Prognosis in early stage cutaneous T-cell lymphoma treated with psoralen plus ultraviolet A irradiation and low-dose interferon-α: Long-term efficacy and survival according to conventional and emerging clinical endpoints.

Patients with early stage cutaneous T cell lymphoma (CTCL) usually have a benign and chronic disease course, characterized by temporally response to conventional skin directed therapies and intrinsic possibility to evolve. Using the combination of psoralen plus ultraviolet A irradiation (PUVA) and low-dose interferon-α (INF), the principal treatment goal is to keep confined the disease to the skin, preventing disease progression. Among 87 patients with early stage IA to IIA MF treated with low-dose IFN-α2b and PUVA in our center, complete remission (CR) were reported in 70 patients (80.5%) and the overall response rate (ORR) was 97.8% (n = 85), with a median time to best response to therapy of 5 months (range, 1-30). Among the responders, only the 8% of patients had a relapse with major event. The median follow-up was 207 months (range, 6-295). Survival data showed a median overall survival (OS) not reached (95% CI; 235-NR months), a disease free survival (DFS) of 210 months (95% CI; 200-226 months) and a median time to next treatment (TTNT) of 38.5 months (95% CI, 33-46 months). The long follow up of this study verifies our preliminary results already published in 2006 and confirms the efficacy of INF-PUVA combination therapy in a real world setting, according conventional (OS and DFS) and emerging (TTNT) clinical endpoint of treatment efficacy.

A validation study of the Simple Psoriatic Arthritis Screening (SiPAS) questionnaire to screen psoriasis patients for psoriatic arthritis.

OBJECTIVES: To validate in a multicentric cohort of patients a self-administered PsA screening tool, called Simple Psoriatic Arthritis Screening (SiPAS) questionnaire, to screen psoriasis patients for signs and symptoms of PsA. METHODS: The SiPAS questionnaire was validated in a multicentric Italian cohort of psoriasis patients referred to two rheumatological centres. RESULTS: A total of 202 psoriasis patients were screened with SiPAS in the validation study. Sixty-two psoriasis patients (30.7%) were diagnosed with PsA. The five screening questions (1. Have you ever had a finger or a toe and/or another joint swollen and painful without any apparent reason?; 2. Occasionally, has an entire finger or toe become swollen, making it look like a 'sausage'?; 3. Do you wake up at night because of low back pain?; 4. Have you had pain in your heels?; 5. Has a doctor ever diagnosed you with psoriatic arthritis?) with a dichotomous response, demonstrated high sensitivity and specificity for predicting PsA. Likelihood ratios for individual parameters varied between 2.06 and 4.75. Using the Bayesian Analysis, the presence of three of five items answered as "yes" showed respectively a sensibility and a specificity of 79% and 87%, with a positive likelihood ratio of 6.14. CONCLUSIONS: The SiPAS questionnaire is able to quickly screen psoriasis patients for PsA. A SiPAS score ≥3 is an indication for referral to a rheumatologist. The SiPAS needs further validation.

Dermoscopy features of atypical fibroxanthoma: A multicenter study of the International Dermoscopy Society.

BACKGROUND/OBJECTIVES: Little is known about the dermoscopic features of atypical fibroxanthoma. METHODS: This was a case-control study. Atypical fibroxanthoma lesions were compared with a control group with non-melanoma skin cancer. RESULTS: Altogether 40 atypical fibroxanthoma were collected. Most developed in men (93%), appearing mainly as nodular (63%), amelanotic (93%) and ulcerated (78%) lesions. Most lesions were located on the scalp (55%) and the ears (13%). Dermoscopically, most atypical fibroxanthoma displayed red (83%) and white (70%) structureless areas and irregular linear vessels (43%). A series of features achieved statistical significance when comparing atypical fibroxanthoma with non-melanoma skin cancer. The presence of red and white structureless areas and white lines, and the absence of yellowish-white opaque scales, hairpin vessels and arborising vessels were predictive of atypical fibroxanthoma in univariate analysis. However, when squamous cell carcinoma was excluded from the analysis, none of the criteria achieved statistical significance. When basal cell carcinoma was excluded, three variables achieved statistical significance in predicting atypical fibroxanthoma: red, structureless areas, the absence of opaque yellowish-white scales and absence of white circles. CONCLUSIONS: Atypical fibroxanthomas seem to be barely distinguishable from basal cell carcinoma dermoscopically, but they are more easily distinguishable from a well to moderately differentiated squamous cell carcinoma. A histopathological examination is needed for the final diagnosis.

Acute generalized exanthematous pustulosis induced by hydroxychloroquine: three cases and a review of the literature.

INTRODUCTION: Acute generalized exanthematous pustulosis (AGEP) is a clinical reaction pattern that is principally drug induced and is characterized by acute, extensive formation of nonfollicular sterile pustules on an erythematous and edematous substrate. Hydroxychloroquine (HHCQ), an antimalarial drug widely used to treat rheumatic and dermatologic diseases, has been described as an uncommon cause of AGEP. OBJECTIVES: This article reports 3 cases of HCQ-induced AGEP and reviews similar cases in the published literature. CASE SUMMARIES: The first case involved a 36-year-old woman with a 10-year history of rheumatoid arthritis and Sjögren's syndrome who had begun a 25-day course of HCQ 100 mg BID due to lack of response to a corticosteroid, with a skin reaction developing 21 days into the new treatment. In the second case, a 70-year-old man with poorly controlled rheumatoid arthritis had begun a course of oral HCQ 100 mg BID 20 days before development of AGEP. The final case involved a 79-year-old woman with polymyalgia rheumatica who had been receiving HCQ 100 mg BID as a steroid-sparing agent for 22 days, with rash developing 20 days after the initiation of HCQ. Sixteen cases of HCQ-induced AGEP were identified in the literature, including some that may have been reported under a different name but were consistent with a clinical diagnosis of AGEP. The US Food and Drug Administration has mandated a change to the labeling for HCQ to include AGEP among potential adverse dermatologic reactions to the drug. CONCLUSIONS: This article reports 3 cases of AGEP related to administration of HCQ. HCQ-induced AGEP is a rare but severe, extensive, and acute reaction. No specific therapy is available, and correct diagnosis generally leads to spontaneous resolution once the causative drug has been withdrawn.

Switching from a biological therapy to another biologic agent in psoriatic patients: the experience of PsOMarche group.

BACKGROUND: Switching is a "hot" topic and the main reasons for switching prior biologic agent are for a primary failure, a secondary failure or drug intolerance, patient's dissatisfaction, physician decision. The aim of the study was to assess the optimization of the switching from a biologic agent to another. METHODS: Five Dermatological Units have participated to PsOMarche working group have studied thirty-eight patients affected moderate to severe chronic plaque psoriasis at time 0 (patient recruitment at time of switching from biological therapy to another), 8 weeks (T8), 16 weeks (T16). RESULTS: Twenty-eight males and 10 females were included in the study. At T0, 18 of 22 patients treated with etanercept had been switched to adalimumab and 4 to ustekinumab. Among 10 patients treated with adalimumab, 5 had been switched to ustekinumab, 2 to golimumab and 3 to certolizumab pegol. One patient treated with Infliximab and 5 patients treated with ustekinumab had been switched to adalimumab. Switching had been performed for primary inefficacy in 9 patients (23.6%) and a secondary failure was evidenced in 29 patients (73.4%). PASI75 was achieved in 53% and in 89.4% of patients after 8 weeks and 16 weeks of switching to the second biologic agent respectively; similarly, PsoDISK score significantly decreased at T8 and T16. CONCLUSIONS: The experience of PsOMarche group have shown that the switching to a biologic agent to another is a valuable treatment choice in patients with moderate to severe psoriasis experiencing a treatment failure with one biologic therapy, leading to a good improvement in skin disease and in patient's quality of life.

Feasibility and diagnostic agreement in teledermatopathology using a virtual slide system.

We investigated the feasibility and diagnostic agreement of a virtual slide system (VSS) in teledermatopathology. Forty-six biopsy specimens from inflammatory skin diseases were selected and scanned with a VSS at the Research Unit of Teledermatology, Medical University of Graz, Graz, Austria. Images were stored on a virtual slide server on which a specific Web application suited for telepathology (http://telederm.org/research/dermatopath/) runs. Twelve teleconsultants from 6 different countries reviewed the 46 cases, working directly on the Web application. Telediagnoses agreed with gold standard and conventional diagnosis with an average of 73% and 74%, respectively. Complete concordance among all teleconsultants with gold standard and conventional diagnosis was found in 20% of the cases. In 10 cases in which complete clinical data were missing, the average agreement of telediagnosis with gold standard diagnosis and conventional diagnosis decreased to 65% and 66%, respectively. Only 3 of 4 cases of inflammatory skin diseases were correctly diagnosed remotely with VSS. The system that we have used, despite its usability, is not completely feasible for teledermatopathology of inflammatory skin disease. Moreover, the performance seems to have been influenced by the availability of complete clinical data and by the intrinsic difficulty of the pathology of inflammatory skin diseases.

Guess what! Pseudomonas aeruginosa sepsis.

A 62-year-old woman affected by end-stage renal disease secondary to Waldenstrom's disease was admitted to place a central venous catheter for hemodialysis purposes. During the admission, she gradually developed a number of necrotic ulcerative and fluctuant nodular skin lesions on the submammary flexures, groins and limbs accompanied by high fever and chills. Yellow-green purulent material could be drained from the site of introduction of the jugular catheter. Skin biopsies were taken from the edge of an inguinal necrotic-ulcerative lesion and from a fluctuant nodular lesion of the thigh, where pus was drained and cultured.

Hemosiderotic clear-cell acanthoma: a pigmented mimicker.

The authors report on a case of a 65-year-old man with pigmented clear-cell acanthoma located on the right thigh. Dermoscopy disclosed a peculiar picture consisting of diffuse black pigmentation with a superficial greyish veil in the central portion, dotted-to-globular dark red-black structures mainly located at the periphery with a homogenous regular reticular arrangement; peripheral translucid desquamation. Dermoscopic features are correlated with the histology, where hemosiderin deposits present in a sheet-like arrangement in the perivascular papillary dermis and in a band-like disposition in the reticular dermis at the base of the lesion can account for the pigmented picture. The lesion arose on a trauma-prone skin site; thus the authors believe that traumatic irritation may be responsible for the clinical and dermoscopic pictures, giving rise to a reaction similar in a way to the Auspitz's sign provocated by trauma for psoriasis. Red blood cells extravasation from extremely superficialized capillaries may have led to hemosiderin deposition in the papillary and the reticular dermis.

Psoriasis-like dermoscopic pattern of clear cell acanthoma.

BACKGROUND: Dermoscopy provides additional criteria for the diagnosis of skin lesions. OBJECTIVE: To investigate the dermoscopic aspect of clear cell acanthoma (CCA). METHODS: Six consecutive cases of CCAs were observed under conventional dermoscopy (Case 1-4) or videodermoscopy (Case 5-6). RESULTS: Common dermoscopic findings were: (i) squamous surface with translucid collarette; (ii) dilated capillary loops, mainly perpendicularly orientated to the skin surface, as dotted, pinpoint-like structures, regularly distributed in a reticular array over the surface. CONCLUSIONS: The vascular psoriasis-like dermoscopic findings seem to be a clue for CCA and provide evidence of an inflammatory process for CCA formation.

Videocapillaroscopic findings in the microcirculation of the psoriatic plaque.

BACKGROUND: Videocapillaroscopy (VCP) is a method to study the morphology and dynamics of microcirculation, but information about capillaroscopic features of the psoriatic plaque is limited. OBJECTIVE: To investigate the distribution, morphology and density of capillaries in lesional and perilesional skin of the psoriatic plaque. METHODS: VCP of a well-delimited plaque of the trunk, arms or legs in 15 consecutive patients with untreated, chronic plaque psoriasis was performed. RESULTS: In the lesional skin, capillaries were tortuous and dilated, homogeneously appearing as 'bushy'. In the perilesional skin, capillary loops seemed to be on a parallel course with respect to the skin surface, with their apex directed towards the marginal zone. The number of capillary loops per area unit was statistically increased in perilesional compared to lesional skin. CONCLUSION: According to the morphology, distribution and density of capillary loops, two different angiogenetic patterns were found in lesional and perilesional skin.

Hereditary benign telangiectasia: videocapillaroscopic findings.

In this article, we provide a detailed depiction of capillary changes at the nailfold and labial mucosa in a patient with hereditary benign telangiectasia. To the best of our knowledge, no such extensive capillaroscopic documentation of this entity has been made previously.