ISSN:1018-8665    

DOI:10.1159/000247336

出版商:S. Karger AG    

年代:1993

数据来源: Karger

ISSN:1018-8665    

DOI:10.1159/000247337

出版商:S. Karger AG    

年代:1993

数据来源: Karger

摘要:

We analyzed the cutaneous reactions to systemic analgesic-antipyretics and nonsteroidal anti-inflammatory drugs reported to the spontaneous reporting system of the Gruppo Italiano Studi Epidemiologici in Dermatologia (GISED). The system has been active since 1988, with periodic intensive surveillance exercises, and 202 dermatologists have collaborated. Up to December 1991, 2,137 reactions had been collected, of which 713 were reactions to systemic analgesic-antipyretics and nonsteroidal anti-inflammatory drugs. A general profile of the reactions was identifiable. It included, in order of frequency, urticaria/angioedema, fixed eruptions, exanthemas, erythema multiforme and Stevens Johnson syndrome. Fixed eruptions and Stevens Johnson syndrome were reported with exceedingly high frequency in association with feprazone. Our system also revealed previously unreported reactions, including fixed eruption to nimesulide, fixed eruption to piroxicam and fixed eruption to flurbiprofen.

ISSN:1018-8665    

DOI:10.1159/000247338

出版商:S. Karger AG    

年代:1993

数据来源: Karger

摘要:

We report a patient with pustular psoriasis (Zumbusch type) with diffuse muscle pain. Evaluation of skeletal muscle metabolism during rest, graded levels of exercise and recovery was performed using 31phosphor magnetic resonance spectroscopy (31P MRS). Our results underline the systemic character of severe pustular psoriasis. The clinical improvement after drug therapy (prednisolone and etretinate) correlated well with the measurable changes in vivo of several metabolites and indices important in muscle contraction, such as phosphocreatine (PCr), inorganic phosphate (Pi), intramuscular pH and the phosphorylation potential (PCr/Pi). Abnormal accumulation of sugar phosphates (phosphomonoester) as well as their slow resolution during recovery reflect profound alteration of metabolism in the sense of block in glycolysis. Our results show that 31P MRS is well suited to identify and measure subtle changes in the muscle metabolism occurring in pustular psoriasis.

ISSN:1018-8665    

DOI:10.1159/000247339

出版商:S. Karger AG    

年代:1993

数据来源: Karger

摘要:

Four women with pustulosis palmoplantaris (PPP) and associated sternocostoclavicular hyperostosis are described. The connection between the two diseases is not coincidental: it is encountered in 9.4% of PPP patients, and was reported in the French literature as part of the SAPHO (synovitis acne pustulosis hyperostosis osteomyelitis) syndrome. This syndrome is linked to the spondyloarthropathies, having an increased prevalence of HLA B27 and occurrence of sacroiliitis. None of our patients had HLA B27, but HLA A26 was found in the 3 patients examined. No conclusion can be drawn from this finding, however, since this antigen is found in 21.7% of Ashkenazi Jews. Recognition of this syndrome by dermatologists will improve diagnosis and treatment.

ISSN:1018-8665    

DOI:10.1159/000247340

出版商:S. Karger AG    

年代:1993

数据来源: Karger

摘要:

The ages at onset of 245 female and 211 male psoriasis (Ps) patients were recorded. The distribution of age of onset in both sexes is bimodal, with separation at the age of 40 years into an early-onset group and a late-onset group. These distributions were normal (Gaussian) with equal variances. These data are compatible with the hypothesis that there are two genotypes for Ps. Further evidence for this hypothesis is provided by the relationship between age of onset and number of affected relatives. The latter, corrected for age at time of study, demonstrates a mixture of two negative binomial distributions, also with likely separation at the age of 40 years. The age distribution of Ps patients reflects the bimodality of age of onset, but with larger means and variances.

ISSN:1018-8665    

DOI:10.1159/000247341

出版商:S. Karger AG    

年代:1993

数据来源: Karger

摘要:

The trunk and the limbs of an 82-year-old man were covered with large sheets of flat-topped erythematous papules arranged in a reticulate pattern which suddenly coalesced assuming erythroderma-like aspects. Body and positional folds were characteristically spared (deck chair sign). Mild eosinophilia was present. The histological and immunohistochemical examinations revealed an unspecific eczematous pattern. After systemic steroids and PUVA treatment the patient recovered from the dermatosis. Papuloerythroderma is a distinctive clinical entity, but it is not clear whether the dermatosis is the skin marker of systemic pathological conditions or a precursor of cutaneous lymphomas.

ISSN:1018-8665    

DOI:10.1159/000247342

出版商:S. Karger AG    

年代:1993

数据来源: Karger

摘要:

We report a new case of Ofuji papuloerythroderma. There was no obvious sign of lymphoma or underlying malignancy. This is the 7th European case of papuloerythroderma. This recently described clinical entity is characterized by a pruritic eruption of widespread red-brown flat papules sparing the skin folds, with eosinophilia. The recognition, report and follow-up of further cases are necessary to clarify its relationship with paraneoplastic syndromes, hypereosinophilic syndrome, or prelymphoma skin eruptions.

ISSN:1018-8665    

DOI:10.1159/000247343

出版商:S. Karger AG    

年代:1993

数据来源: Karger

摘要:

A case of papuloerythroderma in a 74-year-old woman is presented. She had blood eosinophilia (up to 4,130 mm3), elevated serum IgE (2,369 IU/ml) and responded to oral steroids.

ISSN:1018-8665    

DOI:10.1159/000247344

出版商:S. Karger AG    

年代:1993

数据来源: Karger

摘要:

We describe a new case of isolated trichothiodystrophy. This entity is characterized by sparse and brittle hair, low sulfur hair content, tiger tail pattern of the hair under polarizing microscopy, clean transverse fractures through the hair shaft and absent or defective hair cuticles. To our knowledge there are only two reported cases of isolated trichothiodystrophy without associated additional ectodermal or neuroectodermal dysplasias. Polarized microscopy of cut hair showed transverse fracture points (trichoschisis) and alternating dark and bright bands. Low sulfur content of the hairs was confirmed by amino acid analysis. Many acronyms and eponyms have been created to describe sulfur-deficient brittle hair associated with neuroectodermal abnormalities, leading to confusion. We suggest to use a simple classification scheme, according to increasing severity of associated features as proposed by Van Neste.

ISSN:1018-8665    

DOI:10.1159/000247345

出版商:S. Karger AG    

年代:1993

数据来源: Karger