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The characteristics of epidermolysis bullosa (EB) demand higher than average provider support for transition from pediatric to adult care. We administered an online Qualtrics survey to members of the Epidermolysis Bullosa Clinical Research Consortium (EBCRC), a group of providers who care for patients with EB, in order to examine their practices and perspectives on transition of care (TOC) and identify barriers to successful implementation. Sixteen of eighteen medical centers completed the survey. Eighty-eight percent of center representatives expressed concerns about their patients transitioning/transferring from the pediatric to adult-centered care. Thirty-eight percent of providers reported having a formal TOC program in place. Our findings support the desire for formal TOC programs, the need for a team-based approach and, in particular, identification of adult providers to participate in the transition to improve this often challenging time., (© 2024 Wiley Periodicals LLC.)

Background: Epidermolysis bullosa (EB), characterized by skin fragility and blistering, often requires hospitalization. Training for inpatient management of EB is limited, with no unified recommendations available in North America., Objective: To develop consensus-derived best practices for hands-on inpatient management of EB in both the neonatal and postneonatal period., Methods: A modified Delphi method (expert-based input via 2 surveys and a final review) was implemented. Available guidelines from EB Clinical Research Consortium centers were analyzed to determine areas of focus and formulate statements to be voted on by EB Clinical Research Consortium members, experienced EB nurses, and select family members. Study participants evaluated statements using a Likert scale: statements with at least 70% agreement were accepted; statements with 30% or more disagreement were rejected., Results: Ten areas of focus were identified. Delphi participants included 15 dermatologists, 8 nurses, and 6 nonhealth care caregivers. Consensus was established on 103/119 neonatal statements and 105/122 postneonatal statements; no statements were rejected. Most recommendations applied to both age groups., Limitations: Recommendations may require adjustment based on individual patient's clinical context., Conclusion: Using the Delphi method, a consensus-derived resource for hospital-based health care professionals who manage patients with EB has been developed to improve the quality of inpatient care., Competing Interests: Conflicts of interest Dr Garcia-Romero has received the EBCRC grant and has been a speaker for Cerave Latinamerica and Carnot Mexico Laboratories. Dr Price’s’ conflicts of interest are Aegle (PI clinical trial), Rheacell (PI clinical trial) Krystal Biotech (consultant). Dr Perman has served as a consultant for Abeona. Dr Lucky has served as investigator for Krystal Pharma and Phoenicis. Dr McCuaig’s conflicts of interest are AbbVie, Bausch, Boehringer, Galderma, Incyte, J&J, Eli Lilly, Leo, Novartis, Oreal Pfizer, Sanofi, and Sun. Dr Gorell has served as a consultant for Krystal Biotech, Abeona Therapeutics, and Amryt Pharma. Dr Levy’s conflicts of interest are Abeona, Castle Creek, Krystal, and Rheacell. Dr Bruckner’s conflicts of interest are Abeona (consultant), Amryt (consultant and investigator), Castle Creek (consultant and investigator), Krystal Biotech (consultant), Phoenix Tissue Repair (investigator), Phoenicis (investigator), Rheacell (investigator), and TWi Bio (consultant). Dr Paller has been an investigator for AbbVie, Applied Pharma Research, Dermavant, Eli Lilly, Incyte, Janssen, Krystal, Regeneron, and UCB; a consultant for Aegerion Pharma, Azitra, BioCryst, Boehringer-Ingelheim, Bristol Myers Squibb, Eli Lilly, Janssen, Johnson & Johnson, Krystal, LEO Pharma, Novartis, Primus, Regeneron, Sanofi/Genzyme, Seanergy, TWI Biotechnology, and UCB; and on the data safety monitoring board for AbbVie, Abeona, Catawba, Galderma, and InMed., (Copyright © 2024 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.)

Background: Facial angiofibromas (FAs) are a major feature of tuberous sclerosis complex (TSC). Topical rapamycin can successfully treat FAs. A new stabilized cream formulation that protects rapamycin from oxidation has been developed in 0.5% and 1% concentrations., Objectives: To assess the efficacy and safety of a novel, stabilized topical rapamycin cream formulation., Methods: This multicentre double-blind randomized placebo-controlled dose-response phase II/III study with a parallel design included participants aged 6-65 years with FAs of mild or moderate severity according to the Investigator's Global Assessment (IGA) scale. Participants were randomized to one of three treatment arms: topical rapamycin 0.5%, topical rapamycin 1% or placebo. Treatment was applied once daily for 26 weeks. Safety and efficacy measures were assessed at days 14, 56, 98, 140 and 182. The primary endpoint was the percentage of participants achieving IGA scores of 'clear' or 'almost clear' after 26 weeks of treatment. Secondary measures included Facial Angiofibroma Severity Index (FASI) and participant- and clinician-reported percentage-based improvement. Safety measures included the incidence of treatment-emergent adverse events and blood rapamycin concentration changes over time., Results: Participants (n = 107) were randomized to receive either rapamycin 1% (n = 33), rapamycin 0.5% (n = 36) or placebo (n = 38). All treated participants were included in the final analysis. The percentage of participants with a two-grade IGA improvement was greater in the rapamycin 0.5% treatment group (11%) and rapamycin 1% group (9%) than in the placebo group (5%). However, this was not statistically significant [rapamycin 0.5%: odds ratio (OR) 1.71, 95% confidence interval (CI) 0.36-8.18 (P = 0.50); rapamycin 1%: OR 1.68, 95% CI 0.33-8.40 (P = 0.53)]. There was a statistically significant difference in the proportion of participants treated with rapamycin cream that achieved at least a one-grade improvement in IGA [rapamycin 0.5%: 56% (OR 4.73, 95% CI 1.59-14.10; P = 0.005); rapamycin 1%: 61% (OR 5.14, 95% CI 1.70-15.57; P = 0.004); placebo: 24%]. Skin adverse reactions were more common in patients following rapamycin application (64%) vs. placebo (29%)., Conclusions: Both rapamycin cream formulations (0.5% and 1%) were well tolerated, and either strength could lead to clinical benefit in the treatment of FA., Competing Interests: Conflicts of interest: P.A. and L.B. are employees of AFT Pharmaceuticals Ltd. I.S. is a shareholder and director of clinical research of AFT Pharmaceuticals Ltd. H.A. is a shareholder and managing director of AFT Pharmaceuticals Ltd and the named inventor on a patent for a rapamycin cream composition. P.B. has received financial support for conference travel and/or speaker honoraria from Novartis, Biogen, Roche and PTC Therapeutics. K.R. has received honoraria for educational symposia, advisory boards and/or consultancy work from Eisai, LivaNova, Medlink Neurology, Novartis and UCB Australia Ltd. Her institution has supported clinical trials for Biogen Idec Research, Eisai, Epigenyx Therapeutics, GW Research, Janssen-Cilag, Longboard Pharmaceuticals, Marinus Pharmaceuticals, Medicure International, LivaNova, Neurocrine Biosciences, Noema Pharma, Novartis, SK Lifesciences, Takeda Pharmaceutical Company, UCB Australia, UCB Biopharma and Zogenix. C.M., A.F., Y.-H.L., M.I., E.M.-A., D.T., S.T.D., J.J., M.N., P.B., H.N.P., K.H., K.R., S.S., M.M.T., D.B. and B.P. were investigators at NCT03826628 study sites., (© The Author(s) 2023. Published by Oxford University Press on behalf of British Association of Dermatologists.)

Pediatric psoriasis (PsO) and its associated comorbidities carry physical and psychosocial burdens in children and adolescents, which can negatively impact quality of life. However, features distinguishing pediatric PsO from eczema and other common inflammatory skin diseases may not be obvious to primary care providers, which may contribute to underrecognition and misdiagnosis. Accurate diagnosis of pediatric PsO is critical for managing the physical and psychological burdens associated with this disease. This review aims to support pediatricians with enough information to confidently diagnose pediatric PsO, assess associated physical and mental health comorbidities, and recommend first-line treatment options for children with mild to moderate PsO. To accomplish this, we provide information that distinguishes the appearance and symptoms of pediatric PsO from other common pediatric skin conditions. In addition, comorbidities and some of the mental health challenges associated with pediatric PsO are reviewed to help pediatricians provide appropriate care for patients in their clinical practice. Hebert AA, Browning J, Kwong PC, et al. Diagnosis and management of pediatric psoriasis: an overview for pediatricians. J Drugs Dermatol. 2023;22(8):742-752. doi:10.36849/JDD.7531.

There are little published data on the transition of care in EB. We conducted a survey study recruiting EB patients from the Dystrophic EB Research Association (debra) website and centers caring for high numbers of EB patients in the United States and internationally from Sept 17, 2019 to Nov 3, 2021. The majority of participants had not discussed the transition of care with their healthcare providers, nor the healthcare needs to be required as an adult. Ongoing pediatric subspecialty care was reported by 12% of adults, most commonly in pediatric dermatology. Identified barriers to transition included the perceived lack of adult providers' knowledge about EB patient healthcare needs. The results suggest the need for transition guidelines, early discussions with families about transition, and practical information for the adult providers accepting care., (© 2022 Wiley Periodicals LLC.)

Background/objectives: Complications of hematopoietic stem cell transplant (HSCT) include acute graft-versus-host disease (aGVHD). Severe cutaneous aGVHD can present with generalized erythroderma, desquamation, and bullae which can mimic toxic epidermal necrolysis (TEN). TEN occurs in response to a culprit medication. Transplant patients are often on many medications, making it difficult to distinguish between the two conditions. Given that TEN-like aGVHD is rare, we describe a case series of pediatric patients and review the literature., Methods: This is a multi-institutional case series of children who developed TEN-like aGVHD following bone marrow transplantation. Demographic, clinical, and treatment information was collected., Results: Ten patients were identified. Median age at transplantation was 8.5 years (range 0.12-17 years). Median time from transplant to first skin symptoms was 35 days (range 6-110 days) and to first TEN-like symptoms was 40 days (range 16-116 days). 7/10 had other organ GVHD involvement. All patients were on concurrent medications at time of first skin symptoms including immunosuppression for GVHD prophylaxis, infection prophylaxis or treatment, and pain medication. Treatments for TEN-like aGVHD included immunosuppression., Conclusions: We observe that patients with > or equal to 50% BSA involvement of their skin with TEN-like aGVHD, extracutaneous GVHD, and lack of reepithelization tend to have poor outcomes. Given the rarity of this condition, multidisciplinary care of these patients is important for accurate and timely diagnosis and treatment., (© 2022 Wiley Periodicals LLC.)

Background: Psoriasis is a chronic, immune-mediated inflammatory disease with prominent cutaneous features, although the limited number of medications approved for pediatric psoriasis makes treating this population difficult. This review provides an overview of the challenges associated with diagnosing and treating pediatric psoriasis as well as the approved and off-label treatments for children and infants with psoriasis., Methods: Articles relevant to pediatric psoriasis were identified using a series of PubMed searches. Topics relevant to pediatric psoriasis were explored, including disease characteristics, epidemiology, treatment efficacy and safety, and access to care. Publications previously known to the authors were also included., Results: Clinical features of psoriasis can be challenging to identify clinically, and patients face challenges gaining access to treatment. Most medications that have been approved for adult psoriasis lack data and labeling to support safe and effective use in pediatric patients, and therefore access is limited. A growing number of clinical trials using biologic agents for pediatric psoriasis aim to broaden available treatment options but may also raise unique concerns associated with the use of these medications in children., Conclusion: Pediatric psoriasis is underrecognized and often undertreated. Clinicians must balance relative risks and potential benefits when developing a treatment strategy for these patients.

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 < .001). The likelihood of undergoing genetic analysis was greater for junctional EB and recessive dystrophic EB, and the same for dominant dystrophic EB as compared with EB simplex. TEM results in 163 patients were equivocal (55%), concordant (42%), and discordant (3%). IFM results in 185 patients were equivocal (54%), concordant (42%), and discordant (4%)., Limitations: Retrospective design., Conclusions: Diagnostic testing has shifted in favor of genetic analysis. TEM and IFM frequently offer equivocal findings when compared to the specificity afforded by genetic analysis., Competing Interests: Conflicts of interest Dr Bruckner serves as an investigator for Fibrocell, Phoenix Tissue Repair, PROQR/Wings, and Castle Creek and on an ad hoc advisory board for Castle Creek. Dr Pope receives research funding from the EB Research Foundation. Dr Paller serves as an investigator for Castle Creek and Lenus Pharmaceuticals and has been a consultant with honorarium for Abeona. Dr Levy serves on the advisory board for Cassiopea, Regeneron; as an investigator for Fibrocell/Castle Creek, Galderma, Janssen, Pfizer; on the Data Safety and Monitoring Board for Novan; and as a section editor for UpToDate. Dr Lucky serves as an investigator for Lenus Pharmaceuticals and Castle Creek and on the scientific advisory board for EBRP (EB Research Partnership) and Abeona. Dr Glick serves as an investigator for Lenus Pharmaceuticals. Authors Phillips, Augsburger, and Peoples and Drs Huang, Kaplan, Khuu, Tang, Lara-Corrales, Wiss, Levin, Morel, Hook, Eichenfield, McCuaig, Powell, Castelo-Soccio, Price, Schachner, Browning, Jahnke, Shwayder, and Bayliss have no conflicts of interest to declare., (Copyright © 2021 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.)

Erythromelalgia is a rare neurovascular pain condition characterized by erythematous, warm, and painful extremities. Symptoms are exacerbated by heat and relieved by cooling. Treatment is challenging and focuses on symptom control with various medications and therapies targeted toward eliminating destructive cooling behaviors. This pediatric case was notable because the patient's pain dramatically improved after a short-term, low-dose ketamine infusion, allowing her to finally wean off detrimental cooling practices of her extremities. Intravenous ketamine has rarely been described as an adjunctive analgesic strategy for erythromelalgia., (Copyright © 2022 International Anesthesia Research Society.)

Competing Interests: Lindsey M. HoffmanHonoraria: AstraZenecaNo other potential conflicts of interest were reported.

Abstract: A 15-year-old boy presented to the pediatric dermatology department with long-standing patch stage CD8+ mycosis fungoides and subsequent development of recurrent pityriasis lichenoides et varioliformis acuta eruptions. There have been rare reports of patients with chronic, recalcitrant pityriasis lichenoides developing mycosis fungoides, but we believe this to be the second case of mycosis fungoides preceding a diagnosis of pityriasis lichenoides, and the first case reported in the pediatric population., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

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., (© 2020 Wiley Periodicals LLC.)

Background: Pediatric melanoma is rare and diagnostically challenging., Objective: To characterize clinical and histopathologic features of fatal pediatric melanomas., Methods: Multicenter retrospective study of fatal melanoma cases in patients younger than 20 years diagnosed between 1994 and 2017., Results: Of 38 cases of fatal pediatric melanoma identified, 57% presented in white patients and 19% in Hispanic patients. The average age at diagnosis was 12.7 years (range, 0.0-19.9 y), and the average age at death was 15.6 years (range, 1.2-26.2 y). Among cases with known identifiable subtypes, 50% were nodular (8/16), 31% were superficial spreading (5/16), and 19% were spitzoid melanoma (3/16). One fourth (10/38) of melanomas arose in association with congenital melanocytic nevi., Limitations: Retrospective nature, cohort size, and potential referral bias., Conclusions: Pediatric melanoma can be fatal in diverse clinical presentations, including a striking prevalence of Hispanic patients compared to adult disease, and with a range of clinical subtypes, although no fatal cases of spitzoid melanoma were diagnosed during childhood., (Copyright © 2020 American Academy of Dermatology, Inc. Published by Elsevier Inc. All rights reserved.)

Eccrine angiomatous hamartoma (EAH) is a rare benign vascular hamartoma characterized histologically by an increased size and number of mature eccrine glands associated with multiple foci of dilated capillaries in the dermis and subcutis. EAH typically presents in children as discrete, solitary nodules, or plaques most commonly located on the extremities. Some cases of EAH have an agminated distribution involving classic locations, or present as solitary lesions in less common locations such as the face, scalp, or trunk. We report the case of congenital EAH in a child with atypical morphological features and pattern of distribution further expanding on the range of presentations classically described., (© 2019 Wiley Periodicals, Inc.)

Parry-Romberg syndrome (PRS) is characterized by hemiatrophy of facial structures, including skin, subcutaneous fat, muscle, bone, and cartilage. Complications associated with PRS include headaches, seizures, and chronic facial pain. Protocol for the treatment of chronic facial pain is not clear; reports on the use of botulinum toxin A injections for pain reduction in adults but not in the pediatric/adolescent population are available. Here, we discuss two pediatric PRS cases in which treatment with botulinum toxin A injections reduced or eliminated facial pain., (© 2019 Wiley Periodicals, Inc.)

Trichothiodystrophy is a rare autosomal recessive disorder resulting in a broad range of systemic abnormalities. Polarizing microscopy of the hair reveals the pathognomic "tiger tail" of alternating light and dark bands, but the need for a microscope prevents rapid bedside diagnosis. We describe a new technique for the bedside diagnosis of trichothiodystrophy using a handheld polarizing dermatoscope, precluding the need for microscopic examination., (© 2017 Wiley Periodicals, Inc.)

Purpose of Review: The article intends to review recent updates in the management of congenital melanocytic nevi (CMN) and the evolving genomic landscape that has begun to shed light on the nature of nevogenesis, malignant potential, and possible therapeutic targets for those with melanoma and neurologic involvement., Recent Findings: CMN are the result of postzygotic somatic mutations involving key proteins in the mitogen-activated protein kinase pathway, primarily NRAS and BRAF. Complications include a spectrum of neurologic findings and development of melanoma. Patients with extracutaneous involvement may be better termed as having 'CMN syndrome'. MRI findings in high risk patients with multiple CMN may best predict clinical outcome., Summary: The management of patients with CMN is complex. Neurologic involvement and melanoma remain the two most devastating complications. New, genetically targeted therapies for patients with complications may be of value, and research for potential therapies is ongoing.

An estimated 6 to 12 million children are affected by lice annually in the United States.(1) Knowledge of the various treatment options for this diagnosis is essential. This paper aims to provide an overview of the myriad therapeutic options available for lice infestations in children. U.S. Food and Drug Administration-approved drugs, off-label drug therapies, over-the counter-products, and herbal remedies are all discussed in detail. Clinicians may use this information to teach patients and families about the latest available care for the eradication of head lice in pediatric patients., (Copyright © 2015 Elsevier Inc. All rights reserved.)

A new consensus-based classification of congenital melanocytic nevi (CMN) has recently been proposed. It includes categories for projected adult size (PAS) and location, satellite nevi counts, and morphologic characteristics (color heterogeneity, rugosity, nodularity, and hypertrichosis). The objective of the current study was to test the applicability of the new categorization scheme and to correlate classification outcome with the patient's history of melanoma and neurocutaneous melanocytosis (NCM). Children and adults with CMN attending a patient conference in Dallas, Texas, in 2012 were invited to participate in the study. Anamnestical data were collected using a standardized questionnaire. Two dermatologists performed clinical examinations. Of 45 patients enrolled, 33 had a giant CMN (G1 [>40 cm PAS], n = 13; G2 [>60 cm PAS], n = 20), 12 had an NCM (5 symptomatic, 7 asymptomatic), and 1 had a history of melanoma. CMN size was positively correlated with NCM (p < 0.05). The classification system allowed an easy and detailed phenotypic characterization of each individual CMN. CMN size and morphology were difficult to assess in patients after surgical removal, and the number of satellite nevi at birth or during infancy was not always known. Our report provides practical aids for the application of the newly proposed CMN classification. Prospective evaluation of accurately classified patients in CMN registries will reveal the predictive value of the scheme. The small study sample limits meaningful conclusions regarding the correlation between CMN parameters and the risk of NCM and melanoma., (© 2014 Wiley Periodicals, Inc.)

Scabies is a highly contagious infestation with the Sarcoptes scabiei var hominis mite. The variety of clinical presentations make timely, accurate diagnosis problematic. We report the case of a 3-year-old girl with Down syndrome and crusted scabies initially misdiagnosed as erythrodermic psoriasis., (© 2013 Wiley Periodicals, Inc.)

Painful medical procedures in childhood may have long-term negative effects on development and future tolerance of pain. We describe a distraction technique for pediatric pain management that involves "blowing away the pain." This technique may be used for acute pediatric procedures as well as for patients with chronic pain., (© 2014 Wiley Periodicals, Inc.)

Atopic dermatitis (AD) is a common chronic inflammatory skin condition characterized by intense pruritus and a waxing and waning course. AD often presents in infancy and childhood and can persist throughout adulthood. The exact cause of AD is unknown, but it likely reflects an interplay between genetic and environmental factors. AD affects up to 20% of children in the United States, and prevalence may be increasing. Treatment can be effective in alleviating symptoms but serves only to manage the disease, not cure it. Appropriate therapy can also prevent significant complications, such as infection, sleep disturbance, behavioral problems, and growth impairment., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Congenital melanocytic nevi (CMN) are evident in 1% to 6% of neonates. In some studies, nevi with clinical, dermatoscopic, and histologic features identical to CMN have had a prevalence of more than 15% in older children and adults, possibly reflecting the "tardive" appearance of nevi programmed from birth. There is ongoing debate about the magnitude of the risk of melanoma and other complications associated with CMN of various sizes and the best approach to management of these lesions. We review the natural history of CMN, including proliferative nodules and erosions during infancy, neurotization, and spontaneous regression, and features of variants such as speckled lentiginous and congenital blue nevi. The risk of melanoma arising within small-sized (<1.5 cm) and medium-sized CMN is low (likely <1% over a lifetime) and virtually nonexistent before puberty. Recent data suggest that melanoma (cutaneous or extracutaneous) develops in approximately 5% of patients with a large (>20 cm) CMN, with about half of this risk in the first few years of life. Melanoma and neurocutaneous melanocytosis (NCM) are most likely in patients with CMN that have a final size of >40 cm in diameter, numerous satellite nevi, and a truncal location. One-third of individuals with NCM have multiple medium-sized (but no large) CMN. In patients at risk for NCM, a screening gadolinium-enhanced magnetic resonance imaging, preferably before age 6 months, and longitudinal neurologic assessment are recommended. Management of CMN depends on such factors as the ease of monitoring (more difficult for large, dark, thick nevi) and cosmetic and psychologic benefits of excision or other procedures. CMN require lifelong follow-up. Periodic total body skin examinations are necessary for all patients with large CMN, even when complete resection (often impossible) has been attempted., (Copyright 2010. Published by Elsevier Inc.)

A 21-year-old man presented with a life-long history of diffusely thickened skin with a grainy-to-ridged surface, verrucous perioral plaques with radial fissures, and diffuse palmoplantar keratoderma with a stippled appearance. These skin findings were accompanied by sensorineural hearing loss and keratoconjunctivitis, a clinical triad diagnostic of keratitis-ichthyosis-deafness (KID) syndrome. The patient also had a history of recurrent infections and cysts on the scalp. This report draws attention to inflammatory nodules (representing ruptured folliculitis), cysts, and recurrent infections on the scalp as manifestations of KID syndrome and reviews the increasingly recognized risk of follicular tumors and squamous-cell carcinomas in patients with this conditions.