Patients with Spitz naevi in the Greek population: Epidemiologic, Clinical and Histopathological characteristics.

BACKGROUND: Spitz naevi may present with clinical and histopathological atypical features that do not affect patient prognosis but may become worrisome for patients ≥40 years presenting with newly appearing SN. OBJECTIVE: Patient characteristics and sun behaviour patterns were investigated in correlation with age. SN characteristics and histopathological attributes were also investigated in correlation with age. METHODS: Patients with histopathologically confirmed diagnosis of SN were invited for a clinical examination. Data such as skin type, number of banal/atypical naevi, sun exposure patterns and personal/family history were collected. Histopathology preparations were re-examined by two different histopathologists, and characteristics were collected based on a prespecified checklist. Patients were afterwards followed up every 6 months. RESULTS: A total of 110 patients with SN were identified and assigned to three age groups. The most common area of presentation was the trunk, for the ≥40 years age group, and the limbs for the other age groups. Patients ≥40 years had a higher possibility of presenting with a naevus count ≥50 and at least one atypical naevus compared to the other age groups. Patients ≥40 years presented more commonly with a history of painful sunburn (100%) before the appearance of the SN, used less sunscreen, had higher sun exposure times and more clinical signs of solar skin damage compared to the other age groups. Finally, patients ≥40 years presented more commonly with signs of histopathological atypia such as the presence of mitoses, cellular atypia and prominent nucleolus. CONCLUSION: Patients ≥40 were more likely to report a history of longer sun exposure times, of never using a sunscreen and of having a history of painful sunburn. However, the importance of this observation remains to be elucidated as these patients also presented more commonly with lesions located on non-sun-exposed areas (trunk) and higher naevus/atypical naevus counts.

Psoriasis in patients with mycosis fungoides: a clinicopathological study of 25 patients.

BACKGROUND: It has been reported that patients with psoriasis are at increased risk for developing lymphoma including cutaneous T-cell lymphomas (CTCL). However, the comorbidity and the histopathologic correlation of psoriasis and mycosis fungoides (MF) have been less studied. OBJECTIVE: The objective of this study was to investigate the relation between MF and psoriasis. METHODS: We retrospectively reviewed and re-evaluated all MF cases diagnosed and followed in a 16-year period who carried both MF and psoriasis diagnoses. RESULTS: Forty-one of 321 MF patients was the rate of psoriasis' comorbidity according to medical records. Twenty-five patients (7.8%) finally met the inclusion criteria. The rest were excluded due to inadequate evidence. Twenty patients had psoriatic lesions at the time of MF diagnosis. In 23 patients, there was histological confirmation of both diseases. Six patients (24%) were diagnosed with folliculotropic MF, two were diagnosed with pustular psoriasis, and six patients were affected by palmoplantar and nail psoriasis. In four patients, there was a very short time interval between MF and psoriasis diagnosis. Fourteen patients with psoriasis had been previously treated with immunomodulatory regimens. Interestingly, in eight patients, typical histological findings of both diseases were detected in the same biopsy specimen. CONCLUSION: Our results support the opinion that the association between psoriasis and MF does exist. It is most possibly related to the chronic lymphocyte stimulation that occurs during psoriasis that eventually leads to a dominant clone and the evolution to CTCL. Our study suggests that apart from cases of early MF, which are being indeed misdiagnosed as psoriasis, there is another group of patients, where psoriasis truly coexists with - or even progresses to - MF.

CHILD syndrome: the NSDHL gene and its role in CHILD syndrome, a rare hereditary disorder.

BACKGROUND: CHILD syndrome, a rare hereditary disorder of keratinization (MIM 308050, 300275), is the acronym proposed by Happle to name a rare entity, characterized by congenital hemidysplasia, icthyosiform nevus and limb defects, ranging from digital hypoplasia to icthyosiform nevus and ipsilateral limb defects, ranging from digital hypoplasia to complete amelia. PATIENTS AND METHODS: A 9-month-old female infant presented with skin and limb defects involving the right side of her body. Clinical and laboratory evaluation was performed, including DNA sequence analysis of the NSDHL gene. RESULTS: Our patient presented with some of the typical clinical characteristics of CHILD syndrome, i.e. two large erythematous plaques with sharp borders, covered with yellow, wax-like scaling, on the right axilla and on the right groin, dysplastic right hand and alopecia of the right occipital area. The diagnosis was confirmed by DNA screening analysis, that detected a missense mutation c.314C-->T;p-A105V, in the coding region of the NSDHL gene (exon4) of our patient. CONCLUSIONS: This is the first report of CHILD syndrome ever reported in Greece. We suggest that the diagnosis of the syndrome is important for patient information and genetic counselling.

Successful treatment of localized pemphigus foliaceus with topical pimecrolimus.

We report the case of successful treatment of a 79-year-old male patient with recurrent pemphigus foliaceus with pimecrolimus cream 1% once daily for 40 days. The patient initially presented with localized lesions on the scalp and nose area and was treated with systemic corticosteroids. At his fourth relapse within a period of 16 months, he refused any systemic treatment. Pimecrolimus cream was suggested to him as an alternative option.

Immunophenotypic analysis of the p53 gene in non-melanoma skin cancer and correlation with apoptosis and cell proliferation.

BACKGROUND: Sunlight precipitates a series of genetic events that lead to the development of skin cancers such as basal cell carcinoma (BCC) and squamous cell carcinoma (SCC). The p53 tumour suppressor gene, which plays a pivotal role in cell division and apoptosis, is frequently found mutated in sunlight-induced skin tumours. OBJECTIVE: To investigate the immunoreactivity of the p53 gene in non-melanoma skin cancers and to correlate its expression with apoptotic and cell proliferation markers. METHODS: We analysed 35 non-melanoma tumours including 19 BCCs and 16 SCCs from sun-exposed skin areas. p53 protein expression was studied immunohistochemically using the DO7 monoclonal antibody against wild-type and mutant p53 forms. The percentage of p53-immunopositive nuclei was measured by image analysis. Cell proliferation and apoptosis were also assessed by image analysis following Ki-67 immunostaining and application of the TUNEL method on paraffin sections, respectively. RESULTS: The percentage of p53-expressing cells varied from 3.5 to 90 in BCCs (median value 54.4%) and from 3.7 to 94 in SCCs (median value 40.3%). The mean value of Ki-67-positive cells was comparable in both groups of tumours with a mean value of 40.6% in BCCs and 34.6% in SCCs. Conversely, the TUNEL assay showed sporadic staining of apoptotic cells within the tumours with a mean value of 1.12% in BCCs and 1.8% in SCCs. p53 protein expression was correlated positively with cell proliferation (r = 0.75, P = 0.000001) and negatively with apoptosis (r = -0.23, P = 0.05). CONCLUSION: p53 immunoreactivity was high in the majority of the skin carcinomas examined and correlated positively with cell proliferation and negatively with apoptosis. The p53 protein overexpression appears to be related to an inactivated protein resulting from mutations of the p53 gene or other unclear molecular mechanisms.

Impetigo herpetiformis during the puerperium.

We report on a 29-year-old primigravida who developed impetigo herpetiformis 1 day after delivery. To our knowledge, this patient is the second reported case of impetigo herpetiformis presenting during the puerperium. The patient responded quickly to systemic administration of methotrexate and prednisolone.

Classic Kaposi's sarcoma in Greece: a clinico-epidemiological profile.

BACKGROUND: Classic Kaposi's sarcoma (CKS) is not uncommon in Greece with a reported incidence of 0.20 per 100,000 per year. METHODS: Epidemiological, clinical and histological features of all CKS cases, diagnosed in 'A. Sygros' hospital, Athens, Greece during the years 1989-1994, have been recorded and studied prospectively. RESULTS: During the five-year period studied, 66 CKS patients have been diagnosed in our hospital. Incidence among dermatologic patients was 2.11 per 10,000 patients examined, representing 1.35% of total skin malignancies. Patients' age at diagnosis ranged from 53 to 94 years (mean 72 +/- 8.8). The male to female ratio was 2.47:1. A high proportion of the patients were born in Peloponnesos (42.42%) and were residing in Athens (51.51%) or in Peloponnesos (24.24%). Nodules and/or plaques were the most frequent type of lesion, most commonly located on the feet (43.93%) or the hands (28.78%). Accompanying edema was seen in 51.51% of the patients. There were 16 stage I patients (24.24%), 40 stage II (60.60%), 0 stage III and 10 stage IV (15.15%). Involvement of visceral organs was detected in seven patients (10.60%), while 10 had lymph node involvement (15.15%) and three, involvement of the underlying bones (4.54%). Second primary malignancy was diagnosed in 6 cases (9.09%), most often of the reticuloendothelial system (83.33%). CONCLUSIONS: CKS in Greece exhibits some special characteristics, including older age of onset; lower male to female ratio; endemic clustering; disseminated skin disease at diagnosis, often accompanied by lymphedema; not unusual visceral or lymph node involvement and association with second malignancies. We suggest that CKS in Greece possibly represents a distinct endemic subtype of CKS.

A randomized trial of amorolfine 5% solution nail lacquer in association with itraconazole pulse therapy compared with itraconazole alone in the treatment of Candida fingernail onychomycosis.

BACKGROUND: Treatment failures and relapses are not uncommon in onychomycosis. Therefore, it is worthwhile to consider the combination of systemic and topical antifungals to improve the cure rates further and to reduce the duration of systemic treatment. OBJECTIVES: To evaluate and compare itraconazole pulse therapy combined with amorolfine with itraconazole alone in the treatment of Candida fingernail onychomycosis. METHODS: Ninety patients with moderate to severe Candida fingernail onychomycosis were randomized into two treatment groups of 45 subjects each. Group 1 received itraconazole pulse therapy for 2 months and applied amorolfine 5% solution nail lacquer for 6 months, while group 2 received monotherapy with three pulses of itraconazole. The primary efficacy criterion was the result of mycological examination at 3 months. The secondary criterion was the combined mycological and clinical response at 9 months. A pharmacoeconomic analysis was also performed to compare the cost-effectiveness of combined therapy vs. monotherapy. RESULTS: Eighty-five patients were analysed (73 women and 12 men, mean +/- SD age 44.2 +/- 12.9 years). Patients had a mean +/- SD of 3.64 +/- 2.0 nails involved and 228.6 +/- 148.0 mm2 of their nail surface diseased. The mean duration of onychomycosis was 11 months. Paronychial involvement was evident in 71 patients. C. albicans was isolated in 85 cases, C. parapsilosis in three and other Candida species in two cases. Side-effects were uncommon and in only one case led to withdrawal. At the 3-month visit, mycological cure was seen in 32 (74%) of 43 patients in group 1 and in 25 (60%) of 42 patients in group 2. At the 9-month visit, a global cure was seen in 40 patients (93%) in group 1 and in 34 patients (81%) in group 2. Statistical analysis showed no statistically significant difference (P > 0.1) between the two treatment groups. The cost per cure ratio was 1.63 and 1.70euro for groups 1 and 2, respectively. CONCLUSIONS: The combination of amorolfine and oral itraconazole, which interfere with different steps of ergosterol synthesis, exhibited substantial synergy. Compared with oral itraconazole alone, the combination achieved greater mycological cure and increased total cure rate. However, no statistically significant difference was documented for this number of observations. Combination treatment with amorolfine and two pulses of itraconazole is at least as safe and effective as three pulses of itraconazole, with a lower cost per patient. In our opinion, the addition of amorolfine to oral itraconazole pulse therapy is of value in the treatment of moderate to severe Candida fingernail onychomycosis.