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Notes: Epidermolysis bullosa acquisita (EBA) is a rare autoimmune bullous disorder that is often difficult to treat. Few cases have been reported and therapy consists mainly of combinations of systemic steroids, immunosuppressants and, recently, administration of intravenous human immunoglobulin (IVIg). We describe a case of EBA in which our therapeutic choices were limited due to the patient’s poor general condition, including extensive infection of the lesions and a history of pulmonary tuberculosis. The patient was treated with IVIg at a dose of 400 mg/kg per day for 5 consecutive days every 4 weeks. The treatment was well tolerated and the results were satisfactory. It seems that IVIg, due to its possible immunomodulatory mode of action, can be an efficacious therapeutic agent in this rare autoimmune disease.

Notes: Cutaneous larva migrans (CLM) is a characteristic eruption that is caused by penetration and migration in the skin of nematode larvae. Both animal and human hookworms have been implicated in the pathogenesis of the condition, with Ancylostoma braziliensis and A. caninum being the most common among them. CLM is quite common in tropical areas, but, due to travelling, several cases have also been described in Europe. We present a patient with CLM who presumably contacted the disease during a holiday in Singapore. The patient was treated successfully with oral thiabendazole.

Notes: Background  Although a common dermatosis, idiopathic poikiloderma of the face and neck has not been studied in depth for decades.Objectives  To reassess the clinical and epidemiological characteristics of poikiloderma of Civatte (PC).Material and Methods  Fifty consecutive patients with PC. Evaluation included history taking and physical examination. Epidemiological and clinical parameters were recorded and analysed. The literature from 1923 until today, was reviewed thoroughly.Results  The frequency of PC among dermatologic patients was estimated to be 1.4%. There were 34 females (68%) and 16 males in the present study. The mean age at diagnosis was 47.8 years for females and 61.7 years for males. The majority (88%) had skin phototype II or III. Among females, 26 were at their peri-menopausal stage, including three cases of iatrogenic menopause. Four patients reported that other blood-related family members also had PC. The v and the sides of the neck and the upper chest were most often affected in a symmetric distribution. The face (preauricular and parotid region) was involved in 19 patients (38%). The erythemato-telangiectatic clinical type predominated (58%), followed by the mixed (22%) and the pigmented type (20%). Almost half of the patients (46%) were symptomatic (itching, burning and ‘flushing’). The mean duration from onset to diagnosis was 6.2 years according to the patients’ report. The course was usually slowly progressive (82%) and irreversible.Conclusions  PC shows characteristic features, supporting the theory that it represents a distinct entity. It is rather common in Greece. Although menopausal women predominated in our cohort, men were not uncommonly affected and were diagnosed at an older age. Based on the predominating clinical feature, PC can be classified into three clinical forms. Symmetry and sparing of the anatomically shaded areas of the neck are highly characteristic for PC. Face involvement was not as common and as severe as it had been considered in the past. Recognition of clinical type is important for the selection of the most appropriate treatment, which, despite the advent of novel modalities, remains problematic.

Notes: Polymorphous light eruption (PLE) is a common idiopathic photosensitivity disorder with an estimated prevalence of 10–20%. It is characterized by an intermittent skin reaction to ultraviolet (UV) radiation exposure, consisting of non-scarring pruritic erythematous papules, vesicles or plaques that develop on light-exposed skin. Despite the different morphology in different individuals, the eruption tends to have a monomorphous presentation in any single subject. The histopathological features of PLE are distinct and comprise a perivascular lymphocytic infiltrate in the dermis, subepidermal oedema and variable epidermal changes. The pathogenesis of PLE is not well known, but findings suggest that it is a delayed-type hypersensitivity reaction to one or more UV-modified cutaneous antigens. The principal action of PLE is mainly in the UVA region, although some subjects exhibit sensitivity to UVB alone or to both UVA and UVB radiation at the same time. Preventive measures in PLE include the regular use of photoprotective methods combined with graduated exposures to natural sunlight. The induction of immune tolerance by phototherapy and photochemotherapy are useful prophylactic methods in moderate to severe cases. The role of systemic agents in the management of PLE is under investigation. This article reviews the epidemiological, pathogenetic and clinical aspects of PLE and discusses recent advances in the diagnostic approach and management of this condition.

Notes: 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.