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Biomedical subjects

William L Weston

Publications and source records attributed to William L Weston.

17 recordsLinked to original sources
PubMed2006

What dermatologists do not know about smallpox vaccination: results from a worldwide electronic survey.

The risk of a bioterrorist attack with smallpox has increased owing to breakthroughs in the de novo synthesis of long-chain DNA molecules. Although the leading roles of dermatologists in diagnosing recent outbreaks of cutaneous anthrax and monkeypox demonstrate the importance of dermatologist preparedness for bioterrorism, dermatologist knowledge regarding smallpox vaccination has not been extensively examined. We conducted a cross-sectional worldwide electronic survey of all members of the American Academy of Dermatology with available e-mail addresses. The response rate was 23% (1,303/5,723): 34% of respondents were women, 52% were age 50 or older, 85% practiced in the US, and 90% reported English as their primary language. Less than 37% indicated the Centers for Disease Control and Prevention estimated rate of death owing to smallpox vaccination (1 in 1,000,000), and many failed to identify vaccination contraindications: previous myocardial infarction (83%), angina (83%), congestive heart failure (78%), steroid eye drop use (65%), and the non-emergency vaccination of those younger than age 18 (95%). Widespread dermatologist smallpox vaccination knowledge deficits pinpoint opportunities for educational efforts.

Adult↗
PubMed2005

Congenital curvilinear palpable hyperpigmentation.

We report two cases of congenital curvilinear palpable hyperpigmentation on the posterior aspect of bilateral legs in male infants. These lesions appeared shortly after birth and mimicked child abuse with looped cord or postinflammatory hyperpigmentation. Histopathologic features showed lentiginous melanocytic hyperplasia. One of the boys also had severe global developmental delay with abnormal findings from magnetic resonance imaging of the brain. We believe that these lesions represent a new type of congenital hyperpigmentation that we termed "congenital curvilinear palpable hyperpigmentation." Although these lesions do not follow the lines of Blaschko, they may also be associated with neurologic and developmental abnormalities.

Child, Preschool↗
PubMed2004

Demand outstrips supply of US pediatric dermatologists: results from a national survey.

BACKGROUND: The US pediatric dermatology workforce was last examined in 1986 when limited employment opportunity was found. OBJECTIVE: We sought to re-examine pediatric dermatology workforce issues. METHODS: US dermatology chairpersons and residency program directors were surveyed for: (1) agreement with pediatric dermatology workforce statements; and (2) pediatric dermatology faculty and fellow numbers. RESULTS: Respondents agreed that having a pediatric dermatologist or dermatologists on faculty is important, and that a shortage of pediatric dermatologists exists, but did not agree that increasing pediatric dermatology training requirements will increase this shortage. Almost half of the programs (45/94) employed a full-time pediatric dermatologist, and 24 programs had currently been recruiting a pediatric dermatologist for more than 1 year. Only 6 pediatric dermatology fellows were in training. CONCLUSION: Given that open pediatric dermatology faculty positions greatly exceed the number of fellows in training and that formal training requirements will be increasing, the shortage of pediatric dermatologists will likely continue.

Data Collection↗
PubMed2002

Neonatal lupus erythematosus.

Neonatal lupus erythematosus is associated with cutaneous lesions, CHB, hepatic disease, and thrombocytopenia. IgG antibodies to Ro and/or La cross the placenta and participate in the development of the clinical manifestations. Mothers of babies with NLE are likely to develop collagen vascular diseases with time. Infants with NLE are at risk to develop other autoimmune diseases during childhood or adolescence.

Antibodies, Antinuclear↗
PubMedDate not supplied

Giant cell fibroblastoma in a child misdiagnosed as a dermatofibroma.

We report a 9-year-old African-American boy with a giant cell fibroblastoma of the shoulder that was incorrectly diagnosed as a keloid and dermatofibroma. Initial misdiagnosis led to a delay of 4 years in the correct diagnosis, with the tumor producing significant local destruction. We review herein the clinical manifestations, histologic findings, histogenesis, relationship to dermatofibrosarcoma protuberans (DFSP), treatment, and differential diagnosis of giant cell fibroblastoma (GCF). This information is important in correctly diagnosing this uncommon, benign, but locally aggressive and recurrent tumor of childhood. The clinician should consider GCF and DFSP when the pathologic diagnosis of dermatofibroma is made in lesions more than 2 cm in diameter, or when this diagnosis is made in a prepubertal child.

Child↗
PubMedDate not supplied

Rofecoxib-induced instant aquagenic wrinkling of the palms.

An 18-year-old woman presented with a 3-week complaint of exaggerated palmar wrinkling and swelling following brief exposure (1-2 minutes) of her hands to water. She had a history of mixed connective tissue disease and had been started on rofecoxib therapy 1 month prior to the onset of her skin symptoms. Discontinuation of rofecoxib was followed by resolution of symptoms within a period of 3 weeks. Similar palmar skin changes following water exposure have been reported to occur in cystic fibrosis and are thought to be due to increased salt content of the skin and secondary increased water-binding capacity. Rofecoxib is a selective COX-2 inhibitor that has been shown to increase sodium reabsorption in the kidney via effects on prostaglandin E2 and the renal vasculature. The COX-2 protein is also expressed in keratinocytes and plays a role in keratinocyte differentiation. Prostaglandin E2 also plays a role in keratinocyte proliferation and differentiation. Thus rofecoxib may cause increased sodium reabsorption in the skin, as it does in the kidney. The rofecoxib-associated elevation in skin sodium may increase keratin water-binding capacity and cause exaggerated aquagenic wrinkling of the skin, as occurs in cystic fibrosis.

Adolescent↗
PubMedDate not supplied

Self-healing juvenile cutaneous mucinosis.

A healthy 14-month-old black girl presented with a 3-week complaint of "knots" on the face and hands. The lesions were acute in onset and asymptomatic. Multiple, firm, nontender, skin-colored to erythematous nodules were noted on the scalp, forehead, axillae, lower legs, abdomen, and hands. A skin biopsy specimen revealed a well-circumscribed accumulation of mucin in the reticular dermis. Colloidal iron stain was positive. Radiographs showed soft tissue prominence only. Serum protein electrophoresis, thyroid function tests, complete blood count, sedimentation rate, and antinuclear antibody were normal, except for lymphocytosis. Findings were consistent with self-healing juvenile cutaneous mucinosis (SHJCM). SHJCM is a condition of unknown etiology characterized by rapid onset of asymptomatic, indurated papules or nodules. Affected children may have arthralgias, but are otherwise well. Spontaneous resolution is the rule. Most skin lesions in our patient had resolved within 6 months of onset. This patient is unique because of the young age of onset.

Biopsy, Needle↗
PubMedDate not supplied

Cutaneous pustular leukemoid reactions in trisomy 21.

We report two neonates with Down syndrome and postnatal leukemoid reactions who developed acute widespread pustular eruptions. The white blood cell (WBC) counts on the first day of life were markedly elevated, with blasts seen on examination of the peripheral blood smear. The skin eruptions progressed and became pustular. Viral and bacterial cultures were negative. Skin examination revealed pustules on an erythematous base on the cheeks, shoulders, trunk, and proximal extremities. Skin biopsy specimens showed an intraepidermal pustule with an inflammatory infiltrate including neutrophils, eosinophils, and mononuclear cells. The mononuclear cells had atypical, immature-appearing nuclei. In patient 1, these cells were strongly myeloperoxidase positive on immunohistochemistry, indicating myeloid lineage. In patient 2, these cells were CD3-positive T cells. Patient 1 received a 5-day infusion of continuous cytarabine (ara-C) secondary to high WBC counts and symptomatic hyperviscosity. During therapy, the high WBC count and the pustules resolved. The lesions of patient 2 improved with topical mometasone furoate and resolved as her WBC count decreased. Recently, similar cases have been reported. Transient myeloproliferative disorders, or leukemoid reactions, should always be considered when newborns with Down syndrome or trisomy 21 mosaicism develop a pustular eruption.

Biopsy, Needle↗
PubMedDate not supplied

Sarcoptes scabiei infestation misdiagnosed and treated as Langerhans cell histiocytosis.

The inflammatory cell infiltrate in Sarcoptes scabiei infestations often includes Langerhans cells. Scabies infestations in children may mimic Langerhans cell histiocytosis (LCH) clinically as well. We report two children with scabies who were misdiagnosed clinically and histologically as LCH and treated with systemic chemotherapy. Scabies must always be ruled out in infants and children with eczematous eruptions and inflammatory infiltrates that include histiocytes on histologic examination.

Child↗
PubMedDate not supplied

Dermatitis herpetiformis presenting as chronic urticaria.

Childhood dermatitis herpetiformis (DH) is an immunobullous disease associated with gluten-sensitive enteropathy. This disease is rare in children and is typically characterized by intensely pruritic vesicles on the extensor surfaces. Definitive diagnosis of DH depends on the direct immunofluorescence finding of granular or fibrillar IgA deposits along the basement membrane zone of biopsied perilesional skin. We report an 11-year-old boy with an unusual presentation of DH characterized by a 7-month history of chronic urticaria-like skin lesions. He had evanescent, largely asymptomatic, urticarial wheals on his trunk, face, and extremities that were unresponsive to conventional therapy for urticaria. Skin biopsy specimen findings were consistent with DH and direct immunofluorescence of perilesional skin was diagnostic. The patient had no symptoms of gluten-sensitive enteropathy at the time of diagnosis, and his skin lesions rapidly cleared with dapsone therapy. This patient serves to highlight an unusual presentation of childhood DH and the need to consider this diagnosis when evaluating chronic urticarial lesions in children.

Anti-Inflammatory Agents, Non-Steroidal↗
PubMedDate not supplied

Treatment of multiple trichoepitheliomas with topical imiquimod and tretinoin.

An 11-year-old white girl presented multiple flesh-colored dome-shaped papules on the forehead, nose, scalp, and posterior neck. The lesions had been present for 2 years and were asymptomatic. Family history was negative for skin diseases. A skin biopsy specimen revealed histopathology consistent with trichoepitheliomas. Treatment of multiple trichoepitheliomas is usually difficult. Methods reported in the literature include laser treatment, surgery, cryotherapy, electrodessication, and radiation. The parents were concerned about the risk of scarring and wanted to pursue a nonscarring treatment. The patient was initially treated with topical imiquimod three times a week. She progressively increased the frequency of application to twice daily and added topical tretinoin gel once daily to her regimen for more resistant lesions. After 3 years of treatment, the patient experienced approximately 80% clearing of lesions without scarring. The advantage of using this nonsurgical treatment is no scarring, painless, and no need for other invasive procedures such as injection of local anesthetic.

Aminoquinolines↗
PubMedDate not supplied

Atypical IgA/IgG pemphigus involving the skin, oral mucosa, and colon in a child: a novel variant of IgA pemphigus?

Immune-mediated blistering diseases are rare in childhood, with the subset of IgA pemphigus being extremely uncommon. A child with a bullous disorder of the oral mucosa, skin, and colon is reported because of the unusual nature of the clinical and laboratory findings. Immunofluorescence studies demonstrated both intercellular epidermal and basement membrane zone deposition of IgA and IgG antibodies, as well as intercellular colonic deposition of IgA antibodies. IgA and IgG ELISA tests were positive for desmoglein 3 as the intercellular epidermal antigen. The generation of multiple antibodies against several potential antigenic targets suggests epitope spreading may play a role in this disease. We believe this represents a novel variant of IgA pemphigus.

Anti-Inflammatory Agents↗
PubMedDate not supplied

Dermatitis under soccer shin guards: allergy or contact irritant reaction?

We report a retrospective analysis of eight children aged 9 to 16 years who were evaluated for a persistent or recurrent dermatitis that appeared under soccer shin guards. To examine the possibility of contact allergy, all had 51 standard epicutaneous (patch) tests applied and three or four additional tests comprised of pieces of the shin guard components. Tests were read at 48 and 120 hours and all were negative in each subject. Irritant contact dermatitis, not allergy, was the cause of dermatitis in these subjects. It was postulated that sweating and friction contributed to the irritancy.

Adolescent↗