A Rapidly Developing Nodule in a Patient With Hairy Cell Leukemia in Remission: Merkel Cell Carcinoma: A Case Report.

BACKGROUND/AIM: Hairy cell leukemia (HCL) is a well-known lymphoproliferative disease with very effective treatment approaches primarily relying on purine analogues. However, these treatments are associated with profound and prolonged immunosuppression. Merkel cell carcinoma (MCC) is a rare and extremely aggressive skin tumor with an increased incidence in immunocompromised patients. CASE REPORT: We report a case of a patient with HCL who was diagnosed with MCC, while in remission following retreatment with pentostatin, which induced a profound decrease in CD4 (+) T-cells. CONCLUSION: Our case provides further evidence supporting the hypothesis of a significant association between immunosuppression and MCC pathogenesis.

A Retrospective Study on Alopecia Areata in Children: Clinical Characteristics and Treatment Choices.

BACKGROUND: Although children are affected frequently with alopecia areata (AA), data are limited on clinical characteristics and treatment choices. MATERIALS AND METHODS: We retrospectively reviewed the records of the pediatric dermatology department over a 12-year period to identify children with AA. Clinical data were collected. RESULTS: Three hundred and sixty-four children with AA were identified, aged 1-12 years, 214 males and 150 females. The mean age of onset was 6.6 years (±3.3). The disease presented with patches on the scalp in the majority (90.7%), whereas only 6 children had alopecia totalis or universalis. The most commonly prescribed treatment was topical steroids (69.1%), followed by the combination of topical steroids and minoxidil 2% (14.3%). Oral steroids were prescribed in only 16 children. Follow-up at 3 months was available for only 70 children and the majority (84.3%) had some hair regrowth. Hair regrowth was unrelated to the number of plaques (p = 0.257), disease location (p = 0.302), and atopy (p = 0.999). Hair regrowth only correlated with the type of treatment (p = 0.003) with potent topical and intralesional steroids giving the best results. CONCLUSION: AA usually presents with a mild form in children, and potent topical steroids are the mainstay of treatment.

Use of vismodegib for the treatment of multiple basal cell carcinomas in a patient with xeroderma pigmentosum.

A female patient with xeroderma pigmentosum and multiple basal cell carcinomas was treated with a hedgehog pathway inhibitor (vismodegib), which successfully treated the majority of her basal cell carcinomas while preventing the appearance of new lesions. The sum diameter of lesions showed a 61% decrease after 16.5 months of treatment, although after 18.5 months of treatment, a persistent lesion showed progression and metatypical characteristics; adverse events included persistent alopecia muscle cramps, dysgeusia, and amenorrhea. Despite these limitations, vismodegib may have a role in the treatment of some patients with xeroderma pigmentosum.

Six-Year Retrospective Review of Hospital Data on Antimicrobial Resistance Profile of Staphylococcus aureus Isolated from Skin Infections from a Single Institution in Greece.

Objective: To determine the prevalence of resistant strains of Staphylococcus aureus (S. aureus) isolated from Skin and soft tissue infections (SSTI) to various antibiotics. Material and Methods: All culture-positive results for S. aureus from swabs taken from patients presenting at one Greek hospital with a skin infection between the years 2010-2015 were examined retrospectively. Bacterial cultures, identification of S. aureus and antimicrobial susceptibility testing were performed using the disk diffusion method according to Clinical and Laboratory Standards Institute (CLSI) guidelines and European Committee on Antimicrobial testing (EUCAST) breakpoints. EUCAST breakpoints were applied if no CLSI were available. Results: Of 2069 S. aureus isolates identified, 1845 (88%) were resistant to one or more antibiotics. The highest resistance was observed for benzylpenicillin (71.9%), followed by erythromycin (34.3%). Resistant strains to cefoxitin defined as MRSA (methicillin-resistant S. aureus) represented 21% of total isolates. Interestingly, resistance to fusidic acid was 22.9% and to mupirocin as high as 12.7%. Low rates were observed for minocycline, rifampicin and trimethoprim/sulfamethoxazole (SXT). Resistance to antibiotics remained relatively stable throughout the six-year period, with the exception of cefoxitin, fusidic acid and SXT. A high percentage of MRSA strains were resistant to erythromycin (60%), fusidic acid (46%), clindamycin (38%) and tetracycline (35.5%). Conclusions: Special attention is required in prescribing appropriate antibiotic therapeutic regimens, particularly for MRSA. These data on the susceptibility of S. aureus may be useful for guiding antibiotic treatment.

Quality of life, depression, anxiety and loneliness in patients with bullous pemphigoid. A case control study.

BACKGROUND:: Bullous pemphigoid (BP) is a chronic, autoimmune blistering skin disease that affects patients' daily life and psychosocial well-being. OBJECTIVE:: The aim of the study was to evaluate the quality of life, anxiety, depression and loneliness in BP patients. METHODS:: Fifty-seven BP patients and fifty-seven healthy controls were recruited for the study. The quality of life of each patient was assessed using the Dermatology Life Quality Index (DLQI) scale. Moreover, they were evaluated for anxiety and depression according to the Hospital Anxiety Depression Scale (HADS-scale), while loneliness was measured through the Loneliness Scale-Version 3 (UCLA) scale. RESULTS:: The mean DLQI score was 9.45±3.34. Statistically significant differences on the HADS total scale and in HADS-depression subscale (p=0.015 and p=0.002, respectively) were documented. No statistically significant difference was found between the two groups on the HADS-anxiety subscale. Furthermore, significantly higher scores were recorded on the UCLA Scale compared with healthy volunteers (p=0.003). CONCLUSION:: BP had a significant impact on quality of life and the psychological status of patients, probably due to the appearance of unattractive lesions on the skin, functional problems and disease chronicity.

Quality of life, depression, anxiety and loneliness in patients with bullous pemphigoid. A case control study

Abstract: Background: Bullous pemphigoid (BP) is a chronic, autoimmune blistering skin disease that affects patients' daily life and psychosocial well-being. Objective: The aim of the study was to evaluate the quality of life, anxiety, depression and loneliness in BP patients. Methods: Fifty-seven BP patients and fifty-seven healthy controls were recruited for the study. The quality of life of each patient was assessed using the Dermatology Life Quality Index (DLQI) scale. Moreover, they were evaluated for anxiety and depression according to the Hospital Anxiety Depression Scale (HADS-scale), while loneliness was measured through the Loneliness Scale-Version 3 (UCLA) scale. Results: The mean DLQI score was 9.45±3.34. Statistically significant differences on the HADS total scale and in HADS-depression subscale (p=0.015 and p=0.002, respectively) were documented. No statistically significant difference was found between the two groups on the HADS-anxiety subscale. Furthermore, significantly higher scores were recorded on the UCLA Scale compared with healthy volunteers (p=0.003). Conclusion: BP had a significant impact on quality of life and the psychological status of patients, probably due to the appearance of unattractive lesions on the skin, functional problems and disease chronicity.

Cryotherapy versus imiquimod 5% cream combined with a keratolytic lotion in cutaneous warts in children: A randomized study.

BACKGROUND AND OBJECTIVE: Although cutaneous warts are extremely common in children effective treatment does not exist. We combined imiquimod with a salicylic acid solution 15% (SA) on areas, with thick keratin, like palms and soles to increase its penetration through the epithelium. METHODS: Patients were randomly divided into two groups. The first group was subjected to cryotherapy with liquid nitrogen every two weeks for a maximum of 3 months. The second group was subjected to treatment with imiquimod 5% daily for 6-10 h onto the warts for five consecutive days per week for a maximum of 3 months. RESULTS: Eighty-six children were included, 35 girls and 51 boys, 49 in the cryotherapy group and 37 in the imiquimod and SA group. At the end of the third month no statistically significant difference could be noted between the imiquimod 5% and salicylic acid and cryotherapy groups (p = 0.154). Thirty (81.1%) children treated with imiquimod 5% and salicylic acid were free from their warts in comparison to 33 (67.3%) children treated with cryotherapy. CONCLUSIONS: Imiquimod 5% cream as a monotherapy or in combination with SA can be used safely in children with warts and is equally effective and more effective than cryotherapy in plantar warts. LIMITATIONS: Our study is not placebo controlled and spontaneous resolution cannot be ruled out.

Quality of life and psychosocial aspects in Greek patients with psoriasis: a cross-sectional study

Abstract: BACKGROUND: Psoriasis is a common, long-term skin disease associated with high levels of psychological distress and a considerable adverse impact on life. The effects of psoriasis, beyond skin affliction, are seldom recognized and often undertreated. OBJECTIVE: The aim of the study is to evaluate the quality of life, anxiety and depression, self-esteem and loneliness in patients with psoriasis. METHODS: Eighty-four patients with psoriasis were enrolled in the study. The quality of life, depression and anxiety, loneliness and self-esteem of the patient were assessed using the Dermatology Life Quality Index, Hospital Anxiety and Depression Scale, the UCLA loneliness Scale (UCLA-Version 3) and Rosenberg's Self-esteem Scale, respectively. RESULTS: The Dermatology Quality of Life Index score among psoriasis patients was 12.61 ± 4.88. They had statistically significantly higher scores according to the Hospital Anxiety and Depression Scale -anxiety subscale (p=0.032)-compared with healthy volunteers. Moreover, a statistically significant difference was found between the two groups concerning the UCLA-scale (p=0.033) and RSES-scale (p<0.0001). Female patients presented with lower self-esteem than male patients. CONCLUSION: Psoriasis is a distressing, recurrent disorder that significantly impairs quality of life. Therefore, the recognition and future management of psoriasis may require the involvement of multi-disciplinary teams to manage the physical, psychological and social aspects of the condition, as is the case for systemic, long-term conditions.

Management of pemphigus vulgaris: challenges and solutions.

The main objective in the treatment of pemphigus vulgaris is to control the disease, prevent relapses, and avoid adverse events associated with the prolonged use of steroids and immunosuppressive agents. Systemic corticosteroids remain the gold standard treatment for pemphigus vulgaris. Azathioprine and mycophenolate mofetil are the first line of steroid-sparing treatment. Rituximab is extremely effective in recalcitrant pemphigus, when other treatments fail to control the disease. The European Dermatology Forum recommends tapering prednisolone by 25% every 2 weeks after the consolidation phase, and a 5 mg reduction every 4 weeks when the dose is reduced to <20 mg. If the patient relapses, options include increasing steroids back to the previous dose, adding an immunosuppressant if using steroid monotherapy, or replacing a first-line immunosuppressant by another if already on combination therapy.

Quality of life and social isolation in Greek adolescents with primary focal hyperhidrosis treated with botulinum toxin type A: a case series.

Primary hyperhidrosis, although extensively documented in adults, typically has onset that dates back to early childhood. It is an unpleasant and socially disabling problem for the affected child, but little attention has been paid to the disease in adolescents. The objective of the current study was to evaluate the effectiveness of botulinum toxin type A (BTXA) in adolescents with primary palmar and axillary hyperhidrosis and to determine its effect on quality of life and social isolation. Thirty-five individuals (17 girls, 18 boys) with moderate to severe palmar and axillary hyperhidrosis were treated with BTXA (onabotulinum). Patients were examined at baseline and 6 months after treatment. The Hyperhidrosis Disease Severity Scale (HDSS) was used to evaluate disease severity and the Children's Dermatology Life Quality Index (CDLQI) was used to assess quality of life. The University of California at Los Angeles loneliness scale (UCLA version 3) was used to assess personal perception of loneliness and social isolation. The median age of the participants was 14 years, and 48.6% were female. Twenty-one had palmar hyperhidrosis, and 14 had axillary hyperhidrosis. Total CDLQI and social isolation scores decreased significantly after treatment with BTXA (both p < 0.001). There was a significant difference between pre- and post-treatment levels of severity of hyperhidrosis. No statistically significant difference was documented for CDLQI and UCLA scores between boys and girls. Treatment of hyperhidrosis with BTXA resulted in improvement in quality of life, social skills, and activities.

A randomized trial of efficacy of beta-sitosterol and its glucoside as adjuvant to cryotherapy in the treatment of anogenital warts.

INTRODUCTION: All available treatments for anogenital warts have substantial failure rates. An immunomodulating treatment that enhances the patient's own immunity could be valuable as an adjuvant to conventional methods. METHODS: About 123 patients were enrolled in this study and were randomized either to undergo only cryotherapy every 3 weeks (n = 60 patients) or to undergo cryotherapy and to receive a mixture containing 20 mg sterol and 0.2 mg sterolin (BSS-BSSG mixture), known for its immunomodulating properties (n = 63). RESULTS: A complete response after 3 months was demonstrated by 18.3% of the patients in the first group and 30.2% of the second group, while 61.7% of the first group and 79.4% of the second group were lesion free at the end of the 6-month follow-up period. Cox regression analysis of the time until response showed a significant advantage to the mixture treatment group (hazard ratio 2.76, 95% confidence interval 1.61-5.67). LIMITATIONS: The study was not placebo controlled. CONCLUSION: The BSS-BSSG mixture gave promising results as an adjuvant to cryotherapy and may be used in patients with refractory warts.

Quality of life and psychosocial aspects in Greek patients with psoriasis: a cross-sectional study.

BACKGROUND: Psoriasis is a common, long-term skin disease associated with high levels of psychological distress and a considerable adverse impact on life. The effects of psoriasis, beyond skin affliction, are seldom recognized and often undertreated. OBJECTIVE: The aim of the study is to evaluate the quality of life, anxiety and depression, self-esteem and loneliness in patients with psoriasis. METHODS: Eighty-four patients with psoriasis were enrolled in the study. The quality of life, depression and anxiety, loneliness and self-esteem of the patient were assessed using the Dermatology Life Quality Index, Hospital Anxiety and Depression Scale, the UCLA loneliness Scale (UCLA-Version 3) and Rosenberg's Self-esteem Scale, respectively. RESULTS: The Dermatology Quality of Life Index score among psoriasis patients was 12.61 ± 4.88. They had statistically significantly higher scores according to the Hospital Anxiety and Depression Scale -anxiety subscale (p=0.032)-compared with healthy volunteers. Moreover, a statistically significant difference was found between the two groups concerning the UCLA-scale (p=0.033) and RSES-scale (p<0.0001). Female patients presented with lower self-esteem than male patients. CONCLUSION: Psoriasis is a distressing, recurrent disorder that significantly impairs quality of life. Therefore, the recognition and future management of psoriasis may require the involvement of multi-disciplinary teams to manage the physical, psychological and social aspects of the condition, as is the case for systemic, long-term conditions.

Skin diseases in Greek and immigrant children in Athens.

OBJECTIVES: This study aimed to characterize the spectrum of skin diseases affecting children in Greece. METHODS: We retrospectively studied data for 4071 children, aged 0-12 years, who were examined and diagnosed with dermatoses at the outpatient clinic of a university dermatological hospital between December 2005 and August 2007. To evaluate changes in disease patterns, these data were compared with data for a cohort of 12,700 children diagnosed with skin diseases at the same clinic two to three decades earlier (in 1977, 1980, and 1983). RESULTS: The most frequent disease was dermatitis/eczema (34.7%), with atopic dermatitis found in 20.7% of children, contact dermatitis in 6.9%, pityriasis alba in 2.1%, and seborrheic dermatitis in 1.8%. Infections (19.3%), nevi (5.6%), scabies (4.8%), and insect bites (4.3%) followed. More viral (12%) than bacterial (3.7%) and fungal (3.6%) infections were noted. Warts constituted 53.2% of viral infections. Immigrants had an increased risk for bacterial infections and scabies. CONCLUSIONS: Children diagnosed with skin diseases 24-30 years earlier were younger; exhibited lower prevalences of dermatitis/eczema (P = 0.01), viral infections (P < 0.001) and nevi (P < 0.001); higher prevalences of bacterial and fungal infections (P < 0.001) and insect bites (P < 0.01); and similar rates of scabies (P = 0.17). This study documents the high prevalence of atopic dermatitis in the region, the increasing incidence of viral infections and nevi, and the continuing problem of scabies, especially in immigrants.

Condylomata acuminata in children.

We describe a study on 38 children from 1 to 11 years of age, with anogenital warts, 25 were girls. The disease was primarily perianal. Sexual abuse was confirmed in one 9-year-old boy. Most responded to monotherapy with podophyllotoxin, imiquimod 5%, or cryotherapy in a 3-month follow-up period.

Tumors of central and peripheral nervous system associated with inherited genetic syndromes.

There are several genetic syndromes that predispose to the development of tumors of the nervous system. In the present study, we provide a review of the tumors that are associated with neurofibromatosis type 1, neurofibromatosis type 2, tuberous sclerosis complex, von Hippel-Lindau disease, Li-Fraumeni syndrome, Cowden disease, Turcot syndrome, nevoid basal cell carcinoma syndrome (Gorlin syndrome) and rhabdoid predisposition syndrome, which are the most common.

Health related quality of life in patients with anogenital warts.

INTRODUCTION: The health-related quality-of-life (HRQoL) instruments are an important tool for the evaluation of medical outcomes. Sexually transmitted diseases (STDs) influence the patients' life. We aimed to evaluate the HRQoL in patients with anogenital warts at the time of and 1 month after the diagnosis. MATERIALS AND METHODS: We used the short-form (SF)-36 questionnaire to compare the HRQoL of 91 patients with anogenital warts to 53 control subjects with the same socioeconomic characteristics. RESULTS: There was no statistical difference in the overall HRQoL measurement between the anogenital wart patients and controls. However, there was an improvement in the scales of vitality (65.22 ± 15.70 vs. 69.04 ± 14.11, respectively; p < 0.05) and mental health (65.00 ± 20.09 vs. 69.43 ± 18.08, respectively; p < 0.05) in anogenital warts patients between the time of diagnosis and 1 month later. Furthermore, there was a significant deterioration in the scale of social functioning (73.47 ± 22.18 vs. 72.89 ± 19.28, respectively; p < 0.05). The small sample size is a limitation of our study. CONCLUSIONS: HRQoL does not appear to be influenced in anogenital wart patients, as measured by the generic instrument SF-36. It is therefore important to develop specific instruments for the measurement of HRQoL in this group of patients.

The role of cytology and HPV typing as a screening tool in patients with intraanal warts.

BACKGROUND AND AIM: Given that anorectal human papillomavirus (HPV) infection has been related to anal intraepithelial neoplasia (AIN) and rectal cancer, we conducted this study to evaluate the role of cytology of anal smears in the diagnosis of intraanal disease and related AIN and to correlate it to HPV genotypes. METHOD: A total of 72 patients (58 males and 14 females) with perianal warts underwent anoscopy with biopsies and anal cytologic examination. Cytology was carried out for the identification of any dysplasia according to the Bethesda system. All specimens were examined with polymerase chain reaction (PCR) for HPV DNA identification. Exclusion criteria included immunosuppression and high-grade squamous intraepitheliel lesion (HGSIL) or SCC in anal specimens. RESULTS: Seven patients were excluded from the study. Intraanal warts were detected with anoscopy in 57 out of 65 patients, whereas histology showed HPV infection in 56 out of 65 patients and cytology was positive in 52 out of 65 low-grade squamous intraepitheliel lesion (LGSIL) patients. In 43 out of 52 positive patients, simple HPV infection was detected whereas in 9 out of 52 positive patients AIN I. HPV DNA was detected in 51 out of 65 patients, whereas 3 specimens were characterized as invalids. In the majority, HPV 6 could be identified (39/48, 81%), whereas HPV 16 was detected in 4 patients (4/48, 8.3%). One fourth of the positive patients had been infected with more than 1 HPV types (13/48, 27%). Cytology presented a sensitivity 87.5% and specificity 67% in comparison with the histology. CONCLUSIONS: Cytology is highly sensitive in the diagnosis of intraanal warts comparable with histopathology. The combination of the 3 examinations (anoscopy, cytology, and PCR HPV typing) improves diagnostic accuracy and offers a global picture of the anorectal HPV disease.

Effects of botulinum toxin-a therapy for palmar hyperhidrosis in plantar sweat production.

BACKGROUND: Patients with focal hyperhidrosis in multiple areas often report improvement of plantar hyperhidrosis after botulinum toxin A (BTX-A) therapy for palmar hyperhidrosis. OBJECTIVE: To assess sweat production from the soles in patients receiving BTX-A treatment for their palmar hyperhidrosis. PATIENTS AND METHODS: Thirty-six patients with both palmar and plantar hyperhidrosis received 100 U of BTX-A per palm. Sweat production of palms and soles was assessed using a starch iodine test and gravimetry at baseline and 1, 3, and 8 months after treatment. Patients were subjectively assessed using a percentile scale. RESULTS: All patients had significant improvement in their palmar hyperhidrosis that lasted for 6.2 +/- 1.8 months. Gravimetry revealed marginal improvement of plantar hyperhidrosis in 12 patients (from 39.7 +/- 21.3 to 31.5 +/- 18.0 mg/min; p=.01) and statistically significant worsening in 24 patients (from 71.6 +/- 70.60 to 109.94 +/- 82.93 mg/min, p<.001). CONCLUSION: Treatment of palmar hyperhidrosis with BTX-A increased plantar sweating in many patients affected by both palmar and plantar hyperhidrosis in the population under study. Regardless, patients reported satisfaction with the results and were willing to repeat treatment.