New developments in wound care for infants and children.
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Biomedical subjects
Publications and source records attributed to L A Schachner.
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Morphea is a frequently mild, benign, and self-limiting skin disease with a less than 1% reported chance of progressing to systemic scleroderma. Morphea is a sufficient and less terrifying name for these disorders than localized scleroderma.
BACKGROUND: At present, wound treatment of inherited epidermolysis bullosa (EB) is only supportive. OBJECTIVE: To determine the safety and clinical effects of tissue-engineered skin (Apligraf; Organogenesis Inc, Canton, Mass) in the healing of wounds of patients with different types of EB. DESIGN: An open-label uncontrolled study of 15 patients with EB treated with tissue-engineered skin. Each patient received tissue-engineered skin on up to 2 wounds on each of 3 clinic visits: day 1, week 6, and week 12. They were evaluated 7 (+/- 3) days and 6 weeks after each round of treatment. A quality-of-life survey was administered during week 6. SETTING: University of Miami, Miami, Fla. PATIENTS: Volunteers with EB. MAIN OUTCOME MEASURE: Safety and wound healing. RESULTS: A total of 69 different acute wounds received tissue-engineered skin at the day-1 (24 wounds), week-6 (23 wounds), and week-12 (22 wounds) visits. Overall, 63 wounds (79%) were found healed at the day-7 visit. Of the acute wounds, 82% (51/62) were healed 6 weeks after being treated, 75% (27/36) after 12 weeks, and 79% (11/14) after 18 weeks. Nine chronic wounds were also treated. Four were healed at 6 weeks; however, 7 were still open at the last clinic visit (week 18). There were no signs of rejection or clinical infection and no adverse events related to the tissue-engineered skin. The quality of life for most patients improved after treatment. Compared with patients' recollection of wounds treated with standard dressings, healing was faster and less painful. CONCLUSION: In this series of patients, tissue-engineered skin induced very rapid healing, was not clinically rejected, and was devoid of adverse effects. It was felt by the patients and families to be more effective than conventional dressings for EB wounds.
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BACKGROUND: Inherited epidermolysis bullosa (EB) is a mechanobullous disorder. The Dowling-Meara variant, a subtype of EB, is characterized by widespread blister formation that may include the oral cavity and nails. Many patients with the Dowling-Meara phenotype are at increased risk of sepsis and death during infancy. The treatment of EB is generally supportive. The tissue-engineered skin used (Apligraf) is a bilayered human skin equivalent developed from foreskin. It is the only Food and Drug Administration-approved skin equivalent of its kind. It is approved for the treatment of venous ulcers of the lower extremities. It has also been used to treat acute wounds, such as graft donor sites and cancer excision sites. OBSERVATION: To our knowledge, we describe the first case in which a newborn with EB, Dowling-Meara variant, was treated with bilayered tissue-engineered skin. The areas treated with the tissue-engineered skin healed faster than the areas treated with conventional therapy. Most of the areas treated with tissue-engineered skin have remained healed, without developing new blisters. These areas appear to be more resistant to trauma. CONCLUSIONS: Our early success with tissue-engineered skin in this patient may have a significant impact on the future treatment of neonates with EB simplex. Future studies are needed to determine if the beneficial effects of tissue-engineered skin are reproducible in other neonates with EB simplex and in patients of all ages with different subtypes of EB.
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Chronic bullous disease of childhood (CBDC) is an autoimmune blistering disease occurring in prepubertal children. Both CBDC and its adult counter-part, linear IgA bullous dermatosis (LABD), are characterized by linear deposition of IgA along the cutaneous basement membrane zone (BMZ). Circulating IgA antibody in LABD has been found to bind to a 97-kDa BMZ antigen, whereas the antigen in CBDC has not been well characterized. The purpose of this study was to evaluate the immunoreactivity of BMZ IgA antibodies in a series of CBDC patients. We evaluated 12 sera from patients with CBDC with circulating IgA anti-BMZ antibodies on indirect immunofluorescence (IIF), which stained the epidermal side of split skin with titers ranging from 1:20 to 1:640. Immunoblotting was performed against two preparations of BMZ proteins: one enriched with the two bullous pemphigoid antigens (BP230, BP180) and one enriched with the LABD antigen (LABD97). Eight of the twelve sera reacted with a 97-kDa protein that co-migrated with the protein detected in many LABD sera. The intensity of the reaction on immunoblot correlated with serum antibody titers. There was no consistent pattern of reactivity of the IgA anti-BMZ antibodies with either the BP230 or BP180 antigens, although two sera reacted with several higher molecular mass proteins (160-200 kDa). The significance of this reactivity was examined with immunoblotting using BMZ-affinity-purified antibodies, and ELF using nitrocellulose-eluted antibodies. One serum also contained anti-BMZ IgA antibodies that reacted with a 180-kDa protein, corresponding to BP180. We conclude that IgA antibodies in CBDC sera recognize a 97-kDa BMZ antigen present on the epidermal side of BMZ split skin that co-migrates with the antigen previously identified in LABD. These findings suggest that CBDC and LABD are the immunologically related disorders occurring in different age groups.
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BACKGROUND AND DESIGN: Dermatologic practice occurs mainly in the outpatient setting. The reasons for, frequency, and impact of inpatient dermatologic consultation are largely unstudied. In this report, we prospectively studied dermatologic consultation in the major teaching hospital complex of a medical school. Over a period of 8 months, we prospectively recorded the demographics of the patients for whom consultation was requested, the provisional dermatologic diagnosis of the referring service, the final diagnosis of the dermatologic service, and the tests necessary to arrive at a final diagnosis. RESULTS: During a period of slightly over 8 months, dermatologic consultation was requested and delivered to 591 patients who were either hospitalized or being evaluated in the emergency department or other urgent care settings. The services requesting consultation most frequently were medicine (39%), pediatrics (14%), surgery (12%), psychiatry (6%), and neurology (3%). In 51% of consultations, the patients were younger than 45 years of age. Diagnostic tests, including Tzanck smear and potassium hydroxide preparation, confirmed the clinical diagnosis in up to 50% of cases. Dermatologic consultation changed dermatologic diagnosis and treatment in more than 60% of the patients. Generally, the dermatologic diagnoses most frequently missed by the referring service were common conditions with established treatment. CONCLUSIONS: Dermatologic consultation in the hospital setting improves dermatologic diagnosis and has an impact on treatment.
We describe nine patients with eosinophilic pustular folliculitis occurring in infancy. All patients had recurrent crops of pruritic papules primarily affecting the scalp and brow region of the face. Biopsy specimens revealed folliculitis with a predominant eosinophilic infiltrate. Bacterial and fungal cultures of pustules were negative. Most patients had leukocytosis and eosinophilia. Clinical response to topical corticosteroid and/or systemic antibiotic therapy was noted. Eosinophilic pustular folliculitis of infancy must be considered in the differential diagnosis of pustular disorders in infancy.
All forms of cutaneous T-cell lymphoma are rare in children. We describe an 11-year-old girl who had generalized exfoliative erythroderma, intense pruritus, peripheral lymphadenopathy, mycosis cells in the skin and lymph nodes, and Sézary cells in the peripheral blood. Results of a biopsy specimen of involved skin showed changes consistent with mycosis fungoides. A classic case of Sézary syndrome has not previously been reported in childhood or preadolescence.
Human papillomavirus (HPV) type 6 is classically considered a mucosatropic virus. Interestingly, clinical manifestations of HPV 6 infection that involve nonmucosal or nongenital sites have rarely been described. The reasons for this site specific infectivity of HPV 6 are unknown. We describe a patient who had condylomata acuminata-like lesions that involved cutaneous nongenital sites; HPV 6 DNA was detected in skin biopsy specimens with use of the polymerase chain reaction, followed by hybridization with use of type-specific DNA probes.
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A 2 1/2-month-old female infant presented with multiple tense bullae on the hands and feet. Analysis of biopsy specimens confirmed our clinical impression of childhood bullous pemphigoid. Confirmatory data included type IV collagen mapping of the basement membrane zone, a readily available technique that helps distinguish childhood bullous pemphigoid from childhood epidermolysis bullosa acquisita. To our knowledge, our patient is the youngest described with childhood bullous pemphigoid, and we use this opportunity to review the literature and examine the clinical and immunologic features, treatment, and prognosis of this rare childhood immunobullous disorder.
A male infant had three nodules on the left cheek, right forearm, and right neck. Biopsy specimen revealed infantile myofibromatosis (IM). Further evaluation revealed a solitary pulmonary nodule in the right middle lobe located far from mediastinal structures, which had no evidence of tumor enlargement on follow-up tomographic scan. Immunoperoxidase studies were negative for desmin and positive for actin. This is a potentially life-threatening multiorgan system disease; however, if lesions are limited mainly to the skin and soft tissues, the prognosis becomes more favorable. Although many reports suggest spontaneous and complete healing of the cutaneous lesions, our patient had profound atrophy at lesion sites as resolution occurred.