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Acne vulgaris and hidradenitis suppurativa (HS) are chronic inflammatory, multifactorial skin disorders that often develop in adolescence and young adulthood. Both acne vulgaris and HS can cause significant morbidity and psychologic distress, with a negative effect on the quality of life. The relationship among diet, acne, and HS remains somewhat controversial; however, there is increasing evidence that high-glycemic diets and consumption of milk and dairy products promote acne. Studies suggest that weight loss through dietary interventions or bariatric surgery and brewer's yeast exclusion diets have the potential to ameliorate the signs of HS. We review the role of diet in the pathogenesis, prevention, and treatment of HS and acne vulgaris.

Trachyonychia (or twenty-nail dystrophy) is an uncommon chronic disorder manifesting as thin, flattened, brittle nails with excessive longitudinal ridging and loss of luster creating a "sandpaper-like" texture that most commonly presents spontaneously in childhood as an isolated phenomenon; however, it has been historically associated with numerous dermatoses. Rarely, trachyonychia has been reported to occur in families, suggesting a potential hereditary predisposition. We report trachyonychia occurring simultaneously in dizygotic twins, further supporting a possible underlying genetic basis of this idiopathic nail disorder.

Methylchloroisothiazolinone/methylisothiazolinone (MCI/MI) is a combination preservative used in personal care and household products and is a common cause of allergic contact dermatitis (ACD). Recently, MI alone, without MCI, has been increasingly used in consumer products in attempts to minimize allergic reactions. Wet wipes are extensively tested and traditionally believed to be innocuous. MI in wet wipes (“baby wipes”) has not been previously reported to cause ACD in children in the United States. Only 1 previous report of ACD in a child in Belgium has been recently reported. We report 6 children with chronic, perianal/buttock, and facial eczematous dermatitis, refractory to multiple topical and oral antibiotics and corticosteroids. All tested positive to MCI/MI on patch testing. None wore diapers. All patients had been using wet wipes containing MI (without MCI) to affected areas. Discontinuation of wipes resulted in rapid and complete resolution. This is the first report of pediatric ACD to MI in wet wipes in the United States, and the largest series to date. ACD to MI in wet wipes is frequently misdiagnosed as eczema, impetigo, or psoriasis. Wet wipes are increasingly marketed in personal care products for all ages, and MI exposure and sensitization will likely increase. Dermatitis of the perianal, buttock, facial, and hand areas with a history of wet wipe use should raise suspicion of ACD to MI and prompt appropriate patch testing. Rapid resolution occurs after the allergen exposure is eliminated. All isothiozolinones should be avoided in personal care and household products for these patients.

Pediatric psoriasis is a common skin disorder. Although pediatric psoriasis has many similarities to adult psoriasis, there are differences in presentation, particularly in infants. In addition, a more limited therapeutic armamentarium exists for children due to different inherent risks. Nearly all therapies are unapproved in the pediatric population, with far fewer pre- and postmarketing studies having been conducted. Despite these challenges, appropriate treatment should never be denied to a young patient, as physical and psychosocial ramifications of psoriasis in the school age child and adolescent are significant. A partnership with the patient, caregivers, and primary care providers is necessary and rewarding in meeting the challenges of this chronic, incurable disease. We address the presentation, treatment options, and counseling necessary when treating psoriasis in the pediatric population.

Acne vulgaris is a common inflammatory disease of the pilosebaceous follicles that affects patients of all ages, from neonates to adults. We have compared and contrasted the clinical presentation of acne in neonates, infants, children, teenagers, and young adults and review the scenarios in which further systemic endocrine or hormonal tests are indicated. We also discuss age-dependent treatment considerations, including appropriate oral antimicrobial regimens and the proper dosing of isotretinoin in young children versus teenagers and adults.

Background Little is known about the epidemiology of allergic contact dermatitis (ACD) in US children. More widespread diagnostic confirmation through epicutaneous patch testing is needed. Objective The aim was to quantify patch test results from providers evaluating US children. Methods The study is a retrospective analysis of deidentified patch test results of children aged 18 years or younger, entered by participating providers in the Pediatric Contact Dermatitis Registry, during the first year of data collection (2015-2016). Results One thousand one hundred forty-two cases from 34 US states, entered by 84 providers, were analyzed. Sixty-five percent of cases had one or more positive patch test (PPT), with 48% of cases having 1 or more relevant positive patch test (RPPT). The most common PPT allergens were nickel (22%), fragrance mix I (11%), cobalt (9.1%), balsam of Peru (8.4%), neomycin (7.2%), propylene glycol (6.8%), cocamidopropyl betaine (6.4%), bacitracin (6.2%), formaldehyde (5.7%), and gold (5.7%). Conclusions This US database provides multidisciplinary information on pediatric ACD, rates of PPT, and relevant RPPT reactions, validating the high rates of pediatric ACD previously reported in the literature. The registry database is the largest comprehensive collection of US-only pediatric patch test cases on which future research can be built. Continued collaboration between patients, health care providers, manufacturers, and policy makers is needed to decrease the most common allergens in pediatric consumer products.

Small and intermediate congenital melanocytic nevi have a lifetime risk of developing melanoma estimated to range from 0% to 5%. Secondary benign melanocytic proliferations commonly arise in congenital melanocytic nevi; however, some are difficult to definitively distinguish from malignant melanoma based on clinical features and conventional histology. Herein, we describe the use of comparative genomic hybridization in supporting the diagnosis of a deep penetrating nevus developing within a congenital melanocytic nevus of a 3-month-old infant.

In March 1995, the US Food and Drug Administration approved a live attenuated varicella vaccine for use in healthy children 12 months to 12 years old. We report here an 18-month-old girl with cell-mediated immunodeficiency who developed a severe vaccine-associated rash and clinical evidence of vaccine-associated pneumonia 1 month after inadvertent receipt of varicella vaccine.

Granular parakeratosis is an acquired, idiopathic disorder of keratinization typified by retention hyperkeratosis. It usually occurs in women at intertriginous sites. There have been only 2 reports of infants with granular parakeratosis to our knowledge. We describe 3 additional infants with granular parakeratosis. We demonstrate that infantile granular parakeratosis exhibits 2 clinical patterns: bilateral linear plaques in the inguinal folds; and erythematous geometric plaques underlying pressure points from the diaper. A thick, flakelike scale is present in both forms and is characteristic. Diaper wearing appears to play an important role in the genesis of infantile granular parakeratosis but the mechanisms are unclear. Therapeutic responsiveness to topical agents is ambiguous, however, spontaneous clearance after months to 1 year appears to be the rule.

Objective Cutaneous neonatal lupus resembles subacute cutaneous lupus erythematosus (SCLE), and photosensitivity is a common symptom. Tumor necrosis factor α (TNFα) release by ultraviolet light–exposed keratinocytes may be exaggerated in SCLE patients who have the haplotype TNFα −308A;DRB1*03. Accordingly, this study was undertaken to seek genetic and histologic evidence for a role of TNFα in the pathogenesis of cutaneous neonatal lupus. Methods DNA was isolated from 83 children (22 with rash, 35 with congenital heart block [CHB], 26 unaffected siblings) and 58 mothers from the Research Registry for Neonatal Lupus. Results The −308A allele (associated with higher TNFα production), HLA–DRQB1*02, and HLA–DRB1*03 were each present in the majority of children with rash (64%, 68%, and 64%, respectively). The frequency of all 3 6p alleles occurring together in 1 individual was greater in children with rash than in children who had either CHB or no manifestation of neonatal lupus (59% versus 30%; P = 0.02). This association with neonatal lupus rash was equivalent to published findings in a cohort of patients with SCLE, but significantly greater than the association in patients with discoid lupus erythematosus. Prominent TNFα staining in the epidermis was observed in lesional skin from 3 children with rash, but not in skin from a healthy neonate. Conclusion Taken together, the finding of a genetic predisposition to generate increased levels of TNFα following tissue injury and the histologic demonstration of TNFα in the target organ support the notion that this inflammatory cytokine plays a role in the pathogenesis of cutaneous neonatal lupus. Furthermore, the results of these studies provide evidence of a biologic link between neonatal lupus and the rash of SCLE.

Vascular anomalies comprise a widely heterogenous group of tumors and malformations. Great confusion has arisen because of the term hemangioma has been and is continued to be used to represent a multitude of vascular entities. This review presents the updated classification of vascular anomalies with the goal of clarifying the term hemangioma. In addition, newer clinical concepts in hemangiomas and other vascular tumors is presented. Hemangioma subtypes and hemangioma variants are also discussed, and a brief review of pyogenic granuloma and Kaposiform hemangioendothelioma is provided. Finally, the immunohistochemical marker GLUT1 is reviewed, a marker that heralds a new era in vascular anomalies research.

Over 1000 medications are in common usage today. Approximately 700 of these are associated with a variety of cutaneous eruptions. The vast majority of cutaneous drug reactions in children are of 5 types: exanthems, fixed drug reactions, urticarial eruptions, serum sickness-like reactions, and photosensitive eruptions. Among healthy children, adverse drug reactions are generally uncommon and usually minor. Common, serious, and interesting cutaneous drug eruption patterns are discussed in this monograph, with an emphasis on practical information for dermatologists who care for pediatric patients. (Curr Probl Dermatol 2002;14:147-182)

Treatment of a linear verrucous epidermal nevus using topical 0.1% tretinoin cream and 5% 5-fluorouracil in a young patient is described. In 1994, successful topical therapy using this combination was described in the management of an inflammatory linear verrucous epidermal nevus. We report another case in which treatment of a noninflamed epidermal verrucous nevus with 0.1% tretinoin and 5% 5-fluorouracil resulted in significant improvement. An updated summary of the literature discussing management of epidermal nevi is presented.

Background: The recurrent pyogenic infections of patients with hyper-IgM syndrome are controlled by intravenous gamma globulin administration, but patients may suffer from early-onset oral ulcerations and warts. Objective: We have characterized the mucocutaneous manifestations associated with this condition to allow physicians to more readily identify it. Methods: Three male patients with the mucocutaneous manifestations of the hyper-IgM syndrome are described. In one, histopathologic examination of the oral mucosal lesion was performed. Results: Recurrent large, painful oral ulcerations can occur that are not necessarily associated with neutropenia nor do they respond to granulocyte colony-stimulating factor administration. Histopathologic examination of an ulcer showed a heavy infiltrate of mixed inflammatory cells. Warts tend to be widespread and resistant to traditional therapy. Conclusion: Physicians should consider this uncommon condition when examining a male patient with severe oral ulcers or recalcitrant widespread warts. (J Am Acad Dermatol 1998;38:191-6.)

We describe three teenage siblings with confluent and reticulated papillomatosis, all presenting during a 6-month period. Two of the three patients had confirmed tinea versicolor, with positive potassium hydroxide scrapings, in association with this entity. This is the largest series of siblings with confluent and reticulated papillomatosis, and the only report describing family members having both confluent and reticulated papillomatosis and tinea versicolor. This report lends further evidence to the hypothesis that confluent and reticulated papillomatosis may be etiologically linked to tinea versicolor, and also suggests a genetic predisposition for it.

Congenital self-healing reticulohistiocytosis (CSHR) represents the benign end of the spectrum of Langerhans cell histiocytoses, with spontaneous resolution of lesions within the first year of life. However, involvement of organ systems other than the skin has been described occasionally and recurrence of disease at sites distant from the skin has been documented. We report a case of CSHR with eye involvement that spontaneously resolved concurrent with resolution of skin lesions. Because multiple organ systems can be involved and recurrences are possible, long-term follow-up of these patients is indicated.

The substantial clinical and histologic overlap between neurotized congenital melanocytic nevi and the subset of plexiform neurofibromas with hyperpigmentation and hypertrichosis of the overlying skin (pigmented neurofibroma) has led to considerable confusion in the literature. A dark-brown, hypertrichotic plaque covered much of the right lower aspect of the trunk of a 1-year-old girl with a diffuse and plexiform neurofibroma in the same area, numerous cafe-au-lait macules, and intertriginous freckling. The latter findings were diagnostic of neurofibromatosis-1, which was further supported by the presence of unidentified bright objects on magnetic resonance imaging of the brain. Histologic examination of the hyperpigmented plaque revealed melanocytic hyperplasia at the dermoepidermal junction and a proliferation of rounded, pigmented melanocytes dispersed individually and in occasional small nests in the papillary dermis and scattered within underlying neurofibromatous tissue. Immunohistochemical staining with A103 (Melan-A/MART-1) and PNL2 confirmed the melanocytic differentiation of the pigmented cells, whereas glial fibrillary acidic protein and Leu-7 were detected only within plexiform areas and slender neuroid spindle cells. This case draws attention to the pigmented neurofibroma as a distinct clinicopathologic entity resulting from proliferation of melanocytes and neurosustentacular cells in the setting of neurofibromatosis-1.

Infantile systemic hyaloinosis is a rare, progressive, and fatal disease that is inherited in an autosomal recessive fashion. We describe 2 patients in whom thickened skin; small nodules of the perianal region, face, and neck; joint contractures; growth failure; diarrhea; and frequent infections developed within the first few weeks of life. Both patients died before 2 years of age.

We report a case of congenital primary cutaneous rhabdomyosarcoma, solid alveolar type, presenting as a solitary skin lesion on the right upper lip of a 2-week-old infant boy. Rhabdomyosarcoma originates from the embryonic mesenchyme precursor of striated muscle. Histologically it belongs to the group of "small round cell tumors." Its myogenic origin is ascertained by immunohistochemical studies positive for myogenin, muscle-specific actin, desmin, and myoglobin. Malignancy in the neonatal period is uncommon and the clinical management presents considerable challenges. Congenital alveolar rhabdomyosarcoma is a highly malignant tumor with no record of long-term survivors. Treatment options include chemotherapy, excision, and radiotherapy. This infant's tumor was responsive to chemotherapy and surgery and he was free of disease at the 6-month follow-up.

Initial symptoms of Rocky Mountain spotted fever (RMSF), a tick-borne illness caused by Rickettsia rickettsii, are nonspecific and include headache, gastrointestinal disturbances, malaise, and myalgias, followed by fever and rash. The classic triad of fever, rash, and history of tick exposure is uncommon at presentation. Clinical manifestations of RMSF range from virtually asymptomatic to severe. Because of the potentially fatal outcome of RMSF, presumptive clinical diagnosis and empiric antimicrobial therapy can be critical. We present the case of a 3-year-old girl from New York State who presented with fever and rash.

Riga-Fede disease presents in early infancy and is characterized by firm, verrucous plaques arising on the oral mucosal surfaces. These histologically benign lesions occur as a result of repetitive trauma of the oral mucosal surfaces by the teeth. Early recognition of this entity is important, because it may be the presenting sign of an underlying neurologic disorder. We report the case of a 10-month-old boy with extensive Riga-Fede disease involving the lip and tongue that prompted a diagnosis of congenital autonomic dysfunction with universal pain loss.

A 7-month-old infant with cutaneous anthrax developed severe systemic ill- ness despite early treatment with antibiotics. The infant displayed severe microangiopathic hemolytic anemia with renal involvement, coagulopathy, and hyponatremia. These findings are unusual with cutaneous anthrax, but have been described in illness resulting from spider toxin and may delay cor- rect diagnosis. The systemic manisfestations of the disease persisted for nearly a month despite corticosteroid therapy, but resolved.

Cantharidin, a vesicant produced by beetles in the order Coleoptera, has a long history in both folk and traditional medicine. In dermatology, topical cantharidin has long been used to treat warts and molluscum. In 1962, cantharidin lost Food and Drug Administration (FDA) approval owing to the failure of its manufacturers to submit data attesting to cantharidin's efficacy. However, it is expected that the FDA will soon include cantharidin on its "Bulk Substances List," which would permit physicians or pharmacists to compound cantharidin to be used in the office for individual patients. A comprehensive discussion of the origins, folk uses, current FDA status, current dermatologic uses, and effects of cantharidin poisoning has been compiled herein. No cases of systemic intoxication or scarring have been reported with the proper use of cantharidin by a physician. Cantharidin is a safe and valuable medication and should be readded to the dermatologic therapeutic armamentarium.

Trichorhinophalangeal syndrome (TRPS) is characterized by its unique facial features and skeletal abnormalities. A bulbous, pear-shaped nose, elongated philtrum, sparse hair, cone-shaped epiphyses and mild growth retardation are found in both type I (TRPSI) and type II (TRPSII). TRPSII can be distinguished from TRPSI when multiple exostoses or redundant skin are present. While TRPSI is inherited in an autosomal dominant fashion, most cases of TRPSII are sporadic although there are a few cases which are familial. The following is a case report of TRPSII with incomplete penetrance in the index case and exostoses and growth retardation in the patient's two siblings.

mL/kg/h). A second dose of t-PA was administered 3 days later, in an attempt to salvage impending digit loss. Thrombolytic therapy was well tolerated with the exception of bleeding at the site of the intravenous line and mild hematuria. By day 8 of hospitalization, his transfusional requirements ceased, and protein C levels had normalized. Heparin therapy was discontinued 2 days later. There was a significant improvement in his skin lesions and areas of impending gangrene, including his scrotum (Figure, B). However, all of his toes and 7 fingers were autoinfarcted. At discharge, 12 weeks after admission, he was neurologically intact and developmentally appropriate for age. His coagulation and hematologic measurements and renal function were normal. Thrombophilia workup of both parents for inherited predisposition to thrombosis was negative.

Juvenile xanthogranuloma (JXG) is a well-recognized benign disorder of infancy and early childhood characterized by yellowish cutaneous nodules that spontaneously regress over months to years. In the vast majority of children, JXG is limited to the skin and requires no treatment. Over the past two decades, unusual cutaneous and systemic forms of JXG have been increasingly reported. JXGs have been discovered, usually unexpectedly, in every organ system of the body. Correct diagnosis is crucial to prevent unnecessary invasive diagnostic and therapeutic procedures. Unusual clinical and histological variants of JXG often require immunohistochemical studies and/or electron microscopy to establish the diagnosis. Nonlipidized, giant, intramuscular, subcutaneous, and clustered JXG are but some of the variants that are discussed in this article. The immunohistochemistry of JXG, current nosology, and hypotheses regarding the origins of JXG are also reviewed.

We describe a 10-day-old term infant who presented to the emergency room with an acute pustular eruption. Laboratory tests and clinical outcome confirmed the diagnosis of erythema toxicum neonatorum. A full septic workup was performed and all cultures were negative. Wright-stained smear of pustular contents showed a predominance of neutrophils with 10% eosinophils. The white blood cell count was 19,000/mm3 with 10% eosinophils. The eruption resolved spontaneously at 15 days of age leaving no sequelae. This is the first fully documented case of erythema toxicum in a term infant occurring as late as 10 days of age. When erythema toxicum presents in an atypical fashion, diagnostic tests are important to exclude other causes of pustular dermatoses of the neonate.

Three selected cases of transient acantholytic dermatosis were studied because of their definitive correlation with sweating due to fever and/ or bed-ridden situations. Biopsy specimens were serially sectioned and acantholysis was found in the acrosyringium or traced to connect to the acrosyringium in all biopsy specimens. Carcinoembryonic antigen (CEA) and eccrine gland-specific monoclonal antibody, IKH-4, were positive in acantholytic cells. Electron microscopy revealed electron dense material filling the lumen of intraepidermal eccrine ducts. This material leaked into lateral intercellular spaces of the luminal cells, passing tight junctions. Marked edema and numerous lysosomes were reminiscent of those found when eccrine acrosyringium is formed in the embryo; this suggested that an occluded and damaged eccrine intraepidermal duct was being rebuilt via lysosomal digestion.

Berloque dermatitis is a type of photocontact dermatitis. It occurs after perfumed products containing bergamot (or a psoralen) are applied to the skin followed by exposure to sunlight. Striking linear patterns of hyperpigmentation are characteristic, corresponding to local application of the scented product. In the acute phase, erythema and even blistering can be seen. We report a case of berloque dermatitis in a 9-year-old girl that was initially reported as child abuse. To our knowledge, this is the first report of berloque dermatitis mimicking child abuse. Questioning to elicit a history of perfume application coupled with sunlight exposure should help to prevent this misdiagnosis in children.

Figure 1. A seven-month-old male infant was hospitalized with a two-day history of swelling of the left arm and a weeping lesion at the left elbow (Panel A). The patient was afebrile but had a 2-cm open sore, with surrounding erythema and induration, that oozed clear yellow fluid. There was nontender swelling and erythema of the entire arm. The white-cell count was 28,100 per cubic millimeter. Incision and drainage of the lesion produced 10 ml of dark red fluid. A coronal, T1-weighted sequence from a magnetic resonance imaging study (Panel B) demonstrated diffuse, severe edema of the subcutaneous tissues extending . . .