Peeling skin diseases: 21 cases from Turkey and a review of the literature.

BACKGROUND: Peeling skin diseases (PSD) refer to a group of rare autosomal recessive dermatosis which are characterized by spontaneous, continual peeling of the skin. Three different clinical pictures can be distinguished: Inflammatory PSD also referred to as peeling skin syndrome (PSS) type B, non-inflammatory PSD also referred to as PSS type A, and localized forms i.e. acral type PSS. OBJECTIVE: To characterize the clinical and histopathological features of PSD in Turkey. METHODS: We retrospectively reviewed the medical records and clinical photographs of patients who were given diagnosis of PSD and conducted histopathological evaluation of skin biopsies to identify the site of cleavage. Also we evaluated the cases including age, gender, age onset, clinical and histological findings, family history, associated disorders and PSD type. RESULTS: Twenty-one patients with PSD were seen at Gulhane School of Medicine in Ankara between the years 1994 and 2010 in this retrospective study. All patients were men. Their ages were between 20 and 26 years (22.44±2.30, Mean age±SD). Of the patients, eight cases (40%) were type A, eight cases (40%) were type B, and five cases (20%) were acral type PSS. Eleven cases (52%) had parental consanguinity. Keratoderma, cheilitis, keratosis pilaris, melanonichia, clubbing, hyperhidrosis, onychodystrophy were observed in eight cases as an accompanying disorder. CONCLUSIONS: In this case series, PSD occurred rarely and also showed generally mild course of disease in Turkey and most likely related to consanguineous of marriages. Future investigations on PSD will contribute to our progressing alternative targets for pathogenesis-based therapy.

Natural behaviour and surgical treatment of appendiceal carcinoids: an analysis of 2,376 consecutive emergency appendectomies.

OBJECTIVE: Carcinoid tumor of appendix is a rare condition. Its rarity may give rise to variances in its management. We aimed to demonstrate the occurrence and form of presentation of carcinoid tumor of appendix, as well as variations in its treatment. METHODS: All appendicectomies that took place between 2000 and 2008 were considered for study. RESULTS: A total of 2,376 appendicectomies were performed during this period while carcinoid tumours were diagnosed in 27 patients (1.13%). The mean age of patients with carcinoid tumor (26.7 years) was almost the same as that of patients with non-carcinoid pathology (28.1 years). The incidence of male patients was higher than that of females amongst the carcinoid tumor group (female/male ratio: 1/4). None of the carcinoid tumors were identified at operation. One patient (3.7 %) required right hemicolectomy. This patient was followed-up in an inconsistent manner. CONCLUSIONS: Carcinoid tumour of the appendix remains an incidental diagnosis. Patients with carcinoid were significantly younger than those with non-carcinoid conditions in the study. Re-operation rate was low. The interval to definitive surgery was very short and only one patient was followed up. No consideration as to whether the surgery was complete or not was done in the study (Tab. 1, Fig. 1, Ref. 29).

Oral fludarabine treatment of cutaneous infiltration in a patient with B-cell chronic lymphocytic leukaemia.

In B-cell chronic lymphocytic leukaemia (B-CLL), cutaneous infiltration is far less common than in T-cell CLL. However, the effect of fludarabine on cutaneous infiltration in patients with B-CLL is uncertain. We describe a 63-year-old man with B-CLL presenting with cutaneous lesions, who was treated successfully with oral fludarabine. Skin biopsy of one of these lesions revealed diffuse infiltration of uniform lymphocytes. Using PCR analysis, the same immunoglobulin heavy-chain gene rearrangement was found in lymphocytes in all samples (peripheral blood, bone marrow and skin lesion). The patient received four courses of oral fludarabine. Simultaneously with the normalization of the peripheral blood lymphocytosis, the patient became free of the cutaneous infiltration of CLL after four courses of oral fludarabine. Skin lesions had not recurred by the 12-month follow-up examination. To our knowledge, this is the first case of B-CLL leukaemia cutis treated with oral fludarabine. The introduction of fludarabine may contribute to a better outlook for these patients in the future.

Severe dapsone hypersensitivity syndrome.

Dapsone, a potent antiparasitic and anti-inflammatory compound, is mainly used in the treatment of leprosy and a variety of blistering skin diseases. It may cause a severe adverse drug reaction with multiorgan involvement known as dapsone hypersensitivity syndrome. We report the case of a 21-year-old female patient with dapsone hypersensitivity syndrome. The clinical presentation mimicked a viral exanthema.

Muir-Torre syndrome presenting with ileus: a case report.

The Muir-Torre syndrome is characterized by cutaneous neoplasms and visceral malignancies. At least one sebaceous adenoma, epithelioma or carcinoma and at least one internal malignancy are required to make a reliable diagnosis. According to medical literature only two cases of Muir-Torre syndrome with jejunal carcinoma have been reported to date and there is no reported case with intestinal obstruction. Here, we report an unusual case of jejunal carcinoma presenting with ileus.

Spermatocele: a rare complication of rectal surgery and radiotherapy. Case report.

A 47-year-old man developed a presacral mass after abdominoperineal resection, which is combined with pre and postoperative radio-chemotherapy for carcinoma of the rectum. CT-guided biopsy and cytological examination of the mass revealed no malignancy but spermatocele, which is a very unusual complication of rectal surgery.

Follicular mucinosis responding to isotretinoin treatment.

Follicular mucinosis is a rare disorder of unknown etiology characterized by accumulation of mucin in the sebaceous glands and outer root sheaths of the hair follicles. It is divided into a primary benign type and a secondary type mostly associated with lymphomas. No effective standard therapy for follicular mucinosis is available. We describe the case of a 21-year-old Caucasian male who had papules, nodules, and erythematous plaques on his left shoulder, left arm, and right scapular region. He was diagnosed as primary benign generalized follicular mucinosis, and treated with isotretinoin. Almost complete remission was achieved in 4 months.

Comparison of the effects of continuous and intermittent portal triad occlusion (PTO) in rats.

BACKGROUND/AIMS: It has previously been shown that prolonged ischemia of the liver had a mortal course and a method of intermittent occlusion of the hepatic pedicle was defined in order to minimize the damage to the liver. The present experimental study aimed to compare the effects of continuous and intermittent occlusion of the hepatic pedicle on the liver by measuring serum lactate, serum MDA malondialdehyde and glutathione levels and by evaluating the histologic changes in the liver tissue. METHODOLOGY: Thirty male Wistar albino rats weighing 300 +/- 50 g were divided into three groups of ten animals. Group 1 underwent a sham operation. Animals in group 2 underwent continuous portal triad occlusion (PTO group) for 30 minutes following laparotomy. The remaining ten animals in group 3 underwent intermittent occlusion consisting of 10 minutes of occlusion followed by 10 minutes of reperfusion for a total period of 30 minutes of ischemia. Blood samples were collected at the 1st and 6th postoperative hour for analytical evaluation. After sacrificing the animals, liver samples were obtained for histologic evaluation. RESULTS: The serum lactate levels were significantly higher in both portal triad occlusion groups than in the control at the 1st hour. While lactate levels also increased at the 6th hour in the continuous PTO group, it decreased to the level of control values in the intermittent PTO group. The difference between continuous and intermittent groups was also significant. Despite the unchanged malondialdehyde levels in the control group, malondialdehyde levels were significantly increased at the first and sixth hour in both PTO groups and the levels were also significantly higher than control values. Malondialdehyde levels of intermittent PTO groups at the first and sixth hour were both significantly lower than continue PTO groups. Whole blood glutathione levels were not changed in control groups with time, levels increased significantly in both PTO groups. Glutathione levels were higher than control values in both PTO groups at the first hour. While it turned to its basal value in intermittent PTO groups at the 6th hour, it was still significantly higher in the continuous PTO group. When both PTO groups were compared, glutathione levels were found to be significantly higher in the continuous group both at the first and sixth hour than in the intermittent PTO group. Histopathologic evaluation also showed that there was less damage in the intermittent PTO group than in the continuous PTO group. CONCLUSIONS: Our results show that continuous portal triad occlusion resulted in significant oxidative stress and cell damage as confirmed by increased serum lactate and blood malondialdehyde levels. The blood glutathione levels are increased due to a greater requirement in response to increased oxidative stress induced by portal triad occlusion. It is also confirmed that intermittent portal triad occlusion is safer as it causes less oxidative stress and cell damage so that its use is strongly suggested whenever portal triad occlusion is required.

Multiple sclerosis with mass effect: case report.

Multiple sclerosis (MS) may rarely present as a cerebral mass with the clinical features and computed tomography (CT) scan appearance of a cerebral tumor. We report a case of MS with large cranial involvement showing a mass effect. We carried out a complete examination, including contrast enhancement, with neuroimaging studies. The operative procedure associated with medical treatment was performed and we obtained a good result.

Focal epithelial hyperplasia treated with interferon alpha-2a.

BACKGROUND: Focal epithelial hyperplasia (FEH) is an uncommon benign oral condition that occurs mainly in young individuals of certain racial groups. METHODS: A 21-year-old Caucasian man presented with FEH of the oral mucosa. The patient was treated with interferon alpha-2a three times a week for 14 weeks intramuscularly (a total of 162 million units). RESULTS: At 2 months after the end of therapy, the papular lesions showed partial regression. CONCLUSION: This treatment modality should be used in cases with diffuse focal epithelial hyperplasia.

Bilateral male breast cancer and prostate cancer: a case report.

Male breast cancer, consisting only 1% of all breast cancers, is occasionally associated with other primary malignancies, especially in patients with familial breast cancer history. Sporadic male breast cancers with another primary tumor are extremely rare. We report a 67-year-old male with asynchronous bilateral breast cancer and prostate cancer without familial breast cancer history.

Fine needle aspiration biopsy in hepatic Echinococcus multilocularis.

OBJECTIVE: To determine the fine needle aspiration biopsy (FNAB) findings in hepatic Echinococcus multilocularis. STUDY DESIGN: FNAB and tru-cut liver needle biopsy were applied in 14 hepatic E multilocularis cases. Cytologic smears were stained with May-Grünwald-Giemsa and periodic acid-Schiff (PAS) stain. Tissue sections were stained with hematoxylin-eosin (HE) and PAS stain. RESULTS: In tissue sections, homogeneous, thin, cystic structures of various dimensions strongly stained with PAS. Mucoid material was stained with PAS in the cystic structures. Wide, coagulative necrosis was observed in all cases. In some cases there were foreign body-type giant cells at the periphery of the lesion. In all the cytologic smears there were an intense necrotic ground, PAS-positive hyaline cuticular structures and mucoid globules; in some cases there were foreign body-type giant cells. CONCLUSION: The above cytologic characteristics are basic diagnostic criteria for FNAB of E multilocularis.

Examination of periodontal tissues by a cutting-grinding technique.

The decalcification process removes the mineralized part of the dental hard tissues, making the histologic examination of enamel-associated tissues less than optimal. The cutting-grinding technique allows preparation of histologic slides with well preserved dental hard tissues together with their surrounding soft tissue components. In this paper, the histologic appearances of undecalcified teeth and their associated periodontal tissues prepared using the cutting-grinding technique are described.

Secondary oxalosis of bone in a dialysis patient.

Secondary oxalosis of bone is a complication of chronic renal failure. Its frequency and the mechanism of the deposition is unknown. We report the case of chronic renal failure patient on hemodialysis with deposition of oxalate in bone. Possible mechanisms and the significance of the depositions is also discussed.

Fine needle aspiration cytology of malignant lymphoepithelial lesion of the salivary gland. A report of two cases.

Fine needle aspiration cytologic findings in two cases of malignant lymphoepithelial lesion (MLEL) of the parotid glands are presented. Aspirates of both cases showed individual or cohesive clusters of tumor cells intermingling with mature lymphocytes. Cervical lymph node aspirates in both cases were similar to those seen in the parotids. Our findings suggest that neither cytology nor histology of the cervical lymph nodes is conclusive in establishing the primary site of a metastatic lymphoepitheliomalike carcinoma. MLEL of the salivary glands must be included in the differential diagnosis.