Neonatal and infantile erythroderma: a clinical and follow-up study of 42 cases

Erythroderma in neonates and infants is a frequently encountered problem in the daily practice of pediatric dermatology. The objective of this study was to determine the frequency of various causes of this clinical entity, as well as which clinical and laboratory findings are useful in the differentiation of these causes, and to assess the evolution of this disease in this age group. Forty-two patients with erythroderma under 1 year of age were included in this study. A follow-up period of 3-5 years was completed. The study was performed in the Department of Dermatology, Al-Sadr and Alhakeem teaching hospitals and a private section in Najaf governorate, Iraq during the period 1998-2006. The diagnosis was made at an average of 3 months after the onset of the disease. The underlying causes included seborrheic dermatitis in 21.4%, atopic dermatitis in 14.3%, different types of Ichthyoses in 31.5%, psoriasis in 4.7%, pityriasis rubra pilaris in 2.4%, Staphylococcal scalded skin syndrome in 7.14%, Netherton syndrome in 4.7%, immune deficiency syndromes in 4.8% and undetermined erythroderma in 9.5% of the patients. Of 29 cases, histopathological examination of skin biopsy showed non-specific features in 58.7% and could confirm the diagnosis in 41.3% cases. The prognosis was poor with a mortality rate of 26.2% and severe dermatoses persisted in 60% of the survivors. It is difficult to make the etiological diagnosis of neonatal erythroderma from the first examination. Associated immune deficiency should be suspected if the condition associated with skin indurations, severe alopecia, failure to thrive and/or have infectious complications. The prognosis is poor especially in those with immune deficiency or a chronic persistent course.     

Adolescent‐onset ichthyosiform‐like erythroderma with lichenoid tissue reaction: a new entity?

A patient with severe ichthyosiform erythroderma and lichenoid histological changes is presented. We discuss the clinical and histological differential diagnosis, including lupus erythematosus, lichenoid drug eruption, lichen planus, graft-versus-host disease, lymphoma, keratosis lichenoides chronica, Netherton's syndrome and ichthyosiform erythroderma. None of these is consistent with the features in our case, which may represent either a hitherto unreported form of ichthyosiform erythroderma or possibly a new entity.     

Therapeutic options and prognosis of chronic idiopathic erythroderma in older adults

The present authors have previously reported that chronic idiopathic erythroderma (CIE) that lasts for more than 3 months without specific causes occurs almost exclusively in older men. No standardized therapeutic treatments for the condition have been established to date. To assess the efficacies and safeties of current treatments for CIE, the present authors reviewed cases of CIE. Twenty cases were identified, of which CIE was treated with oral corticosteroids, oral cyclosporine A (CsA), ultraviolet therapy, or a combination of these therapies. CIE was cured in 8/20 (40%) of these cases, and the average time to cure was 19.3 months. Oral CsA was the most effective treatment at relatively low doses (50–100 mg/day), which resulted in cures in four cases. Ultraviolet therapy also effectively cured two subjects, and oral corticosteroids cured one patient. In patients that were treated with oral CsA or corticosteroids, the terminations of the treatments within 2 to 3 months resulted in relapses in multiple patients. The maintaining of the treatments for a certain period may be necessary in cases that involve treatments with these orally administered drugs. Although CIE is a chronic and potentially refractory disease, it can evidently be cured or well controlled via appropriate long‐term therapies.     

Prognosis and prognostic factors in adult patients with atopic dermatitis: a long-term follow-up questionnaire study

BACKGROUND: Atopic dermatitis (AD) is a chronic relapsing skin disease. Several investigations concerning the long-term prognosis of AD among children and teenagers have been performed but there are only few data among adults. OBJECTIVES: To investigate the prognosis and prognostic factors in adult patients with AD by a long-term follow-up (25-38 years). The prognostic factors were defined as those factors of importance for the persistence of AD. PATIENTS AND METHODS: A follow-up questionnaire was sent in November/December 1998 to 922 AD patients examined in our outpatient clinic between 1960 and 1973 among 1366 registered patients with AD. The patients were aged 20 years or older when they visited the clinic and 45 years or older when they answered the follow-up questionnaire. RESULTS: The response rate was 90.4%. The age range at the time of follow-up was 45-86 years (mean 55 years). Of the 833 patients who responded, 59% reported AD at some time during the last 12 months, which we defined as persistent AD. The mean value of clearance rate per person-years was 18%. One of the most important factors associated with persistence of AD was a head and neck dermatitis with or without other AD locations at the time of examination according to the old patient records. CONCLUSIONS: This study showed that the majority of adults with AD still had AD when they became older. This applies particularly if negative prognostic factors existed.     

表现为红皮病的挪威疥一例

患者,女,53岁。双上肢红斑、丘疹伴瘙痒1年,泛发全身3个月,曾误诊为"过敏",予抗组胺药、糖皮质激素软膏治疗,皮损未见明显好转。皮肤活检及显微镜镜检发现疥螨,给予氧化锌硫软膏外用。     

以红皮病为首发症状的艾滋病一例

患者,男,40岁。全身皮肤潮红、脱屑伴瘙痒1个月。患者既往无湿疹、银屑病病史。查体：全身弥漫性潮红,表面覆盖黄白色细碎鳞屑,口周、眼周可见裂隙伴渗出,双足背覆盖袜套样黄色、褐色样鳞屑,可见痂样结构。HIV抗体初筛及确诊试验阳性。诊断为艾滋病、红皮病。     

挪威疥误诊为红皮病一例

患者,女,75岁。周身红斑、丘疹6个月伴糜烂、渗出4天,曾于当地诊所予以“西替利嗪片、糠酸模米松乳膏”治疗,症状无好转,皮疹逐渐泛发,我院门诊以“红皮病并皮肤感染”收入院。入院后第二天追问病史,既往曾有周身剧烈瘙痒,以腹部、指缝等部位为著,夜间加重,其家人有典型疥疮样皮损,为患者完善直接镜检见多个疥虫及虫卵。     

Eczematous erythroderma induced by cyanamide

The term cyanamide is applied to both free cyanamide (carbimide) and its calcium salt (calcium carbimide). It is extensively used as a fertilizer, herbicide and chemical intermediate (1). In the medical field, cyanamide was introduced into Canada, Europe and Japan in 1956 as a treatment for chronic alcoholism. It acts by interfering with the hepatic metabolism of ethanol by inhibiting aldehyde dehydrogenase. When this transformation does not take place, there is an increase in acetaldehyde in the blood, resulting in unpleasant sensations (2). The rare adverse skin reactions due to cyanamide include allergic contact dermatitis (3–7), lichen planus (8, 9) and lichen-oid eruption (9).     

Leiner's disease (erythroderma desquamativum): A review and approach to therapy

Leiner's disease (LD) is a rare and serious syndrome of infantile erythroderma of severe and progressive generalized seborrheic‐like dermatitis, recalcitrant diarrhea, malabsorption and wasting, and recurrent local and systemic infections. The purpose of this study is to provide an updated review on management with a summarized review of available peer‐reviewed articles on LD. The mechanisms underlying this disease process remain unclear. The diagnosis includes demonstration of deficient opsonic activity along with the clinical tetrad of erythroderma, persistent gastrointestinal disturbance, superimposed bacterial or candidal infection, and marked wasting. An important correlation between LD and defective yeast and Staphylococcus aureus opsonization has been established. For the familial form of LD, an association of either complement three deficiency or complement five dysfunction has been made. LD should be distinguished from other types of infantile erythroderma, including Omenn syndrome. Treatment includes fluid and nutrition replacement, antibiotics to control infection, and fresh‐frozen plasma therapy. The prognosis is unclear; it depends on treatment. LD is a life‐threatening condition that requires prompt identification and hospitalization. Affected infants who receive vigorous treatment not only have the prospect of surviving, but also generally lead a normal life after infancy.     

Ichthyosiform erythroderma with rickets: report of five cases

We describe five children with ichthyosis and rickets. The association of ichthyosis and rickets is very rare. Four children had lamellar ichthyosis and one child had nonbullous ichthyosiform erythroderma/psoriasis with atopy. All had biochemical and radiological evidence of rickets. Three had clinically evident rickets, of whom two had very severe skeletal deformities. Such a severe skeletal involvement due to rickets in association with ichthyosis is exceptionally rare. We suggest that children with severe ichthyosis, in particular those with pigmented skin, need to be evaluated for rickets, especially in developing countries where there is a background prevalence of vitamin D deficiency.     

Loricrin keratoderma: a cause of congenital ichthyosiform erythroderma and collodion baby

A group of hereditary palmoplantar keratodermas due to heterozygous mutation in the loricrin gene has recently been identified. Of five reported pedigrees, four presented as mutilating keratoderma with ichthyosis (variant Vohwinkel syndrome), and one as progressive symmetric erythrokeratoderma. We report a new Japanese pedigree of loricrin keratoderma. A 14-year-old male and his 11-year-old female sibling had both been born as collodion babies and were initially diagnosed as having non-bullous congenital ichthyosiform erythroderma, but later developed palmoplantar keratoderma with pseudoainhum. Their father was similarly affected. Direct sequencing of genomic DNA revealed a G residue insertion at codon 230-231 of the loricrin gene. Antibody studies confirmed the presence of mutant loricrin in the retained nuclei. We conclude that loricrin gene mutation may present as congenital ichthyosiform erythroderma, and should be included in the differential diagnosis of collodion baby.     

Bullous and non-bullous ichthyosiform erythroderma associated with generalized pustular psoriasis of von Zumbusch type

Bullous ichthyosiform erythroderma (BIE) and non-bullous ichthyosiform erythroderma (NBIE) are rare congenital ichthyoses. Generalized pustular psoriasis (GPP) of von Zumbusch type is a rare and severe form of psoriasis marked by desquamative and pustular erythroderma associated with fever and altered general conditions. We report two adults with an ichthyosis typical of BIE in the first case and NBIE in the second, without any previous history of psoriasis, who presented with a severe and relapsing GPP of von Zumbusch type. Using current knowledge of the genetic relationship between psoriasis and congenital ichthyoses, we discuss the possibility of a common physiopathological link between congenital ichthyoses and GPP, and examine the possible therapeutic problems resulting from this pathological association, especially in BIE.     

Congenital ichthyosiform erythroderma: particulate staining pattern of TGK

A case of late onset non-bullous congenital ichthyosiform erythroderma (CIE) was studied. This patient was not born as a collodion baby and did not have skin abnormalities until 9-10 years of age. She gradually developed erythroderma and fine scales, callosities of her feet, and a mild ectropion. Since recent work has revealed that in the majority of CIE patients, transglutaminase (TGK) is distributed in the cytoplasm of granular cells and horny cells (11), TGK was studied in our case. It was found that TGK was distributed along the cell periphery of horny cells and also in the cytoplasm of granular cells. In the control skins, TGK was stained along the cell periphery of horny cells and granular cells. The marginal band formation was normal. Involucrine and loricrin, the building materials of the marginal band whose-cross-linking is mediated by TGK, were normally stained in the upper epidermis. Cytoplasmic TGK of granular cells and normal development of the marginal band may serve as a helpful diagnostic marker of CIE, particularly because the often confusing collodion baby of lamellar ichthyosis may lack TGK staining and the marginal band altogether.     

Clearing of psoriatic erythroderma following heliotherapy in the Dead Sea area

We report herein a patient suffering from psoriatic erythroderma and psoriatic arthritis treated successfully in the Dead Sea area. Sun exposure (heliotherapy) and emollients, without any additional topical or systemic treatments, resulted in clearing of the erythroderma within 4 weeks of treatment. Additional regimens of climatotherapy and balneotherapy, given for another 2 weeks, led to marked alleviation of his arthritic complaints. The remission of skin disease persisted for 5 months without further therapy.