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Objective: Isotretinoin is generally chosen as the first line treatment of nodulocystic acne. Close laboratory monitoring is a general practice for many dermatologists to be on the safe side due to the laboratory side effects of isotretinoin. We aimed to determine the laboratory side effects of oral isotretinoin and optimal frequency interval for laboratory monitoring during isotretinoin treatment. Materials and Methods: One hundred patients who were under oral isotretinoin therapy were included in the study; serum lipid levels along with liver transaminase levels were recorded at the baseline, 1-, 3- and 6- months of the therapy. Results: We found that there might be slight elevations in serum aspartate transaminase and lipid levels during isotretinoin therapy (p

Keratosis pilaris is a skin disorder which is characterized by follicular hyperkeratosis. It is most commonly seen in patients with atopic dermatitis and ichthyosis vulgaris. Abnormal keratinization of the hair follicle leads to plugging and bumpy, rough appearance. Upper extensor arms, thighs, buttocks and cheeks are most commonly affected by the disease. Even though keratosis pilaris is associated with autosomal dominant inheritance; it may also be seen as a side effect of various targeted cancer therapies including BRAF and tyrosine kinase inhibitors. Herein, we would like to present a case of keratosis pilaris that had developed secondary to nilotinib treatment.

Introduction Chronic spontaneous urticaria (CSU) is defined as recurrent attacks of urticaria present for more than six weeks. The monoclonal anti-immunoglobulin E antibody, omalizumab, was approved for the treatment of CSU in patients who remain refractory to H1-antihistamines. Biologic agents are shown not to increase the risk of COVID-19 infection in different studies. Objective In the present study, we aimed to determine the prevalance of COVID-19 infection in relation to the age, gender, presence of other comorbidities, and treatment given for CSU. Methods We conducted a descriptive cross-sectional study of 233 patients diagnosed with CSU in a tertiary referral hospital. Demographical data, treatment given for CSU, the presence of COVID-19-related symptoms, history of close contact to a person with COVID-19 and COVID-19 real-time polymerase chain reaction (RT-PCR) results were determined via a telephone survey and checked from medical data records. Results One hundred sixty patients were female; whereas 73 were male. The mean age was 44.76. Out of 233 patients with chronic urticaria, 125 had symptoms related to COVID-19 infection. RT-PCR testing for COVID-19 was performed in 156 patients. Of 156 patients with COVID-19 RT-PCR test, RT-PCR result was positive in 15 cases. Conclusions No statistically significant relationship was found between COVID-19 RT-PCR positivity and the type of treatment administered for chronic urticaria when the patients are divided into omalizumab ± oral antihistamines and only oral antihistamines treatment groups (p = 0.150). Omalizumab seems to be safe in the era of COVID-19.

Objective: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are dermatologic emergencies. There is a lack of consensus regarding appropriate management of SJS/TEN. The aim of this study was to evaluate demographic and clinical features, management and outcomes of SJS/TEN patients. Materials and Methods: The data of patients who were ≥18 years old and hospitalized with the diagnosis of SJS, SJS-TEN overlap and TEN at Hacettepe University, between 1992 and 2018 were analyzed retrospectively. Patient demographics, medications, time between the first causative drug intake and the onset of symptoms, mucous membrane involvement, treatment modalities including supportive measures, intravenous immunoglobulin, cyclosporine and systemic corticosteroids, duration of hospitalization, and mortality outcomes were recorded from patient charts. Results: A total of 23 patients (11 men; 12 women) with a mean age of 46.4 ± 19.5 years were included in the study. Twelve patients (52.2%) had SJS, 8 patients (34.8%) had SJS/TEN overlap and 3 patients (13%) had TEN. Mean duration of hospitalization was 12.6 ± 6.5 days. Twenty-two patients (95.7%) were attributed to a specific medication, whereas triggering factor was not detected in 1 patient (4.3%). More than one drug was responsible for 9 patients, including antimicrobials (n=11, 47.8%), anticonvulsants (n=8, 34.8%) and/or non-steroid anti-inflammatory drugs (n=5, 21.7%). The median time between the intake of medication and the onset of symptoms was 20 days (IQR 5.5-30). Sixteen patients used systemic corticosteroids (69.6%) and 4 patients used intravenous immunoglobulin (17.4%) whereas 3 patients used systemic corticosteroids in combination with intravenous immunoglobulin (n=2, 8.7%) or cyclosporin (n=1, 4.3%). The median time between the onset of symptoms and the onset of the rash to the treatment was 5 days (IQR 3-7). Mortality was not observed in our cohort. Conclusion: SJS/TEN was most commonly developed because of drugs, mainly antimicrobials, anticonvulsants and/or non-steroid anti-inflammatory drugs. The absence of mortality in our cohort was considered to be associated with younger age, low rate of TEN, prompt initiation of treatment (mainly corticosteroids) following the rapid discontinuation of the suspected drug.

Objective: Mycosis fungoides is the most common type of primary cutaneous T-cell lymphomas. In this study, we aimed to investigate the biochemical parameters of beta-2 microglobulin, lactate dehydrogenase, CD4/CD8 lymphocyte ratio determined by flow cytometry, and T cell clonality in patients with MF and to correlate these parameters in stage 1A and other stages. Materials and Methods: A hundred sixty-one (87 Male, 74 Female) patients followed-up between January 1995 and January 2019 were retrospectively evaluated. Patients’ demographics, stage of the disease, peripheral lymph node and organ involvement, peripheral blood beta-2-microglobulin, lactate dehydrogenase levels, CD4/CD8 + lymphocyte ratio and presence of T cell clonality in biopsy samples were evaluated. Results: Median beta-2 microglobulin levels were 1608.5 ng/ml for stage 1A and 1970.5 ng/ml for other stages. Peripheral blood median CD4/CD8 lymphocte ratio was 1.60 for stage 1A and 1.85 for other stages. We found statistically significant difference between two groups in terms of beta-2 microglobulin levels and peripheral blood median CD4/CD8 lymphocte ratio (p=0.001, p=0.04). No statistically significant difference was found between two groups in terms of lactate dehydrogenase levels and T-cell clonality (p=0.234, p=0.525). Conclusion: Our study supports that high peripheral blood beta-2 microglobulin level and CD4/CD8 lymphocyte ratio at the time of diagnosis may imply advanced stage and poor prognosis in Mycosis Fungoides.

Acquired reactive perforating collagenosis is a rare skin disorder characterized by the presence of umbilicated pruritic papules and nodules. Transepidermal elimination of altered and perforating bundles of basophilic collagen from the epidermis is a characteristic histologic feature of acquired reactive perforating collagenosis. Along with its well-known association with systemic diseases such as diabetes mellitus, chronic renal failure, and dermatomyositis, there are reports of acquired reactive perforating collagenosis being associated with malignancies. Herein, we present a case of acquired reactive perforating collagenosis associated with chronic lymphocytic leukemia, prostate adenocarcinoma, and Graves's disease. Clinicians are required to be more vigilant in evaluating patients with acquired reactive perforating collagenosis due to its unique association with malignancies and other systemic diseases.

Aim This study aimed to determine the characteristics of dermatology consultation requests from the adult and paediatric emergency departments (EDs) of a university hospital during 8 months of the COVID‐19 pandemic in 2020 and to compare them with the same 8 months of 2019. Materials and Methods Electronic medical records of dermatology consultation requests from adult and paediatric EDs between 15 March 2019 and 15 November 2019, and between 15 March 2020 and 15 November 2020 were retrospectively reviewed. Results The study included 495 consecutive dermatology consultation requests. In total, 283 (57%) consultation requests occurred in 2019, vs 212 (43%) between in 2020 during the COVID‐19 pandemic. The number of consultation requests per day was significantly lower in 2020 (0.9 ± 0.1 per day) than in 2019 (1.15 ± 0.1 per day; P = .002), and was significantly lower in March, April and May 2020, as compared with March, April, and May 2019 (P = .004, P = .001, and P = .001, respectively). The median time from onset of dermatological symptoms to ED presentation was significantly longer in 2020 than in 2019 (4 days in 2019 vs 7 days in 2020; P

BACKGROUND Lichen sclerosus et atrophicus (LSA) is a chronic inflammatory cutaneous disease. AIM To evaluate the characteristics of paediatric LSA patients in Turkey. MATERIAL AND METHODS Data for patients aged

Introduction: Cicatricial alopecia (CA) is a group of disorders that lead to permanent hair loss due to irreversible damage to hair follicles. The aim of this study is to examine clinical and demographic characteristics of patients diagnosed with primary lymphocytic cicatricial alopecia (PLCA).Methods: Patients admitted to our outpatient clinic between 2000 and 2010 and who were diagnosed with PLCA were retrospectively evaluated for demographic characteristics, dermatologic findings, personal and family history, histopathologic results, therapeutic options given to the patients, and patient responses to the treatments.Results: Thirty-six patients were included in this study. Histopathological examination of scalp hair samples revealed that findings were concordant with lichen planopilaris (LPP) in 26 patients, discoid lupus erythematosus (DLE) in 7 patients, pseudopelade de Brocq (PB) in 3 patients. The main first-line treatment most commonly reported was intralesional corticosteroid injection (ILCS, n=16, 44.4%), which was followed by hydroxychloroquine (n=10, 28%). Nineteen of the 36 patients responded to the first-line treatments, whereas 17 patients needed another treatment. Treatments that patients were most likely to respond to were hydroxychloroquine and ILCS for LPP patients; hydroxychloroquine for DLE patients; hydroxychloroquine, systemic corticosteroid, and topical corticosteroid treatments for PB patients.Conclusion: PLCA is an entity widely observed in middle-aged women and is mostly encountered in the form of LPP. In the current study, patients most likely benefited from ILCS and hydroxychloroquine treatments.

Neurofibromatosis type 1 (NF1) is the most common neurogenetic disorder worldwide, and its clinical presentations are highly variable. NF1 is caused by mutations in the NF1 gene, and 50% of NF1 cases are sporadic, which occur in the absence of a family history of the disease and usually result from a new mutation in the germline of a parent. Advanced paternal age may increase the risk for germinal NF1 mutations; however, some dominant conditions, including neurofibromatosis, have shown a lesser association with paternal age, although there are conflicting reports in the literature. We investigated the effects of paternal and maternal age in 241 NF1 patients (121 sporadic and 120 familial cases) who were seen in Hacettepe hospital, a reference center for genetic diseases in Turkey. For statistical analysis, Spearman’s and Chi-square tests were used. In this study, we evaluated paternal and maternal age at birth in sporadic and familial cases of NF1. We also compared the effect of parental age on the appearance and coexistence of various NF1 symptoms. There were no significant statistical differences between paternal age and coexistence of the NF1 symptoms. However, a slightly negative correlation was observed between paternal age and the coexistence of NF1 symptoms in familial cases (p

Background: Evidence regarding the vascular endothelial growth factor A (VEGFA) as a potent mediator of angiogenesis and inflammation in psoriasis has revealed variations in this gene as surrogate markers of psoriasis. Objective: VEGFA gene polymorphisms (−1540 C/A, −1512 Ins18, −460 T/C, and +405 C/G) in psoriasis susceptibility in Turkish population were investigated. Methods: A total of 200 age, sex and ethnicity-matched psoriatic and healthy individuals were examined for clinical type, response to therapy, serum VEGFA and its receptor levels, genotypes and haplotypes. Results: The +405 GG, +405 CG, −1540 CA, and −1512 +Ins18 genotypes conferred a significant risk for developing psoriasis. The C-InsTC haplotype in the controls and C+InsTG, A+InsTC, and A-InsTG haplotypes in psoriatic patients were observed to be significantly high. Increased serum levels of VEGFA were detected in psoriatic patients with the C-InsTC haplotype than that in the controls. The +405 GG genotype was significantly more frequent in psoriatic patients with a positive family history, and the moderate form of psoriasis was more frequent among C+InsTG haplotype carriers than that among the other patients. The +405 GG genotype was found to be more frequent in patients responding to oral retinoids. Serum VEGFR1/FLT1 and VEGFR2/KDR levels were not significantly different when psoriatic patients and controls were stratified based on the risk polymorphic variants. Conclusion: VEGFA gene +405 GG and CG, −1512+Ins18, and −1540 CA genotypes are associated with an increased risk of psoriasis in Turkish population. The G allele at +405 and an 18-bp insertion at −1512 are primarily the risk factors for psoriasis, and this risk is potentiated by the presence of the A allele at the −1540 locus. (Ann Dermatol 28(1) 30∼39, 2016)

BACKGROUND The negative effect of genital and extragenital warts on adult patient quality of life (QoL) is well known; however, the literature lacks data on the effect of extragenital warts on Turkish pediatric patient QoL. The aim of this study was to determine the effect of extragenital warts that persist for ≥6 months on Turkish pediatric patient QoL, as well as to determine the relationship between patient demographic and clinical characteristics, and QoL. METHODS The Pediatric Quality of Life Inventory Version 4.0 (PedsQLTM 4.0) was administered to 85 children and their parents (patient group), and 85 age- and gender-matched children without any skin disease and their parents (control group). Children`s Dermatology Life Quality Index (CDLQI) was administered to the patients. Higher CDLQI and PedsQLTM are indicative of lower QoL. RESULTS Median wart duration and median age at the time of wart onset was 12 months (range 6-84) and 10 years (range 1-16), respectively. In the patient group mean (±Standard deviation [SD]) CDLQI score was 5.20 ± 5.97, and warts had the greatest negative effect on CDLQI symptoms and feelings scores. Mean (±SD) PedsQLTM total score was higher in the affected patients than that for the controls (23.42 ± 12.33 versus 15.81 ± 7.37, P < 0.001), and school, social and emotional functionality subscales exhibited the greatest differences between these groups. Mean (±SD) PedsQLTM total score for the patients` parents was higher than that for the controls` parents (25.94 ± 12.49 versus 17.81 ± 6.87, P < 0.001), and social and emotional functionality subscales exhibited the greatest difference between these groups. CONCLUSIONS The findings show that Turkish children with warts that persist for ≥6 months had lower QoL than the controls.

Background and Design: Alopecia areata (AA) is a frequent type of acquired hair loss with a sudden onset. The aim of this study was to investigate the epidemiological and clinical features of AA and diseases associated with AA in the Turkish pediatric population. Materials and Methods: We retrospectively evaluated the medical data of patients under 16 years of age who were admitted to the pediatric dermatology outpatient clinic at Hacettepe University Hospital between 2006 and 2011 with a diagnosis of AA, alopecia totalis (AT) and alopecia universalis (AU). Results: A total of 89 patients (34 females and 55 males), were included in this study. The mean age of the subjects was 9.8 +/- 3.4 years (range:18 months-16 years). 9% (n=8) patients had other autoimmune diseases: Hashimoto's thyroiditis in 5, vitiligo in 1, juvenile rheumatoid arthiritis (JRA) in 1 and morphea in 1 patient. AA was located on the scalp in 86.5% (n=77) of the patients, 7.9% (n=7) of the patients had AA on the scalp together with eyelashes, eyebrows and the body. AT was noted in 1, AU was noted in 4 patients. Severity of hair loss was

Background and designIn recent years, there are studies about role of dysfunctioning of the peripheral cutaneous nevre fibres in the etiology of chronic urticaria. Similiarly to urticaria, dysfunctioning of peripheral cutaneous nevre fibres was also charged in etiology of fibromiyalgia syndrome which was accepted to be the one of the chronic pain syndromes.The aim of this study was to assess the frequency of fibromiyalgia syndrome in patients with chronic urticaria and to show whether this incidence was affected from clinical findings of urticaria.Material and method50 patients with chronic urticaria and 48 controls were included into the study. Besides an autologous serum skin test, urticaria activity score (UAS) was calculated for the evaluation of the severity of the disease. The diagnosis of fibromiyalgia syndrome was made in accordance to American Collage of Rheumatology 1990 criteria. Patients are also divided into 2 groups according to disease duration and UAS.Results26% of the patients with chronic urticaria and 20.8% of the control group have fibromiyalgia syndrome and no significant difference was observed between each group (p=0.715). There was also no significant difference between patients with positive autologous serum skin test and patients with negative autologous serum skin test. When the patients divided into 2 groups according to UAS were compared with respect to frequency of fibromiyalgia syndrome, no significant difference was observed (p=0.197). Furthermore, when the patients divided into 2 groups according to disease duration were compared with respect to frequency of fibromiyalgia syndrome, there was also no significant difference (p=0.645).ConclusionIn conclusion, contrast to the recently reported study, we observed that frequency of fibromiyalgia syndrome does not increase in chronic urticaria and this frequency was not effected from the severity of urticaria, disease duration and positivity of autologous serum skin test.Key words: chronic urticaria, fibromiyalgia, frequency

We describe seven Turkish children with DOCK8 deficiency who have not been previously reported. Three patients presented with typical features of recurrent or severe cutaneous viral infections, atopic dermatitis, and recurrent respiratory or gastrointestinal tract infections. However, four patients presented with other features. Patient 1-1 featured sclerosing cholangitis and colitis; patient 2-1, granulomatous soft tissue lesion and central nervous system involvement, with primary central nervous system lymphoma found on follow-up; patient 3-1, a fatal metastatic leiomyosarcoma; and patient 4-2 showed no other symptoms initially besides atopic dermatitis. Similar to other previously reported Turkish patients, but in contrast to patients of non-Turkish ethnicity, the patients' lymphopenia was primarily restricted to CD4(+) T cells. Patients had homozygous mutations in DOCK8 that altered splicing, introduced premature terminations, destabilized protein, or involved large deletions within the gene. Genotyping of remaining family members showed that DOCK8 deficiency is a fully penetrant, autosomal recessive disease. In our patients, bone marrow transplantation resulted in rapid improvement followed by disappearance of viral skin lesions, including lesions resembling epidermodysplasia verruciformis, atopic dermatitis, and recurrent infections. Particularly for patients who feature unusual clinical manifestations, immunological testing, in conjunction with genetic testing, can prove invaluable in diagnosing DOCK8 deficiency and providing potentially curative treatment.

Infantile hemangioma (IH) is the most common benign tumor of infantile period. It appears few weeks after birth, grows rapidly in the first 4-6 months. Subsequently, growth stops and involution period which lasts about few years starts. It is more common in girls, premature infants, birthweight < 1500 gr, multiple gestations and Caucasians. Pathogenesis is not clearly known, however role of fetal hypoxic stres is considered. IH is a self-resolving benign lesion, yet in some localisations it may threaten organ function. Periorbital IH may cause ambylopia, astigmatism and strabismus. IH localised to beard area can lead to airway obstruction. Large facial IH can be a component of PHACE syndrome. Mid line lumbosacral IH can be associated with spinal dysraphism. Other complications of IH include ulceration, heart failure, hypothyroidism, cosmetic dysfiguration. Treatment is indicated in ulcerated lesions, life/function threatening situations and lesions that can cause cosmetic nuissance. Corticosteroids, propranolol, interferon, vincristin, laser and surgery are used for this purpose. (Turkderm 2011; 45 Suppl 2: 133-7)

Objective: Leukoclastic vasculitis is an inflammatory disease of the small vessels which frequently presents with skin and joint signs. The aim of the present study is to determine the clinical and demographical characteristics of the patients diagnosed as leukocytoclastic vasculitis in our clinic. Materials and Methods: Patients diagnosed to have leukocytoclastic vasculitis in our clinic between 2000 and 2009 were included in the study. Demographic characteristics of the patients as well as localization and type of the cutaneous lesions, etiology, symptoms, presence of extracutaneous symptoms, histopathological evaluation and treatment options, were retrospectively evaluated.Results: Median age of the 60 patients (36 female, 24 male) was 44 years. Pruritus (25%, n=15) and pain (18.3%, n=11) were the most frequent symptoms. Cutaneous lesions were most frequently localized in the lower extremity and usually presented as palpable pruritic papules and plaques. The most frequent etiologic factor was drugs (25%, n=15). Systemic corticosteroids were the most frequently used therapy of choice (38%, n=23). Conclusion: Leukocytoclastic vasculitis is a benign self limited disease which is frequently triggered by drugs. The most frequent clinical form is palpable pruritic papules or plaques localized in the lower extremity. Defining the etiologic cause is an important step to therapy, while systemic corticosteroid is an effective therapeutic choice.

Erythematous dermatoses are common diseases encountered in the dermatology clinic. Figurate erythemas comprise a huge part of erythematous dermatoses. Herein, figurate erythemas are discussed under the headings of erythema annulare centrifugum, erythema gyratum repens, erythema chronicum migrans, necrolytic migratory erythema, erythema marginatum rheumaticum, familial annular erythema, annular eruption of lupus erythematosus and erythema in chronic granulamatous disease carriers. Additionally, facial and palmar erythema are reviewed.

Aim: Spitz nevus is a benign, mostly acquired melanocytic nevus, which is commonly seen in childhood. The aim of this study was to describe the clinical, dermatoscopic, and histopathological features of Spitz nevus. Methods: Clinical data, histopathological slides, and dermatoscopic images of the patients that were histopathologically or clinically diagnosed with Spitz nevus between 2000-2005 were retrospectively analyzed. For statistical analysis, descriptive statistics and chi-square tests were used. Results: Among the 30 patients included in the study, 56.7% were female. Median patient age was 15.5 years (range: 3-49 years) and 40% were younger than 12 years of age. The most frequent clinical diagnoses before biopsy were Spitz nevus (33%) and melanocytic nevus (30%). Median disease duration was 12 months (range: 1month-20 years) and mean diameter of the lesions was 5 mm. Lesions were most commonly localized on the lower extremities (44.8%) in both children and adults, and lower extremity localization was more common in females (P=0.007). The most common histopathological types noted were Spitz nevus 53.5% (n=15) and Reed nevus 32% (n=9). Dermatoscopy was performed in 16 patients; 31.3% had reticular pattern, 18.8% had starburst pattern, and 18.8% had atypical pattern. Conclusion: Spitz nevi were seen in both children and adults. Pigmented Spitz nevus, which resembles melanocytic nevus, was the most common type and lower extremity localization was more common in females.

Aim: Spitz nevus is a benign, mostly acquired melanocytic nevus, which is commonly seen in childhood. The aim of this study was to describe the clinical, dermatoscopic, and histopathological features of Spitz nevus.Methods: Clinical data, histopathological slides, and dermatoscopic images of the patients that were histopathologically or clinically diagnosed with Spitz nevus between 2000-2005 were retrospectively analyzed. For statistical analysis, descriptive statistics and chi-square tests were used.Results: Among the 30 patients included in the study, 56.7% were female. Median patient age was 15.5 years (range: 3-49 years) and 40% were younger than 12 years of age. The most frequent clinical diagnoses before biopsy were Spitz nevus (33%) and melanocytic nevus (30%). Median disease duration was 12 months (range: 1month-20 years) and mean diameter of the lesions was 5 mm. Lesions were most commonly localized on the lower extremities (44.8%) in both children and adults, and lower extremity localization was more common in females (P=0.007). The most common histopathological types noted were Spitz nevus 53.5% (n=15) and Reed nevus 32% (n=9). Dermatoscopy was performed in 16 patients; 31.3% had reticular pattern, 18.8% had starburst pattern, and 18.8% had atypical pattern.Conclusion: Spitz nevi were seen in both children and adults. Pigmented Spitz nevus, which resembles melanocytic nevus, was the most common type and lower extremity localization was more common in females.

Background and Design: This study is designed to determine the prevalence and the clinical spectrum of skin diseases in renal transplant recipients (RTRs).Material and Method: In this study RTRs who were referred to our department between 2005 and 2007 for dermatologic examination were evaluated. Dermatologic investigation included direct clinical observation and culture or histolopathological investigation when indicated. Patients were divided into three groups: group A, post-transplantation periods £1 year; group B, post-transplantation periods of 1-5 years; and group C, post-transplantation periods >5 years. Results: In this study 88 (M=50, F=38) RTRs were evaluated. The mean age was 37 ± 12 years and the median interval since transplantation was 38.5 months (range=1 month-27 years). Over a 2-year period 298 cutaneous manifestations were identified. Ninety-five immunosuppressive (IS) drug-related manifestations were observed in 58 (%65.9) patients and the most common one was acneiform eruption (n=23). Forty (45.5%) patients developed cutaneous viral infections, consisting of verruca vulgaris (n=29), herpes zoster (n=9), herpes simplex (n=5), molluscum (n=2) and varicella (n=1) infections. Superficial fungal infections were observed in 35(39.2%) patients, most common lesions were dermatophytosis (n = 23) and pityriasis versicolor (n=17). Bacterial infections were observed in 14 (%16) patients, folliculitis was present in 12 of them. Premalignant and malignant lesions were identified in 12 (%13.6) patients, consisting of actinic keratoses (n=9), basal cell carcinoma (n=2), squamous cell carcinoma (n=1) and Kaposi's sarcoma (n=1). There were more premalignant and malignant lesions in patients receiving azathioprine (p=0.002). Cutaneous viral infections were more common in group C (p=0.023) and IS drug-related manifestations were more common in group A (p=0.003). Conclusion: Most common cutaneous manifestation among RTRs was IS drug-related and seen in early post-transplantation period. Warts were the most common manifestation in late post-transplantation period. (Turkderm 2008; 42: 18-21)

The cutaneous features of some genetic diseases that manifest during childhood might be the first signs of predisposition to cancer; therefore, early recognition of such clinical findings as well as necessary follow-up are vital. This review focuses in particular on nevoid basal cell carcinoma, Cowden syndrome, neurofibromatosis type 1, tuberous sclerosis, Peutz-Jegher's syndrome, dyskeratosis congenita, and xeroderma pigmentosum. These genodermatoses, are characterised by predisposition to skin cancer or cancer in other organs; as such this review aimed to highlight their diagnostic clinical features.

Acute generalized exanthematous pustulosis (AGEP) is a rare skin disorder, characterized by acute development of numerous, pin-head sized, nonfollicular pustules on erythematous skin, with high fever and neutrophilia. The condition is frequently caused by hypersensitivity reaction to drugs or viral infections. Diagnosis is established according to clinical and histological criteria. Herein, we report a 17-year-old girl with localized AGEP related to the use of amoxicillin-clavulanate.

Hereditary deficiency of complement component C1q is a rare genetic disorder with susceptibility to recurrent infections with polysaccharide-containing encapsulated microorganisms and a high prevalence of autoimmune diseases, most often systemic lupus erythematosus (SLE). Here, we report a 29-month-old boy who presented with facial rash and history of early death of a sibling with infections, who was found to have a selective deficiency of C1q. The facial rash was composed of patchy erythematous plaques and centrally hypopigmented macules and desquamation. Two siblings had died of severe bacterial infections and his uncle had died of meningitis. Molecular study disclosed a homozygous point mutation in the C1qA chain gene. Five members of the family, including the parents and three healthy siblings, were heterozygous for this mutation.

Mast cell disorders are defined by an abnormal accumulation of tissue mast cells in one or more organ systems. In systemic mastocytosis, at least one extracutaneous organ is involved by definition. Although, systemic mastocytosis usually represents with skin lesion called urticaria pigmentosa, in a small proportion, there is extracutaneous involvement without skin infiltration. Other manifestations are flushing, tachycardia, dyspepsia, diarrhea, hypotension, syncope, and rarely fever. Various medications have been used but there is not a definite cure for systemic mastocytosis. The principles of treatment include control of symptoms with measures aimed to decrease mast cell activation. We describe a case of systemic mastocytosis presenting with hypotension, syncope attacks, fever, and local flushing. In bone marrow biopsy, increased mast cell infiltration was demonstrated. She had no skin infiltration. A good clinicopathological response was obtained acutely with combination therapy of glucocorticoid and cyclosporine.

Chanarin-Dorfman syndrome (CDS) is an autosomal recessive metabolic disorder associated with congenital ichthyosis and a multisystemic accumulation of neutral lipids in various types of cells. Recently, mutations of the ABHD5 gene were identified as the cause of CDS. In this work, we carried out molecular analysis of the ABHD5 gene in 6 unrelated patients. We identified one previously reported mutation, N209X and two novel genetic alterations; a nonsense mutation (p.Y50X) and missense mutation (p.S73A).

Background: Little data exist about the efficacy of phototherapy in childhood dermatoses. Objective: To report our experience with pediatric patients treated with phototherapy. Methods: The study included children ≤17 years of age, who were treated in our phototherapy unit between 1985 and 2005. Data were retrospectively collected. Results: The study included 113 patients (50 boys, 63 girls) with a median of age of 13 years (range: 3–17 yrs). Narrow-band ultraviolet-B (34.6%), and ultraviolet-B (33.1%) were the most common treatments administered to the patients. Indications for phototherapy were psoriasis in 53.5%, vitiligo in 20.5%, pityriasis lichenoides in 14.2%, alopecia areata in 7.9%. Response was achieved in 92.9% of the psoriasis patients treated with narrow-band ultraviolet-B, in 83.3% treated with psoralen plus ultraviolet-A, and in 93.3% with ultraviolet-B. All the pityriasis lichenoides chronica patients who received narrow-band ultraviolet-B responded to the treatment and seven of nine treated with ultraviolet-B had a response. Response was achieved in 57% of the vitiligo patients treated with psoralen plus ultraviolet-A and in 50% of vitiligo patients treated with narrow-band ultraviolet-B. Of the 10 alopecia areata patients, two responded to psoralen plus ultraviolet-A. Among all the patients, erythema was the most common adverse effect. Conclusion: Phototherapy is a well-tolerated treatment for childhood dermatoses, and is especially efficacious in psoriasis and pityriasis lichenoides chronica patients.

Background and Design: This study is designed to determine the prevalence and the clinical spectrum of skin diseases in renal transplant recipients (RTRs). Material and Method: In this study RTRs who were referred to our department between 2005 and 2007 for dermatologic examination were evaluated. Dermatologic investigation included direct clinical observation and culture or histolopathological investigation when indicated. Patients were divided into three groups: group A, post-transplantation periods :51 year; group B, post-transplantation periods of 1-5 years; and group C, post-transplantation periods >5 years. Results: In this study 88 (M=50, F=38) RTRs were evaluated. The mean age was 37 12 years and the median interval since transplantation was 38.5 months (range=1 month-27 years). Over a 2-year period 298 cutaneous manifestations were identified. Ninety-five immunosuppressive (IS) drug-related manifestations were observed in 58 (%65.9) patients and the most common one was acneiform eruption (n=23). Forty (45.5%) patients developed cutaneous viral infections, consisting of verruca vulgaris (n=29), herpes zoster (n=9), herpes simplex (n=5), molluscum (n=2) and varicella (n=1) infections. Superficial fungal infections were observed in 35(39.2%) patients, most common lesions were dermatophytosis (n = 23) and pityriasis versicolor (n=17). Bacterial infections were observed in 14 (%16) patients, folliculitis was present in 12 of them. Premalignant and malignant lesions were identified in 12 (%13.6) patients, consisting of actinic keratoses (n=9), basal cell carcinoma (n=2), squamous cell carcinoma (n=l) and Kaposi's sarcoma (n=l). There were more premalignant and malignant lesions in patients receiving azathioprine (p=0.002). Cutaneous viral infections were more common in group C (p=0.023) and IS drug-related manifestations were more common in group A (p=0.003). Conclusion: Most common cutaneous manifestation among RTRs was IS drug-related and seen in early post-transplantation period. Warts were the most common manifestation in late post-transplantation period. (Turkderm 2008; 42: 18-21)

Autoimmune progesteron dermatitis (AIPD) is a rare disorder which is characterized by cyclical premenstrual flares of cutaneous or mucocutaneous manifestations. We present a 36-year-old woman with a 2-year history of oral ulcers and a rash which presents a week before every menstruation. She also had a history of recurrent herpes labialis infection; however herpes simplex virus could not be detected by PCR in oral lesions. Routine laboratory investigations, as well as serum complement levels were normal, except for elevated ANA titer (1/160). Histopathological examination of one of the papules was consistent with erythema multiforme and immunobullous diseases were ruled out by negative immunofluorescence studies. Based on the history and the clinical features, AIPD was suspected and the diagnosis was confirmed by flare of the lesions after progesterone challenge test. Subsequently, oral tamoxifen was started which controlled her flares significantly and no major side effects except amenorrhea was observed during treatment. Next she was given spironolactone which did not control her symptoms, therefore oral prednisolone had to be introduced. Due to its rare occurence, clinical features, pathogenesis, diagnostic and treatment alternatives of AIPD are discussed in this study based on the review of the medical literature. (Turkderm 2009, 43: 122-5)

Infantile systemic hyalinosis (ISH) is an autosomal recessive, rare disorder in which hyaline deposition occurs in multiple organ systems, including the skin. It is characterised by painful joint contractures, gingival hypertrophy, generalized osteopenia, small pearly papules on the head, flesh nodules in the perianal region, and usually thickened skin. The onset occurs within the first few weeks of life and death occurs by two years of age as a result of recurrent pulmonary infections and diarrhea. Unfortunately, treatment is primarily palliative as there is no cure currently available. Herein, we report an 8-month-old boy who is diagnosed with ISH with the characteristic clinical presentation and histopathological findings. (Turkderm 2009; 43: 112-5)