Abstract：
:Familial progressive hyperpigmentation is rarely described in the literature. We report on five patients from three different families presenting with a peculiar progressive pigmentary disorder. The patients show a progressive diffuse, partly blotchy, hyperpigmentation, intermixed with scattered small hypopigmented macules, a few large hypopigmented areas, occasional café-au-lait spots and, most remarkably, a generalized lentiginosis. Histology revealed different degrees of basal layer hyperpigmentation and pigment incontinence, also in the spots appearing hypopigmented. Ultrastructural analysis showed a normal mode of Caucasian-like melanogenesis with varying content of regular melanosome complexes within the keratinocytes. All families are clustered in a small area around the town of Teublitz in south-east Germany with about 20,000 inhabitants, suggesting a genetic founder effect. Pedigree analysis is compatible with an autosomal dominant mode of inheritance with variable penetrance. Only a few similar, but not identical, cases have been reported in the past. This cluster of cases may therefore represent a rare and perhaps novel variant of a familial progressive disorder of hyperpigmentation.
journal_name
Acta Derm Venereoljournal_title
Acta dermato-venereologicaauthors
Zanardo L,Stolz W,Schmitz G,Kaminski W,Vikkula M,Landthaler M,Vogt Tdoi
10.1080/00015550310005780subject
Has Abstractpub_date
2004-01-01 00:00:00pages
57-60issue
1eissn
0001-5555issn
1651-2057journal_volume
84pub_type
杂志文章abstract：:Lymphocytes in chronic lymphocytic leukaemia and in other malignant lymphomas exhibit an extreme sensitivity to colchicine and are cytolysed in vitro by 10(-6) M colchicine. This characteristic can be quantified with the colchicine sensitivity index which is used for diagnostic purposes. We have demonstrated that lymp...
journal_title：Acta dermato-venereologica
pub_type： 杂志文章
doi：
更新日期：1980-01-01 00:00:00
abstract：:A case of eosinophilic cellulitis (Wells' syndrome) in association with ascariasis is described. The clinical and histopathologic features of the patient responded well to an oral anthelminthic drug. According to our search, this association has not previously been reported. ...
journal_title：Acta dermato-venereologica
pub_type： 杂志文章
doi：10.2340/0001555574292294
更新日期：1994-07-01 00:00:00
abstract：:A 10-year follow-up of patients with juvenile plantar dermatosis and gluteo-femoral eczema disclosed a worse prognosis than previously assumed, with a course in some cases protracted into adult age. The presence of atopy was established with a clinical point score system. It was found that 52% of the patients with juv...
journal_title：Acta dermato-venereologica
pub_type： 杂志文章
doi：
更新日期：1988-01-01 00:00:00
abstract：:Confocal laser scanning microscopy (CLSM) was used for quantitative analysis of CD1a+ cells in epidermis at irritant reactions. Sodium lauryl sulphate (2% and 4%) or non-anoic acid (20% and 80%) were applied to the skin of healthy volunteers under occlusion for 24 h. Skin biopsy specimens were taken after additional 2...
journal_title：Acta dermato-venereologica
pub_type： 杂志文章
doi：101080/000155572348351
更新日期：1992-09-01 00:00:00
abstract：:Patients with clinical features of dorsal macular amyloidosis but without subepidermal amyloid deposits were followed for 2-11 years. The clinical appearance was fairly stable during this period of time, with little tendency of healing. Only 2 of the patients developed typical macular amyloidosis during the follow-up....
journal_title：Acta dermato-venereologica
pub_type： 杂志文章
doi：10.2340/0001555576302304
更新日期：1996-07-01 00:00:00
abstract：:The aim of this study is to assess the associations between chronic spontaneous urticaria (CSU), Helicobacter pylori infection and small intestinal bacterial overgrowth. Forty- eight patients with CSU were studied by scoring the urticaria activity and assesing the quality of life. Patients with H. pylori infection (n=...
journal_title：Acta dermato-venereologica
pub_type： 杂志文章
doi：10.2340/00015555-1373
更新日期：2013-03-27 00:00:00
abstract：:Glycosaminoglycans are important components of all extracellular matrices. One of the glycosaminoglycans is hyaluronan, which is ubiquitously distributed throughout the connective tissue. Hyaluronan is especially abundant in the skin, in which it is of both structural and functional importance. This study describes th...
journal_title：Acta dermato-venereologica
pub_type： 杂志文章
doi：10.1080/000155502320624041
更新日期：2002-01-01 00:00:00
abstract：:Indirect immunofluorescence (IF) studies were performed on skin from a variety of vertebrate specimens and IgG fractions from pemphigoid and pemphigus sera. Pemphigoid antigen was present in fish, amphibian, reptilian, avian and mammalian skin, whereas pemphigus antigen was observed in avian and mammalian skin only. ...
journal_title：Acta dermato-venereologica
pub_type： 杂志文章
doi：
更新日期：1978-01-01 00:00:00
abstract：:The aim of this study was to assess quality of life in patients with scalp dermatitis using the Italian version of the Scalpdex, and to validate the instrument in Italian. The survey was conducted in outpatients with psoriasis, seborrhoeic dermatitis, alopecia, or follicular lichen. Data were completed on 194 patients...
journal_title：Acta dermato-venereologica
pub_type： 杂志文章,多中心研究
doi：10.2340/00015555-1731
更新日期：2014-07-01 00:00:00
abstract：:The incidence of malignant melanoma of the skin has increased rapidly among white people during the last decade. Although the pathogenesis of malignant melanoma is not clear, epidemiologic data and experimental work indicates that ultraviolet (UV) radiation plays a critical role in tumorigenesis. Recent data implicate...
journal_title：Acta dermato-venereologica
pub_type： 杂志文章
doi：
更新日期：1989-01-01 00:00:00
abstract：:Atopic dermatitis (AD) and impetigo are skin conditions where bacterial colonisation and infection, especially with Staphylococcus aureus play an important role. We compared skin bacterial population, resistance patterns and choice of antimicrobial agents in patients diagnosed with AD and impetigo during 2005 and 2011...
journal_title：Acta dermato-venereologica
pub_type： 杂志文章
doi：10.2340/00015555-1996
更新日期：2015-05-01 00:00:00
abstract：:The WR, Kline and VDRL reactions were compared in 496 cases of syphilis. The VDRL was significantly more sensitive than both WR and Kline. ...
journal_title：Acta dermato-venereologica
pub_type： 临床试验,杂志文章
doi：
更新日期：1980-01-01 00:00:00
abstract：:The aims of this study were to compare the performance characteristics and cost-effectiveness of pooling endocervical samples for screening and diagnosis of Chlamydia trachomatis, and to investigate the prevalence of C. trachomatis infection in women in Leningrad Oblast, Russia. A total of 1500 endocervical samples we...
journal_title：Acta dermato-venereologica
pub_type： 杂志文章
doi：10.2340/00015555-0196
更新日期：2007-01-01 00:00:00
abstract：:Pain and discomfort are common and often severe skin symptoms in patients with psoriasis. However, no studies have investigated skin pain and discomfort over time, or factors that explain changes in these symptoms. The aims of the present study were to describe the changes in skin pain, skin discomfort and Psoriasis A...
journal_title：Acta dermato-venereologica
pub_type： 杂志文章
doi：10.2340/00015555-1456
更新日期：2013-05-01 00:00:00
abstract：:The distribution of Fc-receptors for IgG (FcR) on human epidermal cells (EC) was characterized in situ using monoclonal antibodies (MoAbs) by indirect immunofluorescence staining of cryosections. The results showed heterogeneity of FcR expression on Langerhans' cells (LC) and keratinocytes (KC). The MoAb IV.3 against ...
journal_title：Acta dermato-venereologica
pub_type： 杂志文章
doi：102340/0001555570385390
更新日期：1990-01-01 00:00:00
abstract：:Having observed altered itch and flare reactions after histamine application in patients with atopic eczema, we tried to determine these reactions in patients with urticaria and psoriasis. We investigated 16 healthy non-atopic subjects, 16 atopics in an eczema-free interval, 16 with acute atopic eczema, 16 with urtica...
journal_title：Acta dermato-venereologica
pub_type： 杂志文章
doi：10.1080/000155598433458
更新日期：1998-03-01 00:00:00
abstract：:Intraepidermal collections of neutrophils and lymphocytes are unique features of the inflammatory reaction of psoriasis. Migration of leukocytes from dermis to the epidermis suggests a role for chemotactic agent(s). In recent years, increased levels of chemokines such as IL-8 , GRO-a and MCP-1 have been reported in th...
journal_title：Acta dermato-venereologica
pub_type： 杂志文章
doi：10.1080/000155599750011615
更新日期：1999-01-01 00:00:00
abstract：:The ultrastructure of oral lesions in 6 patients with erythema multiforme (EM) and of one apparently clinically healthy oral mucosa in one patient with recurrent EM during remission is described. Alterations were observed in epithelium, basal lamina and lamina propria. Both intercellular and intracellular oedema, intr...
journal_title：Acta dermato-venereologica
pub_type： 杂志文章
doi：102340/0001555570199203
更新日期：1990-01-01 00:00:00
abstract：:Air-borne contact dermatitis caused by Parthenium hysterophorus is a serious problem in India. Patients with this condition have to use corticosteroids regularly in order to maintain clinical remissions, but prolonged usage causes serious side-effects. The weed cannot be eradicated. We have used 3 therapeutic regimens...
journal_title：Acta dermato-venereologica
pub_type： 临床试验,杂志文章
doi：10.1080/000155500750012487
更新日期：2000-01-01 00:00:00
abstract：:This is the first case in the literature describing naevi spili, café-au-lait spots and melanocytic naevi aggregated along-side Blaschko's lines. The pattern of melanocytic lesions in our patient is different from the congenital pigmentary syndromes and the segmental distribution of melanocytic naevi, the quadrant dis...
journal_title：Acta dermato-venereologica
pub_type： 杂志文章,评审
doi：10.1080/000155598443114
更新日期：1998-09-01 00:00:00
abstract：:Treatment of symptomatic oral lichen planus remains a challenging problem. This study compared the efficacy of topical tacrolimus ointment with triamcinolone acetonide ointment in patients with oral lichen planus. Twenty patients (group I) were treated with topical tacrolimus 0.1% ointment 4 times daily, and 20 (group...
journal_title：Acta dermato-venereologica
pub_type： 杂志文章,随机对照试验
doi：10.2340/00015555-0070
更新日期：2006-01-01 00:00:00
abstract：:We evaluated the reproducibility of atopy patch test reactions and the quality and quantity of itch in 16 patients with atopic eczema and a history of a positive atopy patch test reaction, comparing three different application sites. The allergen was re-applied simultaneously on both forearms and the back. Intensity a...
journal_title：Acta dermato-venereologica
pub_type： 杂志文章
doi：10.1080/00015550410024418
更新日期：2005-01-01 00:00:00
abstract：:Three women with hyperkeratosis lenticularis perstans (HLP) are described. The lesions developed after the age of 45 and there was no family history of this disease. The possible role of UV-light in the pathogenesis of this disease is discussed. ...
journal_title：Acta dermato-venereologica
pub_type： 杂志文章
doi：
更新日期：1985-01-01 00:00:00
abstract：:The biosynthesis of prostaglandins by human inflamed skin was studied in 8 patients with primary irritant dermatitis induced by benzalconium-chloride. Inflamed skin from these patients evidenced an increased ability to synthesize prostaglandins. In the presence of exogenous arachidonic acid in the incubation medium, t...
journal_title：Acta dermato-venereologica
pub_type： 杂志文章
doi：
更新日期：1976-01-01 00:00:00
abstract：:Determinations of the urinary excretion of 5-S-cysteinyldopa were performed in 571 patients previously treated by surgery for melanoma or melanoma metastasis. 90% of the 161 patients with metastases showed values exceeding 0.15 mg/24 h, and 9% of the 410 patients without metastases had such values. The increase in 5-S...
journal_title：Acta dermato-venereologica
pub_type： 杂志文章
doi：
更新日期：1979-01-01 00:00:00
abstract：:It has been shown recently that immunotherapy for advanced melanoma is effective. However, in order to improve the efficacy of immunotherapy, the identification of more specific melanoma-associated antigens is urgently needed. Kinesin family member 20A (KIF20A) has been reported to be a promising immunotherapeutic tar...
journal_title：Acta dermato-venereologica
pub_type： 杂志文章
doi：10.2340/00015555-1416
更新日期：2012-11-01 00:00:00
abstract：:A man with AIDS is described in whom a profound weight loss was converted into a weight gain by treatment with megoestrol acetate, a synthetic progesterone. His appetite improved and was accompanied by a feeling of improved well-being. Following abrupt discontinuation of the drug, there was a significant but transient...
journal_title：Acta dermato-venereologica
pub_type： 杂志文章
doi：102340/0001555570520521
更新日期：1990-01-01 00:00:00
abstract：:BRAF and NRAS genetic analyses are time-consuming and can delay treatment choices in patients with metastatic melanomas presenting with acute deterioration. We compared the rapid, real-time, fully automated molecular diagnosis platform Idylla™ with next-generation sequencing (NGS) and immunohistochemistry for detectio...
journal_title：Acta dermato-venereologica
pub_type： 杂志文章
doi：10.2340/00015555-2738
更新日期：2018-01-12 00:00:00
abstract：:A case of juvenile xanthogranuloma in a female newborn infant is reported. Red-brown, flat, well-demarcated lesions were present at birth on her scalp, face and trunk. During the following year of observation the eruptions regressed slowly and spontaneously. Light and electron microscopy revealed histiocytes and Touto...
journal_title：Acta dermato-venereologica
pub_type： 杂志文章
doi：
更新日期：1983-01-01 00:00:00
abstract：:We investigated the usefulness of the restriction enzyme (RE) fingerprinting for epidemiological tracing in gonococcal disease. The RE patterns of three paired gonococcal isolates showed corresponding identical fingerprints. Within each of the three pairs of epidemiologically linked isolates the respective restriction...
journal_title：Acta dermato-venereologica
pub_type： 杂志文章
doi：
更新日期：1985-01-01 00:00:00
