Şükran Tunali

Hypocomplementemic urticarial vasculitis: a rare presentation of systemic lupus erythematosus

Background  Urticarial vasculitis is a small‐vessel vasculitis, presenting clinically as persistent urticarial skin lesions and microscopically as leucocytoclastic vasculitis. Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a distinct type of urticarial vasculitis with multiorgan involvement, whose etiology and link with other diseases are still unknown. Some authors have suggested that HUVS can be accompanied by systemic lupus erythematosus (SLE), and others believe that it is a rare subtype of SLE. Urticarial vasculitis is seen in 7–8% of SLE, while 50% of HUVS patients are diagnosed with SLE.

Generalized Sweet syndrome lesions associated with Behçet disease: a true association or simply co-morbidity?

Sweet syndrome or acute febrile neutrophilic dermatosis is a condition characterized by painful erythematous papules, nodules, and plaques, which demonstrate mature neutrophilic infiltration of the upper dermis histopathologically, and is usually associated with systemic symptoms such as fever and neutrophilia. Generalized Sweet syndrome lesions are usually noted in association with malignancies. Sweet syndrome lesions are rarely seen in patients with Behçet disease and, if present, are usually fewer in number.In this report, we present two female patients who developed generalized Sweet syndrome lesions during follow-up after the diagnosis of Behçet disease. The clinical and histopathologic findings in our patients together with the data in the literature suggest that generalized Sweet syndrome lesions in a patient with clinically inactive Behçet disease should remind the clinician that a strict differential diagnosis needs to be made between Sweet syndrome associated with Behçet disease and the rare cutaneous manifestation of Behçet disease that resembles the lesions of Sweet syndrome.

Polyurethane toilet seat contact dermatitis.

Abstract:  Polyurethane chemicals are produced by the reaction of isocyanates and they may cause allergic contact dermatitis or precipitate asthma attacks. Contact dermatitis to polyurethane toilet seat has not been reported before. Herein we present a case of allergic contact dermatitis to polyurethane toilet seat.

Treatment of advanced classic Kaposi's sarcoma with weekly low‐dose paclitaxel therapy

Treatment of advanced classic Kaposi’s sarcoma with weekly low-dose paclitaxel therapy Several reports have been published recently documenting the efficacy of paclitaxel monotherapy in human immunodeficiency virus (HIV)-associated and post-transplant Kaposi’s sarcoma (KS). The standard dosing and frequency of this therapy has not yet been established, however. We present here an interesting case of classic KS with gastrointestinal involvement and human herpesvirus-8 (HHV-8) positivity responding to weekly low-dose paclitaxel monotherapy, suggesting a tolerable and alternative regimen in the elderly. A 76-year-old woman presented with multiple reddish-blue plaques and nodules of 7 years’ duration distributed widely on the distal upper and lower extremities and face (Fig. 1). Oral examination revealed a reddish-blue plaque, 1.5 cm in size, on the hard palate. No lymphadenopathy or organomegaly was detected by systemic examination. The histopathologic examination of the punch biopsy specimen was compatible with KS (Fig. 2). The patient was HIV negative, but HHV-8 was found to be positive in blood on polymerase chain reaction (PCR) analysis. Systemic evaluation of the patient revealed anemia (hemoglobin, 7.3 g/dL) and occult blood testing was positive. In addition to chronic gastritis, endoscopic examination demonstrated two similar nodules on the descending part of the duodenum which were confirmed histopathologically as KS. Computed tomographic examination of the thorax and abdomen did not show any metastatic lesion or lymphadenopathy. As a result of gastrointestinal involvement and widespread distribution of the lesions, weekly low-dose paclitaxel chemotherapy (60 mg/m in 250 mL of normal saline solution) was initiated. Because of this drug’s marked allergenicity, the patient was premedicated by the infusion of 40 mg dexamethasone intravenously 30 min prior to the paclitaxel infusion. Ten courses were administered. After eight courses of therapy, all lesions, including those on the oral mucosa, had dramatically regressed. The side-effects observed during therapy were alopecia and granulocytopenia. The patient received a total of 10 injections of granulocyte-colony stimulating factor during therapy. She experienced myalgia once after the first course of therapy. At the end of 10 courses, the skin lesions had improved with postinflammatory hyperpigmentation (Fig. 3). Endoscopic examination revealed the disappearance of duodenal lesions. A punch biopsy from the hyperpigmented macules showed no residual disease except for a few ecstatic blood vessels. The patient has been followed up for 15 weeks with no recurrence. The disseminated skin lesions and presence of duodenal involvement in this case warranted the application of chemotherapy as the best approach. Systemic medications that are Food and Drug Administration (FDA) approved are liposomal doxorubicin, paclitaxel, and interferon-α. As a result of previous experience demonstrating its efficacy as a single agent in KS, paclitaxel was preferred. Paclitaxel exerts its antitumor effects by several mechanisms, most importantly by the polymerization of microtubules, inhibition of cell division, and induction of cell death, possibly by the down-regulation of bcl-2 expression. Paclitaxel was

Localized Unilateral Hyperhidrosis and Neurofibromatosis Type 1: Case Report of a New Association

Localized unilateral hyperhidrosis (LUH) is an uncommon but well-defined special form of localized hyperhidrosis with diverse causes. It often occurs within a sharply demarcated area on the face or upper extremities. Here, we report a case of LUH in a 16-year-old male with neurofibromatosis type 1 (NF1) and review related data in the literature. The association of this rare entity and NF1 has not yet been described. Subcutaneous botulinum toxin A injection provided a good clinical response with symptomatic relief.

Effective treatment of relapsing idiopathic nodular panniculitis (Pfeifer-Weber-Christian disease) with mycophenolate mofetil

Relapsing idiopathic nodular panniculitis is the term used to describe a group of diseases that presents as subcutaneous inflammatory nodules, fever and systemic symptoms and histopathologically displays inflammation within the fat lobules. There is no specific test for diagnosis and extensive investigations are required to exclude systemic causes of panniculitis. No uniform effective therapy is available and various drugs used include mainly corticosteroids alone or in combination with other immunosuppressive agents. Presented in this paper is an intractable case of idiopathic nodular panniculitis whose corticotherapy failed and could not be continued because of serious adverse effects. The rapid and good therapeutic response of the patient to mycophenolate mofetil monotherapy is discussed.

Flow Cytometric Analysis of T, B, and NK Cells Antigens in Patients with Mycosis Fungoides.

We retrospectively analyzed the clinicopathological correlation and prognostic value of cell surface antigens expressed by peripheral blood mononuclear cells in patients with mycosis fungoides (MF). 121 consecutive MF patients were included in this study. All patients had peripheral blood flow cytometry as part of their first visit. TNMB and histopathological staging of the cases were retrospectively performed in accordance with International Society for Cutaneous Lymphomas/European Organization of Research and Treatment of Cancer (ISCL/EORTC) criteria at the time of flow cytometry sampling. To determine prognostic value of cell surface antigens, cases were divided into two groups as stable and progressive disease. 17 flow cytometric analyses of 17 parapsoriasis (PP) and 11 analyses of 11 benign erythrodermic patients were included as control groups. Fluorescent labeled monoclonal antibodies were used to detect cell surface antigens: T cells (CD3+, CD4+, CD8+, TCRαβ +, TCRγδ +, CD7+, CD4+CD7+, CD4+CD7−, and CD71+), B cells (HLA-DR+, CD19+, and HLA-DR+CD19+), NKT cells (CD3+CD16+CD56+), and NK cells (CD3−CD16+CD56+). The mean value of all cell surface antigens was not statistically significant between parapsoriasis and MF groups. Along with an increase in cases of MF stage statistically significant difference was found between the mean values of cell surface antigens. Flow cytometric analysis of peripheral blood cell surface antigens in patients with mycosis fungoides may contribute to predicting disease stage and progression.

The efficacy of narrowband UVB treatment in pediatricvitiligo: a retrospective analysis of 26 cases

BACKGROUND/AIM Narrowband UVB (Nb UVB) treatment is commonly used for the management of psoriasis and atopic dermatitis, and is less often used for vitiligo in children. The aim of this study was to evaluate the efficacy and short-term safety of Nb UVB phototherapy in children diagnosed with vitiligo retrospectively. MATERIALS AND METHODS A total of 26 patients younger than 18 years with the diagnosis of vitiligo and managed with Nb UVB phototherapy as documented in archive records were evaluated. Clinical response was assessed according to repigmentation of the lesions: good response when there was more than 75% repigmentation, moderate response when there was 25%-74% repigmentation, poor response when repigmentation was less than 24%, and unresponsive when there was no pigmentation and new lesions occurred. RESULTS A total of 26 patients received Nb UVB treatment; 14 were girls and 12 were boys. The age at onset of the disease varied between 2 and 18 years, with a mean age of onset of 10.07 ± 4.53 years. Repigmentation rate of >75% was detected in 45.4% of cases. CONCLUSION Nb UVB phototherapy seems to be a well-tolerated effective and safe treatment option in children, especially those unresponsive to topical treatment and those with widespread lesions. However, long-term risks such as photocarcinogenesis and photoaging should kept in mind.

Retrospective analysis of the forty-six patients with bullous pemphigoid followed-up in our clinic

Türkderm-Deri Hastalıkları ve Frengi Arşivi Dergisi, Galenos Yayınevi tarafından basılmıştır. Turkderm-Archives of the Turkish Dermatology and Venerology, published by Galenos Publishing. Background and Design: Bullous pemphigoid (BP) is an autoimmune disease characterised by widespread itchy plaques and subepithelial blisterings and usually affects the elderly population. Due to the chronic nature of the disease, to prevent the side effects of chronic steroid treatment, adjuvant immunosuppressive (mycophenolate mofetil, azathioprine, methotrexate) and anti-inflammatory (tetracycline, nicotinamide, dapsone) agents may be used. In this study, we retrospectively evaluated the clinical and demographic characteristics and applied treatments of 46 patients with the diagnosis of BP and compared with literature data. Materials and Methods: We retrospectively evaluated the records of 46 patients who received clinical and histopathological diagnosis of BP and followed up in our clinic between 2006 and 2013. Results: Of the 46 patients, 22 were female and 24 male. The mean age of onset was 69.54 years (range: 18-105). The duration of the lesion ranged from 1 week to 10 months with a median duration of 8 weeks. The most frequent comorbid chronic disease was hypertension detected in 28 (60.8%) patients. Only four patients had a history of malignancy before the onset of the disease. Nineteen patients (42%) used more than 5 agents for comorbid diseases. Thirty-two patients (69.5%) used systemic corticosteroids alone and ten (22%) patients needed additional adjuvant therapies. Conclusion: BP is a major cause of morbidity in the elderly population receiving multiple drug treatment. To avoid the side effects of steroid therapy, especially in patients with severe disease, short-term use of additional immunosuppressive agents appears to be safe and effective.

Efficacy of 0.1% tacrolimus ointment in chronic plaque psoriasis: A randomized double-blind placebo-controlled study

Türkderm-Deri Hastalıkları ve Frengi Arşivi Dergisi, Galenos Yayınevi tarafından basılmıştır. Turkderm-Archives of the Turkish Dermatology and Venerology, published by Galenos Publishing. Background and Design: Despite the development of effective systemic treatments and new biological agents for psoriasis nowadays, topical medications are still the mainstay of treatment. Topical calcineurin inhibitors are currently used in various skin diseases. We investigated the efficacy and safety of tacrolimus, which is an alternative in topical treatment, in comparison with the present medications in plaque psoriasis. Materials and Methods: This prospective double-blind placebo-controlled study was conducted in 24 patients with the diagnosis of plaque psoriasis who were seen in Uludağ University Hospital dermatology outpatient clinic. 0.1% tacrolimus ointment, 0.1% mometasone furoate ointment, 0.005% calcipotriol ointment and placebo in encrypted bottles were randomly applied under occlusion to 27 psoriatic plaques in test chambers, every other day, for a period of 19 days. Clinic sum scores, side effects, and epidermal thickness measured by superficial ultrasound were noted before and after treatment in all microplaques. Besides, histopathologic scoring and epidermal thickness were measured in 9 patients at the end of the study. Results: The clinic sum scores and ultrasonographic epidermal thickness at the end were both significantly lower than the beginning values in all microplaques (p<0.05). The reduction in these two values with tacrolimus were significantly higher than that with placebo (p<0.001), but no difference was observed with calcipotriol (p=0.287, p=0.813, respectively). On the other hand, the reduction in these values with mometasone Amaç: Günümüzde etkili sistemik tedaviler ve yeni biyolojik ajanlar geliştirilmesine rağmen topikal tedaviler psoriasis tedavisinin halen direğidir. Topikal kalsinörin inhibitörleri bugün pek çok deri hastalığında kullanılmaktadır. Çalışmamızda plak tipi psoriasiste topikal tedavide alternatif bir seçenek olarak takrolimusun mevcut tedavilerle karşılaştırmalı etkinlik ve güvenilirliği araştırılmıştır. Gereç ve Yöntem: Prospektif, çift-kör plasebo kontrollü bu çalışma Uludağ Üniversitesi Hastanesi Dermatoloji polikliniğine başvuran, plak tipi psoriasis tanılı 24 hasta ile yapıldı. Yirmi yedi seçilmiş psoriatik plağa 19 gün boyunca, günaşırı, şifrelenmiş şişelerde %0,1 takrolimus merhemi, %0,1 mometazon furat merhemi, %0,005 kalsipotriol merhemi ve plasebo ayrı ayrı lökotestlerle oklüzyon şeklinde, randomize edilerek uygulandı. Tedavi başlangıcı ve sonunda her bir mikroplakta klinik şiddet skorlaması ve yan etkiler kaydedildi, yüzeyel ultrasonografi ile epidermal kalınlık ölçüldü. Ayrıca tedavi bitiminde 9 hastada mikroplaklardan biyopsi alınarak toplam histopatolojik skor ve epidermal kalınlık hesaplandı. Bulgular: Tüm mikroplaklarda hesaplanan tedavi sonu toplam klinik skor ve ultrasonografik epidermal kalınlık tedavi başlangıcındaki değerlerinden düşüktü (p<0,05). Tedavi sonu takrolimus uygulanan plakta toplam klinik skorda ve ultasonografik ölçümde azalma plaseboya göre anlamlı idi (p<0,001), ancak kalsipotriolle fark saptanmadı (sırasıyla, p=0,287, p=0,813). Öte yandan mometazon furat merhemi ile bu ölçümlerde azalma takrolimus merheme göre istatistiksel anlamlı idi (p<0,05). Tedavi sonu toplam histopatolojik skor ve epidermal kalınlığın plaseboya göre farkları karşılaştırıldığında en etkili mometazon furat bulundu. Sonuç: Plak tipi psoriasisli hastalarda takrolimus merheminin özellikle oklüzyonla uygulandığında topikal tedavide alternatif olabilir. Oklüzyon uygulamaları pratikte hasta uyumu gerektirir. Bu nedenle ilacın kalın psoriasis plaklarından emilimini arttıracak yeni bir formülasyon bulmaya yönelik ileri çalışmalar gerektiği kanısındayız. (Türk derm 2015; 49: 107-11) Anahtar Kelimeler: Psoriasis, takrolimus merhem, topikal tedavi