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Biomedical subjects

Ilona J Frieden

Publications and source records attributed to Ilona J Frieden.

At least 19 recordsLinked to original sources
PubMed2006

Keratosis pilaris rubra: a common but underrecognized condition.

BACKGROUND: Keratosis pilaris is a common skin disorder of childhood that often improves with age. Less common variants of keratosis pilaris include keratosis pilaris atrophicans and atrophodermia vermiculata. OBSERVATIONS: In this case series from dermatology practices in the United States, Canada, Israel, and Australia, the clinical characteristics of 27 patients with keratosis pilaris rubra are described. Marked erythema with follicular prominence was noted in all patients, most commonly affecting the lateral aspects of the cheeks and the proximal arms and legs, with both more marked erythema and widespread extent of disease than in keratosis pilaris. The mean age at onset was 5 years (range, birth to 12 years). Sixty-three percent of patients were male. No patients had atrophy or scarring from their lesions. Various treatments were used, with minimal or no improvement in most cases. CONCLUSIONS: Keratosis pilaris rubra is a variant of keratosis pilaris, with more prominent erythema and with more widespread areas of skin involvement in some cases, but without the atrophy or hyperpigmentation noted in certain keratosis pilaris variants. It seems to be a relatively common but uncommonly reported condition.

Adolescent↗
PubMed2006

Complications following pulsed dye laser treatment of superficial hemangiomas.

BACKGROUND AND OBJECTIVE: Pulsed dye laser (PDL) has been reported to be safe and effective in the management of superficial hemangiomas of infancy. We report 12 patients with hemangiomas with complications following PDL. STUDY DESIGN/MATERIALS AND METHODS: Records of patients with hemangiomas and a known adverse outcome following PDL were reviewed. RESULTS: All were treated early (age range: 5 days to 4 months), and all hemangiomas were facial with a superficial component. Eleven were treated with a 585 nm wavelength, fluence range of 4.7-7 J/cm(2), without dynamic cooling. One patient received 7-12 J/cm(2) utilizing a 595 nm wavelength with dynamic cooling. In eight cases, treatment led to severe ulceration with subsequent pain, scarring, and in one instance, life-threatening hemorrhage. In four, permanent atrophic scarring was noted without ulceration. CONCLUSIONS: PDL treatment of superficial hemangiomas may rarely lead to significant complications including atrophic scarring and severe ulceration.

Atrophy↗
PubMed2006

Treatment of vaginal bleeding from a pelvic vascular malformation with desmopressin.

We report the case of a 2-year-old female child with a pelvic vascular malformation complicated by chronic low-grade bleeding. Treatment with intranasal desmopressin significantly reduced vaginal bleeding. Therefore, we suggest that intermittent use of intranasal desmopressin may be useful in the treatment of chronically bleeding low-flow vascular malformations.

Administration, Intranasal↗
PubMed2006

Patterns of infantile hemangiomas: new clues to hemangioma pathogenesis and embryonic facial development.

OBJECTIVES: Large facial infantile hemangiomas have higher rates of complications than small localized hemangiomas, more often require treatment, and can be associated with neurological, ophthalmologic, and cardiac anomalies (PHACE syndrome). The anatomic patterns of these hemangiomas are often referred to as "segmental" despite a lack of precise anatomic definitions. Our study aims to define "segmental" hemangiomas based on clinically observed patterns. Our secondary goal is to relate the observed patterns to currently accepted developmental patterns to gain insight into hemangioma pathogenesis and craniofacial development. METHODS: Photographic data were extracted from a large cohort of patients with infantile hemangiomas. We mapped 294 hemangiomas and recorded common morphologic patterns. Anatomic descriptions of the most common patterns were described and compared with accepted concepts of craniofacial development. RESULTS: Four primary segments were identified (Seg1-Seg4). Seg2 and Seg3 correspond with the previously recognized maxillary and mandibular prominences. Seg1 and Seg4 differ from standard human embryology texts. The frontotemporal segment, Seg1, encompasses the lateral forehead, anterior temporal scalp, and lateral frontal scalp. The segment Seg4, encompassing the medial frontal scalp, nasal bridge, nasal tip, ala, and philtrum, is substantially narrower on the forehead than the previously described frontonasal prominence. CONCLUSIONS: The patterns provide new clues regarding facial development. The observed patterns resemble previously described facial developmental units on the lower face but are distinctly different on the upper face. The patterns suggest that neural crest derivatives may play a role in the development of facial hemangiomas. Finally, these patterns (Seg1-Seg4) help standardize the nomenclature of facial segmental hemangiomas to analyze more effectively hemangioma risks and behavior.

Face↗
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

Neonatal-onset multisystem inflammatory disorder: the emerging role of pyrin genes in autoinflammatory diseases.

Neonatal-onset multisystem inflammatory disorder (NOMID) is a rare congenital disorder characterized by a neonatal-onset urticarial rash, arthropathy, recurrent fevers, and central nervous system disease. We report 3 cases in which patients presented with neonatal-onset urticarial eruption and other organ involvement of varying severity. Genetic testing of 2 of these patients revealed previously unreported genetic mutations in exon 3 of the CIAS1 gene, a recently discovered member of the pyrin gene family. The third patient did not demonstrate a CIAS1 mutation. These cases illustrate the genetic basis of NOMID, an autoinflammatory disorder, and highlight the emerging role of the pyrin gene family in the regulation of nuclear factor kappaB signaling and other pathways involved in inflammation and apoptosis.

Carrier Proteins↗
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

Ultrapotent topical corticosteroid treatment of hemangiomas of infancy.

BACKGROUND: Superficial cutaneous hemangiomas of infancy represent a therapeutic challenge. Two small case series using ultrapotent topical corticosteroids for periocular hemangiomas were reported in the ophthalmologic literature. The use of this therapy for hemangiomas of infancy at other sites on the body has not been reported. OBJECTIVE: We sought to assess the clinical effects of short-term application of ultrapotent topical corticosteroids for the treatment of hemangiomas of infancy. METHODS: The records of 34 infants with proliferating hemangiomas of infancy that were treated with ultrapotent topical steroids were reviewed retrospectively. Treatment response was based on: (1) cessation of growth; (2) shrinkage or flattening of the lesion; and (3) lightening of the surface color. Lesions demonstrating responses of two of the three criteria were judged to have good response; one criterion, partial response; and no improvement, no response. RESULTS: Of the patients, 35% demonstrated good response, 38% partial response, and 27% no response. CONCLUSIONS: Hemangiomas in 74% of the infants demonstrated either good or partial response to treatment with ultrapotent topical corticosteroids. Of the responders, the majority reported cessation of growth before what would have been expected for their age. Improvement varied, with thinner superficial hemangiomas demonstrating better cosmetic improvement than thicker lesions.

Administration, Cutaneous↗
PubMed2005

Kaposiform hemangioendothelioma without Kasabach-Merritt phenomenon.

Kasabach-Merritt phenomenon is a serious coagulopathy associated with kaposiform hemangioendothelioma (KHE), tufted angioma, and possibly other vascular neoplasms. KHE presenting in the absence of Kasabach-Merritt phenomenon is rare, although tufted angioma frequently occurs without thrombocytopenia. We retrospectively reviewed 10 cases of KHE without Kasabach-Merritt phenomenon. The tumors appeared as soft tissue masses with the overlying skin being either normal, erythematous, or violaceous. There were no radiologic or microscopic differences in noncoagulopathic KHE as compared with coagulopathic KHE. Evidence of platelet trapping and hemosiderin deposition was seen histologically, despite normal serum platelet levels. All KHE were less than 8 cm in diameter, suggesting that tumors that grow no larger than this size are less likely to trap platelets in sufficient quantity to cause thrombocytopenia. Our series confirms that KHE appears with a wide spectrum of behavior and response to treatment. The decision as to whether or not to treat a noncoagulopathic KHE should be based on the size and location of the tumor and the possible side effects of therapy.

Child↗
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

Bullous "cellulitis" with eosinophilia: case report and review of Wells' syndrome in childhood.

A 1-year-old girl presented with acute onset of edematous erythematous plaques associated with bullae on her extremities and accompanied by peripheral eosinophilia. She was afebrile, and the skin lesions were pruritic but not tender. The patient was treated with intravenously administered antibiotics for presumed cellulitis, without improvement. However, the lesions responded rapidly to systemic steroid therapy. On the basis of lesional morphologic features, peripheral eosinophilia, and cutaneous histopathologic features, a diagnosis of Wells' syndrome was made. Wells' syndrome is extremely rare in childhood, with 27 pediatric cases reported in the literature. Because it is seen so infrequently, there are no specific guidelines for evaluation and management of Wells' syndrome among children. The diagnosis should be considered for children with presumed cellulitis and eosinophilia who fail to respond to antibiotics. Evaluation should include a directed history, physical examination, complete blood count, and stool testing for ova and parasites, to identify potential triggers. Treatment is with systemic steroid therapy unless disease is limited, in which case medium/high-potency topical steroids may be indicated. If systemic features are prominent or disease is chronic (lasting >6 months), then a referral to hematology/oncology should be considered.

Cellulitis↗
PubMed2005

Neurocutaneous melanosis presenting with hydrocephalus. Case report and review of the literature.

Neurocutaneous melanosis (NCM) is a rare congenital neurocutaneous syndrome characterized by large or multiple congenital melanocytic nevi and benign or malignant melanocytic tumors of the leptomeninges. The authors report the case of a 5-month-old girl with congenital giant melanocytic nevi who presented with symptomatic hydrocephalus. A right frontal ventriculostomy was performed in the patient. Magnetic resonance imaging demonstrated melanocyte accumulation within the hippocampi, medulla, and cerebellum. Cerebrospinal fluid cytology revealed no presence of melanocytes. A ventriculoperitoneal shunt was placed; the patient's neurological condition improved and she was discharged home in good condition. The diagnosis of neurocutaneous melanosis should be considered in a case in which an infant or child presents with hydrocephalus and either large or multiple (> or = 3) congenital melanocytic nevi. Although our patient's neurological status improved following treatment for hydrocephalus, there is no definitive therapy for NCM and symptomatic patients have a poor prognosis. Our case illustrates to the neurosurgeon the importance of recognizing the likelihood of underlying pathological conditions of the central nervous system in a child with cutaneous melanocytic nevi.

Female↗
PubMed2005

Hemangiomas: new insights and classification.

Infantile hemangiomas are the most common tumors of infancy, but less common vascular tumors also can affect young infants and children. In most cases, the diagnosis of IH can be made clinically, but imaging studies and even biopsy may be required in less-than-typical cases. With a careful history and physical examination focusing on the timing, location, and type of hemangioma, as well as extracutaneous signs and symptoms, the general pediatrician will know when to be concerned about a high risk vascular tumor and proceed with referral or further evaluations.

Child, Preschool↗