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.

H syndrome with a novel homozygous SLC29A3 mutation in two sisters.

H syndrome (OMIM 602782) is a recently defined autosomal recessive genodermatosis. Cutaneous findings of H syndrome include hyperpigmentation, hypertrichosis, and induration, while hearing loss, heart anomalies, hepatomegaly, hypogonadism, hyperglycemia (diabetes mellitus), low height (short stature), hallux valgus (flexion contractures), and hematological abnormalities are the extracutaneous abnormalities. We report a novel homozygous missense mutation, c.416T > C p.(Leu139Pro), in the SLC29A3 (NM_001174098.1) gene in two sisters with H syndrome presenting with different phenotypes.

Evaluation of microvessel density with CD31 and CD105 in patients with psoriasis under methotrexate and acitretin therapy.

INTRODUCTION: Methotrexate and acitretin are known to be effective in the treatment of psoriasis, but the mechanisms of the effects of these drugs are not fully known. AIM: To investigate the effect of methotrexate and acitretin on microvessel density (MVD) in psoriasis. MATERIAL AND METHODS: Eighteen patients with psoriasis treated with methotrexate and 9 patients with psoriasis treated with acitretin (AT) were included in this study. MVD was evaluated immunohistochemically by using CD31 and CD105 (endoglin) antibodies. RESULTS: In the methotrexate group, the decrease in CD31 levels after treatment was found to be statistically significant, while in the AT group it was found to be highly significant. In both methotrexate and AT group, there was a statistically highly significant decrease in CD105 levels after treatment. There was no statistically significant difference between CD31 measurements of methotrexate and AT groups. When CD105 levels were measured before and after treatment, no statistically significant difference was found between methotrexate and AT. According to the results of CD31 changes before and after treatment, the CD31 difference was not statistically significant in both groups while the difference was higher in the AT group. CD105 differences were not statistically significant in both treatment groups before and after treatment. CONCLUSIONS: CD31 and CD105 dyes indicate the effects of therapies on vascular proliferation and may be indicators that can be used in daily routine and follow-up studies for psoriasis.

Nineteen-year retrospective evaluation of pemphigus in a single dermatology centre in Istanbul, Turkey.

INTRODUCTION: Pemphigus is an autoimmune intra-epidermal bullous disease of the skin and mucosae. AIM: To retrospectively evaluate the course, prognosis and clinical features of pemphigus. MATERIAL AND METHODS: The files of 196 pemphigus patients admitted to our clinic between December 1995 and December 2014 were collected and analysed. RESULTS: The male to female ratio among patients was 1 : 1.88. Pemphigus vulgaris (PV) was the most common clinical variant observed in 175 (89.3%) of the patients, followed by pemphigus foliaceus (PF) in 14 (7.1%) of the patients. The mean patient age at disease onset was 50 years. PV presented itself as skin lesions in 55 (31.4%) of the patients and as oral mucosa lesions in 120 (68.6%) of the patients. Complete remission and treatment withdrawal were obtained in 112 (57.1%) of the patients, for a mean period of 2.91 ±2.66 years (range: 4 months to 13 years). The mortality rate was 6%, and relapse occurred in 16 (14.3%) of the patients for a mean relapse period of 2.15 ±1.88 years (range: 6 months to 7 years). Mucocutaneous pemphigus (MCP) was the major clinical pattern observed in 96 (49%) of the patients. CONCLUSIONS: Within our study population, pemphigus predominately affected females, and the most common clinical variant was PV, a subtype that frequently occurs in middle-aged individuals. MCP was the most common clinical pattern. Although MCP and higher doses of corticosteroids were needed to control pemphigus, they did not seem to influence the prognosis.

Audiological and vestibular measurements in Behçet's disease.

INTRODUCTION: Behçet's disease (BD) is a vasculitis that involves all small vessels and influences the multiple systems of the human body. This study aimed to evaluate the audio-vestibular system involvement of patients with BD and healthy individuals. MATERIALS AND METHODS: This study was designed as a prospective case-control blinded study. Thirty-one patients with BD and 31 healthy individuals were included. All the subjects were evaluated via pure tone audiometry (PTA), video head impulse test (vHIT), post head shake nystagmus test (PHSNT) and dizziness handicap inventory (DHI) to check for audio-vestibular system involvement. RESULTS: Patients with BD showed higher PTA scores in both speech and high frequencies. The vHIT revealed pathological saccades, particularly in horizontal canals (right ear: p = 0.002, left ear: p = 0.039). The gain values of the patients were slightly lower than those of the control group; however, gain and gain asymmetry differed significantly in a few canals. In the spontaneous nystagmus test and PHSNT, pathological nystagmus was detected to be significantly higher in the patient group than control group (p = 0.001); but the saccade presence in vHIT and nystagmus in PHNT did not differ among the patients (p = 0.106). In addition, the DHI scores of the patients group were higher than those of the control group (p < 0.001). No correlation was found between disease duration and saccade presence. CONCLUSION: The vHIT was used preliminary for evaluating the vestibular system in BD. This study showed the influence of BD on the audio-vestibular system, in particular isolated horizontal canal involvement was discovered in patients with BD. LEVEL OF EVIDENCE: Level III b.

Complementary and Alternative Therapies Used by Patients of Pediatric Dermatology Outpatient Clinics in Turkey: A Multicenter Study.

BACKGROUND: The aim of this study was to determine the frequency of complementary and alternative medicine (CAM) methods used in children and adolescents with chronic skin diseases, which methods parents prefer, and to what extent these methods are useful. MATERIAL AND METHODS: This cross-sectional study was conducted between June and November 2014 in patients with chronic skin diseases attending the outpatient pediatric dermatology clinics of three hospitals in Turkey. Data were collected using self-administered questionnaires from the parents of the patients. RESULTS: A total of 307 patients were included in the study, 168 (54.7%) of whom were female and 139 (45.3%) male. The mean age was 12.3 ± 4.3 years. Eighty of the children (26.1%) had used CAM methods in the previous year. Longer disease duration and having a father with at least a university degree were highly associated with CAM use (p < 0.05). The most used method was herbal remedies available at home. Most patients used CAM methods to support the primary treatment given by their dermatologists. Only eight CAM users (2.6%) reported any side effects. Thirty-nine (48.8%) did not mention CAM use to their doctors. The overall satisfaction rate was 4.5 ± 3.1 on a 10-point rating scale. CONCLUSION: CAM use rates are high in children with chronic skin problems, and parents generally prefer herbal treatments. Satisfaction with the results of CAM use is moderate. Parents generally do not share CAM use information with their doctors unless they are asked.

Plantar Fibromatosis in a Mentally Challenged Man Treated with Intralesional Corticosteroids.

A 21-year-old mentally challenged man presented with a fixed plaque lesion on the right foot, which had been present for 10 years. Dermatologic examination revealed an erythematous, painful, firm, fixed plaque-nodular lesion on the plantar aspect of the right foot (Figure 1A). Nothing of distinction was noted from his family history or his laboratory tests. An incisional biopsy revealed parallel, regular bundles composed of uniform, plump spindle cells. Thin collagen fibers were seen in contact with and located between the spindle cells. This mass was separated from the surrounding soft tissue by an irregular, unclear border (Figure 2A). Immunohistochemically, the spindle cells showed diffuse, strong reactivity to vimentin (Figure 2B) and smooth muscle actin.

Genital involvement in pemphigus vulgaris (PV): correlation with clinical and cervicovaginal Pap smear findings.

BACKGROUND: The frequency of genital involvement in pemphigus vulgaris (PV) has not been clearly defined. OBJECTIVES: We sought to evaluate the frequency of cervical, vaginal, and vulvar involvement in PV and to determine their association with genital symptoms, clinical involvement, and cytological status. METHODS: The current study's sample included 34 female patients with PV. Gynecologic and ear, nose, and throat examinations and indirect immunofluorescence analyses were performed, and Pap smears were collected. RESULTS: Genital involvement was observed in 44.1% of patients. It was significantly associated with disease severity and clinical involvement. Pharyngeal involvement was observed in 61.8% of patients and was the second-most frequently involved mucosal region. Genital involvement was significantly associated with nasal mucosa involvement. Cervicovaginal Pap smears showed acantholytic cells of PV in 35.3% of patients. LIMITATION: The sample size is small. CONCLUSION: Genital involvement in PV is not rare. Genital mucosa is the most affected mucosal region after oral and pharyngeal mucosa. Furthermore, genital involvement is significantly associated with nasal involvement and genital symptoms. The need for complete gynecologic evaluations of patients with PV, nasal involvement, and genital symptoms is emphasized.

A comparative study of histopathological findings in skin biopsies from patients with psoriasis before and after treatment with acitretin, methotrexate and phototherapy.

INTRODUCTION: Psoriasis is a chronic and inflammatory skin disease. Few studies in the literature evaluate the responses to the treatment histopathologically. OBJECTIVES: In this study, we evaluated and compared skin biopsies taken from patients with psoriasis before and after phototherapy and therapy with acitretin and methotrexate. MATERIAL AND METHODS: We included 64 patients with a diagnosis of psoriasis vulgaris in our study. We performed phototherapy on 33 patients (51.6%), while 19 patients (29.7%) were treated with methotrexate and 12 patients (18.8%) were treated with acitretin. RESULTS: All of the patients had chronic plaque psoriasis, and they had skin lesions on more than 10% of their total body surface area and a score of PASI of 7.2-21.8 (average: 12.2). The histopathological parameter scores were similar in the initial evaluations of the pre-treatment treatment groups. When the biopsy specimens of all cases were evaluated together, a significant decrease was observed in terms of parakeratosis, Munro's microabscesses, regular acanthosis, pustules of Kogoj, lymphocyte infiltration in the papillary dermis, loss of the granular layer, spongiosis, suprapapillary thinning, vascularity in the papillary dermis and neutrophile infiltration in the papillary dermis. CONCLUSION: We found in our study that conventional treatment modalities provided histopathologically significant recovery in psoriasis, but they did not have an effect on some histopathological findings. To our knowledge, it is one of the few studies to assess these parameters in psoriasis under the continuous effect of acitretin, methotrexate and phototherapy for three months. There is a need for studies with larger series to examine the histopathological effects of these treatment modalities in terms of immunopathology.

Correlation Between Clinical and Histological Features of Dermatopathic Lymphadenopathy: Analysis of 39 Lymph Nodes.

Background. Dermatopathic lymphadenopathy is a well-defined histopathological entity with an underestimated prevalence in the general population. Objective. The present study was conducted to analyze the characteristics of histologically diagnosed cases of dermatopathic lymphadenopathy at our unit. We also aimed to investigate any association between the clinical features of the patients and the histological findings. Methods. A total of 39 lymph node samples from 39 patients diagnosed with dermatopathic lymphadenopathy were included in this prospective cohort study. Results. Thirty-four (87%) patients had a dermatological disorder. The presence of paracortical eosinophils were significantly higher in patients with dermatological disorders (P = .001), while the presence of dilated sinuses was significantly more common in patients without a dermatological disorder (P = .035). The presence of dilated sinuses and medullary histiocytes were significantly more common in patients with lower body surface area involvement of the disease compared to the ones with a higher body surface area (P = .003, P = .034; respectively). Conclusion. Most of the patients included in the study had one of a broad spectrum of undiagnosed dermatological disorders. The clinical significance of the relation between histological and clinical findings in dermatopathic lymphadenopathy remains to be explained. Dermatopathic lymphadenopathy should always be considered in differential diagnoses of patients with persistent lymph node enlargement even when absolute dermatological disorders are not present. Since various skin disorders may be the cause of lymphadenopathy, performing a full-body examination before lymph node excision might prevent unneccessary procedures.

Skin autoreactivity in Hashimoto's thyroiditis patients without urticaria: autologous serum skin test positivity correlation with thyroid antibodies, sonographical volume and grading.

Recent studies have shown an association between anti-thyroid antibodies and autologous serum skin test (ASST) positive urticaria patients. However, a connection between thyroid and this reliable skin test for mast cell autoreactivity, ASST, has not been reported yet. We investigated ASST in patients with Hashimoto's thyroiditis (HT) without urticaria and compared the results with laboratory and sonographical findings of HT. 154 HT patients, 100 healthy volunteers without HT as a first control group and 46 patients with multinodular goitre but without autoimmune thyroid disease as a second control group underwent testing with ASST. ASST was applied to these groups according to two criteria, first as ASST(new): autologous serum red wheal response 1.5 mm bigger than negative control; second as ASST(old): serum red wheal response 5 mm bigger than negative control accepted as positive. Free triiodothyronine (fT3), free thyroxine (fT4), thyroid-stimulating hormone (TSH), thyroid peroxidase antibody (anti-TPO) and thyroglobulin antibody (anti-Tg) levels were measured. ASST(old), ASST(new) scored positive in 51.3-60.4% of HT patients, with statistically significant differences. Thyroid volume grades were inversely proportional with ASST(old) and (new) positivity. Moderate (+) titers of anti-Tg in ASST(old) and (new) (+) cases were significantly higher than the same titers of anti-Tg in ASST(old) and (new) (-) cases. The prevalence of ASST positivity in HT patients was not affected by the following factors: gender, age at screening, laboratory measurements of thyroid function tests, anti-TPO antibodies and thyroid ultrasound (US) echogenicity. Positivity of ASST in HT has shown that there is a skin mast cell autoreactivity in HT patients independent of autoreactive chronic urticaria (ACU).

The evaluation of thyroid diseases in patients with pemphigus vulgaris.

BACKGROUND: Thyroid disorders may affect all of the organ systems of the body and they are also highly associated with a wide variety of skin disorders. The aim of this study was to investigate the prevalence of thyroid function abnormalities and thyroid autoimmunity in patients with pemphigus vulgaris (PV) and to determine the association between thyroid disorders and clinical involvement and systemic corticosteroid treatment in patients with PV. METHODS: The study consisted of eighty patients with PV and eighty healthy individuals. Thyroid functions (fT3, fT4, and TSH) and thyroid autoimmunity (anti-thyroid peroxidase (anti-TPO), and anti-thyroglobulin (anti-Tg) antibodies) were investigated in both groups. Primary thyroid disease (PTD) was diagnosed with one or more of the following diagnostic criteria: (i) positive antithyroid antibodies, (ii) primary thyroid function abnormalities. RESULTS: Significant changes in the serum thyroid profile were found in 16% (13/80) of the PV group and 5% (4/80) of the control group. Positive titers of antithyroid antibodies (anti-TPO and anti-Tg) were observed in 7 patients (9%) with PV and one in the control group (1,2%). Hashimoto thyroiditis was diagnosed in 9% of PV patients and it was found to be more prevalent in the mucosal form of PV. PTD was found in 13 of (%16) PV patients which was significantly high compared to controls. PTD was not found to be associated with systemic corticosteroid use. Free T3 levels were significantly lower in PV group compared to the control group and free T4 levels were significantly higher in PV group compared to the controls. CONCLUSIONS: PV may exist together with autoimmune thyroid diseases especially Hashimoto thyroiditis and primer thyroid diseases. Laboratory work-up for thyroid function tests and thyroid autoantibodies should be performed to determine underlying thyroid diseases in patients with PV.

Prevalence of metabolic syndrome in patients with psoriasis.

BACKGROUND: Psoriasis is a chronic inflammatory skin disorder in which proinflammatory cytokines including IL-6 and TNF-α increase both locally and systematically. It is thought that chronic inflammation results in metabolic diseases and proinflammatory cytokines give rise to the development of atherogenesis, peripheral insulin resistance, hypertension, and type 2 diabetes. Our aim was to investigate the prevalence of metabolic syndrome in patients with psoriasis vulgaris. METHODS: Study consisted of 115 plaque-type psoriasis patients and 140 healthy individuals. Data including body weight, height, waist circumference, body-mass index, and arterial blood pressure were collected. Fasting blood glucose, triglyceride, and HDL levels were determined. International Diabetes Federation Criteria for Metabolic Syndrome and Insulin Resistance were used for evaluating patients with metabolic syndrome and diabetes. RESULTS: Compared to the control group, metabolic syndrome, diabetes mellitus, and hypertension were found to be higher in psoriasis patients. Metabolic syndrome was increased by 3-folds in psoriasis patients and was more prevalent in women than in men. It was determined that the prevalence of metabolic syndrome was higher in psoriasis patients after the age of 40. Metabolic syndrome was not related to smoking, severity of psoriasis, and duration of disease. CONCLUSIONS: Our findings suggest that psoriasis preconditions occurrence of a group of diseases such as diabetes mellitus, hypertension, and metabolic syndrome. For this reason, patients with psoriasis should be treated early and they should be followed with respect to metabolic diseases.

Evaluation of histopathological changes in control biopsies which taken 48 sessions after NBUVB phototherapy for early-stage mycosis fungoides.

BACKGROUND: There are not many studies investigating histomorphological changes in 48 sessions in patients with early-stage MF after narrowband UVB (NBUVB) treatment. Our purpose is to evaluate histological features of phototherapy after 48 sessions and determine which parameters are more reliable for controlling skin biopsies. METHODS: Biopsies of 32 patients with early stage of MF, who were treated with NBUVB phototherapy, were histologically evaluated before and after the treatments, including epidermotropism, stratum corneum, epidermal thickness, dermal infiltration, papillary dermal fibrosis, vascular alterations, and other dermal changes. We discuss the histomorphological effects of NBUVB phototherapy on skin biopsies by comparing the responders with nonresponders, with before and after the treatment. RESULTS: 9 patients (28%) did not give any response to treatment. Alleviation in epidermotropism, increases in parakeratosis and normal keratosis, perivascular infiltration, and melanophages, decrease in the lichenoid/patchy lichenoid infiltration pattern after the treatment was statistically significant. Comparing by response, normalization of stratum corneum and epidermis, orthohyperkeratosis, decrease in linearly arranged cells, the lichenoid/patchy lichenoid infiltration, the loss of inflammation were statistically significant in responders group. CONCLUSION: We detected a significant decrease in linearly arranged cells after phototherapy, indicating that it is an "important diagnostic parameter" in evaluation of therapeutic response.

Adherence to systemic therapy in patients with psoriasis during the COVID-19 pandemic: A multicenter study.

BACKGROUND: Adherence to treatment is important in chronic dermatological diseases. There are limited data regarding the adherence to treatment in patients with psoriasis during the COVID-19 pandemic. AIMS: We aimed to determine the rates of adherence to systemic treatments in patients with psoriasis and to identify the causes of non-adherence during the COVID-19 pandemic. METHODS: We conducted a cross-sectional multicenter study from May 2021 to August 2021. A questionnaire including items regarding sociodemographic characteristics of the patients, disease-related characteristics, and treatment-related characteristics were filled out by the physicians. The Psoriasis Area Severity Index (PASI), Hospital Anxiety, and Depression Scale, and the Dermatology Life Quality Index were calculated. The rate of adherence and non-adherence to treatment and reasons for non-adherence to treatment were examined. RESULTS: A total of 342 patients with psoriasis were included (182 male/160 female) in the study. The mean age of the patients was 45.9 ± 14.2 years. The average duration of psoriasis was 192 ± 134.7 months. While the rate of adherence to treatment was 57.6%, the rate of non-adherence to treatment was 42.4%. There were no significant differences with respect to adherence to treatment in comparison with oral and injection-therapy groups. The most frequent reasons for non-adherence to treatment were inability to go to the hospital (19.2%), concern about the COVID-19 infection (16.3%), discontinuation of the treatment by the doctor (13.7%), inability to reach the doctor (7.3%), and inability to have access to the medication (7.3%). CONCLUSION: Adherence to oral and injection therapies was fairly high among our patients with psoriasis during the COVID-19 pandemic. Psoriasis severity and duration of medication use had a negative impact on adherence to treatment.

Autologous serum skin test vs autologous plasma skin test in patients with chronic urticaria: evaluation of reproducibility, sensitivity and specificity and relationship with disease activity, quality of life and anti-thyroid antibodies.

Recent concerns have arisen about the specificity and interpretation of the autologous serum skin test (ASST), suggesting that ASST might produce false-positive results, and proposing the use of autologous plasma (APST) instead for intradermal testing in autoreactive urticaria. We investigated autoreactivity to autologous plasma and compared the results for reproducibility, sensitivity, specificity and accuracy and evaluated their association with quality of life and anti-TPO antibodies. 70 adults with chronic spontaneous urticaria (CU) and 62 controls underwent testing with ASST and APST and the tests were repeated two days after the first visit. Blood tests measured anti-TPO levels. Disease activity was assessed by urticaria activity score (UAS-7) and quality of life impairment was assessed by DLQI and CU-Q(2)oL. There were no statistically significant differences between ASST (+) and ASST (-) and also APST (+) and APST (-) patients with regard to disease duration, anti-TPO antibodies, urticaria activity scores, DLQI scores and CU-Q(2)oL scores. The results of first ASST and APST were well correlated with the results of second ASST and APST. The specificity of the two tests was similar, while ASST had a higher sensitivity and accuracy. Our results showed that there is no need to use autologous plasma instead of autologous serum for intradermal testing in CU.

Successful treatment of generalized childhood Schamberg's disease with narrowband ultraviolet B therapy.

Pigmented purpuric dermatoses are a group of dermatoses characterized by petechia and hyperpigmented macules that occur predominantly on the lower extremities. Although it is most commonly seen in adults, this disease can also affect children. Phototherapy has been shown to be effective in some cases in the literature. Here, we present two cases of Schamberg's disease that improved rapidly with narrowband ultraviolet B (UVB) therapy. To our knowledge, they were the first pediatric cases of successful narrowband UVB therapy in this disease.