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Biomedical subjects

Anthony J Mancini

Publications and source records attributed to Anthony J Mancini.

At least 19 recordsLinked to original sources
PubMed2006

Pityriasis lichenoides in childhood: a retrospective review of 124 patients.

BACKGROUND: Pityriasis lichenoides (PL) occurs in all age groups, although predominantly in younger individuals. OBJECTIVE: We sought to study the clinical features of PL in children followed up at our institution. METHODS: The records of 124 children who were given the diagnosis of PL at our institution between 1993 and 2003 were retrospectively reviewed. RESULTS: PL chronica (PLC) was recorded in 37% of the cases, PL et varioliformis acuta (PLEVA) in 57.3%, and clinical features of both disorders were seen simultaneously in the remaining. The median age of onset was 60 months (range: 6-180 months), although the median age of onset of PLEVA (median: 60 months) was significantly younger than that of PLC (median: 72 months) (P = .03). The age distribution showed peaks at 2 to 3 years (24.8%) and 5 to 7 years (32%). A history of infection or drug intake preceded the skin manifestations in 30% and 11.2% of patients with PLC and PLEVA, respectively. The disease began most commonly during winter (35%) or fall (30%). The median duration was 20 months (range: 3-132 months) in patients with PLC and 18 months (range: 4-108 months) in patients with PLEVA. Involvement was diffuse in 74.2% of the patients, peripheral in 20.2%, and central in the remainder. The disease was recurrent in 77% of the patients (n = 80). Of the patients, 59% had pruritus, whereas 32% reported no symptoms; the remainder had fever, arthralgia, or both. Erythromycin estolate or ethylsuccinate was administered to 79.7% of the affected children; 66.6% of these showed at least a partial response. LIMITATIONS: The analyzed data were collected retrospectively and biopsies were not performed in all patients. CONCLUSIONS: PL is not an uncommon disease in childhood, with age peaks in the preschool and early school-age years. It is usually recurrent, and shows a seasonal variation with onset most often in the fall or winter. In childhood PL, erythromycin is an effective initial treatment choice.

Adolescent↗
PubMed2006

The relationship between family medical history and childhood vitiligo.

BACKGROUND: The association between a family history of vitiligo and other autoimmune/endocrine diseases and increased incidence of childhood vitiligo has been described; however, the influence of family history on the clinical characteristics of childhood vitiligo has rarely been investigated. OBJECTIVE: We sought to examine the relationship between family history and the incidence, extent, and course of childhood vitiligo. METHODS: A retrospective chart review and telephone interviews were performed for 137 pediatric patients with vitiligo and 140 control patients (patients with acne, warts, or molluscum contagiosum matched in age, sex, and ethnicity to the study group). Information about the age, sex, ethnicity, age of onset and diagnosis, site of onset, distribution, treatment, course of disease, and family history was obtained. RESULTS: Patients with vitiligo and an extended family history of vitiligo were more likely to have an earlier age of onset of disease than those with a negative family history (odds ratio = 3.70, P = .024). There was no association between family history and site of onset, distribution, or course of disease. LIMITATIONS: A relatively small sample size, recall bias, disease misclassification, and confounding factors are potential limitations of this study. CONCLUSION: Earlier onset of pediatric vitiligo is linked to a family history of vitiligo. Awareness of this association can allow for closer monitoring, earlier detection, and earlier initiation of treatment.

Adolescent↗
PubMed2006

Incontinentia pigmenti in male patients.

BACKGROUND: Incontinentia pigmenti (IP) is a rare X-linked dominant genodermatosis that is typified by distinctive cutaneous findings and often by abnormalities of teeth, hair, nails, eyes, musculoskeletal system, and central nervous system. The gene that is mutated in patients with IP has been mapped to Xq28 and encodes the NF-kappaB essential modulator, NEMO. Female patients with IP show functional mosaicism and cutaneous manifestations follow Blaschko's lines of ectodermal embryologic development. The condition is generally considered to be lethal in utero in male fetuses, suggesting that having some normal gene expression is critical for survival. OBSERVATIONS: We observed 9 boys with IP. All had normal karotypes and no apparent family history of IP. In 8 of these 9 patients, lesions were localized to one extremity at presentation. The diagnosis was confirmed by histopathologic examination that showed eosinophils within intraepidermal, multiloculated vesicles. One of the boys later developed dental and neurologic abnormalities. LIMITATIONS: The case series was small and the workup for these patients from different sites was not uniform. CONCLUSIONS: Male individuals may show cutaneous and noncutaneous features of IP in a limited distribution that allows survival. Postzygotic mutation/somatic mosaicism is the likely mechanism. Given the potential sequelae associated with this condition, continuing follow-up of these patients is recommended.

Diagnosis, Differential↗
PubMed2006

The acanthosis nigricans form of epidermal nevus.

We observed 4 individuals with the acanthosis nigricans (AN) form of epidermal nevus, with clinical and histopathologic features of AN oriented along Blaschko's lines. Three patients had stable lesions since childhood. One patient concurrently had typical bilateral AN lesions. This condition may represent a mosaic form of AN.

Acanthosis Nigricans↗
PubMed2006

Prospective study of infantile hemangiomas: clinical characteristics predicting complications and treatment.

OBJECTIVES: Infantile hemangiomas are the most common tumor of infancy. Risk factors for complications and need for treatment have not been studied previously in a large prospective study. This study aims to identify clinical characteristics associated with complications and the need for therapeutic intervention. PATIENTS AND METHODS: We conducted a prospective cohort study at 7 US pediatric dermatology clinics with a consecutive sample of 1058 children, aged < or = 12 years, with infantile hemangiomas enrolled between September 2002 and October 2003. A standardized questionnaire was used to collect data on each patient and each hemangioma, including clinical characteristics, complications, and treatment. RESULTS: Twenty-four percent of patients experienced complications related to their hemangioma(s), and 38% of our patients received some form of treatment during the study period. Hemangiomas that had complications and required treatment were larger and more likely to be located on the face. Segmental hemangiomas were 11 times more likely to experience complications and 8 times more likely to receive treatment than localized hemangiomas, even when controlled for size. CONCLUSIONS: Large size, facial location, and/or segmental morphology are the most important predictors of poor short-term outcomes as measured by complication and treatment rates.

Child↗
PubMed2005

The price of pruritus: sleep disturbance and cosleeping in atopic dermatitis.

BACKGROUND: Atopic dermatitis is a common skin disorder that most often begins in infancy. Sleep disturbances in children with atopic dermatitis are likely due to itching and scratching and not only impact the afflicted child but may also affect the entire family. Sleep characteristics in young children with atopic dermatitis and their families have not been thoroughly investigated. OBJECTIVE: To evaluate sleep disturbance and cosleeping in young children with atopic dermatitis and evaluate the association between sleep characteristics and features of the disease. DESIGN AND METHODS: Parents of 300 children ranging in age from birth to 6 years with atopic dermatitis responded to 4 questions about sleep characteristics of their child and family. Analyses determined the prevalence of reported sleep disturbance and cosleeping, and their association with features of the patients and disease severity. RESULTS: Sleep disturbance attributed to atopic dermatitis was common; most parents (> 60%) reported that the dermatitis affected how well they or their child slept. Cosleeping because of the skin condition was reported by 30% of families, and most of these parents (66%) were bothered by the cosleeping. Sleep disturbance and cosleeping were directly associated with severity of atopic dermatitis and with the degree to which parents reported that the atopic dermatitis affected the child and family's happiness. CONCLUSIONS: Sleep disturbances were more common in children with atopic dermatitis than have been reported in children overall. These results demonstrate important sequelae of a very common childhood condition that warrant further investigation and the development of intervention strategies.

Adult↗
PubMed2005

Development of the Childhood Atopic Dermatitis Impact Scale: initial validation of a quality-of-life measure for young children with atopic dermatitis and their families.

To measure the effects of atopic dermatitis (AD) on the quality of life of affected young children and their families, we developed a prototype 62-item instrument, the Childhood Atopic Dermatitis Impact Scale (CADIS). The prototype CADIS was developed from a comprehensive conceptual framework based on data from parents and clinicians. The instrument had eight subscales (four each for child and parent): physical health, emotional health, physical functioning, and social functioning. The goal of this work was to test the validity of and to refine the prototype of CADIS. Two hundred seventy parents of children under the age of 6 y with AD responded to the instrument. Content validity was demonstrated by expert and parent reviews of the drafted and refined instrument, and by analyzing parents' responses to open-ended questions about their children's skin disease. Construct validity was assessed in exploratory factor analyses which supported a refinement in the conceptual framework to consist of two dimensions with five domains: child dimensions (symptoms and activity limitation/behavior), and parent dimensions (family/social function, sleep, and emotions). Seventeen items were eliminated, yielding a 45-item refined version of CADIS (score 0-180) with evidence of content and construct validity and suggested use in clinical research.

Adolescent↗
PubMed2005

Pediatric dermatology.

My goal is that the content of this issue be relevant and useful to your clinical practice, whether it be a clinical pearl in diagnosis, a revelation regarding therapy, or an increased comfort level with dermatologic triage. I hope you have as much fun learning as I had putting this issue together with my respected colleagues.

Child↗
PubMed2004

Tacrolimus ointment promotes repigmentation of vitiligo in children: a review of 57 cases.

BACKGROUND: Vitiligo is an autoimmune disorder characterized by loss of pigmentation. Phototherapy and application of topical corticosteroids are most commonly prescribed. However, these therapies are often not effective and use of corticosteroids on the face may lead to cutaneous atrophy, telangiectasia, and ocular complications. OBJECTIVE: We sought to assess the efficacy of topical tacrolimus ointment in the treatment of pediatric vitiligo. METHODS: A retrospective review was performed of 57 pediatric patients with vitiligo at two clinical sites. Patients were treated with tacrolimus ointment for at least 3 months. Clinical responses were documented during clinic visits, and by pretacrolimus and posttacrolimus photography. RESULTS: At least partial response was noted to tacrolimus ointment on the head and neck in 89%, and on the trunk and extremities in 63% of patients. Facial vitiligo of the segmental type showed the best response rate. Two patients initially experienced burning on application. CONCLUSIONS: Topical tacrolimus ointment is an effective alternative therapy for childhood vitiligo, particularly involving the head and neck.

Adolescent↗
PubMed2004

The epidermal nevus syndromes: multisystem disorders.

The epidermal nevus syndromes are a group of congenital syndromes comprising epidermal nevi in conjunction with central nervous system (CNS), ocular, musculoskeletal, and other organ anomalies. We describe a patient with epidermal nevi and multiple organ abnormalities, highlighting the wide spectrum of involvement in epidermal nevus syndromes, and briefly summarize the literature on these rare syndromes.

Bone and Bones↗
PubMed2004

Skin.

Human skin provides a barrier between the host and the physical, chemical, and biological environment. It is also a potential portal of entry for hazardous or infectious agents and a potential target of environmental toxins. Cutaneous vulnerability may take on many forms in the embryo, infant, child, and adolescent. Teratogenic agents may occasionally target skin, as appreciated in the proposed association of the antithyroid medication methimazole, with the congenital malformation known as aplasia cutis congenita. Percutaneous absorption of topically applied substances and the potential for resultant drug toxicities are important considerations in the child. Many topical agents have been associated with systemic toxicity, including alcohol, hexachlorophene, iodine-containing compounds, eutectic mixture of local anesthetics, and lindane. Percutaneous toxicity is of greatest concern in the premature infant, in whom immaturity of the epidermal permeability barrier results in disproportionately increased absorption. Immature drug metabolism capabilities may further contribute to the increased risk in this population. Ultraviolet (UV) radiation exposure, which increases an individual's risk of cutaneous carcinogenesis, may be a particularly significant risk factor when it occurs during childhood. The "critical period hypothesis" suggests that UV exposure early in life increases the risk of eventual development of malignant melanoma. Other risk factors for malignant melanoma may include severe sunburns during childhood, intense intermittent UV exposure, and increased susceptibility of pediatric melanocytes to UV-induced DNA damage. Last, percutaneous exposure to environmental toxins and chemicals, such as insecticides and polychlorinated biphenyls, may differ between children and adults for several reasons, including behavioral patterns, anatomic and physiologic variations, and developmental differences of vital organs.

Child↗
PubMed2003

Segmental neurofibromatosis in childhood.

Segmental neurofibromatosis refers to individuals who have manifestations of neurofibromatosis type 1 (NF-1) limited to one area of the body. It results from a post-conceptional mutation in the NF-1 gene leading to somatic mosaicism. Although it is generally considered a rare condition, this report of 39 children with segmental NF-1 demonstrates that it is commonly seen in a pediatric NF-1 referral center. The mean age at diagnosis was 7.8 years (range: 2-25 years). Twenty-nine patients had only pigmentary manifestations of segmental NF-1, including seven who had only café-au-lait macules and 22 who had café-au-lait macules and freckling. Two patients had isolated plexiform neurofibromas; a third patient had a plexiform neurofibroma of the eyelid in addition to ipsilateral dysplasia of the sphenoid wing and Lisch nodules. A 12-year-old girl had an isolated tibial pseudarthrosis. An 8-year-old boy had an isolated optic pathway tumor, which behaved both biologically and radiographically as an NF1-associated tumor. While most children with segmental NF-1 have only localized pigmentary changes, some children will have isolated plexiform neurofibromas, pseudarthroses, or optic pathway tumors. Accurate diagnosis of segmental NF-1 is crucial for both management and genetic counseling.

Adolescent↗
PubMed2003

Congenital dermatofibrosarcoma protuberans: variability in presentation.

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is an uncommon low-grade fibrohistiocytic tumor that usually occurs on the trunk or proximal extremities and typically appears during the second to fifth decade of life. It most commonly begins as a red-blue plaque that grows slowly and ultimately becomes nodular. The tumor is associated with a high recurrence rate but low metastatic potential. It rarely presents in childhood and is even more rarely present at birth. The clinical diagnosis of DFSP in infancy or childhood may be difficult because, in its early stages, the tumor often resembles a vascular birthmark. OBSERVATIONS: We studied 6 patients with congenital DFSP who were initially thought to have other diagnoses, highlighting the potential clinical variability in presentation. Half of the cases in this series occurred in areas of the body outside of the typically reported distribution pattern of acquired DFSP and in locations that, therefore, may not arouse suspicion of congenital DFSP. CONCLUSIONS: Given the aggressive local potential and high recurrence rate of DFSP, early diagnosis is preferable to facilitate appropriate excision. We recommend that any infant or child presenting with a cutaneous plaque or nodule, even congenital, that does not have characteristic or diagnostic clinical features undergo tissue biopsy for histologic evaluation.

Biopsy, Needle↗
PubMed2002

Extrafacial and generalized granulomatous periorificial dermatitis.

BACKGROUND: Granulomatous periorificial dermatitis is a well-recognized entity presenting most commonly in prepubertal children as yellow-brown papules limited to the perioral, perinasal, and periocular regions. The condition is self-limiting and is not associated with systemic involvement. OBSERVATIONS: We reviewed the medical charts of 5 healthy children presenting with extrafacial granulomatous papules in addition to the typical periorificial papules. These extrafacial lesions were clinically and histologically identical to the facial lesions, were self-limiting, and were not associated with systemic involvement. Resolution seemed to be hastened with the use of systemic antibiotic therapy in 4 of the 5 patients. CONCLUSIONS: Extrafacial lesions can occur in granulomatous periorificial dermatitis and do not appear to adversely affect the duration, response to therapy, or risk of extracutaneous manifestations. Overly aggressive evaluation and inappropriate systemic therapy should be avoided.

Administration, Oral↗