 patients grew both mupirocin-susceptible and mupirocin-resistant SA). SCC was reported in 23 patients in the entire database, of whom 10 had documented wound cultures positive for SA, PA, and Proteus species in 90%, 50%, and 20% of cases, respectively. Conclusions SA and PA were the most commonly isolated bacteria from wounds. Methicillin resistance and mupirocin resistance were reported in 47% and 40% of patients tested, respectively, highlighting the importance of ongoing antimicrobial strategies to limit antibiotic resistance.

Folliculocystic and collagen hamartoma (FCCH) of tuberous sclerosis is a rare entity described in 2012 by Torrelo et al. with only 8 cases described, predominantly in males. It presents since birth or early infancy and in the majority of cases is associated with tuberous sclerosis. The hamartoma presents as an exophytic plaque and has distinctive histopathological features including hair follicles, intact or ruptured epidermal cysts, and an increased number of collagen fibers extending to the subcutaneous tissue. Herein we present an additional case of this rare entity in an 18-year-old female who met clinical criteria for tuberous sclerosis. The patient had an exophytic mass in the left temporal area for many years and wanted surgical excision due to its cosmetic appearance. Histopathology of the surgical specimen showed a hamartomatous lesion with multiple large intact epidermal cysts, hairs and increased thickened collagen. The patient has followed up for 1 year after the excision, with no recurrence. Additionally, we provide a literature review of known cases of FCCH as well as its clinical and histopathological differential diagnosis.

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.

Background/Objectives The past several decades have witnessed unprecedented advances in genomic technology, bringing genetic testing to the forefront of medical practice and moving us towards the practice of personalized medicine. Genetic testing has become an important aspect in preempting and successfully treating diseases in dermatology, yet difficulty remains in regards to obtaining genetic testing for patients. We conducted a survey for pediatric dermatologists in order to try to gauge and understand where difficulties lie in obtaining genetic testing and to analyze how best these issues can be resolved. Methods An 18-question survey was emailed to 480 dermatologists who have attended at least one of the last three annual Society for Pediatric Dermatology (SPD) meetings. Results Virtually all providers encountered at least one situation in which they required genetic testing for a patient (97.3% [n = 108]) and 37.4% indicated needing genetic testing more than six times per year. Of the respondents who had attempted to obtain genetic testing, half were unsuccessful in obtaining coverage more than 75% of the time (45% [n = 32]) and only 7.0% (n = 5) achieved success 75% to 100% of the time. The most common reasons for obtaining genetic testing included the need to provide an accurate diagnosis, followed by the need to provide prognostic information and appropriate medical management. Conclusion The role of genetic testing in the practice of dermatology is expanding, yet obtaining coverage for genetic testing remains a challenge. We propose several solutions as to how this can be remedied.

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.

Distinguishing benign acral nevi from small early acral melanomas may be challenging in certain cases. Dermoscopy is a noninvasive imaging technique that can help clinicians better visualize deeper lesion structures and thus more easily differentiate benign nevi from melanoma. We report the case of a 13-year-old girl with a changing dark brown to black macule with a central papular component on the volar surface of the right third finger. Dermoscopy revealed asymmetrically distributed irregular black blotches on a bluish-black background. Histopathology revealed a traumatized compound melanocytic nevus. Certain melanocytic nevi, although histologically benign, may not conform to the limited selection of reassuring benign dermoscopic patterns. Nevi in children are often dynamic and have a high likelihood of dermoscopic change.

IMPORTANCE: Children with epidermolysis bullosa (EB) comprise a rare population with high morbidity and mortality. An improved understanding of the clinical trajectory of patients with EB, including age at time of clinical diagnosis and major clinical events, is needed to refine best practices and improve quality of life and clinical outcomes for patients with EB. OBJECTIVES: To describe demographics, clinical characteristics, milestone diagnostic and clinical events (such as initial esophageal dilation), and outcomes in patients with EB using the Epidermolysis Bullosa Clinical Characterization and Outcomes Database and to determine what characteristics may be associated with overall EB severity and/or disease progression. DESIGN, SETTING, AND PARTICIPANTS: This cohort study included data on patients with EB who were enrolled in the Epidermolysis Bullosa Clinical Characterization and Outcomes Database from January 1, 2011, to June 30, 2017; 17 participating EB centers in the United States and Canada contributed data to this study. EXPOSURES: Type of EB, including recessive dystrophic epidermolysis bullosa (RDEB), junctional epidermolysis bullosa (JEB), dominant dystrophic epidermolysis bullosa (DDEB), and epidermolysis bullosa simplex (EBS). MAIN OUTCOMES AND MEASURES: Demographic information, clinical characteristics (including age at onset of signs of EB and subsequent clinical diagnosis), types of diagnostic testing performed, and milestone clinical events for patients with RDEB. RESULTS: Of 644 enrolled patients from 17 sites included in this study, 323 were male (50.2%), with a mean (SD) age of 14.4 (11.7) years; 283 (43.9%) had RDEB, 194 (30.1%) had EBS, 104 (16.2%) had DDEB, and 63 (9.8%) had JEB. Signs of disease were present at birth in 202 patients with RDEB (71.4%), 39 with JEB (61.9%), 60 with DDEB (57.7%), and 74 with EBS (38.1%). For those with signs of disease at birth, a clinical diagnosis was made at the time of birth in 135 patients with RDEB (67.0%), 31 with DDEB (52.6%), 35 with EBS, (47.3%) and 18 with JEB (46.2%). Patients with JEB had the highest rate of any confirmatory testing (51 of 63 [81.0%]), followed by RDEB (218 of 283 [77.0%]), DDEB (71 of 104 [68.3%]), and EBS (100 of 194 [51.5%]). For all types of EB, both electron microscopy and immunofluorescence microscopy were performed at younger ages than genetic analysis. Among 283 patients with RDEB, 157 (55.5%) had esophageal dilation, 104 (36.7%) had gastrostomy tube placement, 62 (21.9%) had hand surgery, 18 (6.4%) developed squamous cell carcinoma, and 19 (6.7%) died. CONCLUSIONS AND RELEVANCE: The findings suggest that diagnostic testing for EB is more common for patients with severe phenotypes. Earlier diagnostic testing may enable improved characterizations of patients so that appropriate counseling and clinical care may be offered, especially pertaining to milestone events for those with RDEB.

Infective dermatitis (ID) associated with Human T-cell leukemia virus type-1 (HTLV-1) is a rare form of severe superinfected eczema seen mostly in the Caribbean islands and Latin America. Although rapid response to antibiotic treatment is observed, patients should be monitored for development of complications associated with this retroviral infection, including T-cell leukemia/lymphoma (ATLL) and HTLV-1 associated myelopathy/tropical spastic paraparesis (HAM/TSP). Infective dermatitis is rarely seen in the United States and therefore may be under-recognized by physicians unfamiliar with this condition. Herein, we present an additional case report of an ID associated with HTLV-1 in an 11-year-old girl from Trinidad.

A 15-month-old boy presented with 1-4 cm, pink edematous plaques with overlying round erosions and hemorrhagic bullae in the setting of a gastrointestinal illness and was ultimately diagnosed with bullous-type Sweet syndrome. Despite appropriate treatment with oral steroids, the patient's cutaneous lesions healed with secondary anetoderma. This case should prompt practitioners to be aware of bullous-type Sweet syndrome and the possibility of lesions healing with postinflammatory scarring.

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.

Harlequin ichthyosis (HI) is a rare autosomal recessive congenital ichthyosis associated with mutations in the keratinocyte lipid transporter adenosine triphosphate binding cassette A12 (ABCA12),1 leading to disruption in lipid and protease transport into lamellar granules in the granular layer of the epidermis. Subsequent defective desquamation with compensatory hyperkeratinization follows.2 Historically, there has been a high early mortality rate in infants with HI; however, improved neonatal management and the early introduction of systemic retinoids may contribute to improved prognosis. Death in these patients is most commonly caused by sepsis, respiratory failure, or electrolyte imbalances. We report a case of a neonate with HI treated in the first few days of life with acitretin. The patient initially improved but eventually died of pseudomonas sepsis at 6 weeks of age.

The discovery of propranolol for the treatment of infantile hemangioma in 2008 has revolutionized management of this condition. Hundreds of reports have corroborated the initial published findings that propranolol is a safe and efficacious therapeutic agent. Furthermore, it is well tolerated in infants and can markedly alter the natural history and potential disfigurement of these lesions. The goal of this review is to outline the salient features of infantile hemangioma and the optimal use of propranolol as a first-line treatment modality.

Birthmarks are common skin lesions found in the newborn population. The most commonly encountered birthmarks include the pigmented, the hamartomatous and the vascular birthmarks. Each of these birthmarks may represent normal cutaneous variants with no clinical significance, while others may portend significant clinical diseases. This review elucidates this distinction and describes the appropriate management of some of the most commonly encountered medically significant birthmarks.

Pigmentation of the nail plate, or melanonychia, is typically a benign condition caused by melanocyte activation. Although rare, melanonychia may be the initial presentation of melanoma, thus all cases require an in-depth examination. Evaluation in pediatric patients can prove especially difficult as benign cases have a higher prevalence of atypia compared to adults. Lack of specific treatment guidelines in the pediatric population can make diagnosis and treatment challenging. We report a pediatric patient with melanonychia with atypical features that required significant evaluations and collaboration to ultimately reach a treatment plan.

Perianal lesions in children are common reasons for dermatology clinic visits and a well-defined approach to diagnosis and management is helpful to the practicing clinician. In this article, we review and update various etiologies of perianal lesions in the pediatric population, including infectious, papulosquamous, vascular, and neoplastic. We provide a standard initial approach to diagnosis and updates on current management. Infectious etiologies of perianal lesions discussed in this article include fungal, bacterial, parasitic, and viral. Perianal papulosquamous lesions often encountered in children, and discussed in this article, include acrodermatitis enteropathica, psoriasis, contact dermatitis, and many others. We also discuss the diagnosis and management of other entities including infantile hemangiomas, Langerhans cell histiocytosis, and fibrous hamartoma of infancy.

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

Pigmentation of the nail plate, or melanonychia, is typically a benign condition caused by melanocyte activation. Although rare, melanonychia may be the initial presentation of melanoma, thus all cases require an in-depth examination. Evaluation in pediatric patients can prove especially difficult as benign cases have a higher prevalence of atypia compared to adults. Lack of specific treatment guidelines in the pediatric population can make diagnosis and treatment challenging. We report a pediatric patient with melanonychia with atypical features that required significant evaluations and collaboration to ultimately reach a treatment plan.

Harlequin ichthyosis (HI) is the most severe phenotype of the autosomal recessive congenital ichthyoses. HI is caused by mutations in the lipid transporter adenosine triphosphate binding cassette A 12 (ABCA12). Neonates are born with a distinct clinical appearance, encased in a dense, platelike keratotic scale separated by deep erythematous fissures. Facial features are distorted by severe ectropion, eclabium, flattened nose, and rudimentary ears. Skin barrier function is markedly impaired, which can lead to hypernatremic dehydration, impaired thermoregulation, increased metabolic demands, and increased risk of respiratory dysfunction and infection. Historically, infants with HI did not survive beyond the neonatal period; however, recent advances in neonatal intensive care and coordinated multidisciplinary management have greatly improved survival. In this review, the authors combine the growing HI literature with their collective experiences to provide a comprehensive review of the management of neonates with HI.

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.

Pediatric vascular lesions can be medically threatening and psychologically distressing to patients. This article reviews literature on the laser treatment of two common pediatric vascular lesions, port wine stains and hemangiomas. The purpose of this report was to distinguish the lesions from one another and to present the advantages, disadvantages, complications, and limitations of laser treatment for each lesion type. This review is not a comprehensive inventory but instead highlights the studies that best show promising results or the limitations of laser treatment for the lesions. Overall, port wine stain laser treatment promoted notable clearing with low side effects, whereas hemangioma laser treatment provided inconsistent benefits and severe side effects occasionally. Laser treatment of port wine stains is safe and effective, but laser treatment of hemangiomas remains controversial and is best for lesions without deeper components.

The global population of bed bugs (Cimex lectularius and Cimex hemipterus, family Cimicidae) has undergone a significant resurgence since the late 1990s. This is likely due to an increase in global travel, trade, and the number of insecticide-resistant bed bugs. The global bed bug population is estimated to be increasing by 100–500 % annually. The worldwide spread of bed bugs is concerning, because they are a significant socioeconomic burden and a major concern to public health. According to the United States Environmental Protection Agency, bed bugs are “a pest of significant health importance.” Additionally, 68 % of U.S. pest professionals reported that bed bugs are the most challenging pest to treat. Upwards of 45 disease pathogens have been reported in bed bugs. Recent studies report that bed bugs may be competent vectors for pathogens, such as Bartonella quintana and Trypanosoma cruzi. However, public health reports have thus far failed to produce evidence that major infectious disease outbreaks have been associated with bed bugs. Since many disease pathogens have previously been reported in bed bugs and the worldwide bed bug population is now drastically increasing, it stands to reason to wonder if bed bugs might transmit human pathogens. This review includes a literature search on recently published clinical and laboratory studies (1990–2016) investigating bed bugs as potential vectors of infectious disease, and reports the significant findings and limitations of the reviewed studies. To date, no published study has demonstrated a causal relationship between bed bugs and infectious disease transmission in humans. Also, we present and propose to expand on previous hypotheses as to why bed bugs do not transmit human pathogens. Bed bugs may contain “neutralizing factors” that attenuate pathogen virulence and, thereby, decrease the ability of bed bugs to transmit infectious disease.

Zygomycosis, often referred to as ‘‘mucormycosis’’ or ‘‘phycomycosis,’’ is a rapidly progressive fungal infection which usually occurs in immunocompromised individuals, and is characterized by soft tissue destruction and invasion of blood vessels. The rare and easily misdiagnosed primary cutaneous form may present as a superficial erosion with a painless, gradual onset and slow progression of symptoms or a gangrenous, necrotic ulceration due to rapid tissue and vascular invasion. With the latter form, the mortality rate among affected individuals is high even after aggressive surgical debridement and amphotericin B administration, emphasizing the importance of early recognition and proper diagnosis. We present two instances of gangrenous cutaneous zygomycosis in immunocompromised children and review the literature with regard to etiology, diagnosis and treatment, highlighting the pediatric population.

Prenatal sonographic diagnoses of subcutaneous soft tissue masses include hemangiomas, malformations (capillary, lymphatic, venous, arterial, or mixed), teratomas, and, rarely, sarcomas. 1-5 Prenatally depicted malformations may be seen in association with more complex fetal conditions, including Klippel-Trenaunay-Weber (angio-osteohypertrophy) and Proteus syndromes.6"9 We present an unusual case in which extensive multiple soft tissue tumors encompassing the fetal upper chest, back, nuchal area, and bilateral axillae, considered consistent with multiple large lymphatic malformations noted at 23 weeks' gestation, decreased in size throughout the remainder of gestation. At delivery, relatively small subcutaneous masses remained. Tissue histopathologic findings obtained at biopsy and the neonate's clinical course during which the subcutaneous masses underwent a marked spontaneous decrease in size, were consistent with low-flow mixed lymphatic and venous malformations.

With the increased understanding and application of molecular genetics, exceptions to Mendelian genetics have been characterized. Uniparental disomy and genomic imprinting are two genetic mechanisms that often underlie these unusual patterns of inheritance. Uniparental disomy occurs when both copies of one chromosome pair come from only one parent. Genomic imprinting is the differential expression of allelic genes depending upon the parent of origin. In this review, these topics will be examined with regard to their etiologies and clinical consequences. We will focus on examples from the dermatological literature, including Prader–Willi, Angelman, Silver–Russell, Chediak–Higashi, cartilage-hair hypoplasia, Bloom and Beckwith–Wiedemann syndromes, as well as epidermolysis bullosa and cystic fibrosis.

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.