Risk of mycosis fungoides in psoriatic patients: a critical review.

Psoriasis has been controversially associated with risk of non-Hodgkin lymphoma (NHL) and mycosis fungoides (MF). Also patients who developed MF after systemic treatment for psoriasis have been reported, and some authors suggested that the association between MF and psoriasis is not infrequent. We performed an extensive literature review in order to examine the risk of developing MF in psoriatic patients with a systematic search of the English-language databases. An increased risk for lymphoma overall in psoriatic patients has been found only by three out of seven studies. The risk of developing MF in psoriatic patients has been investigated by different studies in different populations and with different methodologies presenting bias and limitations, and it seems reasonable that misclassification between psoriasis and MF may explain the association reported. In contrast to the large number of psoriatic patients treated with biologicals, only 27 case reports of MF after biological therapy for psoriasis have been reported, and in 10 cases, the initial psoriasis diagnoses were then revised as MF. A true association between MF and psoriasis is possible, but the real incidence and prevalence are still unknown. The reported higher risk of developing MF in psoriatic patients should be reconsidered in the light of the bias of misclassification and the low magnitude reported in previous studies. There is not enough evidence to support a causal relation among biological therapies and MF in psoriatic patients.

European S1 guidelines on the management of lichen planus: a cooperation of the European Dermatology Forum with the European Academy of Dermatology and Venereology.

Lichen planus (LP) is a chronic inflammatory and immune-mediated disease that affects the skin, hair, nails and mucous membranes. Although there is a broad clinical spectrum of lichen planus manifestations, the skin and oral cavity remain the major sites of involvement. A group of European dermatologists with a long-standing interest and expertise in lichen planus has sought to define therapeutic guidelines for the management of patients with LP. The clinical features, diagnosis and possible medications that clinicians can use, in order to control the disease, will be reviewed in this manuscript. The revised final version of the lichen planus guideline was passed on to the European Dermatology Forum (EDF) for a final consensus with the European Academy of Dermatology and Venereology (EADV).

Hymenoptera stings in the head region induce impressive, but not severe sting reactions.

Stings in the head region are considered to be a risk factor for severe systemic reactions to hymenoptera stings. We supposed that stings in skin areas, which are well supplied with blood, lead to more severe reactions and tested our hypothesis in 847 patients with confirmed hymenoptera venom allergy. However, symptom severity was independent from sting site: only 16.3% of patients with severe reactions were stung on the head (P = 0.017). But we confirmed age > 40 years (P < 0.001) as well as elevated basal tryptase levels (P = 0.001) as risk factors. Taking antihypertensive drugs seemed to have an influence: 41.7% of patients taking antihypertensive drugs experienced a severe reaction compared to 29.5% of patients, not taking such drugs (P = 0.019). However, considering patients' age in regression analysis, taking antihypertensive drugs had no effect on symptom severity (P = 0.342). Importantly, in most patients with severe reactions, cutaneous signs were absent (P < 0.001).

Subacute lupus erythematosus during treatment with golimumab for seronegative rheumatoid arthritis.

We report on a 52-year-old woman with a history of severe seronegative rheumatoid arthritis. Several conventional therapies and biological therapy with etanercept and infliximab had been unsuccessful. In 2010 she was given golimumab subcutaneously at a monthly dose of 50 mg. She had a negative ANA titre. After 16 months of uninterrupted therapy and sustained response, she developed skin lesions on the upper trunk, back and upper extremities, which worsened on exposure to the sun. The skin biopsy was compatible with subacute lupus erythematosus. Laboratory findings included an ANA titre 1:640, negative anti-Ro/SSA and anti-DNA antibodies. Topical corticosteroid therapy proved inadequate. The patient's condition improved only after discontinuation of golimumab. The causal relationship between subacute cutaneous lupus erythematosus and golimumab is not dose-related and occurs with some delay (a typical feature of immunological adverse reactions). The association is likely, but not confirmed (because re-challenge was not performed). However, a clear improvement was noted after withdrawal. Based on this case, we hypothesized the aetiological role of golimumab-associated immunogenicity. TNF-α antagonist-induced lupus-like syndrome (TAILS) is a well-known side effect of this class of substances. The British Society of Rheumatology recommends discontinuation of the causal anti-TNF-α treatment in patients with TAILS.

Lucio Leprosy with Lucio's phenomenon, digital gangrene and anticardiolipin antibodies.

Lucio's phenomenon (LPh) is considered a necrotizing panvasculitis and a variant of leprosy Type 2 reaction, clinically characterised by necrotic-haemorrhagic lesions on the extremities and trunk. LPh is observed in diffuse lepromatous leprosy (DLL or Lucio-Latapí leprosy). This is a distinct form of lepromatous leprosy (LL) reported mainly in Mexico. Anti-phospholipid antibody syndrome (APS) has been rarely described in LPh. We report a case of Lucio-Latapí leprosy with LPh observed in a patient from the province of El Oro in Ecuador, who presented clinical manifestations of long standing DLL (non-nodular infiltration of the skin, collapse of the nasal pyramid, madarosis, atrophy of the earlobes), of LPh (necrotic-haemorrhagic macules with irregular shapes) and of APS (necrosis of the right big and second toe). Histopathology showed perineural and periadnexal foamy macrophages with numerous bacilli (diagnostic of LL) in the subcutis, a mild lobular panniculitis with a large subcutaneous vessel infiltrated by macrophages in the wall (typical of LPh) and vessels of the superficial and mid dermis occluded by thrombi but without signs of vasculitis (typical of occlusive vasculopathy as in APS). Our observations suggest that some cases of LPh may be associated with APS. Anti-cardiolipin antibodies (aCL) and lupus anticoagulant (LA) should be tested in patients with LPh because this may have therapeutic implications.

Analysis of clinical, dermoscopic and histopathological features of primary melanomas of patients with metastatic disease--a retrospective study at the Department of Dermatology, Medical University of Graz, 2000-2010.

BACKGROUND: Incidence rates of malignant melanoma have been increasing worldwide and metastatic melanoma is still a significant problem despite widespread prevention programmes. OBJECTIVES: We made a systemic review of all metastasized melanoma patients treated at the Department of Dermatology, Medical University of Graz in the years 2000-2010 and looked at the kind of melanoma type, e.g. if it has been slowly growing superficial spreading melanoma (SSM) or fast growing nodular melanoma (NM). METHODS: Histological slides and clinical images of patients treated at our department between 2000 and 2010, who received chemotherapy because of proven metastatic disease were analysed with regard to growth type of their primary tumours. RESULTS: A total of 88 patients met the inclusion criteria. Mean age of all patients was 57 years (median 59 years, SD ± 15 years). Of these 88 patients 51 patients (58%) (28 male patients and 23 female patients) had SSM; mean age 58 years (median 58 years, SD ± 14 years) and 37 patients (42%) (18 male patients and 19 female patients) had NM; mean age 56 years (median 61 years, SD ± 17 years). Mean Breslow thickness in the SSM group was 2.26 mm (median: 1.6 mm, SD ± 2.11 mm). In the NM group, mean Breslow thickness was 4.59 mm (median: 3.50 mm, SD ± 4.07 mm). When separated by gender, 46 melanomas were seen in the male group (28 SSM and 18 NM) and 42 melanomas in the female group (23 SSM and 19 NM). CONCLUSIONS: Our results showed that more than half of the patients with metastatic disease had SSMs and not, as suspected, NMs. As SSMs are growing over a longer period to become invasive and potentially metastatic, there might be a chance to focus primary and secondary prevention programmes not only on fast growing tumours but also on slowly changes of tumours.

Teledermatology for skin cancer prevention: an experience on 690 Austrian patients.

BACKGROUND: Recent studies investigated the value of teledermatology (TD) as a valid tool for a dermatologist-directed triage systems. OBJECTIVE: To investigate the feasibility of a store-and-forward TD triage system in a large number of patients. METHODS: Previously trained general practitioners selected suspicious skin tumours in the setting of a general preventive medicine screening programme and transmitted their dermoscopic images via virtual private network for decision-making. Within 48 h, two teleconsultants highly experienced in dermoscopy first assessed image quality, then made a diagnosis and answered if lesions were to follow-up, to excise or to be re-evaluated at face-to-face (FTF). RESULTS: A total of 955 lesions were telediagnosed [743 (78%) benign melanocytic, six (0.6%) malignant melanocytic, 186 (19%) benign non-melanocytic and 20 (2%) malignant non-melanocytic]. Excision was recommended for 111 (12%) lesions, 10 lesions (1%) were referred to FTF examination. Follow-up was recommended for 707 (74%) lesions. The vast majority of the lesions (82%) were screened as benign and an intervention was requested in only 18% of cases. Eighty-two patients (12% of the total) were lost at follow-up. The diagnostic accuracy was of 94% with sensitivity of 100% and specificity of 95.8%. CONCLUSIONS: We confirm that TD is suitable to triage skin cancers.

Benign dermoscopic features in melanoma.

BACKGROUND: Various dermoscopic features are usually associated with benign melanocytic lesions. Our objective was to determine frequency and extension of benign dermoscopic features (BDF) in melanoma. METHODS: Retrospective review of dermoscopic images of a consecutive series of 516 histopathologically proven melanomas collected in 6 years in Graz. Correlation of BDF with mean Breslow thickness, with presence/absence of associated benign nevus component and with the pre-operative clinico-dermoscopic diagnosis, as reported on the original histopathologic reports. RESULTS: In addition to melanoma specific criteria, 42% of melanomas showed BDF. In 12.3% cases, the benign features occupied more than the half of the lesion. The BDF typical pigment network, homogeneous pattern and regular globules/cobblestone pattern had the highest frequency. BDF were associated with relatively thinner melanomas (mean Breslow thickness of 0.51 mm). The presence of BDF was observed in 67.1% of histopathologically documented nevus-associated melanoma and in 35.7% of melanoma de novo. A pre-operative clinico-dermoscopic diagnosis of melanoma was achieved in only 54.1% of cases displaying BDF. CONCLUSION: A significant proportion of melanomas may exhibit BDF. Clinicians should be aware of the presence of BDF in melanoma as possible diagnostic pitfall.

UV-induced alterations of the skin evaluated over time by reflectance confocal microscopy.

BACKGROUND: Ultraviolet radiation (UVR) induces various alterations of the skin and plays a decisive part regarding the development of melanoma and non-melanoma skin cancer. For a closer examination of these phenomena in vivo reflectance confocal microscopy (RCM) is one of the most eligible options as it represents a diagnostic tool that allows a non-invasive examination of the skin, showing microanatomical structures and individual cells. OBJECTIVES: The aim of this study was using RCM to observe alterations of the skin induced by UVR and to describe the development of these changes. In addition, the findings were compared with histological examinations of the same area. METHODS: A small area in the gluteal region of 10 healthy subjects was exposed to a threefold individual minimal erythema dose of solar-simulated UVR. The following development of the sunburn reaction was evaluated with RCM 1, 24, 72 h and 1 week after UVR exposure. Furthermore, RCM images of unexposed skin were obtained, serving as a reference. To contrast histological examination with RCM, punch biopsies were performed at each point in time. The obtained data were interpreted regarding histological and RCM-based criteria on sunburn reaction. RESULTS: All important UVR-induced alterations of the skin could be shown in RCM beginning with an inflammatory reaction (inflammatory cells, vasodilatation, oedema), containing the formation of microvesicles, followed by the appearance of apoptotic keratinocytes (sunburn cells), activated melanocytes and at last, loss of the epidermal structure. There was an excellent correlation between RCM and histological features. CONCLUSIONS: Reflectance confocal microscopy is a highly valuable tool for non-invasive monitoring of UVR-induced changes of the skin over time. Furthermore, RCM provides a more detailed visualization of inflammatory cell formation and epidermal blood flow than histological examination can.

Clinical and epidemiological comparison of patients affected by palmoplantar plaque psoriasis and palmoplantar pustulosis: a case series study.

BACKGROUND: In 2007 the International Psoriasis Council proposed that palmoplantar pustulosis (PPP) should be considered a separate condition from psoriasis, despite the presence of certain phenotypes common in both diseases. OBJECTIVES: To describe and compare demographic and clinical characteristics among patients with PPP and palmoplantar plaque psoriasis. METHODS: This was a retrospective case series study from 2005 to 2010. The following data were obtained: age, sex, family history, smoking habits, nail involvement, joint involvement, disease duration, lesion morphology (plaque or pustular), histological diagnosis, comorbidities, and Physician's Global Assessment (PGA) score for extrapalmoplantar lesions. The sample size calculation indicated that 80 patients, 40 patients for each group (palmoplantar plaque psoriasis and PPP) were needed to see clinically relevant differences between groups. RESULTS: Ninety patients were selected, 51 with palmoplantar plaque psoriasis and 39 with PPP. No statistically significant differences were registered between patients affected by PPP and palmoplantar plaque psoriasis as regards age at onset of the disease (48 vs. 44 years; P=0·4), disease duration (6 vs. 10 years; P=0·1), family history of psoriasis (28% vs. 33%; P=0·7), concomitant arthritis (26% vs. 25%; P=1·0), or smoking habits (54% vs. 41%; P=0·2). We observed a female predominance (P=0·01) and a lesser frequency of nail involvement (P=0·03) in patients affected by PPP. CONCLUSIONS: Our data suggest a close relationship between PPP and psoriasis. The existing data concerning epidemiology, clinical presentation, genetics, histopathology and pathogenesis do not permit a clear distinction between these two entities, which seem to coincide in many aspects. PPP appears to have a marked predilection among female smokers.

Imported leprosy in Italy.

BACKGROUND: Leprosy is far from being eliminated with more than 200,000 new cases detected (NCD)/year. OBJECTIVE: Retrospective analysis between 2003 and 2009 to compare the New Case Detected Rate (NCDR) observed in Italy in the immigrant population with the NCDR of the same population in their country of origin to verify if the cases observed are those expected or not. METHODS: Leprosy statistics were retrieved from the Italian leprosy register and from official WHO data. RESULTS: The NCD in Italy were lower than expected, from 2003 when the expected number of NCD was 40.5 between the legally resident immigrants, but only one case was diagnosed (98% of lower from the expected), to 2009 when four NCD were diagnosed and 41 were expected (90% lower from expected). CONCLUSIONS: This study points out a discrepancy between the observed and the expected cases of leprosy in Italy. Specifically, the number of NCD was less than expected for each studied year. Of course our data do not represent a validation, but only an indication of the leprosy diagnosis in Italy. Difficulty in accessing the health systems, fear of segregation, ignorance and illegal immigrant status with consequent fear of police arrest are possible explaining factors. The critical issue anyhow is the medical expertise. The role of the dermatologist is fundamental. For these reasons, there is still a need for wide spread leprosy teaching programmes. Although with few limitations, this study represents a first approach to validate the accuracy in leprosy diagnosis in Italy.

Psoriasis and oral lesions: multicentric study of Oral Mucosa Diseases Italian Group (GIPMO).

OBJECTIVE: This is a multicentric, observational and controlled study designed to verify the existence of a significant association between plaque-type psoriasis and oral lesions, such as geographic tongue and/or fissured tongue. STUDY DESIGN: during a period of 9 months all consecutive patients with plaque-type psoriasis were enrolled using simple nonrandom (sequential) sampling. The control group included healthy subjects presenting to the same Dermatology centers to monitor pigmented skin lesions; the patients were matched for age and sex. All patients were examined for oral lesions. RESULTS: Out of a total of 535 psoriatic patients and 436 control group patients, oral mucosal lesions were detected in 188 (35.1%) and 86 (19.7%) cases, respectively, and the difference is statistically significant. Fissured tongue (FT) and geographic tongue (GT), which were most frequently detected, were seen more frequently in psoriatic patients (FT: 22.6%; GT: 9.1%) than the control group (FT: 10.3%; GT: 5.2%) (p<0.05). CONCLUSIONS: On the basis of the similar studies reported in the literature and the large number of patients involved in our study, we can conclude that FT and GT can be clearly suggested as oral manifestations of plaque-type psoriasis, although the reason for this association is not clear.

Mobile teledermatology for skin tumour screening: diagnostic accuracy of clinical and dermoscopic image tele-evaluation using cellular phones.

BACKGROUND: The ability to diagnose malignant skin tumours accurately and to distinguish them from benign lesions is vital in ensuring appropriate patient management. Little is known about the effects of mobile teledermatology services on diagnostic accuracy and their appropriateness for skin tumour surveillance. OBJECTIVE: To evaluate the diagnostic accuracy of clinical and dermoscopic image tele-evaluation for mobile skin tumour screening. METHODS: Over a 3-month period up to three clinical and dermoscopic images were obtained of 113 skin tumours from 88 patients using a mobile phone camera. Dermoscopic images were taken with a dermatoscope applied to the camera lens. Clinical and dermoscopic images of each lesion together with clinical information were separately teletransmitted for decision-making. Results were compared with those obtained by face-to-face examination and histopathology as the gold standard. RESULTS: A total of 322 clinical and 278 dermoscopic images were acquired; two (1%) clinical and 18 (6%) dermoscopic pictures were inadequate for decision-making. After excluding inadequate images, the majority of which were dermoscopic pictures, only 104 of the 113 skin tumours from 80 of 88 patients could be tele-evaluated. Among these 104 lesions, 25 (24%) benign nonmelanocytic, 15 (14%) benign melanocytic, 58 (56%) malignant nonmelanocytic and six (6%) malignant melanocytic lesions were identified. Clinical and dermoscopic tele-evaluations demonstrated strong concordance with the gold standard (κ = 0·84 for each) and similar high sensitivity and specificity for all diagnostic categories. With regard to the detailed diagnoses, clinical image tele-evaluation was superior to teledermoscopy resulting in 16 vs. 22 discordant cases. CONCLUSIONS: Clinical image tele-evaluation might be the method of choice for mobile tumour screening.

Immunophenotype of skin lymphocytic infiltrate in patients co-infected with Mycobacterium leprae and human immunodeficiency virus: a scenario dependent on CD8+ and/or CD20+ cells.

BACKGROUND: Leprosy occurs rarely in human immunodeficiency virus (HIV)-positive patients. In contrast to tuberculosis, there has been no report to date of an increase in HIV prevalence among patients with leprosy or of differences in leprosy's clinical spectrum. While several studies describe the systemic immune response profile in patients co-infected with HIV and leprosy, the local immune skin response has been evaluated in only a small number of case reports and limited series of patients. OBJECTIVE: To investigate the interaction between Mycobacterium leprae and HIV infection in the skin. METHODS: We investigated the presence and frequency of cells positive for CD4, CD8, CD20, TIA-1, FOXP3 and CD123 in lymphocytic infiltrates from 16 skin biopsies taken from 15 patients with HIV-leprosy co-infection. RESULTS: CD4+ cells were absent in infiltrates from 6 (38%) skin biopsies and present in 10 (62%) cases at low levels (<1·16%) of the lymphocytic infiltrate. CD8+ was the predominant phenotype in the infiltrate (99·4%), followed by TIA-1, expressed by >75% of CD8+ cells. FOXP3+ cells were also present, representing 3·4% of the lymphocytic infiltrate. CD20+ cells were detected in 75% of the cases; however, in two cases (12%) these cells represented 25-50% of the infiltrate, while in the other 10 cases (62%) they were present only focally (<25% of the infiltrate). CD123+ cells were not observed in any of the studied specimens. CONCLUSIONS: Data presented here suggest that cell-mediated immune responses to M. leprae are preserved at the site of disease and that in the absence of CD4+ cells, CD8+FOXP3+ and CD20+ cells may be involved in granuloma formation.