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At least 127 records · Page 7Linked to original sources
PubMed2001

[Clinical aspects and etiology of erythroderma: an analysis of 64 cases].

The difficulty of exfoliative dermatitis lies in the finding of underlying causes. In the present study the profile of 64 patients with erythroderma diagnosed between 1.1.1990 and 31.12.1999 were analysed. Clinical, histological and laboratory findings and their relationship to the aetiology were investigated. A masculine:feminine ratio of 2.6:1 was found. Among these patients the most common causative factors were pre-existing dermatoses (58%) and drugs (16%). In about eleven percent of the patients exfoliative dermatitis was associated with malignancy. Six out of seven malignancies were found in males, five of them suffering from pre-existing dermatosis. In 16 percent of the patients no aetiology of erythroderma could be found. No laboratory or clinical findings were indicative for an underlying malignant condition. Since we demonstrated underlying neoplastic disorders in seven (11%) of the cases, five of them also suffering from pre-existing dermatosis, we consider it absolutely necessary to screen for such diseases in the presence of the diagnosis exfoliative dermatitis.

Adult↗
PubMed1992

Therapeutic options for erythroderma.

In this work we will discuss some of the dilemmas and therapeutic options in the treatment of patients with erythroderma. We will mention treatment modalities for psoriatic erythroderma and review briefly one illustrative case in which a new experimental approach was utilized.

Adult↗
PubMed1987

Recurrent postcoital lower-extremity streptococcal erythroderma in women. Streptococcal-sex syndrome.

Two women with underlying distortion of their lower-body lymphatic systems from neoplasia and surgery or radiation therapy had recurrent episodes of lower-extremity erythroderma temporally associated with sexual intercourse. Both women had vaginal colonization with Streptococcus agalactiae (group B); one was shown to have recurrent bacteremia with this organism at the time of the episodes. Erythroderma developed in these women possibly because of seeding of S agalactiae in the vaginal soft tissues during coitus.

Adult↗
PubMed1985

[Paraneoplastic erythroderma: apropos of a case].

A 63-year-old male patient had severe exfoliative dermatitis (erythroderma) which led to the discovery of squamous cell carcinoma of the larynx. The possibility of paraneoplastic erythroderma is discussed. Although such an aetiology is rare, 5 cases with a similar paraneoplastic syndrome have been previously reported, which cannot be considered as a merely fortuitous association of exfoliative dermatitis and visceral cancer.

Carcinoma, Squamous Cell↗
PubMed1986

A mother and two children with nonbullous congenital ichthyosiform erythroderma.

A mother and her two daughters were all afflicted by congenital nonbullous ichthyosis. The clinical and ultrastructural picture are in accordance with recessive congenital ichthyosiform erythroderma. The mode of inheritance for the children is assumed to be by pseudo-dominance. Nonbullous congenital ichthyosiform erythroderma and lamellar ichthyosis are probably heterogeneous.

Adult↗
PubMed1985

Clinical, histologic, and cell kinetic discriminants between lamellar ichthyosis and nonbullous congenital ichthyosiform erythroderma.

Patients described as having lamellar ichthyosis (LI) may not all have the same disorder. We studied nine patients, four of whom had erythroderma and generalized fine scaling, while the remainder had more marked scaling and hyperkeratosis without erythema. The former group is referred to as having nonbullous congenital ichthyosiform erythroderma (CIE), and the other is said to have LI. The clinical differences were paralleled by cell kinetic and histologic differences. The mean tritiated thymidine autoradiographic labeling indices were 9.36 +/- 4.05 and 19.70 +/- 5.84 for the LI and CIE groups, respectively. The histologic differences were delineated using 10-cm analogue scales and showed statistically significant differences for hyperkeratosis and parakeratosis. These data support the hypothesis that LI and CIE are separate disorders.

Adolescent↗
PubMed1983

Pre-Sézary erythroderma evolving to Sézary syndrome. A report of seven cases.

Seven patients with pre-Sézary erythroderma were followed up for three to 16 years as Sézary erythroderma syndrome developed. With the transition, the clinical and skin manifestations of the disease became chronic, more florid, and more resistant to therapy. Four patients had multiple contact allergies or drug reactions, and one patient had severe atopic dermatitis. Lymphoma developed in one patient, and multiple myeloma developed in a second patient. An increased absolute number of Sézary cells to more than 1,000/cu mm occurred in all patients with Sézary syndrome. Histologic study showed chronic dermatitis in most patients with the pre-Sézary condition and subepidermal lymphocytic band inflammation in most patients with the Sézary syndrome.

Aged↗
PubMed1983

[Treatment of congenital erythroderma ichthyosiforme in mother and son with the oral aromatic retinoid Ro 10-9359. The need for maintenance therapy].

Aromatic retinoid Ro 10-9359 (Tigason) administered in the doses 25-50 mg daily to two patients (mother and son) suffering from congenital non-bullous ichthyosiform erythroderma revealed complete disappearance of the clinical signs of the disease within two weeks. A relapse was noted in one patient 1.5 months after oral treatment was discontinued. The lesions disappeared again after 30 mg of Ro 10-9359 daily within 10 days. No side-effects were recorded. The tolerance of the drug was satisfactory. The obtained data showed a high efficacy of the oral aromatic retinoid in the therapy of congenital ichthyosiform erythroderma and point to the necessity of maintainance treatment with small daily doses of retinoid for longer time periods.

Adult↗
PubMed1994

[A 46-year-old woman with myasthenia gravis associated with macromastia, erythroderma and hypogeusia].

A 46-year-old woman developed blepharoptosis, diplopia and hypogeusia in 1984, and was diagnosed as having myasthenia gravis and malignant thymoma. Her symptoms improved by thymectomy and oral prednisolone administration. In 1988 she complained of breast pain and swelling during menstruation. In 1990 her breast was enlarged to the size of an adult head, and she developed erythroderma and hypogeusia. She was admitted to Nagasaki University Hospital in August, 1990. On admission, erythema was seen in the whole body especially in the face, anterior chest, abdomen and the thigh. Her breast was the size of an adult head. Muscle strength was weak, and ocular movement was limited. She had blepharoptosis. Papillae of the tongue were atrophic. Endoclinological investigations revealed an elevation of PRL to 14.6 ng/ml (normal < 10), antibodies to the acetylcholine receptor to 118.0 nM (normal < 0.5). In TRH stimulation test the response of PRL was amplified. Four intrathoracic masses suspected of recurrence of malignant thymoma were seen in CT. Her macromastia depended upon the amount of PRL, and was reduced by bromocriptine. Erythroderma and hypogeusia were relieved by plasmaphersis. Therefore autoimmune mechanism may be related to these symptoms.

Breast↗
PubMed1993

[Erythroderma disclosing transformation of refractory anemia with excess of blasts into lymphoblastic leukemia].

We report a case of erythroderma revealing lymphoblastic leukaemia occurring immediately after myelodysplasia. The patient was an 87-year old man admitted for poor general condition, lymph node enlargement and pruriginous oedematous erythroderma. Laboratory examinations showed tricytopenia, bone marrow invasion by lymphoblasts in a myelodysplastic environment of the refractory anaemia type with excess of blasts, and a cutaneous lymphocytic infiltrate with non-blastic lymph node reaction. Owing to its morphological and immunohistological features, the lymphocytic infiltrate was regarded as non-specific. We could not help comparing this case to the other non-specific cutaneous lesions of refractory anaemia which usually reflect transformation into leukaemia.

Aged↗
PubMed1996

Carbamazepine induced erythroderma with systemic lymphadenopathy.

We describe a 13-year-old girl with carbamazepine (CBZ)-induced erythroderma associated with marked generalized lymphadenopathy, a very rare form of CBZ hypersensitivity. The results of lymphocyte stimulation test (LST) and patch testing provided valuable evidence for distinguishing CBZ hypersensitivity from immunoblastic lymphadenopathy (IBL) and infectious mononucleosis (IM). An increased LST value was the only clue leading us to suspect a hypersensitivity reaction against CBZ at a peak stage of the skin eruption. In contrast, CBZ patch testing, which was negative at that time, became positive when the erythroderma regressed after discontinuation of CBZ. These findings suggest that LST can be a useful tool in diagnosing similar cases of hypersensitivity reactions to CBZ and other drugs, especially when patch test results are negative.

Adolescent↗
PubMedDate not supplied

Omenn's syndrome: differential diagnosis in infants with erythroderma and immunodeficiency.

The clinical differential diagnosis of erythroderma plus immunodeficiency and failure to thrive in neonates includes graft-versus-host-disease (GVHD), Omenn's syndrome (OS), and Netherton's syndrome (NS). In addition to immunological investigations, skin biopsy is an important part of the diagnostic work-up. We reviewed biopsies from 25 patients that were retrieved from the archives of the Department of Histopathology at Great Ormond Street, of which 9 were OS, 11 were GVHD, and 5 were NS. Five patients had two biopsy specimens. Both OS and GVHD show dyskeratosis and basal vacuolation. OS always shows acanthosis and almost always parakeratosis. GVHD shows a flat epidermis and rarely parakeratosis. OS and GVHD can be distinguished after immunohistochemistry for LCA and CD68 by the relative proportions of lymphocytes and macrophages in the dermal infiltrate (predominantly lymphocytes in OS, relatively more macrophages in GVHD). Skin biopsy diagnosis of OS is difficult before 6 weeks of age because the features are poorly developed. NS can be distinguished by psoriasiform acanthosis, thickening of the basement membrane, prominent dermal blood vessels, absence of dyskeratosis, and basal layer vacuolation, and a dermal infiltrate in which lymphocytes and macrophages are equally represented. Thus, the main difference between GVHD and OS is in the proportion of lymphocytes and macrophages in the infiltrate on immunohistochemical staining for LCA and CD68, while OS and NS may be distinguished on H&E morphology alone.

Adolescent↗
PubMed2005

[Netherton syndrome: a type of infantile erythroderma with failure to thrive, immune deficiency, rickets. Report of 3 cases].

We report the cases of 2 boys and 1 girl suffering from Netherton syndrome. Both boys presented with a non-bullous congenital erythroderma and were diagnosed early as Netherton syndrome with hair biopsies. Both had severe failure to thrive, signs of atopy, several episodes of bacterial infection, and rickets (with a high blood level of vitamin D in the first boy, and vitamin D deficiency in the second). In the third case, the pilar abnormality appeared at the age of 3 years. The girl had ichtyosis linearis circumflexa, failure to thrive and severe constipation. Netherton syndrome is a rare disorder characterized by severe ichtyosis, signs of atopy, immune deficiency and failure to thrive. The disease is severe and comprises many complications in early infancy. It is due to a genetic disorder of recessive autosomal transmission, and the gene, SPINK5, is located in the chromosome 5. Prenatal diagnosis is possible. Two of our patients had rickets, which has never been described in such patients population.

Carrier Proteins↗
PubMed1999

Cholesteatoma in a child with congenital ichthyosiform erythroderma.

A 7-year-old girl had non-bullous congenital ichthyosiform erythroderma (NBCIE) in treatment with retinoids. She also suffered continuous desquamation of the external ears, recurrent external otitis, chronic adenoiditis, tubaric dysfunction and intermittent episodes of otitis media and developed a cholesteatoma in her left ear. Chronic scaling disorders, such as NBCIE and other ichthyosis, can be entities at risk for the development of cholesteatoma in childhood.

Child↗
PubMed2003

Identification of two novel nonsense mutations in the transglutaminase 1 gene in a Hungarian patient with congenital ichthyosiform erythroderma.

Congenital ichthyosiform erythroderma (CIE) belongs together with lamellar ichthyosis (LI) to the group of autosomal recessive congenital ichthyoses (ARCI). Mutations in the transglutaminase (TGase) 1 gene (TGM1) have been identified in several families with LI and in some families with CIE. We report a case of CIE with two new nonsense mutations: a C7780G transversion in exon 11 resulting in a premature stop codon at aminoacid residue Y503X and a C8533G transversion in exon 13 leading to a nonsense mutation at S669X. These mutations were also identified in a heterozygous pattern in the unaffected parents. These two termination-codons result in the translation of a truncated protein at the C-terminal end domain of the TGM 1 molecule. B.C1 monoclonal antibody failed to detect TGase 1 in the patient's skin sample, and TGase activity measured by monodansyl cadaverine-incorporation showed the reduced TGase activity at the distribution of TGase 1 in the epidermis.

Child↗
PubMed2001

Novel mutations of TGM1 in a child with congenital ichthyosiform erythroderma.

We report novel mutations in the transglutaminase (TGase) 1 gene (TGM1) in a Japanese boy with non-bullous congenital ichthyosiform erythroderma (NBCIE). The patient showed fine, grey or light-brown scales on an erythematous skin. An in situ TGase activity assay detected markedly reduced TGase activity in the patient's epidermis. Electron microscopy revealed incomplete thickening of the cornified cell envelope during keratinization in the epidermis. Sequencing of the entire exons and exon-intron borders of TGM1 revealed that the proband was a compound heterozygote for two novel mutations, 9008delA and R388H. In lamellar ichthyosis, most previously reported TGM1 mutations have been located in the central core domain or upstream of the TGase 1 molecule. In the present NBCIE patient, the frameshift mutation 9008delA resulting in a premature termination codon at the tail of the TGase 1 peptide was in the beta-barrel 2 domain (C-terminal end domain) of the peptide, far from the active sites of the TGase 1 molecule, and the mis-sense mutation R388H was in the core domain.

DNA Mutational Analysis↗