A comparative study of efficacy of split-thickness skin grafting versus autologous melanocyte transfer in the management of stable vitiligo.

Background: Vitiligo is a depigmenting skin disorder resulting from the loss of melanocytes in the epidermis. Surgical treatment is indicated in resistant stable vitiligo that does not show adequate response to medical therapy. This study aims at comparing the efficacy of repigmentation by split-thickness skin grafting (STSG) versus autologous non-cultured melanocyte transfer (AMT) in the management of stable vitiligo. Methods: A total of 22 patients were recruited, which included 100 stable vitiligo patches. Fifty patches were subjected to STSG, and the remaining 50 were subjected to AMT. The patients were followed up at monthly intervals for six months to assess the degree of repigmentation. Results: The results at the end of six months were as follows: 40% patches in group A and 42.5% in group B exhibited >75% repigmentation. The outcome was better over the limbs, acral parts and joints. The average donor-to-recipient area ratio for group A was 1:1, whereas that for group B was 1:3.2. Conclusion: Both STSG and AMT are effective surgical modalities in the management of stable vitiligo. However, AMT has cosmetically better good outcomes, requires a smaller size of the donor area and has minimal complications over both recipient and donor areas.

Safety and Efficacy of Restoring Facial Symmetry Using Polydioxanone Thread Face Lift Technique in Patients with Facial Palsy.

Background: Facial nerve palsy often leads to functional and physiological impairment in patients and they often suffer from social stigma. The use of polydiaxone (PDO) threads has long been used to treat facial furrows and age-related sagging in the face. The basic principle of the thread lift procedure is placing cogged threads in the subcutaneous plane along a planned trajectory. The efficacy and ease of this procedure compared to a traditional face lift has made PDO threads a popular option in the past three decades. Methods: We treated 50 patients with a drooping face on one side following facial paralysis with thread lifting and botulinum injections to control contralateral hypertrophy and ipsilateral synkinesis. The Facial Grading System or the serial Sunnybrook Facial Grading System (SB scores) was used to assess at all the visits and serial photographs were recorded. Results: Following the threading procedure, all 50 patients displayed improved facial symmetry. There was significant improvement in the baseline SB scores compared to the scores over following year, with all the scores having significant p value less than 0.05. Conclusion: Based on our results, subdermal suspension with re-absorbable threads in conjunction with botulinum toxin constitutes an efficient and safe procedure for face lifting and rejuvenation of a drooping face as a result of long-lasting facial paralysis.

A Cross-Sectional Observational Study of Clinical Spectrum and Prevalence of Fixed Food Eruption in a Tertiary Care Hospital.

BACKGROUND: Fixed food eruption (FFE) is a rare type of hypersensitivity reaction occurring after ingestion of some food items in the form of recurrent erythematous patches, bullae, vesicle, or pustule at the same site after ingestion of same or related food products. Various items listed responsible for causing FFE include tree nuts, groundnuts, legumes, lentils, eggs, fruits like kiwi, strawberry, tonic water, and tartrazine. Its more commonly reported in developed countries with no Indian studies as of yet. We studied the clinical spectrum and prevalence of FFE in a tertiary care hospital. OBJECTIVE: To study the prevalence and pattern of FFE after eliminating all other possible causes including drug rash. MATERIALS AND METHODS: A cross-section observational study of 27 consecutive patients suspected to have fixed food eruption after eliminating all possibilities of any drug reaction to the best of our knowledge. Informed consent was obtained from the patients, and ethical clearance was taken from the hospital ethical committee. RESULTS: A total of 27 patients were studied out of which 18 (66.66%) were females and 9 (33.33%) were males. The prevalence of fixed food eruption was calculated to be 0.072%.Fixed food eruption was noted secondary to cashew nuts (14.8%), almonds (7.4%), walnut (7.4%), pistachio (3.7%), strawberry (3.7%), kiwi (3.7%), and cheese crisps (3.7%). CONCLUSION: This observational study highlights the varied patterns of fixed food eruptions as well as the burden of disease in population secondary to certain diets.

Biologics in Dermatology: Off-Label Indications.

Skin and subcutaneous diseases affect millions of people worldwide, causing significant morbidity. Biologics are becoming increasingly useful for the treatment of many skin diseases, particularly as alternatives for patients who have failed to tolerate or respond to conventional systemic therapies. Biological therapies provide a targeted approach to treatment through interaction with specific components of the underlying immune and inflammatory disease processes. Advances in the understanding of disease pathophysiology for inflammatory skin diseases and in drug development have ushered in biologic therapies in dermatology. Biologic therapies are molecules that target specific proteins implicated in immune-mediated disease. This review article highlights the increasing evidence base for biologics in dermatology for off-label use.

Impact of Psychosocial Profile on Alopecia Areata in Pediatric Patients: A Case Control Study from A Tertiary Care Hospital in Eastern Uttar Pradesh.

BACKGROUND: Alopecia areata (AA) is a common form of nonscarring alopecia characterized by patchy loss of hair from the scalp and body. It is a complex outcome of factors such as autoimmunity, genetic factors, infectious diseases, as well as psychological factors, such as stress, personality type, familial conditions. Around 20% of patients are in the pediatric age group, and 60% of the patients develop AA before the age of 20 years. AIM: The present study looked into the impact of psychosocial factors in AA. MATERIALS AND METHODS: This was a case-control study conducted over a period of 1 year. One hundred and two patients and age and gender-matched control group between the ages of 2 and 14 years were included. A questionnaire was administered to identify the stress arising due to personal or familial conditions, school-related issues, psychotrauma or illness, and accidents prior to developing AA. Age and gender-matched patients with other dermatoses with low psychosomatic component to it and unlikely to be influenced by stress were selected as control. RESULT: Fifty-three patients (52 %) were male and 49 were female (48 %). Fifty-five (53.9%) patients were in the age group of 10 to 14 years. Forty (39.2%) children had multiple patches. Onset was <5 months in 30 patients (29.4%). Forty-nine (48%) children reported stress due to school-related issues compared to 13 (12.7%) in the control group. Eighteen (17.6%) children had familial issues compared to 6 (0.05%) in the control group. Nineteen children (18.6%) had multiple stressors. Sixty-nine (67.6%) patients related their disease to a stress component compared to 33 (32.3%) who could not relate to any stress. A significant association was noted between examination pressure and academic performance with onset of AA compared to control (P < 0.05%), which was stronger among female compared to male. CONCLUSION: The psychological profile and comorbidities have a significant impact on the onset or recidivism of AA. Impact of a stressful personal or family life, parental pressure to perform better in school, and psychological vulnerability can significantly contribute to the onset or exacerbation of AA.

Real-life Efficacy and Safety of Biosimilar Adalimumab (ZRC-3197) in Patients with Plaque Psoriasis: A Tertiary Care Center Experience.

BACKGROUND: Adalimumab is proven to be safe and effective in treating plaque psoriasis. A biosimilar adalimumab (ZRC-3197; Exemptia™) - approved by Indian Regulators in 2014 - is a 'fingerprint match' of the reference adalimumab in terms of purity, potency, safety, and clinical efficacy. While reference adalimumab remains unavailable, this biosimilar adalimumab (bADA) serves as an accessible, cost-effective option for Indian patients. This is a first-hand, prospective, real-life data on the clinical use of bADA in Indian patients with plaque psoriasis. MATERIALS AND METHODS: Patients with moderate-to-severe plaque psoriasis were prospectively treated with bADA therapy for 16 weeks-80 mg subcutaneously initially, followed by 40 mg every other week from week 1 in real-life setting. Psoriasis Area and Severity Index (PASI) responses, Dermatology Life Quality Index (DLQI) outcomes, and Physician's Global Assessment (PGA) for psoriasis were analyzed. Safety and tolerability evaluations included reported adverse events. RESULTS: A total of 29 patients (15 males) with median age of 38 (25-56) years were included. After 16 weeks of bADA treatment, 93% patients achieved ≥75% reduction in their baseline PASI scores including PASI75, PASI90, and PASI100 responses in 24%, 14%, and 55% patients, respectively. About 52% patients had a DLQI score of 0/1 and 93% patients had a PGA score of 'clear or minimal' at 16 weeks. Treatment was well tolerated with no severe or serious adverse reactions requiring therapy discontinuation. CONCLUSIONS: This report serves as a real-life evidence for the efficacy and tolerability of biosimilar adalimumab administered for 16 weeks in patients with plaque psoriasis.

Thrombophilia prevalence in patients seeking laparoscopic sleeve gastrectomy: extended chemoprophylaxis may decrease portal vein thrombosis rate.

BACKGROUND: Portomesenteric vein thrombosis (PMVT) may occur after laparoscopic sleeve gastrectomy (LSG). Previous studies have shown that PMVT patients may have undiagnosed thrombophilia. We recently changed our practice to check thrombophilia panel in every LSG patient preoperatively. OBJECTIVES: To estimate the thrombophilia prevalence in patients seeking LSG, and determine if extended chemoprophylaxis post LSG reduces PMVT. SETTINGS: University hospital. METHODS: Thrombophilia panels were drawn on every patient seeking LSG after July 2018 at 2 high-volume accredited bariatric surgery centers. A positive panel included factor VIII >150%; protein C <70%; protein S <55%; antithrombin III <83%; and activated protein C resistance <2.13. Patients with a positive panel were discharged on extended chemoprophylaxis. PMVT rates and bleeding occurrences were recorded for LSG patients from August 2018 to March 2019 and were compared with a historic cohort of LSG performed from January 2014 to July 2018. RESULTS: One thousand seventy-five patients seeking LSG had thrombophilia panel checked preoperatively. The cohort was 83% female; mean age and body mass index were 39.2 years and 43 kg/m2, respectively. Of the cohort, 52.4% (563/1075) had positive thrombophilia panel, including factor VIII elevation (91.5%), antithrombin III deficiency (6.0%), protein S deficiency (1.1%), protein C deficiency (.9%), and activated protein C resistance (.5%). Between January 2014 and July 2018, 13 PMVT were diagnosed among 4228 LSG (.3%) and there were 17 bleeding occurrences (.4%). After August 2018, one PMVT was diagnosed among 745 LSG (.1%) and there were 5 bleeding occurrences (.6%). CONCLUSIONS: The estimated thrombophilia prevalence in patients seeking LSG is 52.4%. The majority (91.5%) of these patients have factor VIII elevation. Extended prophylaxis may decrease PMVT postLSG.

Risk Factors Associated with Head Lice Infestation in Rural Pediatric Patients.

INTRODUCTION: Head louse infestation is a common parasitic disease in pediatric age group. It is common in collective centers such as schools, garrisons, campuses and old age homes. It is worldwide in distribution with no strict limitation with respect to age, gender and race. AIMS: Present study was conducted to investigate the socio-economic and lifestyle risk factors for head louse infestation in pediatric patients. MATERIALS AND METHODS: An observational descriptive study at a tertiary care hospital in rural Uttar Pradesh over a period of 1 year wherein measurement of disease/risk factors associated with head louse infestation was performed. It was carried out in all children between 5 and 15 years of age using a pre-validated questionnaire. The method of convenience sampling was used and multiple logistic regression was run to account for potential confounding variables using SPSS software. RESULTS: A total of 165 (71.1%) females and 67 (28.8%) males were infested. Most common age group affected was between 5 and 7 years where 97 (41.8%) children were infected. Ninety-five (40.9%) children were in fourth to seventh standards. Parents of 137 (59%) children were not formally educated. Eighty-one (34.9%) children had six members in the family while per capita income was between 5000 and 2500 in families of 139 (59.9%) children. Eighty-seven (37.5%) children had hair length up to shoulders. One twenty-eight (55.1%) children had no family member affected. Seventy-six (32.7%) children took bath twice a week only. CONCLUSION: Social and economic factors as well as lifestyle and education level of patients play a significant role in epidemiology of head lice.

Correlation of Dermoscopic and Histopathologic Patterns in Leprosy - A Pilot Study.

BACKGROUND: Leprosy is a chronic granulomatous disease caused by Mycobacterium leprae. It is diagnosed based on clinical features and confirmed on the histological findings and peripheral slit-skin smear staining. Dermoscopy is a handy, easily accessible tool to diagnose this granulomatous disease and classify patients based on the immunological and clinical response. METHODS: A single spot observational analysis was conducted in a tertiary hospital in North India. Patients attending the leprosy clinic and admitted patients for institutional therapy on the day of the study were enrolled in the cohort. The clinical and histological findings were correlated with the characteristic dermoscopy findings. A total of 50 patients were included in the study. All patents included in the study were on multidrug therapy and anti-lepra reaction drugs for a duration of less than 6 months. RESULTS: The dermoscopy findings correlated with the clinical and histological findings. Tuberculoid poles of leprosy classically showed loss of hair and skin pigment along with absence of white dots as sweat glands in dermoscopy. Lack of blood vessel changes ruled out any lepra reaction. Lepromatous pole of leprosy on the other hand showed characteristic xerosis and white scaling on dermoscopy in the background of hypotrichosis and hypopigmentation. Leprosy reactions were characterized by blood vessel changes and arborizing blood vessels were characteristic in erythema nodosum leprosum, and a diffuse erythema was a clue toward diagnosing type I lepra reaction. Interestingly, clofazimine-induced pigmentation was picked up characteristically on dermoscopy as a "honey comb pattern". CONCLUSION: Dermoscopy is certainly a handy tool in aiding the diagnosis of leprosy, lepra reactions, and course of therapy. Characteristic patterns during the course of leprosy would certainly facilitate a quick and definitive diagnosis of patients suffering from leprosy. Also, patient drug compliance particularly to clofazimine can also be picked up objectively on dermoscopy.

A Cross-sectional Study to Analyze the Social, Sexual, and Reproductive Challenges among Serodiscordant Couples.

INTRODUCTION: The term "serodiscordant couples" refers to an intimate partnership in which one partner is human immunodeficiency virus (HIV) positive and the other HIV negative. They form a special population which are constantly at risk of acquiring infection, require safer sexual and reproductive options, and are in constant psychological and emotional distress. AIMS: To describe the social, sexual, and reproductive issues and their impact on serodiscordant couples. MATERIALS AND METHODS: A cross-sectional study was conducted on HIV-serodiscordant couples, admitted or attending our outpatient department, where the couples had not separated. A detailed interview of the partners on social, sexual, and reproductive issues was conducted and the data were endorsed in the pro forma. RESULTS: Sixty-four serodiscordant couples were included in the study. Sixty-two (96.8%) males were seropositive compared to 2 (3.1%) females. Sixty-one (95.3%) patients were married and 3 (4.6%) were unmarried. Thirty-six (56.2%) patients were between the age group of 21 and 35 years, 21 (32.8%) between 36 and 55 years, and 7 (10.9%) between 56 and 70 years. Sixty-two (96.8%) patients had a heterosexual orientation compared to 2 (3.1%) patients who were homosexual. Twenty-one (32.8%) patients had a history of sexual encounter outside the relation while 27 (42.1%) were not aware of the source of infection. Fifty-one (79.6%) patients were on antiretroviral therapy (ART) compared to 13 (20.3%) patients who were not on ART. Thirty-one (48.4%) patients admitted to have a constant strain in relation while 16 (25%) were practicing safe sex. Thirty-nine (60.9%) patients had fear of disease transmission while 26 (40.6%) had fear of pregnancy. Forty-nine (76.5%) patients had children at the time of detection while 15 (23.4%) had no issue. Forty-one (64%) patients expressed desire to have children as compared to 23 (35.9%). CONCLUSION: The unique requirements of serodiscordant couples in terms of providing them safer sexual and reproductive options to prevent the transmission of HIV to the seronegative partner or the child during pregnancy need to be addressed for better patient management.

A Cross-Sectional Observational Study of Geriatric Dermatoses in a Tertiary Care Hospital of Northern India.

INTRODUCTION: Geriatric dermatoses are one of the most common reasons for day-to-day consultation in the elderly. Over the past few years, understanding of the pathophysiology of skin changes in the geriatric age group has improved and has paved the way for better therapeutic options. There are only a few studies conducted in India about the geriatric dermatoses. This article reviews the various physiological and pathological changes of aging, dwelling on the role of intrinsic and extrinsic factors in the pathogenesis of aging skin thus better understanding of this emerging branch in dermatology leading to enhance resource management for elderly population. MATERIALS AND METHODS: This is a cross-sectional observational study carried out on 500 consecutive patients aged 60 years and above in Department of Dermatology of a Tertiary care hospital of Northern India after meeting the inclusion and exclusion criteria. RESULTS: Out of 500 patients studied with male to female ratio of 1.4, wrinkles followed by cherry angiomas were the most common physiological cutaneous manifestations, and infective dermatoses followed by allergic contact dermatitis were the most common pathological conditions seen. Few rare cases were also seen during the study such as cutis marmorata, delusion of parasitosis, and sweet syndrome in case of acute myeloid leukemia. CONCLUSION: Geriatric dermatology is an emerging branch in dermatology, and an update on this, will go a long way to effectively manage these patients. A thorough knowledge of the epidemiology as well as gender distribution of dermatological diseases in geriatric population in the tertiary care hospital will help in assessing health status and health care needs related to skin for better allocation of resources, distribution of material and manpower, and help health care providers in better decision-making resulting in higher clientele satisfaction.

A Descriptive Study to Analyze Chemotherapy-Induced Hair Loss and its Psychosocial Impact in Adults: Our Experience from a Tertiary Care Hospital.

BACKGROUND: Hair loss is one of the most commonly reported and psychologically distressing adverse effects of chemotherapeutic agents. Studies on its impact on psychosocial aspect of cancer patients are lacking at present. OBJECTIVE: To study the chemotherapeutic agents causing hair loss and its psychosocial implications in adults. MATERIALS AND METHODS: Observational study was done for a period of 1 year, wherein all cancer patients, more than 18 years of age who developed hair loss while on chemotherapy were assessed for type of malignancy, details of chemotherapy protocol, their knowledge about chemotherapeutic agents induced hair loss, and its impact on their social life and patterns of adjustment to deal with it. A prevalidated closed-ended questionnaire was used as a data collection tool. RESULTS: Out of 179 patients, 96 (53.6%) were males as against 80 (44.6%) females, and 49 (27.3%) patients were between 18 and 30 years of age. Carcinoma lung was the most common malignancy seen in 46 (25.6%) patients followed by rectosigmoid carcinoma in 41 (22.9%) patients. Combination of cyclophosphamide and doxorubicin was the most common combination resulting in hair loss in 49 (27.3%) cancer patients. A total of 101 (56.4%) patients felt that hair loss was the worst side effect of chemotherapy, while 29 (16.2%) had to continue because it was life-saving. A total of 129 (72%) patients said hair loss is affecting their social life; 37 (20.6%) patients were using hair accessories while 69 (38.5%) did not even attempt to hide hair loss as they were too occupied with fear of disease. CONCLUSION: Chemotherapy-induced hair loss is a common adverse effect in cancer patients undergoing treatment. A thorough counseling about it and methods to deal with it should be a part of management of the patients.

An Observational Study to Describe the Clinical Pattern of Dermatological Emergencies from Emergency Department and Intensive Care Unit: Our Experience from a Tertiary Care Hospital in Northern India.

BACKGROUND: A large number of skin diseases have the potential to culminate into potentially fatal "acute skin failure." The concept of dermatological intensive care unit (ICU) has largely evolved as a result of increased number of emergencies encountered by dermatologists these days. Dermatological emergencies comprise 8-20% of cases presenting to the emergency department. A wide variety of these conditions require a collective effort by intensivists, surgeons, physicians, and nursing staff in association with the treating dermatologist to reduce the associated mortality and morbidity. Dermatology ICU along with state-of-the-art nursing care is required to manage these cases, which result in acute skin failure. MATERIALS AND METHODS: A prospective study conducted in a tertiary care center with a round the clock emergency department and a state-of-the-art dermatology ICU over a period of 12 months. Patients requiring primary dermatological consultation in the emergency department and patients admitted in the dermatology intensive care unit were evaluated, and their clinical variables were statistically analyzed. RESULTS: In total, 327 cases were seen in the emergency department, out of which 54 (16.5%) cases were admitted in dermatology ICU, 239 (73.1%) were treated as outpatient cases, and 34 (10.4%) were managed as inpatients in other wards of the hospital. The most common condition in out-patient cases was acute urticaria and angioedema in 71 (29.7%), while vesiculobullous disorders in 16 (29.6%) patients was the most common condition requiring admission in dermatology ICU. CONCLUSIONS: At present, only few studies are available in the literature on the spectrum of dermatological disorders reporting to emergency department, and further requiring intensive care under ICU setting. This prospective study highlights the varied patterns of dermatosis reporting to emergency outpatient department and those managed in the ICU.

Disease Pattern among Sexually Transmitted Infection Clinic Attendees: A Hospital-Based Study.

BACKGROUND: Sexually transmitted infections (STIs) have a definite role in the facilitation of HIV infection, which, in turn, also increases susceptibility to other STIs. Further, the social stigma and secrecy surrounding STIs create difficulty in estimating its true incidence and prevalence. OBJECTIVE: The objective of this study was to know the pattern of diseases including different clinical presentations and final diagnosis among the STI clinic attendees. MATERIALS AND METHODS: This study was a hospital-based study; a predesigned and pretested schedule was used to collect data from 140 patients after obtaining their consent. The study was carried out between January 1, 2016, and December 31, 2016. STATISTICAL ANALYSIS: Analysis was done using tabulation and proportion. RESULTS AND OBSERVATION: Genital ulcer (31.43%) was the most common clinical presentation among the attendees. Primary syphilis was the most common (17.14%) STI detected among the participants followed by herpes genitalis (16.43%). The most common mixed infection was primary syphilis with chancroid (36.36%) followed by herpes genitalis with primary syphilis (18.18%). CONCLUSION: Ulcerative STIs singly or in combination are more frequent than the non-ulcerative STIs. Further studies with large sample sizes are needed to obtain a more vivid picture.

A Clinico-epidemiological Study of Childhood Herpes Zoster.

Herpes zoster (HZ) is a viral infection believed to be caused by the re activation of varicella zoster virus (VZV) or human herpes virus type 3 (HHV 3) that persists in the posterior nerve root ganglion. HZ is rarely reported in the pediatric age group with an intact immunity. Past infection with VZV and immunization with chickenpox vaccine are key markers in the onset of varicella zoster in children. Our aim was to study the clinicoepidemiological pattern of HZ infection in children aged less than 12 years and to start an early management to prevent long term complications. A prospective observational study over a total duration of 2 years was conducted in a tertiary hospital, and all children less than 12 years of age with diagnosed HZ were included in the study. A total of 39 children were diagnosed to have pediatric HZ infection during the study period. The children were followed up over 4 weeks post diagnosis and were treated with oral acyclovir therapy along with symptomatic management. All children had an uneventful benign course, and their siblings and close pediatric contacts were also screened for the development of HZ or chickenpox during the incubation period. All children were screened for an underlying immunodeficiency and two cases of HIV co infection were detected. HZ is a rare disease in childhood. Varicella in early childhood is a risk factor for HZ in both immunocompromised and immunocompetent children. The appearance of HZ in a young child does not always imply an underlying immunodeficiency or malignancy, but the children should be screened for immunodeficiency. In general, the prognosis is good in healthy children.

Nilotinib-induced Perforating Folliculitis: Two Cases.

Cutaneous adverse effects of chemotherapy are widely known but underreported. A significant advancement is made in the field of oncology with the advent of new classes of drug being added to the existing classes at a fast pace. Most of these cutaneous adverse effects are self-limiting and subsides on suspending the drug either temporarily or permanently. Some of these effects are merely overlooked by the patients and the treating physician hence goes un-noticed. Nilotinib is a newer second-generation tyrosine-kinase inhibitor approved for the management of chronic myeloid leukemia. This drug is rapidly establishing itself as afirst-line therapy for chronic myeloid leukemia. Like other chemotherapeutic agents, a wide array of cutaneous adverse effects is noted with this drug. We report two cases of perforating folliculitis induced by nilotinib.

Granuloma Annulare Skin Lesions in a Case of Sarcoidosis.

We report the case of a 32-year- old man with a short 3-week history of erythematous, annular, non scaly plaques on palmar and dorsal aspect of his hands, who was concurrently diagnosed as a case of sarcoidosis on the basis of findings of generalized lymphadenopathy and radiological and histological features of pulmonary sarcoidosis. His skin biopsy was consistent with the diagnosis of granuloma annulare. Sarcoidosis and granuloma annulare are two separate diseases, which involve the skin and have a mononuclear histiocytic cellular reaction, although their aetiology is still unknown. Granuloma annulare has been associated with the concomitant diagnosis of sarcoidosis in only two more case reports and this association can be evaluated further to study a common link in the aetipathogenesis of these two granulomatous skin diseases.

Rare Variant of Ankyloblepharon-ectodermal Defect-cleft Lip/Cleft Palate Syndrome: Curly Hair-ankyloblepharon-nail Disease Syndrome.

Ankyloblepharon-ectodermal defect-cleft lip/cleft palate (AEC) syndrome is one of the variants of ectodermal dysplasia. It is an autosomal dominant disorder comprising of ankyloblepharon, ectodermal dysplasia, and cleft palate or cleft lip. In 1976, it wasfirst described by Hay and Wells, therefore also known as Hay-Wells syndrome. The characteristic feature of this syndrome is "ankyloblepharon filiforme adnatum", which refers to the partial thickness fusion of the eyelid margins. The "curly hair-ankyloblepharon-nail disease (CHAND) syndrome" is a clinical variant of AEC syndrome. We report a rare case of a 7-year-old girl child who presented with history of abnormal dentition, 20 nail dystrophy, and light-colored, sparse curly hairs since birth. Parents gave history that at the time of birth, her both eyelids were fused partially, which was surgically corrected by an ophthalmologist at 1 month of age. There was no history of hypohidrosis or anhidrosis, heat intolerance, cleft lip or cleft palate. Microscopy of the hair shaft found "bubbly hair" morphology. This case is unique as it is a rare presentation, and awareness should be there for this constellation of findings so that the systemic associations can be investigated. "Bubble hair" morphology on microscopy is a unique feature in this rare autosomal recessive condition.

Factor VIII elevation may contribute to portomesenteric vein thrombosis after laparoscopic sleeve gastrectomy: a multicenter review of 40 patients.

BACKGROUND: Portomesenteric vein thrombosis (PMVT) has been increasingly reported after laparoscopic sleeve gastrectomy (LSG). Factor VIII (FVIII) is a plasma sialoglycoprotein that plays an essential role in hemostasis. There is increasing evidence that FVIII elevation constitutes a clinically important risk factor for venous thrombosis. OBJECTIVES: To report the prevalence of FVIII elevation as well as other clinical characteristics in a multicenter series of patients who developed PMVT after LSG. SETTING: University hospitals. METHODS: A retrospective review was conducted of all patients that developed PMVT after laparoscopic bariatric surgery from 2006 to 2016 at 6 high-volume bariatric surgery centers. RESULTS: Forty patients who developed PMVT postoperatively, all after LSG, were identified. During this timeframe, 25,569 laparoscopic bariatric surgery cases were performed, including 9749 LSG (PMVT incidence after LSG = .4%). Mean age and body mass index were 40 years (18-65) and 43.4 kg/m2 (35-59.7), respectively. Abdominal pain was the most common (98%) presenting symptom. Of patients, 92% had a hematologic abnormality identified, and of these, FVIII elevation was the most common (76%). The vast majority (90%) was successfully managed with therapeutic anticoagulation alone. A smaller number of patients required small bowel resection (n = 2) and surgical thrombectomy (n = 1). There were no mortalities. CONCLUSIONS: A high index of clinical suspicion and prompt diagnosis/treatment of PMVT usually leads to favorable outcomes. FVIII elevation was the most common (76%) hematologic abnormality identified in this patient cohort. Further studies are needed to determine the prevalence of FVIII elevation in patients seeking bariatric surgery.