Family History Of Psoriasis Research Articles

Acceptance of chronic illness in psoriasis vulgaris patients

Acceptance of an illness is regarded as a considerable problem in patients with chronic diseases. Lack of acceptance can lead to lower adherence to medical treatment and delayed clinical improvement. Psoriasis, being a chronic skin disease, is known to cause considerable distress to patients. To examine whether selected demographic factors and personal resources have an impact on acceptance of illness in psoriasis vulgaris patients. Self-reported data on acceptance of illness, self-efficacy, health locus of control, optimism and coping strategies were collected from 100 psoriasis vulgaris inpatients and PASI was calculated by a dermatologist. The following measures were employed: acceptance of illness scale, generalized self-efficacy scale, multidimensional health locus of control scale, life orientation test and mental adjustment to disease scale. Physical factors such as sex, age, disease duration and severity, and family history of psoriasis had no effect on acceptance of illness. However, based on multiple regression analysis, higher levels of optimism, lower conviction of others' influence on one's health and the less frequently employed coping strategy concentration on emotions, together with more severe disease expressed by PASI, were correlated with higher acceptance of disease in psoriasis patients. The results obtained seem to confirm that personal resources play an important role in acceptance of chronic illness. Enhancement of optimism, and minimizing one's conviction that one's health depends on others could lead to higher acceptance of illness.

Flare and change of psoriasis morphology during the course of treatment with tumour necrosis factor blockers

Tumour necrosis factor (TNF) blockers represent an exciting advance in the management of psoriasis. However, the safety profile of these drugs is not completely established. We present a review of the literature, and report on eight patients: two with the unexpected appearance of psoriasis, and the remaining six with exacerbation and change in morphology of their existing psoriasis, all of which occurred during treatment with the TNF blockers adalimumab, etanercept and infliximab. The two new cases, neither of whom had any personal or family history of psoriasis, developed pustular psoriasis on the palms and/or soles. The other six patients, previously diagnosed with severe chronic plaque psoriasis (four patients), generalized pustular psoriasis (one) and erythrodermic psoriasis (one), developed eruptive guttate psoriasis between 15 days and 18 months after the beginning of therapy. These patients had never before presented guttate-type psoriatic lesions, and the lesions appeared in areas of the body that were free of psoriatic plaques at baseline.

Classification criteria for psoriatic arthritis: Development of new criteria from a large international study

To compare the accuracy of existing classification criteria for the diagnosis of psoriatic arthritis (PsA) and to construct new criteria from observed data. Data were collected prospectively from consecutive clinic attendees with PsA and other inflammatory arthropathies. Subjects were classified by each of 7 criteria. Sensitivity and specificity were compared using conditional logistic regression analysis. Latent class analysis was used to calculate criteria accuracy in order to confirm the validity of clinical diagnosis as the gold standard definition of "case"-ness. Classification and Regression Trees methodology and logistic regression were used to identify items for new criteria, which were then constructed using a receiver operating characteristic curve. Data were collected on 588 cases and 536 controls with rheumatoid arthritis (n = 384), ankylosing spondylitis (n = 72), undifferentiated arthritis (n = 38), connective tissue disorders (n = 14), and other diseases (n = 28). The specificity of each set of criteria was high. The sensitivity of the Vasey and Espinoza method (0.97) was similar to that of the method of McGonagle et al (0.98) and greater than that of the methods of Bennett (0.44), Moll and Wright (0.91), the European Spondylarthropathy Study Group (0.74), and Gladman et al (0.91). The CASPAR (ClASsification criteria for Psoriatic ARthritis) criteria consisted of established inflammatory articular disease with at least 3 points from the following features: current psoriasis (assigned a score of 2; all other features were assigned a score of 1), a history of psoriasis (unless current psoriasis was present), a family history of psoriasis (unless current psoriasis was present or there was a history of psoriasis), dactylitis, juxtaarticular new bone formation, rheumatoid factor negativity, and nail dystrophy. These criteria were more specific (0.987 versus 0.960) but less sensitive (0.914 versus 0.972) than those of Vasey and Espinoza. The CASPAR criteria are simple and highly specific but less sensitive than the Vasey and Espinoza criteria.

Food‐Induced Acute Generalized Exanthematous Pustulosis in a Pregnant Woman

A 21-year-old woman of Romany origin, in the third trimester of her fourth pregnancy, was admitted to the hospital because of a generalized erythematous and pustular eruption and desquamation involving her face, neck, trunk, and extremities. The skin changes were accompanied by fever (100.4 degrees F [38 degrees C]) and malaise. The patient was convinced that the dermatitis was induced by the consumption of "spoilt" pork sausage (bad smell, changed taste) approximately 24 hours earlier. Clinical examination revealed a woman with phototype III skin, black eyes, and black hair, in good general health. Widespread, symmetrical, moderately intense erythema and isolated or coalescing targetoid lesions studded with discrete, pinhead-sized, nonfollicular pustules in the center or at the periphery were distributed over her face, trunk, groins, and upper and lower extremities (Figures 1). On the neck and abdomen, lamellar desquamation was observed (Figure 2). Palms, soles, scalp, mucous membranes, hair, and nails were not affected. Nikolsky's sign was negative. The patient complained of very slight skin burning and itching. The pregnancy was proceeding without any complications and her obstetric status was normal. The woman had neither any accompanying diseases, nor previous personal or family history of psoriasis, nor any known allergies. She had taken no systemic medication (not even vitamins). She had three pregnancies; two ended with the delivery of healthy babies and one of them was aborted at her will. Laboratory studies revealed leukocytosis (13.2 x 109/L), neutrophilia (8 x 109/L), anemia (hemoglobin, 108 g/L), and an elevated erythrocyte sedimentation rate (68-110 mm/h). The results from the following investigations were normal: urinalysis, renal and hepatic function, serum albumin, Ca, Na, K, aspartate aminotransferase titer, cryoprotein, hepatitis B surface antigen, and serum markers for syphilis. Bacterial and fungal cultures of pustular content were sterile. A skin biopsy specimen of lesional skin revealed subcorneal pustules containing leukocytes and necrotic keratinocytes and a mixed perivascular inflammatory infiltrate with isolated eosinophils in the dermis (Figure 3). The patient was treated with systemic methylprednisolone in gradually reduced doses, fluocinonide cream 0.05%, and emollients. As a result, her fever disappeared and her erythema faded. Frequent obstetric examination and cardiotocography were normal and showed no evidence of placental insufficiency. At 40 weeks' gestation, the patient spontaneously gave birth without any complications to a healthy boy. She was discharged with complete resolution of the skin lesions, preceded by massive desquamation of the epidermis. The 1-year follow-up of the patient revealed no relapses or new pustular eruptions.

Severe Acute Generalized Exanthematous Pustulosis in a Pregnant Woman

A 23-year-old woman was admitted to the Department of Dermatology 4 days postpartum with multiple disseminated pustules. A week earlier, she had received 500 mg of amoxicillin with 125 mg clavulanic acid for pharyngitis. After three doses, she noticed the first pustules appear on the chest and back, and the antibiotic treatment was discontinued. Three days later, during the 37th gestational week, she delivered her second child prematurely, but in generally good condition. After delivery, her skin lesions began to spread, and the patient developed fever up to 39 degrees C (102.2 degrees F). Her medical history was significant for similar pustular lesions at age five, diagnosed as a bacterial skin infection despite negative skin cultures. Antibiotics were introduced with no clinical improvement over several months. Her skin lesions resolved after discontinuation of antibiotics. No personal or family history of psoriasis was present. On admission, generalized pustules and flaccid blisters with pus on an erythematous background were present (Figure 1). The patient complained of burning of the skin lesions and fever (38.2 degrees C [100.8 degrees F]) but was otherwise in good condition. Laboratory tests revealed leukocytosis (15,000/mL) with granulocytosis (82%) and an extremely high C-reactive protein level (323.4 mg/L; normal range, 0-7 mg/L). Bacteriologic culture of the pus was negative. The histopathology revealed a subcorneal blister filled with neutrophils and a few epidermal cells. In the dermis, a scant perivascular inflammatory cell infiltrate was noted (Figure 2). Direct immunofluorescence revealed small amounts of IgM at the dermoepidermal junction. Because of the very diffuse distribution of pustules, two doses of hydrocortisone 200 mg IV b.i.d. for 2 days was administered, followed by prednisone 40 mg q.d. with rapid tapering. Rapid improvement of skin lesions was observed and the patient's skin practically cleared within 7 days. Corticosteroids were discontinued after 14 days.

Psoriasis onset during infliximab treatment: description of two cases

The authors describe two patients with no personal or family history of psoriasis who developed psoriatic lesions during infliximab treatment: a woman affected by seronegative rheumatoid arthritis and a man affected by ankylosing spondylitis.

Vascular endothelial growth factor gene polymorphisms increase the risk to develop psoriasis

We investigated the relationship between eight polymorphisms in the gene encoding for vascular endothelial growth factor (VEGF) (-1540C > A, -1512Ins18, -1451C > T, -460T > C, -160C > T, -152G > A, -116G > A and +405G > C) and plaque-type psoriasis stratified for age at onset, gender and family history of dermatosis. For this purpose, 117 patients with chronic plaque-type psoriasis and 215 healthy subjects were enrolled. We found that being homozygous -1540AA, -1512InsIns, -1451TT, -460CC and -152AA conferred a significant risk in developing psoriasis compared with heterozygous (-1540CA, -1512 + Ins, -1451CT, -460CT and -152AG) and homozygous genotypes (-1540CC, -1512 + +-1451CC, -460TT and -152GG) grouped together [odds ratio (ORs) = 1.73, 1.73, 1.73, 1.77 and 1.87, respectively]. Conversely, having the -116AA or +405GG genotype did not significantly increase the risk of disease expression compared with other genotypes of the same loci. Interestingly, we found that -1540AA, -1512InsIns, -1451TT, -460CC and -152AA homozygous genotypes have a significant two-fold increased risk in developing psoriasis after the age of 40 years (late-onset psoriasis) (ORs = 2.19, 2.19, 2.19, 2.05 and 2.26; P = 0.02, 0.02, 0.02, 0.04 and 0.02, respectively) as compared with controls. On the contrary, we found no phenotype-genotype association of the same magnitude among the patients in whom psoriasis developed at or before the age of 40 years (early-onset psoriasis) compared with controls. Genotype distributions were not significantly different when cases and controls were stratified either by gender or family history of psoriasis. Finally, VEGF plasma concentration was not significantly different between patients and controls and was not correlated with the severity of the disease.

Amicrobial pustulosis with antinuclear antibodies and rheumatoid factor

Conflicts of interest: none declared. Sir, Amicrobial pustulosis (AP) is a recurrent, aseptic pyoderma involving mainly the skin folds such as the external acoustic meatus, scalp, pubis and digits of young females and is often associated with autoimmune diseases or immunological abnormalities.1 We describe a patient with AP who had positive antinuclear antibodies (ANA) and rheumatoid factor (RF). A 29‐year‐old woman presented with a 1‐year history of exudative eruptions and hair loss on the scalp. Examination showed exudative erythema with pustules, erosions and hair loss on the scalp (Fig. 1a). Moreover, erythema with pustules was observed on the axilla (Fig. 1b), vulva, external acoustic meatus, around the umbilicus and on the second and third fingertips of both hands (Fig. 1c). In addition, loss of axillary hair was observed. The patient had no past or family history of psoriasis. Laboratory data showed red blood cell count 4·39 × 1012 L−1, white blood cell count 7·5 × 109 L−1 (neutrophils 78·6%, lymphocytes 15·8%, monocytes 4·3%, eosinophils 1·2%, basophils 0·1%) and C‐reactive protein 0·5 mg dL−1. Liver and renal function tests were normal. ANA (× 80, speckled pattern) and RF (21 IU mL−1, normal 0–12) were positive, although lupus band test (LBT) was negative. Other autoantibodies, however, were negative and there were no symptoms of collagen diseases, including rheumatoid arthritis. Staphylococcus aureus was cultured only once from a pustule on the axilla but not from any other lesions. Skin biopsy from the scalp showed infiltration of neutrophils, eosinophils and lymphocytes in the dermis around the hair follicles (Fig. 2a,b), and skin biopsy from the axilla showed parakeratosis, psoriasiform epidermal hyperplasia and neutrophilic spongiform pustules, which were similar to pustular psoriasis (Fig. 2c,d). Moreover, infiltration of neutrophils was detected in the dermis (Fig. 2c). On the basis of the clinical and histopathological findings, we diagnosed the patient as having AP.1 The effect of an oral antibiotic was limited, but topical application of mometasone furoate ointment, a class II glucocorticoid, markedly reduced the eruptions within 2 weeks and subsequently induced complete regrowth of the scalp hairs. Continued occasional applications of mometasone furoate have resulted in good control without scarring alopecia for the past 6 years.

Drug-Induced Psoriasis: An Evidence-Based Overview and the Introduction of Psoriatic Drug Eruption Probability Score

ABSTRACTPsoriasis is a common skin disorder that needs a long-term management, not only because, of its prevalence but also because of the profound impact it can have on patients quality of life. Drugs may result in exacerbation of a preexisting psoriasis, in induction of psoriatic lesions on clinically uninvolved skin in patients with psoriasis, or in precipitation of the disease in persons without family history of psoriasis or in predisposed individuals. The knowledge of the drugs that may induce, trigger, or exacerbate the disease is of primary importance in clinical practice. By reviewing the literature, there are many reports on drug-induced psoriasis, but the data are not univocal. We propose, when possible, the use of a probability score from the authors to obtain a better classification and further understanding of drug-induced psoriasis.

Relationship Between Smoking and the Clinical Severity of Psoriasis

To evaluate the association between different components of smoking history and the clinical severity of psoriasis. A hospital-based cross-sectional study. Inpatient wards of a hospital for skin diseases in Rome, Italy. A total of 818 adults with psoriasis. The Psoriasis Area and Severity Index was used to assess the clinical severity of psoriasis between February 21, 2000, and February 19, 2002. After adjustment for potential confounders (sex, age, body mass index, psychological distress, family history of psoriasis, duration of psoriasis disease, and alcohol consumption), high intensity of smoking (>20 cigarettes daily) vs a lower level of consumption (< or =10 cigarettes daily) was associated with a more than 2-fold increased risk of clinically more severe psoriasis (odds ratio [OR], 2.2; 95% confidence interval [CI], 1.2-4.1). Cigarette-years, measured as the product of the intensity and duration (years) of smoking, significantly increased the risk of clinically more severe psoriasis after adjustment for confounding factors (OR,1.3; 95% CI, 1.0-1.6, for a 600-U increase in cigarette-years). Separate analyses for men and women showed that the effect of cigarette-years was stronger for women (OR, 1.8; 95% CI, 1.2-2.6, for a 400-U increase in cigarette-years) than for men (OR, 1.2; 95% CI, 0.9-1.6, for a 700-U increase in cigarette-years). Smoking is associated with the clinical severity of psoriasis and highlights the importance of smoking cessation in patients with psoriasis.

Psoriasis induced by anti–tumor necrosis factor therapy: A paradoxical adverse reaction

Administration of anti-tumor necrosis factor (anti-TNF) agents is beneficial in a variety of chronic inflammatory conditions, including psoriasis. We describe 5 patients in whom psoriasiform skin lesions developed 6-9 months after the initiation of anti-TNF therapy for longstanding, seropositive rheumatoid arthritis (etanercept or adalimumab), typical ankylosing spondylitis (infliximab), and Adamantiades-Behçet's disease (infliximab). In all 5 patients, the underlying disease had responded well to anti-TNF therapy. Four patients developed a striking pustular eruption on the palms and/or soles accompanied by plaque-type psoriasis at other skin sites, while 1 patient developed thick erythematous scaly plaques localized to the scalp. In 3 patients there was nail involvement with onycholysis, yellow discoloration, and subungual keratosis. Histologic findings from skin biopsies were consistent with psoriasis. None of these patients had a personal or family history of psoriasis. In all patients, skin lesions subsided either with topical treatment alone, or after discontinuation of the responsible anti-TNF agent. The interpretation of this paradoxical side effect of anti-TNF therapy remains unclear but may relate to altered immunity induced by the inhibition of TNF activity in predisposed individuals.

Vitamin D receptor gene polymorphisms, particularly the novel A-1012G promoter polymorphism, are associated with vitamin D3 responsiveness and non-familial susceptibility in psoriasis

Psoriasis is a genetically determined disease characterized by hyperproliferation and disordered maturation of the epidermis. Th1 lymphocytes are implicated in its pathogenesis. The vitamin D receptor (VDR) is a candidate modifying gene, having immunosuppressive effects and being involved in anti-proliferative and pro-differentiation pathways in keratinocytes. There is suggestive evidence that the A allele of the A-1012G polymorphism is associated with down-regulation of the Th1 response, via GATA-3. The F and T alleles of Fok1 and Taq1 have been associated with increased VDR activity. The present study aimed to test the hypothesis that the A allele of A-1012G is protective for occurrence and severity of psoriasis and enhances therapeutic response to vitamin D analogues and that these effects would be additive to those of Fok1 and Taq1. The study group comprised 206 psoriasis patients who had received topical calcipotriol treatment and 80 controls. There was no significant linkage disequilibrium between any pair of the three polymorphic sites (P=0.3-0.8). The A, F and T alleles were positively associated with calcipotriol response: AA genotype (compared to AG/GG), odds ratio (OR)=2.18 (P=0.04); TT, OR=1.97 (P=0.03); AAFF genotype combination, OR=4.11 (P=0.03); AATT, OR=5.64 (P=0.005); and FFTT, OR=3.22 (P=0.01). Comparing patients without, to patients with, a family history of psoriasis, the A allele was under represented (P=0.01) and the AAFF genotype combination even more so (compared to residual genotypes) (OR=0.24; P=0.005). AAFF was also under-represented in patients without a family history compared to controls (OR=0.31; P=0.04). There were no associations of family history with Fok1 and Taq1. There were no associations of severity of psoriasis with any polymorphism. In conclusion, the A-1012G, Fok1 and Taq1 VDR polymorphisms were associated with response to calcipotriol. A-1012G and Fok1 were associated with susceptibility to non-familial psoriasis.

Gene expression profiles in psoriasis: analysis of impact of body site location and clinical severity

Psoriasis is characterized by symmetry of plaques and modulation of multiple genes within those plaques. We compared gene expression profiles of plaques of psoriasis at different anatomical sites for both symmetrical and asymmetrical disease to ascertain whether the same genes were expressed. Gene expression profiles were analysed in biopsies from lesional and uninvolved skin from two groups of patients with either predominantly symmetrical or truncal plaques of psoriasis vulgaris, and from normal skin of healthy volunteers. Genomic analyses were performed using cDNA array and kinetically monitored reverse transcriptase-initiated polymerase chain reaction (kRT-PCR) approaches. A cluster of genes upregulated in involved psoriasis skin as compared with normal skin was identified using each of these two technologies. Clustering of patients based on their gene expression profile did not reveal any correlation with family history of psoriasis, age at onset or association of psoriasis with arthritis. There was no difference in gene expression profile between the type (symmetrical vs. truncal) or location (left vs. right side of body) of psoriatic plaques. Gene expression profiles of involved psoriatic skin analysed by kRT-PCR analysis did correlate with both global (Psoriasis Area and Severity Index) and local (erythema, desquamation and plaque elevation) clinical severity. These results indicate that it may be feasible to analyse the molecular effects of pharmacological agents on psoriatic skin in 'minizone' protocols, that the obtained data can be correlated with clinical severity and that plaques of psoriasis in the same individual express the same genes.

Peripheral Blood T‐Cell Responses to Keratin Peptides that Share Sequences with M Proteins are Largely Restricted to Skin‐Homing CD8+ T Cells

The association of psoriasis with throat infections by streptococcus pyogenes suggests a potential antigenic target for the T cells that are known to infiltrate dermis and epidermis of psoriatic skin. Streptococcal M protein shares an extensive sequence homology with human epidermal keratins. Keratins 16 (K16) and 17 (K17) are mostly absent from uninvolved skin but are upregulated in psoriatic lesions. There is increasing evidence that CD8+ T cells play an important effector role in psoriasis and M protein‐primed T cells may recognize these shared epitopes in skin via molecular mimicry. To identify candidate epitopes, peptides with sequences from K17 were selected on the basis of predicted binding to HLA‐Cw6 and sequence similarities with M6 protein. Matched peptides from the sequence of M6 protein and a set of peptides with poor predicted binding were also selected. Cw6+ individuals with psoriasis and Cw6+ healthy controls, having a family history of psoriasis, were recruited. PBMCs were incubated with the peptide antigens. T‐cell activation in the CD4+, CD8+ and later the skin‐homing cutaneous lymphocyte‐associated antigen (CLA)‐expressing subset of CD8+ T cells was evaluated by CD69 expression and intracellular IFN‐γ accumulation using flow cytometry. We demonstrate that Cw6+ psoriasis patients had significant CD8+ T‐cell IFN‐γ responses to peptides from K17 and M6 protein selected on the basis of sequence homology and predicted HLA‐Cw*0602 binding. These responses were about 10 times more frequent in the skin‐homing cutaneous lymphocyte‐associated antigen‐expressing (CLA+) subset of CD8+ T cells. CD4+ T cells showed only borderline responses. CD8+ T cells from Cw6 + nonpsoriatic individuals responded to some M6 peptides but very rarely to K17 peptides, and this also applied to the CLA+CD8+ subset. These findings indicate that psoriatic individuals have CD8+ T cells that recognize keratin self‐antigens and that epitopes shared by streptococcal M protein and human keratin may be targets for the CD8+ T cells that infiltrate psoriatic skin lesions.

HLA-DQA1 and DQB1 alleles are associated with genetic susceptibility to psoriasis vulgaris in Chinese Han.

Psoriasis vulgaris is a chronic skin disorder characterized by infiltration of inflammatory elements, keratinocyte hyperproliferation and altered differentiation. Although the pathogenesis of psoriasis is not fully understood, there is solid evidence of a susceptibility locus in the human leukocyte antigen (HLA) region. To investigate whether HLA-DQA1 and DQB1 alleles are associated with genetic susceptibility to psoriasis vulgaris in Chinese Han. The polymerase chain reaction-sequence-specific primer (PCR-SSP) method was used to analyse the distribution of HLA-DQA1 and DQB1 alleles in 189 patients with psoriasis and 273 healthy controls. The HLA-DQA1*0104 (OR = 2.33, P = 0.0001154, Pc = 2.0 x 10-3), DQA1*0201 (OR = 3.36, P < 1.0 x 10-7, Pc < 1.0 x 10-6), DQB1*0201 (OR = 1.64, P = 0.0192, Pc > 0.05) and DQB1*0303 (OR = 1.55, P = 0.0377, Pc > 0.05) alleles were more prevalent in patients with psoriasis vulgaris than in controls, and HLA-DQA1*0501 (OR = 0.30, P = 0.0000039, Pc < 4.0 x 10-5) alleles were less prevalent. The HLA-DQA1*0104 (OR = 2.42, P = 0.0001159, Pc < 2.0 x 10-3), DQA1*0201 (OR = 3.74, P < 1.0 x 10-7, Pc < 1.0 x 10-6) and DQA1*0501 (OR = 0.30, P = 0.0000374, Pc < 4.0 x 10-4) alleles were only associated with type I psoriasis. HLA-DQA1*0104 and DQA1*0201 were more prevalent in patients with or without a family history of psoriasis. However, the DQA1*0501 allele was only more prevalent in patients without a family history of psoriasis. HLA-DQA1*0104 and DQA1*0201 alleles may be psoriasis susceptibility genes or may be in close linkage with the susceptibility genes. The HLA-DQA1*0501 allele seems to have a protective effect against the development of psoriasis vulgaris in Chinese Han. There may be a difference in genetic background between psoriasis patients with and without a family history of psoriasis.

Self-reported stress reactivity and psoriasis-related stress of Nordic psoriasis sufferers.

The purpose of the study was to investigate the perceived influence of stress on psoriasis onset and disease severity in a large sample of psoriatics and to compare stress reactors and non-reactors with respect to psoriasis-related stress, disease severity, family history of psoriasis and sociodemographic factors. A total of 5795 members of the Nordic psoriasis associations and 702 patients recruited from Nordic dermatologists or university clinics were asked whether their first outbreak of psoriasis occurred during times of worry and stress. They were also asked to rate the degree to which their psoriasis was influenced by stress and to complete the Psoriasis Life Stress Index, the Psoriasis Disability Index and a number of additional questions concerning sociodemographic factors. Seventy-one per cent of the members and 66% of the patients reported that their psoriasis was exacerbated by stress, and 35% in both groups reported that the onset of their psoriasis occurred during a time of worry and stress. Stress reactors, scoring above the median on stress reactivity, reported greater disease severity, psoriasis-related stress and impairment of disease-related quality of life. They also reported more frequent use of tobacco, tranquillizers and antidepressants. More women than men were stress reactors, and stress reactors were more likely to have a family history of psoriasis. Our findings confirm and extend the results of previous studies and indicate that a subgroup of psoriatics may be more psychologically reactive to their disease and its influence on everyday life. Whether this group is also physiologically more reactive to psychosocial stress remains to be investigated.

Subcorneal Pustular Dermatosis in Association with Chronic Lymphocytic Leukaemia

Sir,Subcorneal pustular dermatosis (SPD) is a chronic,relapsing, pustular eruption, the aetiology and patho-genesis of which are unknown. It has previously beenreported in association with rheumatoid arthritis (1),Crohn’s disease (2), pyoderma gangrenosum, Sweet’ssyndrome and other neutrophilic dermatoses, benignmonoclonal gammopathies (3) and IgA myeloma (4).Here we describe a patient with SPD in association withchronic lymphocytic leukaemia (CLL) and discuss thepossibility of a pathogenetic link. No cases havepreviously been reported of SPD and CLL.CASE REPORTA 78-year-old woman was referred to the DermatologyDepartment in August 1996 with a pruritic rash affect-ing her scalp, axillae, inguinal and infra-mammaryflexures. She had no personal or family history ofpsoriasis. Examination showed erythema, papules andpustules. A biopsy was inconclusive, showing in someareas hyperkeratotic, parakeratotic and mildly acan-thotic epidermis with spongiosis and focal neutrophilexocytosis, while in other areas there were well-formedsubcorneal pustules. Special stains were negative forbacteria and fungi. The patient was treated with topicalsteroids, emollients, tar preparations and calcipotriol,each with little benefit. She then received oral acitretin(10–25 mg daily for a total of 29 months), bath PUVA(twice weekly for 3 months) and oral methotrexate (upto 20 mg once weekly for 6 months); each produced atbest a short-lived partial improvement in the rash.In September 1999 the patient had a widespreaderythematous pustular eruption affecting the trunk andlimbs with a flexural distribution. She was systemicallywell and denied any joint or gastrointestinal symptoms.On this occasion, a mild lymphocytosis (white cellcount 11.8610

Psychological Distress Impairs Clearance of Psoriasis in Patients Treated With Photochemotherapy

To assess whether psychological distress affects treatment outcome in psoriasis. Cohort study of patients with psoriasis receiving psoralen-UV-A (PUVA) photochemotherapy. Two university hospital dermatology departments. One hundred twelve patients with chronic plaque psoriasis. We assessed clinical severity of psoriasis, psychological distress, and other potential confounders of treatment outcome such as skin phototype, family history of psoriasis, and alcohol intake before starting PUVA therapy. Clinical severity of disease and response to therapy were assessed at every fourth appointment. Pathological or high-level worry was the only significant (P =.01) predictor of time taken for PUVA to clear psoriasis. Event curves of time to clearance significantly differed between high- and low-level worry groups (log rank test, 6.64; df = 1; P =.01). Patients in the high-level worry group cleared with PUVA treatment at a rate 1.8 times slower than that of the low-level worry group (ExpB = 1.81; 95% confidence interval, 1.13-2.90). Fiftieth percentile time to clearance of psoriasis in the high- and low-level worry groups showed a median difference of 19 days. Psychological distress, in the form of excessive worrying, has a significant and detrimental affect on treatment outcome in patients with psoriasis. Patients with psoriasis who are classified as high-level worriers may benefit from adjunctive psychological intervention before and during treatment. These findings provide further evidence of the existence of a brain-skin axis.

Do the Physical and Histologic Features and Time Course in Acute Generalized Exanthematous Pustulosis Reflect a Pattern of Cytokine Dysregulation?

Background: Acute generalized exanthematous pustulosis (AGEP) is characterized by fever and an indurated erythematous eruption early, with the development of nonfollicular pinhead sterile pustules on an erythematous background. The eruption progresses and resolves relatively rapidly. Although drugs are believed to be the major etiologic agents, other immune modulators, including infections, heavy metals, and radiation, have been implicated. Objective: The purpose of this study was to document underlying diseases in patients with AGEP and to determine if this data and the histologic features suggested an underlying pattern of immune dysregulation. Methods: Twenty-one patients with new or recurrent episodes of AGEP were questioned concerning underlying diseases. The histopathologic features seen in the biopsy sections and the approximate time of biopsy during the course of their eruptions were recorded. Results: Two patients had a history of psoriasis and one patient had a family history of psoriasis, two patients had diagnoses of sarcoid, two patients had inflammatory bowel disease, one had autoimmune thyroiditis, and one patient had multiple sclerosis. Biopsies done at the onset of the eruption showed marked to moderate papillary dermal edema and a mixed dermal inflammatory infiltrate. Shortly thereafter, biopsies showed spongiform pustules within the epidermis and occasional dyskeratotic cells with residual perivascular dermal edema. Although no definitive vasculitis was seen, there was leukocytoclasis within the dermal infiltrate in the majority of biopsy specimens performed more than 48 hours after the onset of the eruption. Conclusion: The histologic features seen in AGEP and the disease associations suggest that patients who develop this eruption may have an underlying tendency for development of a pattern of immune dysregulation characterized by a T helper-1 cytokine pattern.

Do the physical and histologic features and time course in acute generalized exanthematous pustulosis reflect a pattern of cytokine dysregulation?

Acute generalized exanthematous pustulosis (AGEP) is characterized by fever and an indurated erythematous eruption early, with the development of nonfollicular pinhead sterile pustules on an erythematous background. The eruption progresses and resolves relatively rapidly. Although drugs are believed to be the major etiologic agents, other immune modulators, including infections, heavy metals, and radiation, have been implicated. The purpose of this study was to document underlying diseases in patients with AGEP and to determine if this data and the histologic features suggested an underlying pattern of immune dysregulation. Twenty-one patients with new or recurrent episodes of AGEP were questioned concerning underlying diseases. The histopathologic features seen in the biopsy sections and the approximate time of biopsy during the course of their eruptions were recorded. Two patients had a history of psoriasis and one patient had a family history of psoriasis, two patients had diagnoses of sarcoid, two patients had inflammatory bowel disease, one had autoimmune thyroiditis, and one patient had multiple sclerosis. Biopsies done at the onset of the eruption showed marked to moderate papillary dermal edema and a mixed dermal inflammatory infiltrate. Shortly thereafter, biopsies showed spongiform pustules within the epidermis and occasional dyskeratotic cells with residual perivascular dermal edema. Although no definitive vasculitis was seen, there was leukocytoclasis within the dermal infiltrate in the majority of biopsy specimens performed more than 48 hours after the onset of the eruption. The histologic features seen in AGEP and the disease associations suggest that patients who develop this eruption may have an underlying tendency for development of a pattern of immune dysregulation characterized by a T helper-1 cytokine pattern.