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Biomedical subjects

Lawrence F Eichenfield

Publications and source records attributed to Lawrence F Eichenfield.

At least 19 recordsLinked to original sources

Cutaneous manifestations of Costello syndrome.

Cutaneous findings are common in Costello syndrome, but have not been extensively reviewed in the dermatology literature. We present the cutaneous and histopathologic findings from two cases of Costello syndrome and review previously described cutaneous features of this syndrome. Both patients had manifestations of Costello syndrome with thick, lax skin on the dorsal aspects of hands and feet, deep palmar and plantar creases, curly hair, hyperkeratoses, acanthosis nigricans, papillomas, and multiple pigmented lesions. One patient had multiple syringomas on the forearms, a finding not previously reported. Pigmented lesions have previously been reported as nevi in the literature though no biopsies have been reported. We conclude that thick, loose skin on the dorsal aspects of hands and feet and deep palmar and plantar creases are cardinal manifestations of Costello syndrome and benign tumors of ectodermal origin such as papillomas, calcified epitheliomas, dermoid cysts, mammary fibroadenosis, and syringomas are important features of this syndrome.

Abnormalities, Multiple↗

Allergic contact dermatitis from pimecrolimus in a patient with tacrolimus allergy.

A 15-year-old male with previously documented allergic contact dermatitis from tacrolimus was allergic to pimecrolimus. This was demonstrated by double-blinded, right-versus-left provocative use testing with pimecrolimus cream 1% versus inactive vehicle applied twice daily to normal skin. The active cream but not its vehicle caused preauricular dermatitis starting after 1 week and caused isolated papules on the extensor wrist starting after 2 weeks. Patch testing on the patient's back was weakly positive (1+) with pimecrolimus cream 1% and negative with the vehicle. Higher concentrations of pimecrolimus were not available for testing. Patch tests on 30 control patients with pimecrolimus cream 1% were negative.

Adolescent↗

Digitocutaneous dysplasia.

Digitocutaneous dysplasia is a rare X-linked dominant genetic syndrome characterized by multiple digital fibromas, atrophic plaques, dental anomalies, dysmorphic features, and bone anomalies. We report the case of a 2-year-old Mexican girl with this rare condition and discuss the clinical, histologic, and genetic features.

Child, Preschool↗

Periorificial dermatitis in children and adolescents.

BACKGROUND: There has been very little evaluation of the history, morphology, or disease course of perioral/periorificial dermatitis in children. OBJECTIVE: We sought to elucidate the clinical manifestations and treatment outcomes in this condition. METHODS: A retrospective chart review with telephone follow-up was used to study 79 children and adolescents. RESULTS: Patients ranged from 6 months to 18 years of age. The average duration of the rash at presentation was 8 months. Seventy-two percent had a history of topical, inhaled, or systemic steroid exposure. Seventy percent of patients had perioral involvement, 43% perinasal, and 25% periocular involvement. A perivulvar rash was reported in 1% of patients. Treatment with topical metronidazole was associated with clearing on follow-up examination. LIMITATIONS: This is a retrospective study without case controls and is subject to interviewer and memory bias. CONCLUSION: Perioral dermatitis appears at all ages in childhood and adolescence and may be associated with topical corticosteroid use. It may be responsive to topical metronidazole in children and adolescents and is more appropriately termed periorificial dermatitis.

Administration, Oral↗

Two randomized, double-blind, placebo-controlled studies of fluticasone propionate lotion 0.05% for the treatment of atopic dermatitis in subjects from 3 months of age.

A novel lotion formulation of fluticasone propionate 0.05% has recently become available. Two large, randomized, placebo-controlled, double-blind studies involving 438 subjects demonstrated its efficacy and safety when applied once daily in the treatment of atopic dermatitis in subjects from 3 months to 87 years of age. The studies were limited to 4 weeks duration of use of fluticasone lotion and did not assess longer term efficacy or side effects.

Administration, Topical↗

Congenital localized scleroderma.

OBJECTIVES: Juvenile localized scleroderma (JLS) usually has its onset during later childhood. This report describes the clinical and serologic features of six children with congenital localized scleroderma (CLS). STUDY DESIGN: A large, multinational study was conducted among pediatric rheumatology and dermatology centers by collecting information on demographics, family history, triggering environmental factors, clinical features, laboratory reports, and treatment of patients with JLS. Patients with onset at birth were carefully examined. RESULTS: Among 750 patients with JLS, 6 patients (0.8%) had scleroderma-related lesions at birth. Female-to-male ratio was 2:1. All patients had linear scleroderma, in four involving the face with en coup de sabre appearance. Two patients were misdiagnosed as having skin infection, one nevus, one salmon patch, and two undefined skin lesions. The mean diagnostic delay was 3.9 years. In comparison with the group of 733 patients with late-onset JLS, CLS presented a significantly more prolonged disease duration at diagnosis and a higher frequency of en coup de sabre subtypes. CONCLUSIONS: Congenital localized scleroderma is a rare and probably underestimated condition in neonates. The linear subtype was the exclusive manifestation of the disease. CLS should be included in the differential diagnosis of infants with cutaneous erythematous fibrotic lesions to avoid functional and aesthetic sequelae and to allow prompt therapy.

Atrophy↗

The epidemiology of molluscum contagiosum in children.

Molluscum contagiosum (MC) is a viral disorder of the skin and mucous membranes characterized by discrete single or multiple, flesh-colored papules. Although MC as a clinical entity is well defined and commonly observed, few data regarding its epidemiology in the pediatric population exist. Our purpose was to collect epidemiologic data on children with MC with regard to age, gender, ethnicity, degree of involvement, relation to pre-existing atopic dermatitis (AD), and immune status. A retrospective chart review was conducted. All subjects were seen at 3 tertiary pediatric dermatology referral centers with two of the sites based at a Children's Hospital. A total of 302 patient charts with the Current Procedural Terminology code diagnosis of MC seen over a 6- to 8-month period were reviewed. Approximately 80% of the patients were younger than 8 years old. The majority of patients (63%) had more than 15 lesions. All but one patient were otherwise healthy, as determined by history and clinical examination. Approximately 24% of the patients presented with a history of previous or active coexistent AD. However, children with AD were at risk for an increased number of lesions. These data provide valuable updated information on the demographics and clinical presentation of MC in pediatric patients in the United States. Limitations include that this was a retrospective study with a population limited to tertiary pediatric dermatology referral centers.

Age Distribution↗

Pulmonary thromboembolism associated with Klippel-Trenaunay syndrome.

Klippel-Trenaunay syndrome (KTS) is a rare congenital anomaly characterized by unilateral limb overgrowth, venous varicosities, and capillary malformations (port wine stains) of the affected limb or limbs. Large venous malformations such as those observed in KTS are rare, and many physicians are unfamiliar with the potential complications, which include hypercoagulability, thrombosis, and pulmonary embolism (PE). As a result, patients may suffer from delayed diagnosis of a potentially life-threatening thromboembolic event. We present 2 cases of children with KTS complicated by PE, and we review the English-language literature regarding pathophysiologic features, interventions, and outcomes of PE in the setting of KTS among both pediatric and adult patients, with emphasis on issues relevant to pediatricians.

Acute Disease↗

General anesthesia for pediatric dermatologic procedures: risks and complications.

OBJECTIVE: To assess the safety and adverse events associated with the use of general anesthesia in children undergoing elective dermatologic procedures. DESIGN: A multicenter retrospective review. SETTING: Children's Hospital and Health Center, San Diego, Calif, and Northwestern University School of Medicine, Chicago, Ill. PATIENTS: The study population comprised 269 children and adolescents ranging in age from 2 months to 18 years (881 procedures performed by 6 pediatric dermatologic and laser surgeons). MAIN OUTCOME MEASURES: The risk of an adverse event occurring during general anesthesia for pediatric dermatologic procedures. RESULTS: The risk of general anesthesia in elective pediatric dermatologic procedures was low: 90% of patients experienced no clinically relevant complications. The most common clinically relevant adverse effect of general anesthesia was perioperative nausea and emesis, which was noted in 4% of patients. There were no serious life-threatening events noted, and the mortality rate was 0%. CONCLUSION: The use of general anesthesia for dermatologic procedures in a children's hospital setting appears safe, with a low rate of complications.

Adolescent↗

Use of pimecrolimus cream 1% (Elidel) in the treatment of atopic dermatitis in infants and children: the effects of ethnic origin and baseline disease severity on treatment outcome.

BACKGROUND: Pimecrolimus cream 1%, a cell-selective inhibitor of inflammatory cytokines, has been shown to be effective in treating atopic dermatitis (AD). This report examines the effect of ethnic origin and baseline disease severity on treatment outcomes in pediatric patients with AD treated with pimecrolimus cream 1%. METHODS: The analysis included 589 patients aged 3 months to 17 years from three 6-week, randomized, multicenter studies of similar design. Patients were treated with pimecrolimus cream 1% or vehicle twice daily. Efficacy, safety and tolerability in Caucasian and non-Caucasian groups were compared. In addition, the effect of baseline disease severity on treatment outcome was investigated. RESULTS: A total of 321 Caucasian and 268 non-Caucasian patients [Blacks, Asians and others (including Hispanics)] with mild, moderate or severe disease at baseline were included. Baseline characteristics were comparable between the pimecrolimus and vehicle control groups and between Caucasian and non-Caucasian groups. Significantly higher efficacy [measured by Investigators' Global Assessment and Eczema Area and Severity Index (EASI) scores] was achieved in the pimecrolimus-treated group, compared with the vehicle group, irrespective of ethnic origin. Baseline disease severity had no effect on treatment outcome: patients with both mild and moderate AD responded well to pimecrolimus (absolute change from baseline in EASI score -2.60 and -5.48, respectively; both P < 0.001). Pimecrolimus cream 1% was safe and well tolerated in all ethnic groups and at all levels of disease severity. CONCLUSIONS: Ethnic origin and baseline disease severity had no effect on treatment outcome with pimecrolimus cream 1% in patients with AD.

Adolescent↗

Prevalence of scalp scaling in prepubertal children.

OBJECTIVE: To determine the prevalence and most common etiologies of scalp scaling in infants and prepubertal children and the specificity of head and neck lymphadenopathy for the diagnosis of tinea capitis associated with scalp scaling. DESIGN/METHODS: A cross-sectional study of 300 children, 200 from an urban general pediatric practice and 100 from 2 urban pediatric dermatology practices, was conducted. Half of the subjects were <2 years old, and half were 2 to 10 years old. Demographic data, medical history, and clinical data noting the presence of scalp scaling and other scalp signs and symptoms, as well as adenopathy of the head and neck, were collected. RESULTS: Scalp scaling was seen in 66 (22%) children. There was an insignificantly higher prevalence of scalp scaling in those <2 years old compared with the 2- to 10-year-old group. In those <2 years old with scalp scaling, the most common diagnoses were seborrheic dermatitis (thick, adherent, greasy scale predominantly in the frontal and/or vertex areas of the scalp) and atopic dermatitis/eczema. Among those 2 to 10 years old with scalp scaling, the most common diagnoses were nonspecific (fine, white) scaling, seborrheic dermatitis, and atopic dermatitis/eczema. Nine (3%) patients were culture-positive for a dermatophyte, all of whom were black, and grew Trichophyton tonsurans. More than half (52.7%) of all patients had head and neck adenopathy. The presence of posterior nodes was significantly associated with atopic dermatitis/eczema and marginally associated with a positive dermatophyte culture. The presence of scalp scaling plus posterior adenopathy was significantly associated with seborrheic dermatitis and a positive dermatophyte culture in the entire study population and with atopic dermatitis in the pediatric dermatology clinics. CONCLUSIONS: Scalp scaling was common in children 0 to 10 years old. Infantile-type seborrheic dermatitis was noted in both age groups: it was 3 times as likely in children <2 years old (18%) than in those 2 to 10 years old (6%). Contrary to traditional teaching, seborrheic dermatitis can be found in preadolescent children. Atopic dermatitis/eczema was associated with scalp scaling in both age groups. Head and neck adenopathy was very common and nonspecific for any 1 diagnosis. Posterior adenopathy with concurrent scalp scaling was significantly associated with seborrheic dermatitis, a positive dermatophyte culture, and atopic dermatitis. In this nonselected pediatric population, scalp scaling with adenopathy was not associated exclusively with tinea capitis.

Age Distribution↗

Allergic contact dermatitis from tacrolimus.

A 9-year-old boy developed allergic contact dermatitis from tacrolimus ointment. Tacrolimus was proven to be the allergen by right-versus-left double-blinded provocative use testing of tacrolimus ointment 0.1% versus inactive vehicle applied twice daily to normal preauricular and antecubital skin. Facial dermatitis appeared after 1 week and antecubital dermatitis after 7 weeks. Furthermore, patch testing of each individual ingredient was positive only with tacrolimus; a concentration of 2.5% in ethanol was required. Forty control patients had negative patch tests with tacrolimus 5% in ethanol. We hypothesize that the unusually long time required to elicit a positive use test on the arm and the high patch test concentration required on the back are caused by low percutaneous absorption through normal extrafacial skin. This is likely to be caused in part by the high molecular weight of tacrolimus. A similar phenomenon may occur when patch testing with neomycin sulfate.

Child↗

Venous malformations in blue rubber bleb nevus syndrome: variable onset of presentation.

Blue rubber bleb nevus syndrome (BRBNS) is a rare disorder characterized by discrete venous malformations of varying size and appearance that are present on the skin and within the gastrointestinal tract. The characteristic cutaneous lesions consist of deep-blue, soft, rubbery blebs, which are easily compressible. A serious complication is gastrointestinal bleeding. Because venous malformations were described historically as cavernous hemangiomas, the lesions of BRBNS were also inappropriately called hemangiomas in the literature. We describe 3 cases to delineate the venous malformations of BRBNS and to highlight their variable onset of presentation and progression. In one case, a venous malformation was noted during a prenatal ultrasound evaluation at 5 months gestation. The other 2 cases demonstrated a lack of the classic cutaneous lesions at birth. BRBNS consists of multiple venous malformations, rather than hemangiomas as described. Subcutaneous venous malformations may occasionally be the sole presenting finding in patients with this unusual syndrome, and may be evident even in the prenatal period.

Female↗