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Biomedical subjects

Lawrence F Eichenfield

Publications and source records attributed to Lawrence F Eichenfield.

33 records · Page 2Linked to original sources
PubMed2003

The congenital lingual melanotic macule.

BACKGROUND: Circumscribed oral melanotic macules in adults are a well-defined entity. Congenital oral melanotic macules, however, are rare and not well characterized. We report 5 cases of congenital lingual melanotic lesions with consistent clinical and histologic features. OBSERVATIONS: Congenital lingual melanotic macules presented in all cases at birth, followed by proportional growth. Clinical findings were well-circumscribed, brown, single or multiple macules on the tongue, 2 to 5 mm in size, without history of bleeding, ulceration, or trauma. Family history was negative for similar pigmented lesions. A literature review showed only 2 previous clinical case reports with similar features. Histopathologic examination showed increased melanin pigmentation in the basal epidermal layer with varying degrees of overlying hyperkeratosis and subepidermal pigment-laden macrophages. No appreciable increase in melanocyte number, junctional nests of melanocytes, or cell atypia was noted. Two retrospective histopathology reviews mention 4 corresponding cases but report no clinical data. CONCLUSION: The congenital lingual melanotic macule represents a clinically distinct, benign, pigmented oral melanotic lesion, and may be more common than the literature suggests.

Female↗
PubMed2003

Current management of atopic dermatitis and interruption of the atopic march.

Treatment of atopic dermatitis requires a comprehensive approach that includes evaluation of potential triggers and education of the patient and family regarding proper avoidance measures. Hydration of the skin and maintenance of an intact skin barrier remain integral to proper management. Although topical corticosteroids have been a mainstay of anti-inflammatory therapy, the newer topical calcineurin inhibitors offer advantages for treatment of this chronic, relapsing disease. Studies aimed at defining optimal combination therapy and early intervention might change the treatment paradigm for atopic dermatitis.

Anti-Inflammatory Agents↗
PubMed2003

Genetic heterogeneity in erythrokeratodermia variabilis: novel mutations in the connexin gene GJB4 (Cx30.3) and genotype-phenotype correlations.

Erythrokeratodermia variabilis is an autosomal dominant genodermatosis characterized by persistent plaque-like or generalized hyperkeratosis and transient red patches of variable size, shape, and location. The disorder maps to a cluster of connexin genes on chromosome 1p34-p35.1 and, in a subset of families, results from mutations in the gene GJB3 encoding the gap junction protein connexin-31 (Cx31). A recent report suggested the involvement of another connexin gene (GJB4) in the etiology of erythrokeratodermia variabilis. In this study, we sequenced the coding region of GJB4 in 13 unrelated erythrokeratodermia variabilis families without detectable mutations in GJB3. Mutation analysis revealed six distinct missense mutations in five families and a sporadic case of erythrokeratodermia variabilis, all of which were not found in controls. Mutation G12D, identified in an extended Dutch family, lies in the predicted amino-terminus and may interfere with the flexibility of this domain, connexin selectivity, or gating polarity of gap junction channels. Other mutations (R22H, T85P, F137L, F189Y) were located in the transmembrane domains of Cx30.3, and are predicted to hinder regulation of voltage gating or alter the kinetics of channel closure. Affected individuals of two unrelated families harbored point mutations leading to amino acid substitution F137L, which was also reported in GJB3, yet the extent and severity of hyperkeratosis was milder compared to the corresponding mutation in GJB3. Two mutations (T85P, F137L) were associated with the occurrence of rapidly changing erythematous patches with prominent, circinate, or gyrate borders in affected children but not in adults, supporting the notion that this feature is specific to Cx30.3 defects. Nevertheless, we observed highly variable intrafamilial phenotypes, suggesting the strong influence of modifying genetic and epigenetic factors. In addition to pathogenic mutations, we identified several missense mutations and a 4 bp deletion within the GJB4 coding region, which might represent either inconsequential polymorphisms or recessive mutations. In conclusion, our results demonstrate genetic heterogeneity in erythrokeratodermia variabilis, and emphasize that intercellular communication mediated by both Cx31 and Cx30.3 is crucial for epidermal differentiation.

Connexins↗
PubMed2003

Elidel (pimecrolimus) cream 1%: a nonsteroidal topical agent for the treatment of atopic dermatitis.

Elidel is a steroid-free cream containing a 1% strength of the topical immunomodulator pimecrolimus. Elidel was specifically developed as a treatment for atopic dermatitis (AD) and is approved for use in children as young as 2 years of age. The production of inflammatory cytokines by activated T cells in skin is thought to play an important role in the pathogenesis of AD. Elidel potently suppresses cytokine production by dermal T cells without significantly impairing systemic immune responses. Elidel does not cause steroid-associated local effects, such as dermal atrophy, striae, or telangiectasia. In randomized controlled clinical studies, twice-daily application of Elidel was shown to significantly improve the signs and symptoms of AD in infants, children, and adults. The clinical effect of Elidel on pruritus, the most troublesome symptom of AD, can be observed within 1 week of therapy and is maintained for the duration of treatment. Elidel is well tolerated; the risk of application-site reactions, such as itching or burning, is comparable with that of the vehicle. Adverse effects were generally mild in patients receiving Elidel and occurred at rates comparable with those in patients receiving vehicle treatment. In a 1-year study, Elidel significantly reduced the incidence of flares when used at the first signs and symptoms of acute AD. As a result, overall corticosteroid use to treat flares was significantly lower in patients using Elidel for early intervention.

Administration, Oral↗
PubMed2003

Bullous aplasia cutis congenita.

A tense yellow vesicle was noted on the back parietal scalp of a female newborn. This proved to be a bullous variant of aplasia cutis congenita. Only 16 cases of this apparently rare disorder have been previously reported. Histologic evaluation of such lesions reveals a distinct pattern containing fibrovascular stromas, edematous stroma, or both. Identical histologic findings are found in encephaloceles and meningoceles, supporting the recently proposed hypothesis that this variant of aplasia cutis may represent the form fruste of a neural tube closure defect. This disorder should be included in the differential diagnosis of vesicobullous lesions in the neonate. Bullous aplasia cutis congenita is a rare clinical subtype of aplasia cutis congenita with distinctive histologic findings. We present a new case, and summarize the clinical and histologic findings of the 16 cases previously reported in the English-language literature. Bullous or membranous aplasia cutis congenita may represent a form fruste of a neural tube defect.

Ectodermal Dysplasia↗
PubMed2003

Fluocinolone acetonide 0.01% in peanut oil: therapy for childhood atopic dermatitis, even in patients who are peanut sensitive.

BACKGROUND: Fluocinolone acetonide 0.01% in a blend of refined peanut and mineral oils has been used as treatment for scalp psoriasis for several years, but only more recently for atopic dermatitis. OBJECTIVE: We sought to study the effectiveness for atopic dermatitis, potential for adrenal axis suppression, and safety of the fluocinolone acetonide 0.01% in oil in children with atopic dermatitis, including children with atopic dermatitis and peanut allergic sensitivity. METHODS: Three separate studies were performed in children aged 2 to 12 years with atopic dermatitis: multicenter double-blind, randomized, and vehicle-controlled trial; cortisol stimulation testing; and prick testing, patch testing, and monitored medication use in children with peanut allergic sensitivity. RESULTS: Improvement of >/=50% was demonstrated within 2 weeks in 81% to 87% of 81 patients treated with active medication versus 39% of 45 children treated with vehicle oil alone. No adrenal suppression occurred after 4 weeks of therapy in 32 patients. None of 9 patients who were peanut sensitive reacted to either the full formulation or vehicle in prick or patch testing; 20 children who were peanut sensitive showed no allergic reactions after application of the medication. CONCLUSION: Fluocinolone 0.01% in peanut oil is an effective alternative to the use of topical corticosteroid agents in ointment, cream, and lotion forms in children. No evidence of adrenal suppression or adverse local effects were demonstrated in these studies. The medication was well tolerated in patients with peanut allergic sensitivity.

Administration, Topical↗
PubMed2003

Long-term safety of tacrolimus ointment in children treated for atopic dermatitis.

Atopic dermatitis (AD) is a chronic, relapsing, highly pruritic inflammatory skin disease that is particularly prevalent in the paediatric population. Emollients and topical corticosteroids have represented the standard of treatment for patients with AD, despite their numerous adverse effects and patients' tendency towards steroid resistance. Topical tacrolimus marks the introduction of an entirely new class of medications, the non-corticosteroid topical immunomodulators. Numerous short- and long-term and ongoing paediatric and adult studies have demonstrated the excellent efficacy and superb safety profile of this anti-inflammatory agent. The authors review many of these landmark studies and discuss the mechanism of action and safety profile of tacrolimus, in the context of the pathophysiology of AD.

Administration, Cutaneous↗
PubMed2003

Atopic dermatitis and asthma: parallels in the evolution of treatment.

OBJECTIVES: To review epidemiologic correlations between asthma and atopic dermatitis (AD), identify common features in disease pathophysiology, and review steps involved in the development of asthma therapy guidelines to assess the appropriateness of a similar process and approach for AD. METHODS: A 7-member panel representing specialists in dermatology, allergy, asthma, immunology, and pediatrics from around the United States convened to review the current literature and evolving data on AD. Participants presented reviews to the panel on the epidemiology of asthma and AD, the genetic predisposition to allergic disease, the current understanding of the immunopathophysiology of AD, interrelationships between the pathologic pathways of asthma and AD, evolving treatment concepts and options in AD, and the applicability of the asthma treatment model and how it may be adapted for guideline development for AD. Commentary and criticism were recorded for use in document preparation. RESULTS: There are clear epidemiologic parallels in asthma and AD. Importantly, AD frequently is the first manifestation of an atopic diathesis, which occurs in genetically predisposed individuals and also includes asthma and allergic rhinitis. Up to 80% of children with AD will eventually develop allergic rhinitis or asthma later in childhood. This classic "atopic triad" has numerous pathophysiologic elements in common, including cyclic nucleotide regulatory abnormalities, immune cell alterations, and inflammatory mediators and allergic triggers. New therapeutic options that target underlying immune mechanisms are available, and their place among treatments for AD is becoming established. Guidelines of care have been developed for asthma. The panel noted that the National Institutes of Health/National Heart, Lung, and Blood Institute guidelines for diagnosis and management of asthma, first issued in 1991, had a tremendous positive impact on many aspects of asthma treatment. It not only created a heightened awareness that asthma is a disease of chronic inflammation, but it also provided unified approaches for therapy and opened new areas of basic science and clinical research. In addition, the guidelines spurred interactions among physicians of various specialties and stimulated a great quantity of research in asthma therapy. It is anticipated that AD therapy guidelines would have similar positive outcomes. CONCLUSIONS: The panel concluded that, on the basis of current information and evolving therapeutic options, a clear rationale exists to support AD guideline development. The many parallels between AD and asthma suggest that processes and approaches used for the asthma therapy guidelines would be appropriate for AD.

Administration, Topical↗
PubMed2002

Safety and efficacy of pimecrolimus (ASM 981) cream 1% in the treatment of mild and moderate atopic dermatitis in children and adolescents.

BACKGROUND: The ascomycin derivative pimecrolimus (ASM 981) is a cell-selective cytokine inhibitor, specifically developed for the treatment of inflammatory skin diseases. OBJECTIVE: When applied topically, pimecrolimus cream 1% has shown promise as a treatment for inflammatory skin conditions, including atopic dermatitis (AD) in children and adults, allergic contact dermatitis, and chronic contact irritant hand dermatitis in adults. METHODS: In two independent 6-week, randomized, multicenter studies of identical design, the efficacy and safety of pimecrolimus cream 1% in children with predominantly moderate AD were compared with vehicle. Pooled data from a total of 403 patients were used in the analysis. The primary efficacy parameter was the Investigator's Global Assessment (IGA) score. Secondary parameters included Eczema Area and Severity Index (EASI) and severity of pruritus scores. Subjects were also asked to assess their disease control as uncontrolled, limited, good, or complete. RESULTS: Significant therapeutic benefits relative to vehicle were observed in the pimecrolimus-treated group at the first efficacy assessment, 8 days after initial application of the study medication (eg, relief of pruritus). At each subsequent postbaseline visit, pimecrolimus-treated patients showed significant improvement relative to controls in all efficacy measures. The medication was well tolerated. CONCLUSION: Pimecrolimus cream 1% appears to be a safe and effective alternative to currently used therapies for AD.

Administration, Topical↗
PubMed2002

A clinical study to evaluate the efficacy of ELA-Max (4% liposomal lidocaine) as compared with eutectic mixture of local anesthetics cream for pain reduction of venipuncture in children.

OBJECTIVE: A double-randomized, blinded crossover trial was performed to assess the efficacy of ELA-Max (4% liposomal lidocaine) as compared with eutectic mixture of local anesthetics (EMLA) for pain relief during pediatric venipuncture procedures. Safety was assessed by evaluation for topical or systemic effects and measurement of serum lidocaine concentrations. METHODS: A total of 120 children who were scheduled for repeat venipuncture for non-study-related reasons at 2 sites participated in the study. Patients were doubly randomized to treatment regimen (study medication application time of either 30 or 60 minutes) and to the order of application of the topical anesthetics for each venipuncture. The primary outcome measures were the child's rating of pain immediately after the venipuncture procedures using a 100-mm visual analog scale (VAS) tool and the parent's and blinded research observer's Observed Behavioral Distress scores. RESULTS: Both ELA-Max and EMLA seemed to alleviate venipuncture pain. There was no clinically or statistically significant difference in the patient VAS scores within the 30-minute or 60-minute treatment groups, and there was no clinical or statistical difference in VAS scores between the 30-minute ELA-Max treatment without occlusion and the 60-minute EMLA treatment with occlusion. There were no clinically or statistically significant differences between treatment with ELA-Max and EMLA in parental or blinded researcher Observed Behavioral Distress scores, the most frequent response at any observation time being "no distress." CONCLUSION: This study demonstrates that a 30-minute application of ELA-Max without occlusion is as safe and as effective for ameliorating pain associated with venipuncture as a 60-minute application of the prescription product EMLA requiring occlusion.

Adolescent↗
PubMedDate not supplied

A practical approach for the use of oral isotretinoin for infantile acne.

Infantile acne is a rare occurrence. It is more common in boys and predominately occurs on the cheeks in infants between the ages of 1 and 16 months. Clinically, the lesions range from comedones to inflammatory papulopustules to cysts. Successful therapies include topical tretinoin, benzoyl peroxide and topical and oral erythromycin. For more serious cases, oral isotretinoin (Accutane) has been reported to successfully treat recalcitrant infantile cystic acne. We describe two additional patients with infantile cystic acne treated successfully with oral isotretinoin. The dose of isotretinoin used ranged from 0.2 mg/kg/day to 1.5 mg/kg/day. The treatment duration varied from 5 to 14 months. Careful monthly monitoring is recommended because of the many side effects reported with isotretinoin. Practical tips for the administration of oral isotretinoin in infants are reviewed.

Acne Vulgaris↗
PubMedDate not supplied

What is standard of care in the evaluation of elastosis perforans serpiginosa? A survey of pediatric dermatologists.

Elastosis perforans serpiginosa is a rare chronic dermatosis characterized by extrusion of altered elastic fibers through the epidermis. It often occurs in association with a variety of connective tissue disorders, and may develop following penicillamine therapy; however, it may also present without comorbidities. There are currently no well-established protocols for the investigation of possible associated disorders in patients who present with elastosis perforans serpiginosa. We describe three patients with idiopathic elastosis perforans serpiginosa seen at our clinic and review the standard of care among 31 pediatric dermatologists surveyed who have cared for such a patient within the last 2 years. Based upon the results of our survey, we conclude that most pediatric dermatologists limit their evaluation of such patients to a thorough patient history and physical examination. This limited approach may be a sufficient evaluation in affected patients who are otherwise healthy.

Adolescent↗