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Immune mechanisms are considered to be responsible for the pathogenesis of cicatricial alopecia in lichen planopilaris (LPP) and discoid lupus erythematosus (DLE) diseases. CD200 has an immunomodulatory function in hair follicles. The functions of Merkel cells (MCs) in hair follicles remain to be fully understood.This study aimed to determine the number and distribution of MCs as well as CD200 expression in patients with DLE and LPP.Using immunohistochemistry, the number and distribution of MCs (staining with CK20) and CD200 expression in biopsy specimens of LPP and DLE patients were compared with control group patients.The number of follicular MCs, total MCs, mean follicular MCs, and CD200 expression were significantly lower in the case groups compared to the control group. In CD200MC loss might play a role in immune privilege collapse in hair follicles. This study is novel in terms of investigating MCs in DLE and LPP patients.

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.

The features of chronic rejection (CR) in full-face vascularized composite allotransplantation (VCA) are not well-known. Herein, we report a full-face transplant patient that experienced two episodes of acute rejection (AR) and three episodes of AR/CR over the course of 6-years. The patient noticed a small, round patch of hair loss in his beard 9 months after the second AR episode, which occurred 21 months post-transplantation. Biopsy of the alopecic patch showed lichen-planopilaris-like features, which were suggestive of early CR. Despite an increase in immunosuppressive dosages, the alopecia progressed. Following the second and third AR/CR episodes, the alopecia became more pronounced, with the addition of hyperpigmentation as well as sclerosis and telangiectasia. The findings of multiple biopsies showed CR. Based on these findings we think that alopecia with lichen-planopilaris-like histopathological features similar to grade III AR features, particularly in hair follicles appears to be an early finding of CR in the presented patient. The findings further indicate that follicular involvement may be a significant feature of CR in VCA patients and that it can present prior to sclerosis, vasculopathy, or loss of adnexa. The present case is uniquely important because of the distinctive presentation of CR, with hair follicles clinically and histopathologically affected, leading to progressive and irreversible alopecia with lichen-planopilaris-like histopathology.

Poikiloderma with neutropenia (PN), Clericuzio-type is a rare autosomal recessively transmitted genodermatosis caused by biallelic mutations in the USB1 gene and is characterized by early-onset poikiloderma and chronic neutropenia. Nail dystrophy, palmoplantar hyperkeratosis, hypogonadotropic hypogonadism, and recurrent infections can be associated with the disease. Herein, we present a 27-year-old Turkish male patient newly diagnosed as PN, Clericuzio-type after confirmation of a c.531delA (p.His179MetfsX86) homozygous deleterious mutation in exon 5 of the USB1 gene. The presented case highlights the importance of genetic testing for avoiding misdiagnosis based solely on clinical findings, as well as the benefit of a multi-disciplinary diagnostic approach, as he was initially misdiagnosed as Rothmund-Thompson syndrome and subsequently diagnosed as PN, Clericuzio-type at age 27 years.

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.

Epidermolysis bullosa simplex migratory circinate erythema (EBS-Migr) is an uncommon subtype of EBS. We report a case of EBS-MIGR with a novel heterozygous pathogenic mutation in exon 9 (frameshift deletion c.1650delC) and likely benign heterozygous mutation in exon 2 (missense c.591C > A) of keratin 5. This novel pathogenic mutation in KRT5 expands the molecular spectrum of this rare subtype of EBS.

Unnecessary dermatology consultation requests from emergency departments (EDs) are a common occurrence worldwide.This study aimed to analyze the demographic and clinical characteristics of patients consulted to the dermatology department for dermatologic disorders by a university hospital's pediatric ED (PED) and adult ED (AED).The electronic medical records of 2316 dermatology consultation requests from the PED and AED during a 5-year period were retrospectively reviewed. Patient demographic and clinical characteristics, dermatological diagnoses, and time of day of dermatology consultation requests from the PED and AED were retrospectively analyzed.The electronic medical records of 1845 consultation requests with complete data were included in the study. There were 969 (52.5%) consultation requests from the PED and 876 (47.5%) from the AED. Mean time from onset of dermatological symptoms to ED presentation was 31.6 d. Herpes zoster infections (18.5%), adverse cutaneous drug reactions (8.1%), and urticaria with angioedema (7.9%) were the most common skin disorders resulting in consultation requests from the AED, versus non-specific viral infections (9.2%), insect bites (8.3%), and atopic dermatitis (8.2%) from the PED. In all, 11.5% of ED patients that received dermatology department consultation required hospitalization due to dermatologic disorders.As patients commonly present to EDs with non-urgent dermatological diseases, ED physicians should receive training on common dermatological diseases so as to decrease the number of unnecessary dermatology consultation requests.

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 Rosacea is a chronic disease that is characterized by facial skin inflammation and vascular abnormality. Vascular endothelial growth factor (VEGF) is a potent mediator of vascular permeability and inflammation that might play a role in the pathogenesis of rosacea. Objective This study aimed to determine the association between VEGF gene polymorphisms and rosacea. Methods A case-control study design was used to compare 100 patients with rosacea and 100 age- and gender-matched control subjects in terms of VEGF polymorphisms based on polymerase chain reaction and the serum level of VEGF and VEGF receptors based on enzyme-linked immunosorbent assay. Results Heterozygous and homozygous +405C/G polymorphism of the VEGF gene was observed to increase the risk of rosacea 1.7-fold (95% confidence interval 1.2-4.2) and 2.3-fold (95% confidence interval 1.2-4.2), respectively. There was a significant positive correlation between the severity of rosacea and +405C/G polymorphism of the VEGF gene in patients with erythematotelangiectatic rosacea. Limitations Serum VEGF and VEGF receptor levels were measured in the limited number of patients. Conclusion The present findings indicate that +405C/G polymorphism of the VEGF gene increases the risk of rosacea.

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

The vast majority of primary immunodeficiencies (PIDs) occur due to the defects in cells originating from hematopoietic stem cells, while in some PIDs, there are defects in various genes responsible for non-leucocyte immune response such as seen in epidermodysplasia verruciformis (EV). EV caused by the mutations in TMC6, TMC8, and CIB1 genes is called "typical." "Atypical" EV may develop in patients with primary immunodeficiencies originating from hematopoietic stem cells, which include severe T-cell failure, caused by inactivating biallelic mutations of STK4, RHOH, CORO1A, ITK, TPP2, DCLRE1C, LCK, RASGRP1, or DOCK8 genes. Here, we present a family with TMC8 gene mutation leading to disseminated epidermodysplasia verruciformis including laryngeal papilloma and recurrent cutaneous squamous cell carcinomas. Typical EV with impaired local, keratinocyte-intrinsic immune response should be considered when routine immunological examinations are normal in patients presenting with clinical signs of EV. Although it is not possible to prevent EV lesions, early and appropriate surveillance for malignancy is mandatory.

The risk of active tuberculosis is still a concern in patients receiving biologics. To determine the risk of latent tuberculosis infection (LTBI) reactivation by Quantiferon-TB Gold (QFT) assay in psoriatic patients treated with biologics in 11 years' follow-up, along with chest radiography alterations. This retrospective study included 279 patients with plaque-type and/or pustular, or nail psoriasis who were treated with biologics, and had results for ≥2 LTBI tests. The QFT outcomes were defined according to the baseline and the follow-up QFT results; seroconversion as from negative to positive, seroreversion as from positive to negative, persistently seronegative as invariantly negative, persistently seropositive as invariantly positive, and other any result was accepted as indeterminate. Demographic features, the presence and the type of any chest X-ray abnormality was noted during the follow-up. Of 279 baseline QFT tests, the vast majority were negative (n = 193; 69%), with a less of positive (n = 86; 31%). Ten (5.2%) out of 193 patients converted from negative to positive QFT status after starting biologic therapy (P < 0.001) during 11 years' follow-up. Although these 10 patients exhibited seroconversion of QFT from negative to positive, only one patient was diagnosed with active TB. There was no statistically significant difference among biologics as regards with QFT seroconversion risk (P = 0.09). This study showed that 5.2% of patients showed seroconversion. Annual QFT testing remains a necessary and mandatory tool to prevent further TB reactivation in psoriasis patients taking biologic therapy although only one patient was diagnosed with active TB in this cohort. This article is protected by copyright. All rights reserved.

Recent reports indicate that hydroxychloroquine is a potential new treatment option for alopecia universalis; thus, we aimed to report on the safety and efficacy of hydroxychloroquine in 6 patients with refractory alopecia universalis that were treated with 400 mg/d continuously for ≥6 months. The treatment outcome was retrospectively evaluated using the Severity of Alopecia Tool (SALT), and at the end of 6 months, patients with a ≥50% decrease in the SALT score were considered as strong responders, a 5%-50% decrease as intermediate responders and a

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.

Alopecia areata (AA) is a common disease that results in nonscarring hair loss. Recently, tofacitinib (TOFA) has been a novel promising therapy for AA. The aim of this study is to determine the efficacy of oral TOFA treatment for alopecia areata (AA), and alopecia universalis (AU). Data of nine patients treated with oral TOFA with either AA or AU were retrospectively evaluated. Treatment outcome, disease severity, and therapeutic response were evaluated by Severity of Alopecia Tool (SALT) scores along with regular photographic surveillance done at third and sixth months. Treatment response was established on four categories: complete response (90% change in latest SALT score), intermediate response (51-90% change), moderate response (6-50% change), and nonresponse (

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 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 Recurrent aphthous stomatitis (RAS) is a common disease of the oral mucosa with an unknown etiology. This study aimed to determine if food additives play a role in the etiology of RAS as well as to determine if patch testing can be used to detect which allergens cause RAS. Methods This prospective study included 24 patients with RAS and 22 healthy controls. All the participants underwent patch testing for 23 food additives. Results In total, 21 (87.5%) RAS patients and 3 (13.6%) controls had positive patch test reactions to ≥1 allergens; the difference in the patch test positivity rate between groups was significant (P < 0.05). The most common allergen that elicited positive patch test results in the patient group was cochineal red (n = 15 [62.5%]), followed by azorubine (n = 11 [45.8%]) and amaranth (n = 6 [25%]). Conclusions The present findings show that food additives might play a role in the etiology of RAS and that patch testing could be a method for determining the etiology of RAS.

Background/Objective Diaper dermatitis (DD) is a common infantile dermatosis with a highly variable prevalence and incidence. This study aimed to present the demographic and clinical features of babies with DD. Methods Data, including demographic and clinical features, obtained from DD patient forms were retrospectively analyzed. Results The study included 63 babies with DD (female: n = 35 [55.6%]; male: n = 28 [44.4%]; mean age 11.6 mos). Most of the patients (43.5%) were 0 to 6 months of age. In all, 76.3% were breastfed, of whom 31.7% were exclusively breastfed. The number of previous episodes of DD was significantly lower in breastfed babies. The most common diaper area cleansing method was wet wipes (54.1%). Diaper creams were used in 86.2% of patients. DD was severe in 22.2% of patients, moderate in 57.4%, and mild in 20.4%. Candida infection was noted in 77.4% of patients. The median number of previous episodes of DD was significantly higher in patients with Candida infection than in those without (p = 0.02). Treatment yielded greater than 50% improvement (complete and moderate response) in 59.4% of patients at the first follow-up visit (3–5 days) and 82.6% at the second follow-up visit (10 days). Conclusion Most babies with DD were 0 to 6 months of age. Breastfed babies had fewer previous episodes of DD, so mothers should be encouraged to breastfeed their babies to reduce the incidence of DD. Candida infection was common; clinicians should be aware of its association with DD.

Narrowband ultraviolet B (UVB) provides complete response (CR) in 54-91% of early mycosis fungoides (MF) patients. Data concerning relapse rate and relapse-free interval after discontinuation of therapy need clarification. The purpose of this study was to evaluate the relapse rate and the relapse-free intervals of early MF patients after achieving complete response with narrowband UVB phototherapy.Retrospective evaluation of data, which belong to early MF patients treated with narrowband UVB phototherapy between May 2000 and July 2010, and followed-up until May 2012 in a single institution, for the occurrence of relapse and the time to relapse.Of 31 patients, who were followed-up for a mean of 56.5 ± 30.2 months (median 55 months, range 20-120 months), relapse was observed in 11 (35.5%) patients, within a mean of 28.8 ± 18.2 months (median 33 months, range 4-59 months), whereas 20 (64.5%) patients stayed relapse-free for a mean of 54.2 ± 28.8 months (median 55.5 months, range 20-119 months). Patients received maintenance phototherapy with a median duration of 12 months (range 1-30 months) after achieving complete response.Results indicate that narrowband UVB phototherapy may induce low relapse rates and long relapse-free intervals for early MF.

Pityriasis lichenoides (PL) is not uncommon in childhood, but current knowledge about the efficacy of oral erythromycin therapy for its treatment in children is limited. To investigate the role of oral erythromycin therapy in the treatment of PL in children, the records of 24 children with PL who had been started on oral erythromycin treatment at our institution between 2005 and 2008 were retrospectively reviewed. The study included 24 patients (14 male, 10 female) with a median age of 7 years (range 2-14) of whom 15 (62.5%) had PL chronica (PLC), six (25%) PL et varioliformis acuta (PLEVA), and three (2.5%) PLEVA-PLC overlap. History of upper respiratory tract infection was reported in 33% (n = 8) of the patients. History of drug intake and vaccination was noted in 20% (n = 5) and 20% (n = 5), respectively. The disease began during spring (30%, n = 7) or fall (30%, n = 7) in the majority of patients. The median duration of the disease was 11 months (range 1-48 months). Fifteen (68.2%) patients had more than 100 lesions. Distribution was diffuse in 82% (n = 18) of the cases and peripheral in the remainder (n = 6). Oral erythromycin was started at a dosage of 30 to 50 mg/kg per day in three to four divided dosages for 1 to 4 months. Good response was recorded in 64% and 73% of patients in the first and second months of therapy, respectively. Response rate rose to 83% in the third month. In those for whom follow-up data were available (n = 16), relapse was recorded in 12.5% (n = 3). Oral erythromycin may be an effective and well-tolerated treatment option for PL in children and should be continued for at least 3 months.

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.

Leiomyosarcoma and epidermodysplasia verruciformis are rarely encountered in children. The association of either leiomyosarcoma or epidermodysplasia verruciformis with immune deficiency has previously been documented. A 6-year-old girl, who has had multifocal leiomyosarcomatosis after the previous diagnoses of epidermodysplasia verruciformis and immune defect, represents an interesting association of these features.

Current knowledge about the efficacy of narrowband UVB therapy for the treatment of pityriasis lichenoides is limited.The aim of this study was to investigate the efficacy of narrowband UVB therapy for the treatment of pityriasis lichenoides chronica (PLC).Data were retrospectively collected following a review of patient phototherapy and medical charts, and telephone interviews were performed for follow-up information.The study included 25 patients (14 male, 11 female) with a mean age of 34 +/- 13 years. Diagnosis was confirmed by histopathological examination in 92% (n = 23) of the patients. The median duration of the disease was 24 months (range: 2-192 months). The median number of sessions until response was 25 (8 weeks) (range: 9-77 sessions), with a median cumulative dose of 15 J/cm(2) (range: 2-158 J/cm(2)). Complete response and partial response were achieved in 48% and 44% of the patients, respectively, while 8% of the patients achieved no response. In those patients for whom follow-up data were available (n = 17), 10 (58%) relapsed within a median of 9.5 months (range: 1-44 months).Our results suggest that narrowband UVB is an effective and well-tolerated treatment option for PLC.

Background Pityriasis lichenoides (PL) occurs in all age groups, although predominantly in younger individuals. Objective We sought to study the clinical features of PL in children followed up at our institution. Methods The records of 124 children who were given the diagnosis of PL at our institution between 1993 and 2003 were retrospectively reviewed. Results PL chronica (PLC) was recorded in 37% of the cases, PL et varioliformis acuta (PLEVA) in 57.3%, and clinical features of both disorders were seen simultaneously in the remaining. The median age of onset was 60 months (range: 6-180 months), although the median age of onset of PLEVA (median: 60 months) was significantly younger than that of PLC (median: 72 months) ( P = .03). The age distribution showed peaks at 2 to 3 years (24.8%) and 5 to 7 years (32%). A history of infection or drug intake preceded the skin manifestations in 30% and 11.2% of patients with PLC and PLEVA, respectively. The disease began most commonly during winter (35%) or fall (30%). The median duration was 20 months (range: 3-132 months) in patients with PLC and 18 months (range: 4-108 months) in patients with PLEVA. Involvement was diffuse in 74.2% of the patients, peripheral in 20.2%, and central in the remainder. The disease was recurrent in 77% of the patients (n = 80). Of the patients, 59% had pruritus, whereas 32% reported no symptoms; the remainder had fever, arthralgia, or both. Erythromycin estolate or ethylsuccinate was administered to 79.7% of the affected children; 66.6% of these showed at least a partial response. Limitations The analyzed data were collected retrospectively and biopsies were not performed in all patients. Conclusions PL is not an uncommon disease in childhood, with age peaks in the preschool and early school-age years. It is usually recurrent, and shows a seasonal variation with onset most often in the fall or winter. In childhood PL, erythromycin is an effective initial treatment choice.

We report an unusual case of an inherited disorder of the desmosomal protein plakophilin 1, resulting in ectodermal dysplasia–skin fragility syndrome. The affected 6-year-old boy had red skin at birth and subsequently developed skin fragility, progressive plantar keratoderma, nail dystrophy, and alopecia. Skin biopsy revealed widening of intercellular spaces in the epidermis and a reduced number of small, poorly formed desmosomes. Mutation analysis of the plakophilin 1 gene PKP1 revealed a homozygous deletion of C at nucleotide 888 within exon 5. This mutation differs from the PKP1 gene pathology reported in 8 previously published individuals with this rare genodermatosis. However, all cases show similar clinical features, highlighting the importance of functional plakophilin 1 in maintaining desmosomal adhesion in skin, as well as the role of this protein in aspects of ectodermal development.

Bullous pemfigoid (BP), an autoimmune disorder, can also be induced by some medications. Vildagliptin is a new drug used to treat diabetes mellitus (DM). Recently, a few cases of vildagliptin-induced BP have been described in the literature. We report a patient with BP in which vildagliptin was thought to be as a possible causative agent. The awareness of BP development risk during gliptin therapy can prevent unnecessary usage of systemic drugs with serious side effects.