LEKTI demonstrable by immunohistochemistry of the skin: a potential diagnostic skin test for Netherton syndrome

BACKGROUND: Netherton syndrome (NS) is a rare autosomal recessive condition characterized by ichthyosiform erythroderma, trichorrhexis invaginata and atopic manifestations. Confirming the diagnosis may be difficult in the early stages. Mutations in the SPINK5 gene which encodes for the serine protease inhibitor LEKTI are associated with NS. These mutations create premature termination codons which result in absent or abnormal expression of LEKTI in patients with NS. OBJECTIVES: To investigate the expression of LEKTI in the skin of patients with NS in comparison with normal controls and patients with other skin conditions, namely atopic dermatitis, psoriasis and nonbullous ichthyosiform erythroderma. METHODS: Immunohistochemistry was performed on skin sections from four patients with NS, four normal controls, four with atopic dermatitis, two with psoriasis and two with nonbullous ichthyosiform erythroderma, using a primary rabbit polyclonal antibody against LEKTI. RESULTS: LEKTI was localized to the stratum granulosum in normal skin. All four skin sections from patients with NS showed absent or very reduced staining for LEKTI. Staining in the other disorders showed positive LEKTI expression in varying patterns. CONCLUSIONS: NS can be difficult to diagnose especially in the early stage, which can lead to inappropriate treatments particularly if it is misdiagnosed as atopic dermatitis. Immunohistochemistry of skin with an antibody against LEKTI is a potentially useful diagnostic test for NS.     

Altered lamellar body secretion and stratum corneum membrane structure in Netherton syndrome: differentiation from other infantile erythrodermas and pathogenic implications.

BACKGROUND: The infant with Netherton syndrome (NS) typically displays a generalized erythroderma covered by fine, translucent scales, which can be difficult to distinguish clinically from erythrodermic psoriasis, nonbullous congenital ichthyosiform erythroderma, or other infantile erythrodermas. Some infants with NS develop progressive hypernatremic dehydration, failure to thrive, and enteropathy. Such complications can be fatal. Diagnosis is typically delayed until the appearance of a pathognomonic hair shaft anomaly, trichorrhexis invaginata (bamboo hair). To facilitate the early diagnosis of NS, we obtained biopsy specimens from 7 patients with erythrodermic NS and compared their morphologic findings to those of 3 patients with erythrodermic psoriasis and 2 with congenital ichthyosiform erythroderma. Biopsy specimens were processed for light and electron microscopy using postfixation with osmium tetroxide and ruthenium tetroxide. OBSERVATION: In NS, and often in congenital ichthyosiform erythroderma and erythrodermic psoriasis, the stratum corneum layer was largely replaced by parakeratotic cells. A distinctive feature--premature secretion of lamellar body contents--occurred only in NS. Furthermore, lamellar body-derived extracellular lamellae and stratum corneum lipid membranes were separated extensively by foci of electron-dense material. Finally, transformation of lamellar body-derived lamellae into mature lamellar membrane structures was disturbed in NS. CONCLUSIONS: Premature lamellar body secretion and foci of electron-dense material in the intercellular spaces of stratum corneum, features not observed in other erythrodermic disorders, appear to be frequent and relatively specific markers for NS. These ultrastructural features could permit the early diagnosis of NS before the appearance of the hair shaft abnormality. These abnormalities could explain the impaired permeability barrier in NS, and account for hypernatremia and dehydration in infants with NS.     

Betapapillomavirus in multiple non‐melanoma skin cancers of Netherton syndrome: Case report and published work review

Netherton syndrome (NS) is a rare genetic disease presenting with ichthyosiform erythroderma, hair alterations and atopy. NS is due to SPINK5 gene mutations, which cause absent or decreased expression of the encoded protein lymphoepithelial Kazal‐type‐related inhibitor (LEKTI) in all stratified epithelia. We report a 43‐year‐old man affected with NS, who developed several squamous and basal cell carcinomas on the face, ears and scalp and papillomatous lesions of hips, groin and genitoanal area. Molecular analysis of the SPINK5 gene revealed homozygosity for the recurrent mutation c.238dupG. Human papillomavirus (HPV) DNA detection and genotyping on patient skin carcinomas and hyperplastic lesions found betapapillomavirus DNA in 10 of 12 (83%) carcinomas and in a hip papilloma, with multiple betapapillomavirus types being identified. Immunohistochemistry showed upregulated expression of p16^INK4a protein in nine of 12 (75%) patient carcinomas, in line with findings reported in HPV‐related cancers. LEKTI and filaggrin immunostaining was strongly decreased in patient skin. A published work search for NS cases with skin cancers and HPV infection identified 15 NS patients, five of them showing mucosal or cutaneous HPV infection. Overall, our results confirm the increased susceptibility to skin carcinomas of some NS patients and provide further evidence of an association between HPV and non‐melanoma skin cancers in NS. The highly impaired skin barrier function, hallmark of NS, could facilitate HPV infection, in turn increasing the risk for cancer development.     

Congenital Erythrodermic Psoriasis: Case Report and Literature Review

Abstract: A 4-year-old girl was seen in our department for erythroderma, palmoplantar hyperkeratosis, and scalp desquamation present since birth. The dermatosis had run an intermittent course, with exacerbations after Infections and spontaneous remissions. A specimen from a skin biopsy performed at 1 year of age showed the characteristic features of psoriasis, findings that were confirmed in our biopsy specimen. Treatment with acltretin controlled the outbreaks. At 7 years of age she has developed, for the first time, plaque type psoriasis. Congenital erythroderma is an unusual form of psoriasis with a wide differential diagnosis.     

Netherton syndrome in two Japanese siblings with a novel mutation in the SPINK5 gene: immunohistochemical studies of LEKTI and other epidermal molecules

BACKGROUND: Netherton syndrome (NS) is a severe autosomal recessive disorder characterized by ichthyosiform erythroderma, bamboo hair and atopy. The disease is caused by mutations in the SPINK5 gene, which encodes a putative serine protease inhibitor, LEKTI (lymphoepithelial Kazal-type-related inhibitor). Previous studies have clearly shown a crucial role for LEKTI in skin barrier formation. OBJECTIVES: To identify pathogenic mutations in two Japanese siblings with NS, and further to investigate the consequences of the mutations at the protein level. METHODS: To screen for mutations in the SPINK5 gene, all of its exons and splice junctions were amplified by polymerase chain reaction and directly sequenced. In addition, immunohistochemical staining of LEKTI, desmoglein (Dsg) 1 and elafin was performed with their specific antibodies. RESULTS: Mutation analysis resulted in the identification of compound heterozygous mutations, Q713X and R790X, in the SPINK5 gene of both patients. The former one is a novel mutation. Immunohistochemical studies in one patient demonstrated a complete absence of LEKTI and a strong expression of elafin in the patient's skin. Dsg1 was normally expressed in our patient. CONCLUSIONS: In this report, we describe compound heterozygous mutations in the SPINK5 gene in two Japanese siblings with NS. The result of immunohistochemistry shows LEKTI deficiency and upregulation of elafin in the skin of one patient. Furthermore, our data indicate that degradation of Dsg1 does not always occur in NS.     

Neonatal and infantile erythrodermas: a retrospective study of 51 patients

OBJECTIVE: To determine the frequency of the various underlying causes of erythroderma in newborns or infants, as well as which clinical or laboratory findings were relevant for the etiological diagnosis. PATIENTS: Fifty-one patients who presented with exfoliative erythroderma during their first year of life were included in this retrospective study. SETTING: Department of Pediatric Dermatology at a university hospital. RESULTS: On average, the etiological diagnosis was established 11 months after the onset of erythroderma. The underlying causes observed included immunodeficiency (30%), simple or complex ichthyosis (24%), Netherton syndrome (18%), and eczematous or papulosquamous dermatitis (20%). Five patients (10%) had erythroderma of unknown origin. The following parameters were of value in determining the underlying cause of erythroderma: congenital onset, skin induration and the presence of large scaling plaques, alopecia with or without hair dysplasia, evolution, response to topical corticosteroid therapy, presence of infections, and failure to thrive. Histological analysis confirmed the diagnosis in only 19 (45%) of 42 cases. However, it proved of great value for the detection of significant lymphocyte infiltration or keratinocyte necrosis indicating a diagnosis of Omenn syndrome or immunodeficiency. The prognosis was poor in this series: the mortality rate was 16%, and severe dermatosis persisted in 29 (67%) of the survivors. CONCLUSIONS: The etiological diagnosis of neonatal erythroderma is difficult to make; some clinical features may be helpful, but no one feature is characteristic of a cause. An immunodeficiency must be suspected in cases of severe erythroderma with skin induration, severe alopecia, failure to thrive, infectious complications, or evocative histological findings. The prognosis is poor, with a high rate of mortality in immunodeficiency disorders and severe chronic disease in Netherton syndrome and psoriasis.     

Netherton syndrome in one Chinese adult with a novel mutation in the SPINK5 gene and immunohistochemical studies of LEKTI

Background:

Netherton syndrome (NS) is a severe autosomal recessive ichthyosis. It is characterized by congenital ichthyosiform erythroderma, trichorrhexis invaginata, ichthyosis linearis circumflexa, atopic diathesis, and frequent bacterial infections. The disease is caused by mutations in the SPINK5 (serine protease inhibitor Kazal-type 5) gene, a new type of serine protease inhibitor involved in the regulation of skin barrier formation and immunity. We report one Chinese adult with NS. The patient had typical manifestation of NS except for trichorrhexis invaginata with an atopic diathesis and recurrent staphylococcal infections since birth.

Aims:

To evaluate the gene mutation and of its product activity of SPINK5 gene in confirmation of the diagnosis of one Chinese adult with NS.

Materials and Methods:

To screen mutations in the SPINK5 gene, 33 exons and flanking intron boundaries of SPINK5 were amplified with polymerase chain reaction (PCR) and used for direct sequencing. In addition, immunohistochemical staining of LEKTI (lymphoepithelial Kazal-type-related inhibitor) with specific antibody was used to confirm the diagnosis of NS. The results were compared with that of healthy individuals (twenty-five blood samples).

Results:

A G318A mutation was found at exon 5 of patient''s SPINK5 gene which is a novel missense mutation. The PCR amplification products with mutation-specific primer were obtained only from the DNA of the patients and their mother, but not from their father and 25 healthy individuals. Immunohistochemical studies indicated there was no LEKTI expression in NS patient''s skin and there was a strong LEKTI expression in the normal human skin.

Conclusion:

In this report, we describe heterozygous mutation in the SPINK5 gene and expression of LEKTI in one Chinese with NS. The results indicate that defective expression of LEKTI in the epidermis and mutations of SPINK5 gene are reliable for diagnostic feature of NS with atypical clinical symptoms.     

Netherton syndrome: report of two Taiwanese siblings with staphylococcal scalded skin syndrome and mutation of SPINK5

Netherton syndrome (NS) is a severe autosomal recessive ichthyosis. It is characterized by congenital ichthyosiform erythroderma, trichorrhexis invaginata, ichthyosis linearis circumflexa, atopic diathesis and frequent bacterial infections. Pathogenic mutations in SPINK5 have recently been identified in NS. SPINK5 encodes lymphoepithelial Kazal-type-related inhibitor (LEKTI), a new type of serine protease inhibitor involved in the regulation of skin barrier formation and immunity. We report two Taiwanese brothers with NS. The patients had typical manifestations of NS with an atopic diathesis and recurrent staphylococcal infections, including staphylococcal scalded skin syndrome (SSSS) since birth. Horny layers were obtained by skin surface biopsy for electron microscopy from lesional skin of both patients and from normal controls. All 33 exons and flanking intron boundaries of SPINK5 were amplified for direct sequencing. The ultrastructure of the stratum corneum (SC) was characterized by premature degradation of corneodesmosomes (CDs) with separation of corneocytes. A homozygous 2260A --> T (K754X) mutation of SPINK5 was found in both patients. Staphylococcal exfoliative toxin A (ETA) is a serine protease capable of cleaving desmoglein 1, an important adhesive molecule of CDs, and can cause separation of the SC, resulting in SSSS. The premature degradation of CDs found in our patients may be attributable to insufficient LEKTI, and possibly also to colonization/infection of ETA-producing Staphylococcus aureus. Mechanisms involved in the pathogenesis of the skin barrier defect in NS are proposed. Further study is needed to prove this hypothesis.     

rAAV2-mediated restoration of LEKTI in LEKTI-deficient cells from Netherton patients

Background

Netherton syndrome (NS, MIM 256500) is a potential live threatening autosomal-recessive skin disorder clinically characterized by the trias of congenital erythroderma, hair shaft anomalies and atopic diathesis. It is caused by mutations in the gene SPINK5 resulting in a deficiency of its processed protein named lympho-epithelial Kazal-type related inhibitor (LEKTI). LEKTI controls the activity of several serine proteases in the skin that are involved in terminal differentiation. Loss of LEKTI results in protease hyperactivity, increased degradation of intercellular junctions, reduced stratum corneum adhesion and impaired skin barrier function. Today NS can only be treated symptomatically.

Objective

Does gene transfer offer a therapeutic option for NS in the future?

Methods

A recombinant adeno-associated virus type 2 vector was constructed containing the full length cDNA (rAAV2/C-SPINK5) of functional human LEKTI. Infectious virus particles were used for transfection of LEKTI-deficient-keratinocytes of NS patients in vitro.

Results

Gene transfer of SPINK5 in NS-keratinocytes led to a five-fold increase in mRNA expression of SPINK5 reaching almost 75% of normal value. The functionality of the expressed LEKTI was proven in a hydrolytic activity assay demonstrating that the activity of LEKTI after gene transfer increased closely to the level seen in keratinocytes of healthy individuals.

Conclusion

The results provide first evidence that gene transfer of SPINK5 results in increased LEKTI activity in NS-keratinocytes, thus offering a rational to further pursue such a gene therapy approach for NS.     

Lamellar ichthyosis with episodic psoriasiform reaction pattern

The skin of a girl born with the typical appearance of "collodion baby," evolved into an exfoliative erythroderma that clinically was lamellar ichthyosis. However, biopsy specimens done in early infancy showed psoriasis. Over the ensuing sixteen years she has continued to have clinical lamellar ichthyosis with rare occasions of febrile episodes and superficial pustules. Some biopsy specimens have been diagnosed as showing lamellar ichthyosis, while others have again shown psoriasis.     

Netherton综合征与SPINK5

Netheton综合征是一种严重的常染色体隐性遗传病,其预后较差死亡率高,目前尚无有效的治疗方案。研究发现,Nethetn综合征的发生可能与SPINK5突变导致的LEKTI缺陷和皮肤屏障功能障碍有关。免疫组化检测LEKTI、SPINK5基因突变分析对Netheton综合征的诊断有重要意义,胚胎植入前遗传学诊断可用于Netheton综合征的产前筛查,并有效避免患病新生儿的出生。以往对Netheton综合征的治疗以对症处理为主,随着Netheton综合征遗传学的研究进展,基因转移、局部给予LEKTI生物活性碎片和免疫球蛋白替代疗法可能是将来的发展方向。     

Exacerbation of pityriasis rubra pilaris under efalizumab therapy

A 60-year-old woman, diagnosed as having psoriasis vulgaris in 2004 and unresponsive to standard therapies, received weekly subcutaneous injections with efalizumab. Within 9 weeks of treatment a massive aggravation of skin lesions occurred with widespread orange-tinged erythroderma, islands of normal skin on the back and the inner side of the forearms and palmoplantar hyperkeratosis. A biopsy confirmed the clinical diagnosis of pityriasis rubra pilaris. After discontinuation of efalizumab and treatment with oral corticosteroids, acitretin (30 mg/day) and PUVA therapy, the skin lesions continuously improved. Efalizumab, a fully humanized monoclonal antibody against CD11a, inhibits various T cell processes important in the pathogenesis of psoriasis. Efalizumab has been approved for the treatment of moderate to severe psoriasis, but there are no reports in the literature on the use of efalizumab for pityriasis rubra pilaris.     

Correlation between SPINK5 gene mutations and clinical manifestations in Netherton syndrome patients

Netherton syndrome (NS) is a congenital ichthyosiform dermatosis caused by serine protease inhibitor Kazal-type 5 (SPINK5) mutations. Tissue kallikreins (KLKs) and lymphoepithelial Kazal-type-related inhibitor (LEKTI) (SPINK5 product) may contribute to the balance of serine proteases/inhibitors in skin and influence skin barrier function and desquamation. SPINK5 mutations, causing NS, lead to truncated LEKTI; each NS patient possesses LEKTI of a different length, depending on the location of mutations. This study aims to elucidate genotype/phenotype correlations in Japanese NS patients and to characterize the functions of each LEKTI domain. Since we were unable to demonstrate truncated proteins in tissue from patients with NS, we used recombinant protein to test the hypothesis that the length of LEKTI correlated with protease inhibitory activity. Genotype/phenotype correlations were observed with cutaneous severity, growth retardation, skin infection, stratum corneum (SC) protease activities, and KLK levels in the SC. Predominant inhibition by LEKTI domains against overall SC protease activities was trypsin-like (Phe-Ser-Arg-) activity by LEKTI domains 6-12, plasmin- and trypsin-like (Pro-Phe-Arg-) activities by domains 12-15, chymotrypsin-like activity by all domains, and furin-like activity by none. KLK levels were significantly elevated in the SC and serum of NS patients. These data link LEKTI domain deficiency and clinical manifestations in NS patients and pinpoints to possibilities for targeted therapeutic interventions.     

Genetic analysis of a severe case of Netherton syndrome and application for prenatal testing

Netherton syndrome (NS) is a rare autosomal recessive disease with variable expression. It is defined by a triad of symptoms: congenital ichthyosiform erythroderma, trichorrhexis invaginata and atopy. Recently, genetic linkage has been established to the SPINK5 gene locus on chromosome 5q32 encoding the serine protease inhibitor LEKTI. In this study, we present a recurrent homozygous mononucleotide deletion (153delT) resulting in a severe case of NS exhibiting exfoliative erythroderma with lethal outcome at the age of 4 months and its application in prenatal testing in a subsequent pregnancy of the mother.     

Sézary syndrome without erythroderma: A case report and review of published work

Sézary syndrome (SS) is defined by erythroderma and circulating atypical T cells, with or without lymphadenopathy. Recently, Thompson et al. identified a distinct population of SS patients with an atypical presentation: a high blood tumor burden of Sézary cells fulfilling criteria for SS but without fulfilling the criteria for erythroderma at the diagnosis. Here, we report a case of a 49‐year‐old Japanese man with SS who did not present with erythroderma initially, but exhibited erythematous itchy papules symmetrically located on the legs and arms. We also reviewed reported cases of SS without initial erythroderma. The skin manifestations at diagnosis varied from patches to tumors often seen in mycosis fungoides, and other rarer findings such as excoriation, palmoplantar keratoderma and alopecia. Pruritus was reported in most patients (86%), unlike early mycosis fungoides, and could be the main clue to the diagnosis of SS. Notably, three patients were reported to have presented with papular lesions, similar to our case. Little is known about why skin lesions in SS without erythroderma vary and why these cases did not exhibit erythroderma initially. Attenuated stimulation by colonized Staphylococcus aureus, impairment in recruitment of malignant T cells and suppression of inflammatory response induced by malignant T cells with regulatory phenotype may be associated with skin manifestations. Further studies are necessary to elucidate the etiology of this entity.     

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.     

LEKTI is localized in lamellar granules, separated from KLK5 and KLK7, and is secreted in the extracellular spaces of the superficial stratum granulosum

Lympho-epithelial Kazal-type-related inhibitor (LEKTI) is a putative serine protease inhibitor encoded by serine protease inhibitor Kazal-type 5 (SPINK5). It is strongly expressed in differentiated keratinocytes in normal skin but expression is markedly reduced or absent in Netherton syndrome (NS), a severe ichthyosis caused by SPINK5 mutations. At present, however, both the precise intracellular localization and biological roles of LEKTI are not known. To understand the functional role of LEKTI, we examined the localization of LEKTI together with kallikrein (KLK)7 and KLK5, possible targets of LEKTI, in the human epidermis, by confocal laser scanning microscopy and immunoelectron microscopy. In normal skin, LEKTI, KLK7, and KLK5 were all found in the lamellar granule (LG) system, but were separately localized. LEKTI was expressed earlier than KLK7 and KLK5. In NS skin, LEKTI was absent and an abnormal split in the superficial stratum granulosum was seen in three of four cases. Collectively, these results suggest that in normal skin the LG system transports and secretes LEKTI earlier than KLK7 and KLK5 preventing premature loss of stratum corneum integrity/cohesion. Our data provide new insights into the biological functions of LG and the pathogenesis of NS.     

Erythroderma as a paraneoplastic cutaneous disorder in systemic anaplastic large cell lymphoma

Background Paraneoplastic cutaneous disorders (PCDs) or dermadromes are skin conditions that have an association with internal malignancies but are not themselves malignant. We report the first two cases of systemic anaplastic large cell lymphoma (s‐ALCL) accompanied by erythroderma and multiple leg ulcers as PCDs. Case 1. A 52‐year‐old Japanese man presented with disseminated itchy papular erythemas which he had over his entire body for the preceding 5 years that later exacerbated to erythroderma. Multiple punched‐out ulcers also developed on his lower legs. Superficial lymph nodes (LNs) were swollen, and a left axillary LN biopsy demonstrated dense CD30⁺ atypical large cell (ALC) infiltration. By contrast, lymphocytes infiltrating into the erythroderma and leg ulcers were CD30^?, and T‐cell receptor β (TCRβ) chain gene rearrangement was negative in skin biopsy specimens. Thus, he was diagnosed with s‐ALCL. Not only his s‐ALCL but also his erythroderma and leg ulcers responded well to chemotherapy. Case 2. A 71‐year‐old Japanese woman presented with erythroderma that persisted for approximately 20 years after mastectomy. At her initial hospital visit, she was diagnosed with s‐ALCL by biopsy of swollen left inguinal LNs. Similar to Case 1, CD30⁺ ALCs were negative in skin samples with normal TCRβ chain gene rearrangement. As the erythrodermic skin lesion responded well to chemotherapy for s‐ALCL, it was considered a PCD. Conclusion s‐ALCL development may be predicted by the precedence and concurrence of intractable paraneoplastic erythrodermic and ulcerative skin lesions, as reported in our two cases.     

Pityriasis rubra pilaris: the clinical context of acantholysis and other histologic features

Background Acantholysis has been described in biopsies of pityriasis rubra pilaris (PRP), but this has not been emphasized in the dermatology literature. It is helpful for dermatologists to associate acantholysis with PRP in the correct clinical setting. Objective This study aims to further elucidate the clinical context and associated histologic features of acantholysis in PRP. Methods Eight cases of PRP with acantholysis, 16 other cases of PRP, 26 cases of psoriasis, and 17 cases of erythroderma of different causes were studied in an academic setting. Results The presence of acantholysis initially confounded the diagnosis in two cases of PRP. Acantholysis was focal or extensive and resembled Darier’s disease, Hailey–Hailey disease, or pemphigus vulgaris. Acantholysis was seen in biopsies from early, isolated papulosquamous lesions from the trunk as well as from erythroderma. By comparison, 26 cases of psoriasis and 17 cases of erythroderma showed only focal acantholysis in two cases of erythroderma. Conclusions Acantholysis is a histologic feature of PRP and can serve as a histologic clue to the diagnosis of PRP before the onset of erythroderma. Eosinophils and/or a lichenoid infiltrate may also be evident.     

红皮病108例临床分析

目的探讨红皮病的病因、诱因、治疗方法及效果。方法回顾性分析108例红皮病患者的临床资料。结果108例患者,占皮肤科总住院患者的1.75%。平均发病年龄51.76±21.73岁,男女之比为3.7:1。68.5%继发于原有皮肤病,其中由银屑病所致者占39.8%;其他致病原因依次为病因不明占16.7%,药物过敏12.0%,继发于恶性肿瘤2.8%。不合理治疗是诱发和加重红皮病的主要因素。结论患者以老年居多,病因主要为继发于原有皮肤病,其中大多数继发于银屑病,年龄小的患者以药物过敏为主。不规则使用糖皮质激素是主要诱因。