Search

Bullous dermolysis of the newborn is a dominant or recessive inherited subtype of dystrophic epidermolysis bullosa characterized by the tendency to spontaneously stop blistering within the first months of life. Here we report two siblings with bullous dermolysis of the newborn who were born prematurely and have a novel recessive mutation, p.Pro2259Leu, in the triple helix domain of type VII collagen. We discuss the possible relationship between genotype and prematurity and clinical manifestations in these patients.

Background: Some results from laboratory and epidemiologic studies suggest that diet may influence the risk of melanoma, but convincing evidence for a role of single nutrients or food items is lacking. Diet quality, which considers the combined effect of multiple food items, may be superior for examining this relation. Objective: We sought to assess whether diet quality, evaluated with the use of 4 different dietary indexes, is associated with melanoma risk. Methods: In this population-based case-control study, we analyzed the relation between 4 diet quality indexes, the Healthy Eating Index 2010 (HEI-2010), Dietary Approaches to Stop Hypertension (DASH) index, Greek Mediterranean Index (GMI), and Italian Mediterranean Index (IMI), and melanomarisk in a northern Italian community, with the use of data from 380 cases and 719 matched controls who completed a semiquantitative food frequency questionnaire. Results: In the overall sample, we found an inverse association between disease risk and the HEI-2010 and DASH index, but not the Mediterranean indexes, adjusting for potential confounders (skin phototype, body mass index, energy intake, sunburn history, skin sun reaction, and education). However, in sex stratified analyses, the association appeared only in women (P-trend: 0.10 and 0.04 for the HEI-2010 and DASH index, respectively). The inverse relations were stronger in women younger than age 50 y than in older women, for whom the GMI and IMI scores also showed an inverse association with disease risk (P-trend: 0.05 and 0.02, respectively). Conclusions: These results suggest that diet quality may play a role in cutaneous melanoma etiology among women. JN utr2015;145:1800‐7.

Milia en plaque (MEP) is an uncommon finding characterized by numerous tiny milia within an erythematous area. Despite its benign and asymptomatic nature, MEP raises cosmetic concerns; moreover, the available treatment modalities for MEP are limited. In view of the few cases described in the literature, no consensus has been reached, with respect to the optimal treatment for MEP, and the choice of therapy should be individualized. We report a case of eyelid milia en plaque successfully treated with a new CO2 fractional laser that is able to ensure superficial ablation of the epidermis remodeling tissue in-depth, with minimal thermal damage and extremely rapid recovery time. The results obtained after only two treatments were good, no scarring or dyschromic changes have been registered. At 1 year, just few recurrent milia were present.

Cutaneous melanoma incidence has been increasing during the last few years, and diet has been suggested as one of the lifestyle factors responsible for this increase. Since antioxidant nutrients such as ascorbic acid might prevent skin carcinogenesis, we investigated the risk of cutaneous melanoma related to vitamin C intake in a population-based case-control study in Northern Italy based on 380 melanoma patients and 719 matched controls, to whom we administered a semiquantitative food-frequency questionnaire. After adjusting for potential confounders, odds ratio of melanoma were 0.86 (95 % confidence interval 0.65 - 1.15) and 0.59 (95 % confidence interval 0.37 - 0.94) in the intermediate and highest categories of vitamin C dietary intake respectively, compared with the bottom one. The association between vitamin C and decreased risk persisted after adjustment for some potential confounders. In age- and gender-stratified analyses, this association was seen in young females (< 60 years old), and was found to be enhanced in subjects with phototypes II and III. These results suggest a possible protective activity of vitamin C intake against cutaneous melanoma in specific subgroups of this population of Northern Italy.

Health status, health services utilization and mortality differ by marital status for both sexes in most conditions, but little is known about dermatological diseases. We evaluated whether marital status is associated with the impact that dermatological diseases have on quality of life (QoL). Data from two surveys on dermatological outpatients were pooled. Marital status, sex, age, and educational level were analysed in relation to QoL (using the scales of the Skindex-29 questionnaire: emotions, symptoms, and functioning) and psychological well-being (using the GHQ-12 questionnaire). Data on 5,471 patients (59% females, 46% married) were obtained. Married patients in univariate analysis had lower mean values on the emotions scale and higher mean values in the symptoms scale of the Skindex-29 compared to singles. Statistically significant differences were identified only in men, for the emotions scale and for the GHQ-12. Females had significantly higher mean scores than males on each of the Skindex-29 scales and on the GHQ-12. Married patients had a lower disease impact on the emotions scale even if they suffered a higher impact on the symptoms scale. However, after multiple adjustment, gender seems to be more relevant than marital status in the evaluation of health status.

Background In dental diseases, significant discrepancies were observed in the oral health-related quality of life evaluation between patients and providers. Few studies have been performed specifically on the impact of oral mucosal diseases on patients' health. Objective We sought to compare the evaluation of the severity of oral mucosal conditions in providers and patients. Methods Patients with an oral mucosal condition were recruited at the oral health care unit of a dermatologic hospital. Severity was evaluated both by the physician and by the patient, using a global severity assessment score on a 5-point scale. The 14-item Oral Health Impact Profile was used to evaluate oral health-related quality of life, the 12-item General Health Questionnaire for psychologic problems, and the 20-item Toronto Alexithymia Scale for alexithymia (ie, the difficulty in identifying and expressing feelings). Results Data were complete for 206 patients. The agreement between patients' and providers' evaluation was very low (Cohen κ = 0.18). Severity was particularly underestimated by the physician in patients with alexithymia (43% compared with 25% of patients with no alexithymia) and with psychologic problems (44% vs 25%). Limitations Because of the high number of different conditions, and thus the small figures in each group, it was not possible to analyze the concordance between patient and provider in each single condition. Conclusion Even in the severity assessment of his or her own disease, it is plausible that a patient does not provide a simple clinical evaluation, but includes subjective aspects. It is important for the physician to take into account the severity the patient perceives in making treatment decisions, and in evaluating clinical improvement.

Background: Among the pigmented lesions with a central area of scar, we found a group of cases histologically characterized by striking architectural alteration of the melanocytic component, but with no cytological atypia and mitotically quiescent. The aim of the current study was to assess the biological nature of such lesions. Methods: We selected 19 of these melanocytic neoplasms that had the following characteristics: (a) a clinically evident whitish central area suggestive of regression (with no history of a previous surgical procedure or trauma), (b) histological features of fibrous scar-like tissue at the center of the lesion, (c) the presence of large, confluent and unusually shaped melanocytic nests at the dermoepidermal junction and in the dermis, (d) a pagetoid spread of melanocytes above the epidermal basal layer and (e) remnants of nevus tissue at the border of the scar. The lesions showed no evidence of cytological atypia, expansive nodules of melanocytes, significant numbers of mitoses or cellular necrosis. Results: All the cases have been followed up and none have recurred or metastasized. Histologically, these neoplasms have important similarities with the so-called recurrent nevus, nevi on lichen sclerosus and nevi developed during or following cutaneous inflammatory and sclerosing processes. The origin of the scar in each case was obscure but was probably related to minor unnoticed trauma or to chronic friction on a nevus. In few cases, the fibrosis was probably the result of partial regression of the nevus or a sequel to folliculitis. The pseudomelanomatous features appear to be related to the presence of the scar, as already reported for nevi that are involved in fibrotic or scarring processes. In our study, the nevi involved in the fibrotic process were congenital nevi and common or dysplastic nevi. One case was a Spitz nevus. Conclusions: From our data we concluded that, despite their worrisome clinical and histological aspect, the lesions described in this case series were most probably benign melanocytic nevi, involved by a fibrotic process combined with pseudomelanomatous proliferation. The lack of cytological atypia, mitoses and expansive nodules allowed us to differentiate these lesions from regressing melanomas.

Atypical fibroxanthoma (AFX) is a spindle cell neoplasm with low metastatic potential but high tendency to recur after surgery. Because of the rarity of this lesion and its aspecific clinical features, AFX could be easily misdiagnosed and undertreated by many clinicians who encounter them. Dermoscopy represents a valuable tool for easily assessing skin lesions, even though histological examination is required for final diagnosis. We report a case of a cheek lesion with dermoscopic “blue amber pattern”, easily recognisable and not observed in others skin tumours, which could represent an additional feature useful in differentiating this tumour from other skin neoplasms.

La dermatite atopica (DA) e una malattia infiammatoria della cute geneticamente determinata, caratterizzata essenzialmente da una piu o meno intensa flogosi cutanea ad andamento cronico-recidivante, in soggetti geneticamente predisposti e affetti da atopia.

ejd.2013.2129 Auteur(s) : Calogero Pagliarello calogero.pagliarello@libero.it, Carla Antonella Pepe, Mara Lombardi, Claudio Feliciani, Giuseppe Fabrizi, Sergio Di Nuzzo Section of Dermatology, Department of Surgical Sciences, Parma Medical School, Via Gramsci 14, 43100 Parma, Italy Although Sweet's syndrome (SS) rarely occurs during pregnancy, the relationship with miscarriage is under-reported. We report a case of SS in a pregnant patient who had an early miscarriage. Dermatologists should be aware [...]

Current therapy for vitiligo is largely unsatisfactory; hence there is a need for new treatment combinations to be assessed. Whereas stable vitiligo can benefit from surgical therapies, such as suction-blister epidermal grafting (SBEG) (1), the non-segmental counterpart usually shows a poor response to this treatment (2). To overcome this issue, for high-risk patients affected by both focal and generalized vitiligo, a combined ap-proach of epidermal graft and systemic corticosteroids has been advocated in order to reduce the risk of graft depigmentation (3, 4).We report here the case of a patient affected by ge-neralized vitiligo who was treated successfully with SBEG after a prolonged course of topical 0.1% tacro-limus treatment (Protopic, Astellas Pharma US, Inc.). It is noteworthy that maintaining topical tacrolimus treatment allowed and preserved graft pigmentation for more than one year, whereas, after its discontinuation, graft depigmentation began to develop.CASE REPORTA 62-year-old Caucasian woman presented with well-defined white patches on her face (Fig. 1a), hands and elbows of 2 years duration. She had first noted the skin depigmentation as a side-effect during interferon α-2a treatment for chronic active hepatitis C. This treatment was successful, since viral eradication was reached, but the skin lesions persisted after discontinuation of interferon. She had not had diabetes or any autoim-mune disease such as thyroiditis, as documented by extensive laboratory work-up. She was diagnosed with generalized vitiligo and she commenced applying 0.1% tacrolimus ointment to the facial lesions at bedtime, combined with narrow-band ultravio-let B (NB-UVB) phototherapy twice a week. Topical tacrolimus used in conjunction with ultraviolet (UV) light is effective in increasing the extent of overall repigmentation and in reducing the cumulative NB-UVB dose needed to achieve a therapeutic benefit (5, 6). After one-year treatment she achieved an acceptable level of repigmentation (Fig. 1b) with no further improvement, although treatment continued for an-other year. This finding was consistent with the reported time to reach signi-ficant pigmentation during tacrolimus treatment (7).We proposed a trial with SBEG to co-ver her left forehead, after we had fully informed her about the high chance of graft depigmentation reported for generalized vitiligo. After the grafting procedure, the patient followed the same therapeutic regimen, based on topical tacrolimus at bedtime plus NB-UVB phototherapy twice a week. After 3 months the grafts reached a good pigmentation, so phototherapy treat-ment was stopped (Fig. 1c). We recom-mended the patient to apply tacrolimus treatment each night to help centrifugal pigmentation to spread from the grafts.

Report of a Case A woman in her 30s presented with hidradenitis suppurativa (HS), Hurley stage II. She was treated with oral contraceptives (drospirenone/ethinyl estradiol), spironolactone (50 mg/d), topical clindamycin, andmonthly intralesional corticosteroids (triamcinolone acetonide, 10 mg/mL). Previously, she had received rifampicin (600 mg/d), first with minocycline (100 mg/d), then with clindamycin (300 mg/d), and finally with moxifloxacin (400mg/d). After these treatments failed, she received isotretinoin (0.5 mg/kg/d) for 2 more years to achieve remission, but without success. Because shehaddecided tobecomepregnant, shewas searching for an alternative treatment, devoid of teratogenic effects, to safely replace oral contraceptives. Effective therapy was paramount because theHS seriously interferedwith sexual intercourse and indirectly with her planned pregnancy. Surgical treatmentwas offered (local incision anddrainage, deroofing, limited local orwide excision), which she declined.

Calogero Pagliarello, MD, PhD; Andrea Paradisi, MD, PhD; Caterina Dianzani, MD, PhD; Mauro Paradisi, MD; Paolo Persichetti, MD, PhD; Health Services Research Unit, IDI-IRCCS (Dr Pagliarello), Dermatology Department, Catholic University of Sacred Heart (Dr A. Paradisi), and Dermatology (Drs Dianzani and M. Paradisi) and Plastic and Reconstructive Surgery (Dr Persichetti) Units, Campus Bio-Medico Medical School, Rome, Italy