Crescendo response to rituximab in oral pemphigus vulgaris: a case with 7-year follow-up.

Pemphigus vulgaris (PV) is an autoimmune blistering disease affecting the skin and mucous membranes. Rituximab, a CD20 chimeric monoclonal antibody, has efficacy in PV management. We report a case of severe oral PV that showed a progressive response to repeated courses of rituximab, culminating in a rapid response within 4 weeks following severe relapse 4 years after initial therapy. It demonstrates the progressively shorter time to achieve partial or complete remission following rituximab infusions, combined with minimal adjuvant therapy over a 7-year follow-up period.

A cutaneous lymphoma international prognostic index (CLIPi) for mycosis fungoides and Sezary syndrome.

BACKGROUND: There is no prognostic index for primary cutaneous T-cell lymphomas such as mycosis fungoides (MF) and Sezary syndrome (SS). METHOD: Two prognostic indices were developed for early (IA-IIA) and late stage (IIB-IVB) disease based on multivariate data from 1502 patients. End-points included overall survival (OS) and progression free survival (PFS). External validation included 1221 patients. FINDINGS: Significant adverse prognostic factors at diagnosis consisted of male gender, age >60, plaques, folliculotropic disease and stage N1/Nx for early stage, and male gender, age >60, stages B1/B2, N2/3 and visceral involvement for late stage disease. Using these variables we constructed two separate models each defined using 3 distinct groups for early and late stage patients: 0-1 (low risk), 2 (intermediate risk), and 3-5 factors (high risk). 10 year OS in the early stage model was 90.3% (low), 76.2% (intermediate) and 48.9% (high) and for the late stage model 53.2% (low), 19.8% (intermediate) and 15.0% (high). For the validation set significant differences in OS and PFS in early stage patients (both p<0.001) were also noted. In late stage patients, only OS differed between the groups (p=0.002). INTERPRETATION: This proposed cutaneous lymphoma prognostic index provides a model for prediction of OS in early and late stage MF/SS enabling rational therapeutic choices and patient stratification in clinical trials.

The profile of dermatological problems in primary care.

BACKGROUND: Cutaneous disease is thought to account for 10-15% of patient consultations with general practitioners, but relatively little is known about the demography of dermatological conditions in primary care. AIM: To assess the proportion and diagnostic profile of dermatological conditions seen in primary care in the southeast of Scotland, and to draw comparisons with secondary dermatological care. METHODS: General practitioners in 13 general practices were asked to note all skin-related consultations during a 2-week period. The case notes of these patients were reviewed, and diagnosis and treatment was recorded. Patients who had consulted for the same skin disorder on >or= 3 occasions during the previous year were invited for assessment by a consultant dermatologist. Where possible, the case notes from 10% of all consultations during the 2-week study period were examined to assess accuracy of recording. RESULTS: The percentage of consultations relating to cutaneous disorders varied between practices, ranging from 3% to 18.8%, with a mean of 8.4%. Eczema accounted for 22.5%, infections 20.3% and benign tumours for 11.4% of consultations with a dermatological basis. In contrast, in secondary care, benign tumours accounted for 23.8%, malignant tumours for 16.4% and eczema for 16.3% of dermatological consultations. CONCLUSIONS: Dermatological disorders make up a significant proportion of general practitioners' workload. The diagnostic profile of primary-care dermatology differs markedly from that of hospital practice. General practitioners may benefit from training specifically tailored to the common primary-care dermatological conditions.

Eosinophilic folliculitis: an important differential diagnosis after allogeneic bone-marrow transplant.

Eosinophilic folliculitis (EF) is a descriptive histopathological term applied to a heterogeneous group of disorders. In EF, the characteristic histopathological features are eosinophilic spongiosis and pustulosis involving the infundibular region of the hair follicle. EF may be seen in association with bacterial and fungal infection, drug reactions and haematological disorders. However, in those conditions, the histopathological changes are rarely restricted to the infundibula but in most cases include a moderate to dense perifollicular or even diffuse dermal infiltrate of lymphocytes, or eosinophilic or neutrophilic granulocytes. We present two cases of EF after mini-allogeneic bone-marrow transplantation (BMT) in order to highlight this rare and perhaps under-recognized clinical association.

The changing face of dermatological practice: 25 years' experience.

BACKGROUND: In order to plan appropriate delivery of dermatology services we need periodically to assess the type of work we undertake and to examine changing trends in the numbers and type of referrals and the workload these referrals generate. OBJECTIVES: To quantify outpatient workload in hospital-based and private practice; to assess reasons for referral to secondary care and to examine the changes over 25 years in the diagnostic spectrum of conditions referred. METHODS: During November 2005, all outpatient dermatological consultations in the south-east of Scotland were recorded. Demographic data, source of and reason for referral, diagnoses, investigations performed, treatment administered and disposal were recorded, and comparisons made with four previous studies. RESULTS: During the 1-month study, attendances were recorded for 2118 new and 2796 review patients (new/review 1 : 1.3, female/male 1.3 : 1, age range 0-106 years). Eighty-nine per cent of new referrals came from primary care and 11% from secondary care. Fifty-seven per cent of referrals were for diagnosis and 38% for management advice. Benign tumours accounted for 33.4%, malignant tumours 11.6%, eczema 16% and psoriasis 7.4% of new cases. For return patients, 20% had skin cancer, 16.5% eczema, 13.4% psoriasis and 9% acne. The referral rate has risen over 25 years from 12.6 per 1000 population in 1980 to 21 per 1000 in 2005, with secondary care referrals increasing from 61 in November 1980 to 230 in November 2005. CONCLUSIONS: Attendances for benign and malignant skin tumours have increased sixfold since 1980. Patients with eczema and psoriasis account for one third of clinic visits. New referrals have risen by 67%, with those from other hospital specialties almost quadrupling since 1980 to 11% of the total in 2005. These results confirm the demand from both primary and secondary care for a specialist dermatology service.

The quality of life of 790 patients with photodermatoses.

BACKGROUND: Polymorphic light eruption and erythropoietic protoporphyria (EPP) have been demonstrated to have a moderate and large impact on the quality of life (QoL) of patients, respectively. However, there is little information available about the impact of other photodermatoses on QoL. OBJECTIVES: To assess and compare the impact of all forms of photodermatoses on patients' QoL using the standard 1-week Dermatology Life Quality Index (DLQI) questionnaire and a modified questionnaire to assess the impact over the previous year. METHODS: All patients with photodermatoses seen between 2001 and 2005 at five U.K. photobiology centres were contacted by post on the same day during a forecasted sunny week across the U.K. and asked to complete DLQI questionnaires. RESULTS: A total of 1877 patients were contacted. Seven hundred and ninety-seven (42%) patients replied, with a range from 30% to 48% for the five individual centres. Nearly two-thirds of patients with actinic prurigo (AP) and more than one-third of patients with photoaggravated dermatoses (PAD), chronic actinic dermatitis, EPP and solar urticaria had a DLQI of > 10, confirming a very large effect of the disorders on QoL. Of the cutaneous porphyrias, both variegate porphyria (median DLQI 3) and porphyria cutanea tarda (median DLQI 1.5) had a much lower impact on QoL than EPP. CONCLUSION: This is the first large-scale study to attempt to measure the impact of a range of photodermatoses on QoL. Photodermatoses have a major impact on QoL. This impact is highest in AP and PAD.

A patient with Rothmund-Thomson syndrome and tongue cancer--experience of radiation toxicity.

We describe a male patient with Rothmund-Thomson syndrome (RTS) given postoperative radiotherapy for squamous carcinoma of the tongue. This was well tolerated. This is only the second reported case of oral cancer and radiotherapy in RTS.

IGF-1 regulates cardiac fibroblast apoptosis induced by osmotic stress.

In this study we have determined the ability of IGF-1 to protect cardiac fibroblasts against osmotic-induced apoptosis and investigated the potential mechanism(s) underlying this protection. Treatment with IGF-1 (1-100 ng/ml) promoted a dose dependent increase in cell survival against osmotic cell death. Both Akt and ERK1/2 were rapidly phosphorylated by IGF-1 and blocked by wortmannin and PD98059, inhibitors of their upstream activators respectively. However, IGF-1-induced protection was mediated via a wortmannin-dependent but PD98059-independent pathway as determined by cell survival assay suggesting a role of PI3-K/Akt. Furthermore, IGF-1 appeared to reduce the activation of a number of early components in the apoptotic pathway in a wortmannin dependent manner including the osmotic stress-induced perturbation in mitochondrial membrane potential, cleavage and activation of caspase-3 and DNA fragmentation. Thus, the results suggest that IGF-1 regulates osmotic stress-induced apoptosis via the activation of the PI3-K/Akt pathway at a point upstream of the mitochondria and caspase-3.

Verrucas. Guidelines for management.

Most people will experience infection with human papilloma virus (HPV) at some point in their life. Diagnosis, based on clinical examination, is usually straight forward. Treatment can, however, be challenging. Indications for treatment include pain, interference with function, cosmetic embarrassment, and risk of malignancy. Clearance rates are highest in young, healthy individuals with short duration of infection. Treatment may be with destructive agents (keratolytics, cryotherapy, curettage and cautery, laser, photodynamic therapy), with antimitotic agents (podophyllin, bleomycin, retinoids), with immune stimulants (topical sensitizers, cimetidine), or with topical virucidal agents [formaldehyde (formalin), glutaral (gluteraldehyde)]. As yet, there is no single totally effective treatment for viral warts. Some patients may choose to leave their warts untreated until spontaneous resolution. In those who seek intervention, simple, well tolerated therapies should be chosen initially in preference to more complicated, potentially harmful agents. It is likely that future research will be directed to developing an antiviral agent specific for HPV which would be safe, effective and not prohibitively expensive.

A tri-linear pattern of scleroderma 'en coup de sabre' following Blaschko's lines.

Linear scleroderma 'en coup de sabre' is characterized by atrophy and furrowing of the skin of the frontoparietal region. In most cases it occurs as a single, paramedian line that may be associated with hypoplasia of underlying structures and hemiatrophy of the face. Rarely, lesions form two lines, either both on the same side or bilaterally. We describe an 18-year-old male with an unusual tri-linear pattern of scleroderma 'en coup de sabre' following Blaschko's lines.

Generalized pustular psoriasis following withdrawal of oral cyclosporin treatment for palmo-plantar pustulosis.

We report the cases of two patients who developed generalized pustular psoriasis on the withdrawal of oral Cyclosporin treatment for persistent palmoplantar pustulosis. This complication does not appear to have been reported previously.