ICHTHYOSIS LINEARIS CIRCUMFLEXA: NOT ALWAYS A PART OF NETHERTON SYNDROME.

Ichthyosis Linearis Circumfiexa (ILC) is a rarely seen autosomal recessive keratinization disorder and is characterized by erythematous, polycystic, plaques with 'double-edged' scales. Its histological features resemble psoriasis. A triad of ichthyosis (usually Ichthyosis Linearis Circumfiexa), trichorrhexis invaginata (a distinctive hair shaft disorder) and atopy is named as Netherton syndrome. Herein, we report a 12 year-old girl presenting with ILC not accompanied with typical atopy findings and hair shaft disorder.

The use of complementary and alternative medicine among dermatology outpatients in Eastern Turkey.

INTRODUCTION: Over the past decade, complementary and alternative medicine (CAM) has become increasingly popular around the world. OBJECTIVES: In this study, we aim to investigate how frequently CAM is used and the types of CAM methods used for dermatological disease in Eastern Turkey. MATERIALS AND METHODS: We recruited 1610 patients from our clinic for this study. The sociodemographic features and the CAM methods were investigated with a survey. RESULTS: The most common dermatological disorders included contact dermatitis (21.4%), acne vulgaris (17.5%), fungal infections (10.9%), eczema (6.3%), and warts (5.7%). The ratio of patients using at least one CAM method was 43.7% and that of those using two or more CAM methods was 20.8%. The most commonly used CAM methods were those using henna, cologne, moisturizing cream, prayer, and herbal therapy. Some patients were found to use some interesting and unusual CAM methods, such as putting out a cigarette over the skin on the back for anthrax, applying raw meat for furuncle, using fuel oil and nitric acid for contact dermatitis. CONCLUSIONS: CAM methods are commonly used in our population. CAM methods often cause adverse reactions that may alter diagnostic skin findings and interfere with the efficacy of other medical therapies. Therefore, physicians should ask their patients about the use of CAM methods while collecting patient history. Physicians have a critical role in preventing improper use of CAM. In addition, further investigations into the efficacy, benefits, and risks of CAM methods should be carried out for better insight into those CAM methods.

GAPO syndrome associated with pyoderma vegetans: an unreported co-existence.

GAPO syndrome is a rare autosomal recessive disease and an acronym composed of growth retardation, alopecia, pseudoanodontia, optic atrophy. Approximately 38 cases have been reported in literature until now. Pyoderma vegetans is a chronic inflammatory disease characterized with vesicopustular, exudative and vegetative lesions usually localized on face, scalp, axilla and genitalia. Pyoderma vegetans is attributed to a bacterial infection frequently occurring in individuals with an underlying immunosuppressive condition. A 30-year-old female patient was admitted to our clinic with complaint of a hemorrhagic, crusted, exudative vegetative two plaques on the scalp. On her physical examination, she had a prematurely aged face, predominant lower lips, total tooth loss, frontal bossing, enlarged anterior fontanelle, marked scalp veins, micrognatia, depressed nasal bridge, short stature, growth retardation. She was diagnosed as GAPO syndrome as the result of her physical examination. Result of the biopsy taken from scalp was evaluated as pyoderma vegetans. And the diagnosis of pyoderma vegetans was established based on the correlate of both clinical and histopathologic findings. Pyoderma vegetans coexisting with GAPO syndrome has not been reported previously. Thus we wished to report it.

A lamotrigine induced toxic epidermal necrolysis in a child.

Toxic epidermal necrolysis (TEN) is rare, life-threatening skin disorder that usually is caused by an adverse drug reaction. The exact pathogenesis of TEN is still unknown. Many treatments including prednisolone, cyclosporine and intravenous immunoglobulin (IVIG) can be used to halt the disease process. We present a 12-year-old girl with epilepsy who developed TEN after about 14 days of lamotrigine treatment. Lamotrigine was immediately discontinued. After receiving systemic corticosteroid treatment, the patient had a complete recovery. Antiepileptic-induced TEN can be mortal in some cases. Thus, we would like to point out the importance of early diagnosis and treatment.

A case of fulminant varicella infection with purpura fulminans, hepatitis, and rhabdomyolysis.

Varicella zoster virus causes varicella which is a common disease. Generally it is self-limiting, and treatment is often unnecessary, but severe or life-threatening complications are rarely seen. We report a case of fulminant varicella complicating with purpura fulminans, hepatitis, and probable rhabdomyolysis in a previously healthy child.

Incontinentia pigmenti with ocular involvement: two cases.

Incontinentia pigmenti (IP) is a rare, X-linked dominant disorder that presents at or soon after birth and presents with cutaneous signs and symptoms. Besides its typical skin signs presenting in all patients, central nervous system (CNS), ophthalmologic and dental involvement are encountered as well. In this report, we present two patients at the verrucous stage of IP and review the literature concerning the diagnosis and course of IP.

Fixed drug eruption induced by topical olopatadine ophthalmic solution.

Fixed drug eruption (FDE) usually develops after oral administration and is described as a cutaneous reaction recurring at the same location each time the drug is taken. Olopatadine is both a H1 histamine receptor antagonist and a mast cell stabilizer, indicated for the treatment of allergic conjunctivitis. Here, we report a 14-year-old male patient who developed FDE localised on the lateral side of periorbital rim bilaterally, whilst applying olopatadine 0.1% ophthalmic solution for the treatment of allergic conjunctivitis. As far as we know, FDE due to olopatadine has not been previously reported in the literature. We deem it appropriate to report this case because FDE that results from the application of topical drugs is a rare event in the literature.

Immunoregulatory effects of isotretinoin in patients with acne.

BACKGROUND: In vitro studies have shown that retinoids influence T-cell differentiation. OBJECTIVES: To study the effect of isotretinoin on T-cell differentiation markers in patients with acne. METHODS: A total of 37 patients with acne vulgaris (25 female, 12 male, age 19.6 ± 3.7 years) and 30 age- and sex-matched healthy controls (19 female, 11 male, age 20.5 ± 4.4 years) were included in the study. Screening for biochemical parameters in serum samples were done just before initiation (pretreatment) and after 3 months of isotretinoin treatment (post-treatment) in the acne group. RESULTS: Baseline levels of tumour necrosis factor (TNF)-α (P<0.0001), interleukin (IL)-4 (P<0.0001), IL-17 (P<0.0001) and interferon (IFN)-γ (P=0.002) were significantly higher in the acne group compared with the control group. TNF-α (P<0.0001), IL-4 (P<0.0001), IL-17 (P<0.0001) and IFN-γ (P<0.0001) levels decreased after isotretinoin treatment. TNF-α and IL-4 values after isotretinoin treatment were similar to those of the control group. However, levels of IL-17 (P<0.0001) after isotretinoin treatment were higher than those of the control group, despite a significant decline after treatment. Levels of IFN-γ (P<0.0001) after isotretinoin treatment were lower than those of the control group. CONCLUSIONS: This study shows that isotretinoin treatment significantly decreases TNF, IL-4, IL-17 and IFN-γ levels in patients with acne. We failed to show that isotretinoin redirects naive T helper (Th) differentiation preferentially towards the Th2 cell lineage.

Nine case series with phototoxic dermatitis related to Chenopodium album.

Chenopodium album L. ssp. album is a common plant species worldwide, especially in humid areas. Sun exposure after oral intake of the plant can lead to sunburn-like rashes owing to its furocoumarin content. In this article, we reported nine patients who developed dermatitis with edema, erythema and necrosis on the face and dorsum of the hands. Each was exposed to sunlight after eating selemez (the vernacular name of C. album L. ssp. album). The plant samples analyzed by an expert botanist were described as C. album L. ssp. album belonging to Chenopodiacea family.

Thyroid autoimmunity associated with recurrent aphthous stomatitis.

BACKGROUND: Recurrent aphthous stomatitis (RAS) is an autoimmune disorder characterized by the periodic appearance of aphthous lesions on the oral mucosa. TH1 cytokines plays a key role in the aetiopathogenesis. Autoimmune thyroid disease (ATD) is the most common autoimmune disease and is frequently accompanied by various other autoimmune diseases. OBJECTIVE: To investigate the frequency of ATD which has not been studied in the patients with RAS. METHODS: Ninety patients and 30 healthy volunteers were included into the study. The serum samples were assayed for thyroid stimulant hormone (TSH), free and total triiodothyronine (fT3, TT3), free and total thyroxine (fT4, TT4), thyroglobuline, anti-thyroid peroxidase antibody (anti-TPO) and anti-thyroglobuline antibody (anti-TG) levels. Thyroid ultrasonography was performed as well. RESULTS: In RAS patients, the fT3, TT3 levels were higher; whereas the fT4 levels were lower that the control group (P < 0.05). The anti-thyroid antibody was positive in 31.11% of the patients with RAS, and in only 10% of the individuals in the control group (P < 0.05). The mean anti-TG level was also higher in the RAS group. Ultrasonography revealed nodules in 28.8% of the patients with RAS and in 16.7% of the individuals in the control group (P < 0.05). The sT4 levels were lower and the TSH, anti-TPO and anti-TG levels were significantly higher in the RAS patients with thyroid nodules than the RAS patients without nodules (P < 0.05). DISCUSSION: These results may be related to either the advance age of the patients or the increased duration of the autoimmune activation which may affect the thyroid. CONCLUSIONS: The frequency of thyroid autoimmune-related problems was higher in patients with RAS. It would be worthy of searching autoimmune thyroid disorders in patients with RAS.

Nonsteroidal anti-inflammatory drugs-induced generalized fixed drug eruption: two cases.

Fixed drug eruption (FDE) is a drug-induced cutaneous reaction that occurs at the same site with each exposure to a specific medication and usually manifests as round or oval, sharply demarcated erythematous or edematous plaques. The exact mechanism is unknown. The most common causative agent is co-trimoxazole. Other major categories of causative agents of FDE include antibiotics, antiepileptics, and nonsteroidal anti-inflammatory drugs (NSAIDs). FDE usually causes localized eruptions and very rarely generalized lesions. We report two cases of developing generalized FDEs after exposure to diclofenac and naproxen.

Mucocutaneous disorders in children with down syndrome: case-controlled study.

OBJECTIVE: Down's syndrome is the most frequent chromosomal anomaly that can affect a multiple organ systems, including skin. In this study, we compared the frequency of mucocutaneous disorders in children with Down's syndrome with normal children. METHODS: We recruited fifty children with Down's syndrome and 50 healthy children as a control in our study. Mucocutaneous abnormalities were examined in both groups. FUNDINGS: Of 50 patients, 22 were girls and 28 were boys. The skin findings such as xerosis and Mongolian spots were the most frequently seen in the patients with Down's syndrome. Seborrheic dermatitis, cheilitis and fissured tongue were seen frequently, as well. The rare findings were plantar hyperkeratosis, alopecia areata, geographic tongue, café-au-lait macula, livedo reticularis, cutaneous infections, pityriasis capitis simplex, cutis marmorata, neurotic excoriation, trichotillomania, keratosis pilaris and diaper dermatitis. No mucocutaneous finding was seen in 16 patients. The prevalence of the skin disorders including Mongolian spot, seborrheic dermatitis, cheilitis, fissured tongue was significantly higher in patients with Down syndrome than normal individuals (p<0.05). CONCLUSION: Therefore, large-scaled epidemiological studies covering the entire population are needed. We believe that these studies could provide better understanding the dermatological diseases in children with Down's syndrome that would help to practitioners to treat these problems.

Alström syndrome with acanthosis nigricans: a case report and literature review.

Alström syndrome (AS) is a very rare autosomal recessively inherited disorder that can lead to infantile-onset dilated cardiomyopathy, blindness, hearing impairment, obesity, diabetes, hepatic and renal dysfunction. AS is caused by mutations in the ALMS1 gene, which is located at chromosome 2p13. The life span of patients with AS rarely goes beyond an age of 40 years. There is no specific therapy for AS, but early diagnosis and intervention may moderate the progression of the disease and may improve the length and quality of the patient's life. We report a 10 year-old boy presenting with Alström Syndrome and acanthosis nigricans.

Cutaneous anthrax in Eastern Turkey: a review of 85 cases.

BACKGROUND: Anthrax is a zoonotic disease diminishing worldwide. Although a very rare disease in developed countries, anthrax is still endemic in developing countries. AIM: To evaluate the clinical history and features, treatment and outcome of our patients with anthrax and emphasize the importance of the disease in our region. METHOD: In this study, the records of all patients diagnosed with cutaneous anthrax admitted to Yüzüncü Yil University Medical Faculty between March 2002 and March 2007, were reviewed, and data on age, gender, occupation, clinical symptoms and findings, location and type of lesions, clinical history, laboratory findings, treatment and outcome were recorded. RESULTS: There were 85 patients [46 (54.1%) male, 39 (45.9%) female; mean age 30.6 years, range 6-72]. All the patients had a history of contact with infected animals or animal products. The infectious agent was found using direct examination of Gram-stained smears from 17 patients (20%), and Bacillus anthracis was isolated from vesicle fluid cultures from 11 patients (12.9%). Diagnosis was based on clinical findings in the remaining 57 patients (67.1%). All patients, except for two with respiratory tract obstruction, made a full recovery. CONCLUSION: Anthrax continues to be an endemic disease in Turkey, and should be considered in patients with a relevant contact history having a painless ulcer and vesicular skin lesions surrounded by a zone of oedema. Gram staining and simple culture methods are useful aids to diagnosis, but diagnosis may have to be based on clinical grounds in the majority of patients.

Oesophageal involvement during attacks in pemphigus vulgaris patients.

BACKGROUND: Pemphigus vulgaris (PV) is a rare autoimmune bullous skin disorder characterized by frequent involvement of the mucous membranes, usually beginning at the mouth. AIMS: To investigate the oesophageal involvement in patients with PV and to explore the primary relationship of the disease with symptoms such as dysphagia, odynophagia and retrosternal burning. METHODS: Oesophageal involvement was investigated by upper gastrointestinal endoscopy and biopsy during the early phase of the attacks in 26 patients with PV (12 men, 14 women, age range 24-63 years). RESULTS: Histopathological examination and direct immunofluorescence of the oesophageal biopsy specimens revealed pemphigus involvement in 12 of 26 patients (46.15%). CONCLUSION: The oesophagus is an important predilection zone for PV, thus care must be taken to detect these lesions at an early stage.

Anti-inflammatory effects of tretinoin (all-trans-retinoic acid) 0.1% and adapalene 0.1% in rats.

In this study, the anti-inflammatory effects of tretinoin (all-trans-retinoic acid) 0.1% cream and adapalene 0.1% gel were compared in rats to determine whether there was a difference between these agents. Thirty-six rats of either sex were divided into six groups (two control groups, and an etodolac, indomethacin, tretinoin and adapalene group) of six animals each. Each group was given different drugs or chemicals. The inhibitory activities of the drugs were determined on carrageenan-induced rat-paw oedema. The inhibition rate (53.48%) in the tretinoin group was found to be higher than adapalene and controls (P < 0.05). Adapalene was found to have an inhibition rate of 10.28%, and when compared with the other groups, was found to have no statistically significant anti-inflammatory activity. We conclude that tretinoin has a higher anti-inflammatory activity than adapalene and thus should be preferred for the treatment of inflammatory lesions.

Hairy tongue: a case report.

Hairy tongue or black hairy tongue is a benign condition characterized by hypertrophy of the filiform papillae that give the dorsum of the tongue a furry appearance. The color of the elongated papillae varies from yellowish white to brown or black. The etiology is unclear, but the disorder has been associated with numerous predisposing conditions such as heavy smoking, poor oral hygiene, use of topical or systemic antibiotics, systemic corticosteroid therapy, yeast infections, and radiotherapy for head and neck malignancies. A case is presented in which a hairy tongue appeared in a 67-year-old man.