Cutaneous findings in patients with acromegaly and its relationship with concomitant endocrinopathies.

OBJECTIVES: Skin changes in acromegaly are often the first sign of the disease. The aim of this study was to describe the cutaneous findings in patients with acromegaly. In addition, a secondary aim was to investigate the possible association of these findings with remission status and concomitant endocrinopathies. DESIGN, PATIENTS, AND MEASUREMENTS: In this prospective multicenter study, 278 patients over the age of 18 years with acromegaly who were followed up in 14 different tertiary healthcare institutions were included. These patients, who were followed up by the Endocrinology Department, were then referred to a dermatologist for dermatological examination. The frequency of skin lesions was investigated by detailed dermatologic examination. Dermatological diagnosis is reached by clinical, dermatological and/or dermoscopic examination, and rarely skin punch biopsy examinations in suspicious cases. The possible association of the skin findings between remitted and nonremitted patients and with concomitant endocrinopathies were evaluated. RESULTS: The most common skin findings in patients with acromegaly in our study were skin tags (52.5%), cherry angiomas (47.4%), seborrhoea (37%), varicose veins (33%), acneiform lesions (28.8%), hyperhidrosis (26.9%) and hypertrichosis (18.3%). Hypertrichosis was significantly more prevalent in patients nonremitted (p: .001), while xerosis cutis was significantly more prevalent in patients remitted (p: .001). The frequency of diabetes mellitus and hypothyroidism was significantly higher in patients with varicose veins and seborrhoeic keratosis than those without. Additionally, the coexistence of hypothyroidism, hyperthyroidism and galactorrhea was significantly higher in patients with Cherry angioma than in those without Cherry angioma (p-values: .024, .034 and .027, respectively). The frequency of hypogonadism in those with xerosis cutis was significantly higher than in those without (p: .035). CONCLUSIONS: Cutaneous androgenization findings such as skin tag, seborrhoea, acne and acanthosis nigricans are common in patients with acromegaly. Clinicians should be aware that skin findings associated with insulin resistance may develop in these patients. It can be said that the remission state in acromegaly has no curative effect on cutaneous findings. Only patients in remission were less likely to have hypertrichosis. This may allow earlier review of the follow-up and treatment of acromegaly patients presenting with complaints of hypertrichosis. Additionally, it can be said that patients with skin findings such as cherry angioma may be predisposed to a second endocrinopathy, especially hypothyroidism. Including dermatology in a multidisciplinary perspective in acromegaly patient management would be beneficial to detect cutaneous findings earlier.

Screening Fabry's disease in chronic kidney disease patients not on dialysis: a multicenter study.

OBJECTIVES: Fabry's disease is an X-linked inherited, rare, progressive, lysosomal storage disorder, affecting multiple organs due to the deficient activity of α-galactosidase A (α-Gal A) enzyme. The prevalence has been reported to be 0.15-1% in hemodialysis patients; however, the information on the prevalence in chronic kidney disease not on dialysis is lacking. This study aimed to determine the prevalence of Fabry's disease in chronic kidney disease. METHODS: The patients older than 18 years, enclosing KDIGO 2012 chronic kidney disease definitions, not on dialysis, were enrolled. Dried blood spots on Guthrie papers were used to analyze α-Gal A enzyme and genetic analysis was performed in individuals with enzyme activity ≤1.2 µmol/L/h. RESULTS: A total of 1453 chronic kidney disease patients not on dialysis from seven clinics in Turkey were screened. The mean age of the study population was 59.3 ± 15.9 years. 45.6% of patients were female. The creatinine clearance of 77.3% of patients was below 60 mL/min/1.73 m2, 8.4% had proteinuria, and 2.5% had isolated microscopic hematuria. The mean value of patients' α-Gal A enzyme was detected as 2.93 ± 1.92 µmol/L/h. 152 patients had low levels of α-Gal A enzyme activity (≤1.2 µmol/L/h). In mutation analysis, A143T and D313Y variants were disclosed in three male patients. The prevalence of Fabry's disease in chronic kidney disease not on dialysis was found to be 0.2% (0.4% in male, 0.0% in female). CONCLUSION: Fabry's disease should be considered in the differential diagnosis of chronic kidney disease with unknown etiology even in the absence of symptoms and signs suggestive of Fabry's disease.

Rosacea treatment with intermediate-dose isotretinoin: follow-up with erythema and sebum measurements.

Isotretinoin is one of the therapeutic options for rosacea. However, the response of erythema to treatment with isotretinoin is usually slow and incomplete with common (0.5-1 mg/kg/day) or low (10 mg/day) doses. This study investigated the efficacy of, and relapse on, 20 mg/day isotretinoin treatment in rosacea, with the aid of instrumental measurement of facial erythema and sebum levels. A 20 mg/day dose of isotretinoin was given for 4 months, and then the dose was tapered off within the following 6 months. A total of 25 patients were included in the study. Papule and pustule counts, erythema index, sebum level, dermatologist's and patient's erythema scores, and dermatologist's sebum scores were significantly lower in the first month of therapy compared with pre-treatment values (p < 0.05). Within a median follow-up of 11 months (95% confidence interval: 8.4-13.5 months) 45% of patients had a relapse. In conclusion, 20 mg/day isotretinoin was rapidly efficient for reducing both inflammatory lesions and erythema in rosacea.

Plasmacytoid dendritic cell tumor: A case report.

A 62-year-old man presented with a painless eruption on his arms and trunk. Physical examination showed 2 well-demarcated erythematous plaques on the anterior trunk and 6 purple-red papules on the back and upper extremities. Blood chemistry and computed tomography results were normal. Herein we describe a patient with plasmacytoid dendritic cell neoplasm in the absence of systemic symptoms.

Green staining of clothing: a signal for pseudomonal infection.

Anticonvulsant hypersensitivity syndrome (AHS) is a nondose-related idiosyncratic reaction to aromatic antiepileptic drugs and is a cause of drug discontinuation. Pseudomonas aeruginosa is a gram-negative bacillus that can produce infections in many different organs, including the skin and soft tissue. We report a patient with erythroderma and AHS who developed a pseudomonal infection. Green staining of the underwear served as a diagnostic clue for severe P aeruginosa infection that had developed because of a local flexural skin infection that spread due to a damaged skin barrier. Inspection of the patient's clothes may give information about any exudate from the skin and should be done routinely as part of the physical examination.

Investigation of the inflammatory mechanisms in alopecia areata.

We aimed to investigate the profile of the inflammatory infiltrate in lesional and nonlesional tissue in alopecia areata (AA) and look for possible associations between inflammatory mechanisms, neuropeptide expressions, and various clinical features. Twenty-four patch-type AA patients were included. Forty-eight lesional and nonlesional skin samples were stained immunohistochemically with antibodies for CD1a, CD3, CD4, CD8, CD20, CD57 (for natural killer cells), mast cell tryptase, nerve growth factor receptor (NGFR), and substance P (SP). Various clinical findings were recorded. Psychological distress levels and stress-related hormones were measured. Lesional skin showed statistically more CD3(+), CD8(+), and CD57(+) lymphocytes, mast cells, Langerhans cells, and more prominent immunoreactivities of NGFR and SP (P < 0.003). Most nonlesional skin showed CD3(+) and CD57(+) cells, mast cells, and NGFR(+) nerve fibers. NGFR and SP, and SP and perivascular mast cell infiltrates were correlated, whereas peribulbar mast cells and anagen follicle counts were inversely correlated in nonlesional skin (P < 0.05). Near half of the patients' distress levels were high. No relationship among biochemical, psychological, and clinical parameters could be shown. AA may involve the entire skin in which lesions occur as a result of local T cell-mediated cytotoxic inflammatory response initiated by Langerhans cells and mast cells activated via neuropeptides.

Cutaneous metastatic adenocarcinoma arising from a malignant sacrococcygeal teratoma in an adult.

We present a patient with cutaneous metastasis caused by an adenocarcinoma arising from a malignant teratoma. A 37-year-old woman seen for the complaint of swelling in the genital region also complained of a draining mass in her gluteal region present since birth. Physical examination showed marked edema in the labia majora, multiple hyperkeratotic papules in the left labium majus, and erythema, induration, and swelling in the left femoral and inguinal regions. A soft tumor that exhibited sinus tracts was palpated in the left gluteus. Excision of the gluteal tumor revealed a teratoma. Vulvar skin biopsy confirmed a mucinous adenocarcinoma which had derived from this teratoma. A tumor that arises from pluripotent germ cells, teratoma rarely shows malignant transformation. The patient presented is a rare example of a cutaneous metastasis originating from a congenital sacrococcygeal teratoma in an adult.

Nature of cell kinetics in psoriatic epidermis.

BACKGROUND: Psoriasis vulgaris is a common chronic inflammatory dermatosis. Disorders in keratinocyte proliferation, differentiation, inflammation and immune dysregulation are the major factors implicated in the pathogenesis of psoriasis vulgaris. METHODS: The study was performed in skin specimens of 25 patients with psoriasis vulgaris and a control group of 10 individuals without a skin disease. Biopsy specimens from lesional and normal skin were analyzed by immunohistochemical method for expressions of Ki-67, Bcl-2, terminal deoxynucleotidyl transferase-mediated dUTP-biotin nick-end labeling (TUNEL), tumor necrosis factor alpha (TNF-alpha) and nuclear factor kappa B (NF-kappaB). In addition, densities of mast cell infiltration were also investigated. RESULTS: Ki-67 and TUNEL indexes and TNF-alpha and NF-kappaB expressions were significantly higher in psoriatic epidermis than in normal epidermis (p < 0.05). There was no significant difference at Bcl-2 reactivity between the normal and the psoriatic epidermis (p > 0.05); however, Bcl-2 staining intensity of lymphocytes was higher in psoriatic lesions than in normal dermis (p < 0.05). Additionally, the number of mast cells was significantly higher in psoriatic dermis than in normal skin (p < 0.05). CONCLUSIONS: There were several complex factors involved in the pathogenesis of psoriasis. We conclude that cellular damage and apoptosis temporarily coincide with epidermal proliferation during the course of psoriatic hyperplasia.