Mehmet Akif Ciftcioglu

A 13-year-old girl with recessive dystrophic epidermolysis bullosa presenting with squamous cell carcinoma.

Abstract:  Recessive dystrophic epidermolysis bullosa (RDEB) is an uncommon and severely disabling genetic disorder characterized by trauma‐induced blisters, intractable skin ulcers, scarring, milia, and nail dystrophy. Patients with RDEB have an increased tendency for fast‐growing and early metastasizing squamous cell carcinoma (SCC). We report here a 13‐year‐old girl with RDEB who developed a large SCC on the left knee. At 6 months of evolution it was resected and covered with an autologous skin graft. To our knowledge, this is the youngest patient with RDEB complicated by SCC to be reported, and therefore may serve to emphasize the importance of vigilance in surveying RDEB patients for SCC.

Androgen Receptor Levels of Oral and Genital Ulcers and Skin Pathergy Test in Patients with Behçet’s Disease

Background: Hormonal factors have long been proposed to play a role in Behçet’s disease (BD). Male sex, systemic onset, HLA-B51 positivity and a younger age of onset in BD are associated with severer disease, and the disease generally runs a milder course in women. Vascular involvement is more common, and the skin pathergy test (SPT) is more strongly positive in men. BD rarely develops before puberty or after the age of 50 years. Clinical manifestations of the disease, with the exception of eye symptoms, tend to improve with time. Therefore, BD may be androgen driven to some degree. Objectives: We aimed to investigate androgen receptor (AR) levels of oral ulcers (OU), genital ulcers (GU) and SPT areas and compared them with those of adjacent normal-appearing skin/mucosa from patients with BD. Methods: Thirty-eight patients with BD (16 female, 22 male; mean ± SD age, 36.45 ± 10.2 years), diagnosed according to the criteria of the International Study Group for Behçet’s Disease, were included in the study with blind histological examination. Biopsies from OU of 10 patients, GU of 11 patients, SPT areas of 17 patients and adjacent (approximately 2 cm distant) normal-appearing skin/mucosa in patients with BD were performed. Nuclear AR levels were studied by an immunohistochemical technique, using monoclonal antibodies. The percentage of positively staining cells was recorded as the AR index (ARI). In addition, the prevalence and the positivity rate of SPT has also been evaluated. Results: ARI values in the lesional and control (non-lesional adjacent) skin/mucosa were found to be 14.5 versus 18% for OU, 28.7 versus 25.5% for GU and 36.3 versus 21.8% (p = 0.068) for SPT areas. The positive SPT areas in male patients showed a higher ARI than those of female patients (43.36 and 23.33%; p = 0.078). The ARI values of SPT areas in male patients but not in female patients were found to be significantly higher as compared with non-lesional skin (21.63%; p = 0.039). The SPT positivity was also more common in male patients compared with female patients (86.4% and 62.5%), although the difference was not significant (p = 0.88). SPT have been found to be more strongly positive among the males (4.63 ± 3.3) compared with female patients (3.18 ± 1.9), and the difference was statistically significant (p = 0.022). Conclusions: Our findings indicate that androgens seem to play a role both in the formation and increased positivity of the SPT areas in male patients with BD.

Prognostic value of Ki-67, CD31 and epidermal growth factor receptor expression in basal cell carcinoma.

Recurrence of basal cell carcinoma following treatment is common, and the majority of recurrences appear in the first 3 years. We examined the original tumors of 26 basal cell carcinoma cases, 14 of whom had a recurrence after an average of 3.7 years, and 12 of whom had no recurrence during an average of 4.4 years follow‐up. Using immunohistochemistry, we tested for Ki‐67, CD31 and epidermal growth factor receptor expressions in the tumor tissue. The percentages of expression for Ki‐67, CD31 and epidermal growth factor receptor were significantly higher in the recurrent tumors than in the non‐recurrent ones. Expression of Ki‐67 and CD31 was 271.57 ± 17.91 and 58.1 ± 9.37 for the recurrent group and 187.08 ± 21.48 and 23.9 ± 5.45 for non‐recurrent group respectively (p<0.0001; p<0.0001). Expression of epidermal growth factor receptor was positive in all basal cell carcinoma cells. The staining intensity was strong in 57% of recurrent and 8.3% of non‐recurrent tumors (p=0.014). These results show that Ki‐67, CD31 and epidermal growth factor receptor expression differ between basal cell carcinomas which later recur and those that do not recur.

Treatment of papuloerythroderma of Ofuji with Re‐PUVA: a case report and review of the therapy

Papuloerythroderma of Ofuji (PEO) is a disease of elderly men, characterized by intensely pruritic and widespread, red, flat‐topped papules with sparing of the body folds and creases (the so‐called ‘deck‐chair’ sign). The etiopathogenesis of the disease remains unknown. Psoralen plus UVA (PUVA), topical and systemic corticosteroids, etretinate, cyclosporin and interferon are the main approaches in the treatment of this rare disease. A case of PEO in a 60‐year‐old man who responded to retinoid plus PUVA (Re‐PUVA) treatment is reported here and a review of the therapy with other relevant cases is presented.

The divergent roles of growth differentiation factor-15 (GDF-15) in benign and malignant skin pathologies

GDF-15 (Growth Differentiation Factor-15) is a member of the transforming growth factor β (TGF-β) superfamily. GDF-15 is not only involved in cancer development, progression, angiogenesis and metastasis, but also controls stress responses, bone formation, hematopoietic development, adipose tissue function and cardiovascular diseases. GDF-15, which is regulated by p53, has shown antitumorigenic and proapoptotic activities in vivo and in vitro. Also, GDF-15 is involved in skin biology and histamine-induced melanogenesis; it is overexpressed in melanoma cells and is associated with depth of tumor invasion and metastasis. GDF-15 level is increased in patients with systemic sclerosis and is related with the degree of skin sclerosis and intensity of pulmonary fibrosis. In the future, GDF-15 may be a potential target for therapy in benign disorders with skin fibrosis and malignant lesions of the skin.

Recurrent bullous lesions associated with familial Mediterranean fever: a case report.

Familial Mediterranean fever (FMF) is an inherited, recurrent, inflammatory disease. Of its various cutaneous features, erysipelas‐like erythema is the best known and most common skin lesion. We present a new case of FMF with recurrent bullous lesions. A 41‐year‐old woman was admitted to our clinic with tense bullae, 20 × 20 mm in diameter on the left shin. The patient had a history of fever, abdominal pain, peritonitis attacks and infertility. A lesional skin biopsy revealed subepidermal bullae and neutrophilic infiltration around dermal vessels. Direct immunofluorescence analysis was negative. Over the period of investigation, the lesion regressed spontaneously; 1 month later, a similar lesion appeared on the right wrist. Diagnosis of FMF was made according to the Tel‐Hashomer criteria. Recognition of this peculiar skin lesion may lead to an earlier diagnosis of the disease.

Presence and subcellular localizations of surfactant proteins A and D in human spermatozoa

OBJECTIVE To investigate the presence of surfactant protein-A (SP-A); molecular weight 34 kDa and surfactant protein-D (SP-D); and molecular weight 43 kDa in human spermatozoa. DESIGN Prospective, research study. SETTING Two universities in Turkey. PATIENT(S) Semen specimens (n = 10) were obtained from normozoospermic donors. INTERVENTION(S) Human sperm were exposed to an anti-human SP-A polyclonal antibody, and monoclonal antibody, to human SP-D protein. MAIN OUTCOME MEASURE(S) Presence of SP-A and SP-D proteins in human beings. RESULT(S) Indirect immunofluorescence assays of human sperm indicated the presence of SP-A in the mid piece, the tail, and sometimes at the equatorial region of spermatozoa. A brilliant green light detected SP-D in the tails and acrosome of some sperm. The anti-SP-A antibody detected a single band corresponding to the molecular weight values of 34 kDa in spermatozoa, whereas no band was observed in the negative control. The anti-SP-D antibody showed the expected band at 43 kDa in spermatozoa. CONCLUSION(S) This is the first report and a novel finding of the presence of surfactant glycoproteins on human spermatozoa.

The Effect of Erythropoietin on Anastomotic Healing of Irradiated Rats

ABSTRACT Aim: The aim of the present study is to evaluate the possible protective effects of erythropoietin (EPO) on anastomotic wound healing after preoperative radiotherapy according to its pleiotropic mechanism of action. Methods: Thirty-two male Wistar albino rats were randomized into four groups containing eight rats each: ANAS group, standard resection plus anastomosis; RT+ANAS group, radiation plus standard resection plus anastomosis; ANAS+EPO group, standard resection plus anastomosis plus EPO; RT+ANAS+EPO, radiation plus standard resection plus anastomosis plus EPO. All animals were sacrificed by cardiac puncture, and anastomotic healing was measured by bursting pressure, hydroxyproline (OHP) levels, myeloperoxidase (MPO) activity and histopathological evaluations. Malondialdehyde (MDA), tumor necrosis factor-alpha (TNF-α), and matrix metalloproteinase-9 (MMP-9) were also measured in serum specimens. Results: OHP levels in the RT+ANAS + EPO group were significantly increased compared with other groups (p < .05). In contrast, MPO activity in the RT+ANAS+EPO group was significantly decreased compared with other groups (p < .05). Serum MDA levels were found to be decreased in the ANAS+EPO and RT+ANAS+EPO groups (p < .05). Group comparisons demonstrated that bursting pressure was significantly higher in EPO treated rats (p < .05). The histopathology results revealed that EPO treatment improves anastomotic wound healing though decreased necrosis and inflammatory cell infiltration and increased fibroblast activity. Conclusion: The findings of the present study indicate that EPO contributes to wound healing and the strength of colon anastomosis following radiation due to its antioxidant and anti-inflammatory effects, but further studies are needed to explore the significance of these effects.

Evidence of surfactant protein A and D expression decrement and their localizations in human prostate adenocarcinomas

Abstract Aim: Surfactant proteins (SP-A and SP-D) were originally described in the lung; however, they are also present in the prostate. Purpose of this study, therefore, was to determine how surfactant proteins are altered in prostate adenocarcinomas (PCa) and find out any connection exists between their expressions and their staining patterns, prostate-specific antigen (PSA) values, Gleason score, age, tumor volume and tumor, node, metastases (TNM) clinical stage. Methods: Thirty-five tissue samples were obtained during radical prostatectomy. All specimens were classified to three groups based on the Gleason score <7, 7 and Gleason score >7. Surfactant proteins’ expressions were tested by immunohistochemical and Western blotting methods. Results: Immunoreactivity was detected in the cytoplasm from both basal cells and secretory epithelial cells in malignant and non-malignant areas. About 80% of the malignant basal cells were characterized as either weak or strong while non-malignant epithelial cells demonstrated strong immunoreactivity for SP-A. Also malignant (81.8%) and non-malignant cells (90.6%) were characterized as either weak or strong for SP-D. Decrement of SP-A and SP-D immunostaining tended to associate with an increasing Gleason score (p > 0.05, p < 0.05), tumor volume (p < 0.05, p > 0.05) and age (p > 0.05, p > 0.05). There was a strong positive correlation between Gleason score and tumor volume (p < 0.01). Also, either none or weak SP-A and SP-D immunoreactivity was observed specimens with Gleason score 7 or higher. SP-A and SP-D reacted with 34 kDa (SP-A) and 43 kDa (SP-D) immunoreactive single bands were decreased in tumor tissues. Conclusions: The development of prostate cancer may be related to decreased level of surfactant protein A and D.

Nevoid basal cell carcinoma syndrome associated with unilateral renal agenesis: acceleration of basal cell carcinomas following radiotherapy

To the Editor We report here a new case of nevoid basal cell carcinoma syndrome (NBCCS) in association with unilateral renal agenesis. Radiotherapy had been given to the patient, causing a significant increase in the number of basal cell carcinomas (BCCs) per year. Our case emphasizes the importance of treatment selection and of surveying these cases for the development of multiple BCCs. A 38-year-old male patient was referred to our clinic because of multiple tumoral masses. He had a history of multiple, purple– black tiny papules on the head and neck region since he was 29. He developed multiple tumoral masses on the scalp, face and trunk in the following years. Several biopsies revealed BCCs, and they were treated by simple surgical excision or cryotherapy. New BCCs appeared throughout these years. Two years prior to admission, radiotherapy (1000 cGy) was given for the treatment of a BCC around the left eye, after which the old lesions on the face showed rapid enlargement and a significant number of new lesions appeared. There was no family history of any similar skin condition. Total vision loss of the left eye, micrognathia and scoliosis were detected in the physical examination. Dermatological examination revealed sharply bordered, red– purple coloured papules and infiltrated plaques on the scalp, forehead, cheeks, neck and trunk (figs 1 and 2). Palmoplantar pittings were noted. Histopathological examination revealed uniform cell groups with narrow cytoplasm and large basophilic nucleus and characteristic palisades of the cells at the periphery. Table 1 Patients