Epidermal consumption in benign and malignant melanocytic neoplasms.

Consumption of the epidermis associated with effacement of the rete ridge pattern has been cited as a useful criterion in the diagnosis of melanoma, but the significance of consumption in the absence of rete ridge effacement is unknown. We evaluated 701 melanocytic neoplasms for presence and 'grade' of consumption by melanocytic nests relative to diagnosis, body location, gender and age. We defined 1+ consumption as collections of melanocytes occupying greater than two thirds of the viable epidermis, with or without loss of the rete ridge pattern. Nests extending to the bottom of, within, and through the granular layer were graded 2+, 3+ and 4+, respectively. Consumption was more frequent and higher grades were found in melanomas followed by Spitz nevi compared with conventional melanocytic nevi (p < 0.001). Melanomas with higher Breslow thickness showed higher grades (p < 0.05). In conventional nevi, consumption occurred most frequently in back (13.7%), acral (11.9%) and scalp (9.8%) locations. Consumption without the requirement for rete ridge effacement occurs more frequently and at higher grades in melanoma. Higher grades correlate with higher Breslow thickness. Consumption is also common in Spitz nevi and occurs at lower grades in conventional (non-Spitz) nevi, especially on the back, the scalp and at acral sites.

A Newborn with Congenital Mixed Phenotype Acute Leukemia After In Vitro Fertilization.

Congenital leukemia is a rare disease. The majority of cases of this disease are acute myelogenous leukemia (AML). Congenital acute lymphoblastic leukemia (ALL) is rare and most often is of B cell lineage. Rarely, some cases have been designated biphenotypic or mixed phenotype acute leukemia (MPAL). Herein, we report a preterm newborn referred to us as a result of the appearance of blue-violaceous dermal nodules on her body at birth. She was a twin and the product of an in vitro fertilization (IVF) pregnancy. Physical examination showed jaundice, hepatosplenomegaly, and peripheral facial nerve palsy in addition to dermal nodules. Bone marrow aspiration showed 40% blasts of lymphoid lineage; skin biopsy and its immunohistochemistry revealed myeloblastic infiltration of the dermis. Cytogenetic analysis (46,XX), fluorescence in situ hybridization (FISH) analysis, and cranial magnetic resonance were normal. The patient was diagnosed with congenital MPAL, and an association between IVF and congenital leukemia was suggested.

Vitamin D receptor gene polymorphisms and haplotypes (Apa I, Bsm I, Fok I, Taq I) in Turkish psoriasis patients.

BACKGROUND: Psoriasis is an inflammatory disease characterized by increased squamous cell proliferation and impaired differentiation. Vitamin D, Calcitriol, and its analogues are successfully used for psoriasis therapy. However, it is unknown why some psoriasis patients are resistant to Vitamin D therapy. Vitamin D mediates its activity by a nuclear receptor. It is suggested that polymorphisms and haplotypes in the VDR gene may explain the differences in response to vitamin D therapy. MATERIAL/METHODS: In this study, 102 psoriasis patients and 102 healthy controls were studied for VDR gene polymorphisms. The Fok I, Bsm I, Apa I and Taq I polymorphisms were examined by PCR-RFLP, and 50 subjects received vitamin D therapy to evaluate the association between VDR gene polymorphisms and response to vitamin D therapy. Existence of cutting site is shown by capital letters, and lack was shown by lower case. The haplotypes were analysed by CHAPLIN. RESULTS: There was significant difference in allele frequency of T and genotype frequency of Tt between cases and controls (p values 0.038 and 0.04, respectively). The Aa and bb genotypes were significantly higher in early onset than late onset psoriasis (p values 0.008 and 0.04, respectively). The genotypes Ff, ff and TT are significantly different between vitamin D3 therapy responders and non-responders (p values 0.04, 0.0001, 0.009, respectively). To the best of our knowledge, this is the first report showing importance of VDR gene haplotypes in psoriasis, the significance of the Wald and LR (Likelihood Ratio) statistics (p=0,0042) suggest that FfBbAatt is a disease-susceptibility haplotype. CONCLUSIONS: Haplotype analysis is a recent and commonly used method in genetic association studies. Our results reveal a previously unidentified susceptibility haplotype and indicate that certain haplotypes are important in the resistance to vitamin D3 therapy and the onset of psoriasis. The haplotypes can give valuable data where genotypes unable to do.

Calcinosis cutis in a newborn with transient pseudohypoparathyroidism.

Pseudohypoparathyroidism (PHP) is a heterogenous group of disorders characterized by hypocalcemia with hyperphosphatemia, increased serum concentration of parathyroid hormone (PTH), and insensitivity to the biological activity of PTH. Calcinosis cutis, the cutaneous deposition of calcium salts in the dermis, is a rare clinical symptom in infancy. The deposition of calcium in the skin may be classified as dystrophic, metastatic, idiopathic, and iatrogenic. Although a few infants with PHP and calcinosis cutis have been reported, to the authors' knowledge, the combination of neonatal transient PHP and calcinosis cutis associated with calcium treatment has not been previously reported. The authors report a newborn boy with transient PHP presenting with early hypocalcemia, hyperphosphatemia, increased PTH levels, and calcinosis cutis after intravenous treatment of calcium gluconate.

HIV-negative case of bacillary angiomatosis with chronic hepatitis B.

Bacillary angiomatosis (BA) is an infectious disease which occurs predominantly in immunosuppressive patients and rarely in immunocompetent individuals. We had a case of BA who presented with a red cutaneous nodule on his left leg of which histopathological examination revealed marked lobular proliferation of capillaries throughout the interstitium and clusters of bacilli with hematoxylin-eosin and Warthin-Starry stains, respectively. Antibody titer against Bartonella henselae was 1/32. The only systemic disease the patient had was chronic hepatitis B. HIV was negative and no other immunosuppressive status was established. In this case we believe that the immunological differences secondary to chronic hepatitis B could have caused a tendency for the disease development.

The EEC syndrome and SHFM: report of two cases and mutation analysis of p63 gene.

The p63 gene is a transcription factor and a member of the p53 family. Heterozygote mutation of the p63 gene is suggested in a number of human syndromes including limb development and/or ectodermal dysplasia. The EEC syndrome, consisting of ectrodactyly (E), ectodermal dysplasia (E) and cleft lip (C) with or without cleft palate, is the prototype of these syndromes with the presence of heterozygote mutation in the p63 gene in most of the patients. Nonsyndromic split hand/foot malformation (SHFM) is one of the EEC-like syndromes, and the p63 gene mutation was reported in only a few patients. Five different loci have been mapped to date, but the etiology is yet to be explained in the rest of the patients. Here, we report two cases. Case 1, diagnosed with EEC syndrome, had type 2 urogenital sinus and a new heterozygous mutation of 934G>A (D312N) in exon 8 of the p63 gene. Case 2 was diagnosed as SHFM with no mutation in the p63 gene. Genotype and phenotype correlation of these two cases among the reported patients is discussed in this report.

Two cases of Cushing's syndrome due to overuse of topical steroid in the diaper area.

Topical use of corticosteroids may cause immunosuppression and iatrogenic Cushing's syndrome via hypothalamic-pituitary-adrenal axis. We report two cases with iatrogenic Cushing's syndrome with different clinical outcomes due to abuse of same potent topical steroid clobetasol propionate. One of them died because of fatal disseminated cytomegalovirus infection. The other patient recovered completely. Physicians and parents should be informed about the adverse effects of such potent topical corticosteroids and physicians should prescribe less potent agents, especially during infancy.

Epidemiology of atopic dermatitis in primary schoolchildren in Turkey.

The present study aimed to investigate the prevalence of atopic dermatitis in primary schoolchildren in Denizli, Turkey, and to determine the possible risk factors for atopic dermatitis in home environment. A self-administered questionnaire was handled to the parents of 2,100 children aged 7 to 15 years, from three randomized primary schools and 1,644 (78.9%) completed and returned the questionnaire. The questionnaire included the United Kingdom Working Party diagnostic criteria and asked about conditions that could affect the course of atopic dermatitis. The prevalence of atopic dermatitis and coexisting factors that may affect the course of the disease were evaluated in 1,644 children (825 girls and 819 boys). The prevalence of atopic dermatitis was detected as 4.9%. Passive smoking, heating systems either in the house or in the child's bedroom, and the number of people living in the house had no significant effect (p > 0.005). The difference in prevalence of atopic dermatitis between developed and developing countries is striking. The determination of the factors that have an influence in this issue will probably enable us to change the course and frequency of atopic dermatitis.

The role of essential and non-essential elements in Mal de Meleda.

Mal de Meleda (MDM) is a rare autosomal recessively inherited palmoplantar keratoderma. In 1826 Stulli described the disease on the Dalmatian island of Meleda. Hyperkeratosis involves other parts of the body such as elbows and knees, in addition to the palms and soles. Zinc deficiency is known to cause hyperkeratozis in addition to other skin changes. We studied the levels of essential and nonessential elements in blood and tissues /nail, hair, skin/ in MDM patients and healthy controls living in Köprüçay area, Turkey. The results show no significant difference in blood zinc, iron, copper, cadmium, and lead levels between MDM patients and healthy controls. The zinc and copper levels of nail and hair in MDM patients did not differ from those of healthy controls.

[Biotyping of Propionibacterium acnes strains isolated from patients with acne vulgaris]. / Akne vulgaris hastalarindan izole edilen Propionibacterium acnes suslarinin biyotiplendirilmesi.

Propionibacterium acnes is the main bacterial etiologic agent in acne vulgaris pathogenesis. The rate of antibiotic resistant P. acnes isolates is less than 10% in our country. The aim of this study was to biotype P. acnes strains which were isolated from acne vulgaris patients and to investigate the biotype distribution in our region. P. acnes isolates from 64 patients (18 male, 46 female; mean age 19 +/- 3.1 years) were included in the study and biotyped by sugar-specific pH changes using sorbitol, erythritol and ribose. Results were compared with the global acne grading scores and clinical types of the patients. The frequency rates of P. acnes biotypes were found as follows; 40 strains (62.5%) were biotype I, 3 (4.7%) were biotype II, 20 (31.3%) were biotype III and one (1.6%) was biotype IV, whereas biotype V was not detected. The acne cases who were admitted to dermatology outpatient clinics mostly complained of papulopustuler and nodulocystic lesions. Therefore the detection of antibiotic resistance rates of P. acnes strains by mass screening of the community has a growing importance. Since P. acnes biotype III is the main biotype which causes the most severe acne reactions, the 31.3% rate of frequency found in our study, can not be overlooked. As a result, this data should be considered as a guide while prescribing antibiotics for the treatment of acne patients.

Relapsing Henoch-Schönlein purpura in an adult patient associated with hepatitis B virus infection.

Henoch-Schölein purpura is usually a disease of children presenting with arthralgia, abdominal pain, renal involvement, and palpable purpura. Viral and bacterial infections may have a role in its etiology. We present a 32-year-old male patient with recurrent Henoch-Schölein purpura in association with a chronic hepatitis B infection of ten years duration. The patient had received lamuvidine and interferon-alpha for the treatment of hepatitis B infection for a year. The skin lesions disappeared with the treatment of the hepatitis B infection. Four months after discontinuation of the therapy, the purpuric papules reappeared with reactivation of the hepatitis B infection. Although rarely reported, hepatitis B virus infection should be considered in patients with Henoch-Schölein purpura.

Skin tags and atherosclerotic risk factors.

An association between skin tags and insulin resistance, obesity, impaired carbohydrate and lipid metabolism has been suggested. However, there still is a need for comprehensive and controlled clinical studies. We aimed to evaluate the atherogenic risk factors in patients with skin tags. Thirty-six patients with skin tags who were admitted to the dermatology department and 22 healthy controls were included in this study. Possible subjects who were taking systemic drugs or who had a systemic disease that may be associated with lipid or carbohydrate metabolism abnormalities were excluded from the study. All the measurements were completed in 26 patients. Standard oral glucose tolerance tests were performed on the patient and control groups. Serum insulin, total cholesterol, triglyceride and HDL-cholesterol levels were measured. LDL-cholesterol and VLDL-cholesterol ratios and HOMA-IR and body mass indices were calculated. The mean levels of body mass index, HOMA-IR, and total cholesterol were significantly higher in patients than in controls. In conclusion, skin tags may not be innocent tumoral proliferations; instead, follow-up of such patients with regard to the development of diseases associated with atherosclerosis may be beneficial.

Prevalence of dermatomycoses in Mal de Meleda patients: a field study.

Mal de Meleda is a rare autosomal recessive form of palmoplantar keratoderma characterized by hyperkeratosis of the palms and soles. The presence of yeast and dermatophytes was investigated in 29 mal de Meleda patients from Koprucay canyon, Turkey, a newer geographical focus, and was found in 62.0% and 20.7% of cases, respectively. Antifungal resistance of isolates was not detected.