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Biomedical subjects

Beth A Drolet

Publications and source records attributed to Beth A Drolet.

At least 19 recordsLinked to original sources

Intertriginous eruption associated with chemotherapy in pediatric patients.

BACKGROUND: Cutaneous eruptions commonly occur in children receiving chemotherapy, and the clinical situation often demands immediate diagnosis and initiation of treatment. Several patterns of cutaneous eruptions to chemotherapy have been reported; however, the nomenclature used to describe these entities has been derived from the histologic findings. The morphologic characteristics, distribution, and natural history of these reactions have not been well established. OBSERVATIONS: We report the clinical features of 16 pediatric patients with a distinctive chemotherapy-induced eruption. The eruption is most prominent in or limited to intertriginous regions and areas of occlusion. We were not able to identify any single chemotherapeutic agent or even a group of agents in the same pharmacologic family that seemed to be associated with this reaction. The eruption did not appear to be related to sex, age, ethnicity, underlying malignancy, or genetic disease. CONCLUSIONS: Recognition of this distinct clinical pattern can help rule out more serious entities, avoid a biopsy, and reassure the physician and patient of the benign and self-resolving clinical course. This entity may be observed with many chemotherapeutic agents and underlying diseases, but most often with high-dose chemotherapy protocols.

Abdomen↗

Early stroke and cerebral vasculopathy in children with facial hemangiomas and PHACE association.

PHACE association is a rare neurocutaneous syndrome that may include posterior fossa malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, eye abnormalities, and sternal defects. The arterial abnormalities may be developmental or acquired and usually involve the cervical and cerebral vasculature. We believe that infants with PHACE association are at increased risk of arterial ischemic stroke and describe 5 infants with facial hemangiomas and structural and acquired arterial anomalies, all of whom suffered an arterial ischemic stroke during infancy.

Central Nervous System Vascular Malformations↗

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↗

Linear scleroderma en coup de sabre with associated neurologic abnormalities.

Linear scleroderma represents a unique form of localized scleroderma that primarily affects the pediatric population, with 67% of patients diagnosed before 18 years of age. When linear scleroderma occurs on the head, it is referred to as linear scleroderma en coup de sabre, given the resemblance of the skin lesions to the stroke of a sabre. Here we describe 3 pediatric patients with linear scleroderma en coup de sabre who presented with neurologic abnormalities before or concurrent with the diagnosis of their skin disease. Our patients' cases highlight the underrecognized relationship between neurologic complications and linear scleroderma en coup de sabre and illustrate the importance of a thorough skin examination in patients with unexplained neurologic disease.

Bell Palsy↗

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↗

Asymptomatic inflammatory bowel disease presenting with mucocutaneous findings.

Although inflammatory bowel disease (IBD) typically presents with gastrointestinal complaints, mucocutaneous lesions are commonly associated and can precede gastrointestinal symptoms, thereby alerting the clinician to the diagnosis of IBD before the onset of gastrointestinal symptoms. Nine children are reported who had no gastrointestinal symptoms suggestive of IBD but presented with mucocutaneous findings of IBD and were subsequently diagnosed with Crohn's disease or ulcerative colitis based on characteristic features on gastrointestinal endoscopy and/or biopsies. The majority of the patients had oral and perianal lesions. We believe that IBD is a common etiology for persistent oral lesions in the pediatric population. In addition to a good history, children with unexplained oral mucous membrane lesions should have an examination of the rectal and genital mucosa as well as tests for complete blood count, iron levels, sedimentation rate, albumin, and occult blood in the stool with endoscopy and biopsies to rule out IBD if indicated.

Child↗

Lichenoid eruptions in children.

Lichenoid eruptions are quite common in children and can result from many different origins. In most instances the precise mechanism of disease is not known, although it is usually believed to be immunologic in nature. Certain disorders are common in children, whereas others more often affect the adult population. Lichen striatus, lichen nitidus, Gianotti-Crosti syndrome, and lichen spinulosus are examples of lichenoid lesions that are more common in children than adults. Distinguishing these diseases is necessary for prediction of the course of the eruption and for optimal management. In most cases, certain clinical characteristics enable the clinician to reach a diagnosis, whereas in other cases biopsy is required for a definitive answer. Many of these lesions are self-limited and only require symptomatic treatment, although corticosteroids can hasten resolution in certain disorders. Discontinuation of the medication is often sufficient for resolution of lichenoid drug eruptions.

Acrodermatitis↗

Skin lesions in the neonate.

Neonatal skin provides physical protection and assists in fluid balance,immunosurveillance, and thermoregulation; thus, playing a vital role in the newborn's transition from an aqueous to an air-dominant environment. Worried parents often seek medical attention from their child's physician regarding skin lesions. Thus,a working knowledge of both normal and abnormal cutaneous lesions of the neonate is required to properly address these issues. In this article, the authors briefly discuss transient benign lesions,pustular and vesicular infections, "birthmarks" (vascular and pigmentary lesions), common congenital abnormalities, select blistering disorders, and various other skin conditions.

Hemangioma↗

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↗

Dermatologic manifestations of Crohn disease in children: response to infliximab.

Dermatologic extraintestinal manifestations of Crohn disease may be refractory to treatment with corticosteroids and immunomodulators. The authors describe four children with Crohn disease with dermatologic manifestations: pyoderma gangrenosum, orofacial involvement, erythema nodosum, and idiopathic lymphedema. These dermatologic conditions were unresponsive to conventional therapy but had rapid and sustained response to the anti-TNF-alpha antibody infliximab. No adverse reactions occurred. Infliximab should be considered for treating the extraintestinal dermatologic manifestations of Crohn disease in children.

Adolescent↗

Cutaneous manifestations of hemophagocytic lymphohistiocytosis.

BACKGROUND: Hemophagocytic lymphohistiocytosis is a rare, rapidly progressive, and potentially fatal disorder of activated histiocytes. The initial clinical presentation commonly includes fever, hepatosplenomegaly, and pancytopenia. Skin eruptions are described in up to 65% of patients. Information regarding the morphological features, configuration, and distribution of these eruptions is lacking and is typically reported as nonspecific and "maculopapular." The aim of this report is to better delineate the cutaneous manifestations of the disorder to assist in differentiating the process from other systemic diseases. OBSERVATION: A case report of a neonate with hemophagocytic lymphohistiocytosis with generalized purpuric macules is described. The clinical features of 5 other patients with hemophagocytic lymphohistiocytosis at Children's Hospital of Wisconsin, Milwaukee, are summarized. Clinical images of 1 additional neonatal patient with hemophagocytic lymphohistiocytosis are presented as well. These observations demonstrate the varied cutaneous manifestations of hemophagocytic lymphohistiocytosis: erythroderma, generalized purpuric macules and papules, and morbilliform eruptions. CONCLUSION: Awareness of cutaneous involvement can assist in the initial diagnosis of hemophagocytic lymphohistiocytosis and potentially signify recurrences.

Antineoplastic Combined Chemotherapy Protocols↗

Familial nonmembranous aplasia cutis of the scalp.

Aplasia cutis of the scalp is often a sporadic condition, but familial occurrences with an autosomal dominant inheritance have been documented. Aplasia cutis of the scalp may be seen in two main clinical variants: oval-shaped membranous aplasia cutis and irregular, larger defects. We report six families in whom more than one member has aplasia cutis of the scalp, all of them with large irregular defects located over the vertex or anterior to the vertex along the sagittal suture. We review previous reports of this entity with clinical pictures and note that in most instances, the defects are of the nonmembranous variant.

Ectodermal Dysplasia↗

Twin nevus?

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Female↗