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Biomedical subjects

Walter H C Burgdorf

Publications and source records attributed to Walter H C Burgdorf.

16 recordsLinked to original sources

Cancer-associated genodermatoses: a personal history.

Cancer-associated genodermatoses are a group of genetic disorders inherited in an autosomal-dominant fashion in which unique cutaneous findings are a reliable marker for the risk of developing internal malignancies. The historical, clinical and dermatopathological aspects of basal cell nevus syndrome, Muir-Torre syndrome, Cowden syndrome, Carney complex and Birt-Hogg-Dubé syndrome are reviewed in a personal and informal fashion. The latest advances in the molecular genetics of the disorders are also summarized.

Basal Cell Nevus Syndrome↗

[Disseminated juvenile xanthogranuloma].

Juvenile xanthogranuloma (JXG) is the most common type of non-Langerhans cell histiocytosis occurring predominantly in infants and children. Typical lesions are asymptomatic red-yellow papules and nodules on the scalp or in the axillae or groins. Multiple lesions are more common in children than in adults. A 2-year old girl presented with approximately 20 red-brown lesions, leading to the diagnosis of disseminated JXG. Internal involvement was excluded. Spontaneous regression occurred over 6 months of follow-up. A wait-and-see strategy is recommended for cutaneous JXG.

Child, Preschool↗

Analysis of original contributions in three dermatology journals.

In a cross-sectional analysis, the three top ranking dermatology journals ( Archives of Dermatology , British Journal of Dermatology , and the Journal of the American Academy of Dermatology ) were evaluated for their scientific content, the characteristics of their authors, and funding aspects in 2002. A flood of scientific papers are published each year covering a broad variety of dermatologic topics. Aside from the actual content, a scientific article provides information about the number of authors, their nationality and affiliations, and, with some limitation, about previous presentations and funding. The present study analyzes this situation in dermatology by evaluating the content in the three top dermatologic journals.

Advertising↗

Indeterminate cell histiocytosis: fact or fiction?

Indeterminate cell histiocytosis is a rare disorder, in which the predominant cells have the characteristics of both Langerhans cells and macrophages. We, in this study, describe 18 patients and compare them with those previously published. Most patients were adults with either solitary or multiple red-brown papules or nodules. While most lesions were confined to the skin, both conjunctival and bony involvement was seen. Histologically, the lesions showed patterns resembling those described for xanthogranulomas, with predominantly oncocytic (nine patients), spindled (five patients), scalloped (two patients) or vacuolated (two patients) macrophages. The accompanying infiltrate was mainly lymphocytic, although eosinophils and occasionally plasma cells were seen. All lesions were positive for macrophage markers, such as KP1 (CD68) and Ki-M1p, as well as for S-100 protein and showed variable reactivity for CD1a. No Birbeck granules were seen ultrastructurally in one patient. Some patients shared features with sinus histiocytosis with massive lymphadenopathy. It is unclear whether this disorder is a separate entity or represents various macrophage disorders identified at various time points in the inflammatory response. Ratzinger G, Burgdorf WHC, Metze D, Zelger BG, Zelger B. Indeterminate cell histiocytosis: fact or fiction? A clinicopathological series of 18 patients.

Adult↗

Lessons from atopy patch testing in atopic dermatitis.

The exposure of atopic eczema (AE) patients to their relevant protein allergens (eg, from house dust mite, cat dander, grass pollen, or food allergens) can trigger an exacerbation or maintain the disease. Diagnostic procedures are needed to specify allergen avoidance recommendations for the individual patient. Skin prick tests and specific serum IgE tests might be helpful in pointing out potential trigger factors, but relevance needs to be confirmed (eg, with food provocation tests). The atopy patch test (APT) involves the epicutaneous application of intact protein allergens in a diagnostic patch test setting with an evaluation of the induced eczematous skin lesions after 24 to 72 hours. The APT targets the cellular component of AE and helps round out the AE test spectrum. As a number of apparently minor test modifications greatly influence the sensitivity, specificity, and reproducibility of the APT, the European Task Force on Atopic Dermatitis (ETFAD) has developed a standardized APT technique. It consists of purified allergen preparations in petrolatum, applied in 12-mm diameter Finn chambers mounted on Scanpor tape to non-irritated, non-abraded, or tape-stripped skin of the upper back. The APT is read at 48 and 72 hours according to the test criteria and reading key of the ETFAD for appearance of erythema, and number and distribution pattern of the papules. In contrast with skin prick tests, the APT might even detect a relevant sensitization in the absence of specific IgE. Many studies have been undertaken to objectify the sensitivity and specificity of the APT to show its diagnostic use in clinical practice.

Advisory Committees↗

Dermatofibroma-a critical evaluation.

Dermatofibromas are common cutaneous lesions. In most cases, they can be readily identified clinically and show a typical histology. In a small percentage of cases they show unusual clinical and more often histologic features that may cause differential diagnostic problems. In addition there are reactive fibrous lesions with neural or smooth muscle features that we speculate may represent dermatofibroma variants.

Biomarkers, Tumor↗

Viral infections in atopic dermatitis: pathogenic aspects and clinical management.

A number of different widespread and disseminated viral infections can occur in patients with atopic dermatitis. Eczema molluscatum is troublesome but not dangerous. Although eczema vaccinatum is rare, it is life-threatening and of increased concern as smallpox vaccinations are reintroduced as a response to possible bioterrorism. There is little information on the course of smallpox itself in atopic dermatitis. Eczema herpeticum is the most common member of this group; recent advances in understanding its pathogenesis might contribute to a more successful management of this serious complication.

Dermatitis, Atopic↗

Principles of dermatoscopy of pigmented skin lesions.

There has been a dramatic increase in the incidence of malignant melanoma in most parts of the world. Because the tumor thickness is the most important prognostic factor for the prognosis of the malignant melanoma, the early detection of thin melanomas is essential. Dermatoscopy allows the physician to discriminate between melanocytic and nonmelanocytic lesions with high diagnostic accuracy and to detect initial malignant melanomas. We review the principles of dermatoscopy and the differential diagnosis of pigmented skin lesions. Before using the ABCD rule of dermatoscopy to classify melanocytic lesions into benign, suspicious, or malignant, the distinction between melanocytic and nonmelanocytic lesions is necessary. An essential prerequisite for the usefulness of this technique is adequate training.

Dermatology↗

Identification of human papillomavirus DNA in cutaneous lesions of Cowden syndrome.

BACKGROUND: Cowden syndrome (CS) or multiple hamartoma syndrome is a cancer-associated genodermatosis inherited in an autosomal dominant pattern. One of the diagnostic criteria is facial papules which are felt to be trichilemmomas, benign hair follicle tumors, which some consider to be induced by human papillomavirus (HPV). OBJECTIVE: To search for HPV in skin tumors, especially trichilemmomas, from patients with CS. METHODS: Skin lesions from patients with CS were classified histologically. Each tumor was then analyzed for HPV DNA by polymerase chain reaction with different primer sets; positive amplicons were typed by direct sequencing. RESULTS: Twenty-nine biopsies from 7 patients with CS were investigated. Only 2 of 29 tumors clinically suspected of being trichilemmomas were confirmed histologically. In addition, 3 sclerotic fibromas, also typical of CS, were found, as well as 1 sebaceous hyperplasia. The other 23 lesions showed histological features of HPV- induced tumors in various stages of development. HPV DNA was found in 19 of 29 cutaneous lesions. Tumors without any histological signs of HPV induction were negative for HPV DNA. Two tumors which were histologically classified as common warts contained HPV types 27 and 28. All the 17 other HPV types belong to the group of epidermodysplasia-verruciformis-associated types. CONCLUSIONS: The majority of cutaneous lesions in CS contain HPV DNA. They may have a variety of histological patterns. Trichilemmomas are not clinically distinctive and can be difficult to identify in CS patients.

Adolescent↗

Long-lasting "christmas tree rash" in an adolescent: isotopic response of indeterminate cell histiocytosis in pityriasis rosea?

A 13-year-old girl developed a non-pruritic pityriasis rosea-like rash, which did not respond to topical corticosteroids or UV therapy but persisted for 2 years. The lymphohistiocytic infiltrate in the upper dermis showed mononuclear cells immunoreactive with S100, CD68, factor XIIIa and CD1a. Electron microscopic evaluation of these cells demonstrated lamellated dense bodies but no Birbeck granules, lipid vacuoles or cholesterol crystals. Two diagnoses were made: a primarily clinical diagnosis of generalized eruptive histiocytosis and a more cell-biology-based diagnosis of an indeterminate cell histiocytosis. Three years later, the lesions are showing spontaneous resolution, with loss of erythema and flattening. Our patient's indeterminate cells fulfil Rowden's classical definition (dendritically shaped epidermal non-keratinocytes without identifying cytoplasmic features), as well as Zelger's newer definition (cells with features of both macrophages and dendritic cells). A Christmas tree pattern has not been previously described in indeterminate cell histiocytosis. Development of indeterminate cell histiocytosis in the lesions of a healing pityriasis rosea might explain the unusual distribution pattern. The development of a skin disorder at the site of an unrelated, already healed skin disease is known as an isotopic response. Key

Adolescent↗

Immunomodulatory macrolactams for topical treatment of inflammatory skin diseases.

Tacrolimus (FK-506; Fujisawa Pharmaceutical Co Ltd) and pimecrolimus (SDZ-ASM-981; Novartis AG) are topical immunomodulators, which provide an alternative to glucocorticosteroids for the topical treatment of atopic dermatitis and other inflammatory dermatoses. Both substances form a complex with cytosolic immunophilins. The complex blocks calcineurin and inhibits the transcription of NF-AT-dependent pro-inflammatory cytokine genes. Multicenter, randomized, double-blind clinical trials have shown the efficacy of both substances in atopic dermatitis. We review the physicochemical characteristics, mode of action, pharmacokinetic data, side effects, results of the clincal trials and further indications for tacrolimus and pimecrolimus.

Adjuvants, Immunologic↗