Clinical factors associated with remission of obese acanthosis nigricans after laparoscopic sleeve gastrectomy: a prospective cohort study.

BACKGROUND: Acanthosis nigricans (AN) involves skin hyperpigmentation in body folds and creases. Obesity-associated AN (OB_AN) is the most common type of AN. The skin condition of obese patients with AN can be improved through bariatric surgery, such as laparoscopic sleeve gastrectomy (LSG), after weight loss. However, the contributing factors to the remission of AN after surgery are still not fully determined. The authors aimed to assess the metabolic and pathological factors associated with remission of AN following LSG in obese individuals. METHODS: The study included 319 obese patients who underwent LSG at our hospital. The subjects were divided into obesity (OB) only (OB, n =178) or OB with AN (OB_AN, n =141) groups. The basic clinical and metabolic indices and the dermatological features via reflectance confocal microscopy and histology were collected from patients prior to and after LSG. RESULTS: OB_AN patients had higher fasting plasma glucose, homeostatic model assessment for insulin resistance, and testosterone levels than OB patients. LSG could significantly improve the biochemical and histopathological features of OB_AN patients. The remissive rate of OB_AN patients was about 86.5% (122 out of 141) after surgery. The remission of OB_AN skin lesions was positively correlated with testosterone levels ( P <0.01). In addition, there was a significant positive correlation between changes in AN scores and epidermal thickness and skin pigmentation scores after surgery ( P <0.01). CONCLUSION: The remissive rate of OB_AN after LSG is associated with improved testosterone levels and reduced epidermal thickness and skin pigmentation levels.

Improved Insulin Secretion Response and Beta-cell Function Correlated with Increased Prolactin Levels After Laparoscopic Sleeve Gastrectomy in Morbidly Obese Patients with Acanthosis Nigricans.

OBJECTIVE: To explore insulin secretion patterns, ß-cell function, and serum prolactin (PRL) concentrations in Chinese morbidly obese patients with Acanthosis nigricans (AN) and their alterations after laparoscopic sleeve gastrectomy (LSG). METHODS: A total of 138 morbidly obese subjects undergoing LSG were categorized as simple obesity without AN (OB group, n = 55) and obesity with AN (AN group, n = 83). Oral glucose tolerance test (OGTT), PRL, and related metabolic indices were performed pre- and 12 months post-LSG. Insulin secretion patterns were derived from insulin secretion peak time during OGTT: type I (peak at 30 or 60 min) and type II (peak at 120 or 180 min). RESULTS: Preoperatively, AN group showed significantly higher proportions of type II insulin secretion pattern, fasting insulin (FINS), and homeostatic model assessment of insulin resistance (HOMA-IR) whereas lower oral glucose insulin sensitivity (OGIS), insulinogenic index (IGI), and disposition index (DI) than OB group, which were improved significantly at 12 months postoperatively in both groups, more pronounced in AN group. Intriguingly, serum PRL declined substantially in AN group than OB group at baseline whereas elevated only in the AN group post-LSG. After adjusting for confounding factors, elevated PRL correlated significantly with increased IGI and DI, and decreased HOMA-IR in both genders, as well as increased OGIS in females, which was detected only in the AN group CONCLUSION: Morbidly obese patients with AN presented delayed insulin secretion response, impaired insulin secretion, and ß-cell dysfunction, which were significantly improved by LSG and might benefit from elevated PRL.

Clinical signs, interventions, and treatment course of three different treatment protocols in patients with Crouzon syndrome with acanthosis nigricans.

OBJECTIVE: Crouzon syndrome with acanthosis nigricans (CAN) is a rare and clinically complex subtype of Crouzon syndrome. At three craniofacial centers, this multicenter study was undertaken to assess clinical signs in relation to the required interventions and treatment course in patients with CAN. METHODS: A retrospective cohort study of CAN was performed to obtain information about the clinical treatment course of these patients. Three centers participated: Erasmus Medical Centre, Rotterdam, the Netherlands; John Radcliffe Hospital, Oxford, United Kingdom; and Hôpital Necker-Enfants Malades, Paris, France. RESULTS: Nineteen patients (5 males, 14 females) were included in the study. All children were operated on, with a mean of 2.2 surgeries per patient (range 1-6). Overall, the following procedures were performed: 23 vault expansions, 10 monobloc corrections, 6 midface surgeries, 11 foramen magnum decompressions, 29 CSF-diverting surgeries, 23 shunt-related interventions, and 6 endoscopic third ventriculostomies, 3 of which subsequently required a shunt. CONCLUSIONS: This study demonstrates that patients with the mutation c.1172C>A (p.Ala391Glu) in the FGFR3 gene have a severe disease trajectory, requiring multiple surgical procedures. The timing and order of interventions have changed among patients and centers. It was not possible to differentiate the effect of a more severe clinical presentation from the effect of treatment order on outcome.

Beare-Stevenson Syndrome With Blepharoptosis as a Complication of Front-Orbital Advancement and Remodeling.

Beare-Stevenson syndrome (BSS) is an extremely rare genetic disorder characterized by a broad range of congenital malformations including craniosynostosis, cutis gyrata, facial deformities, and abnormal genitalia. The authors report a case of a 7 month old female who developed a mechanical ptosis secondary to dermatochalasis as a complication of fronto-orbital advancement and remodeling (FOAR) surgery which subsequently required multiple lid surgeries to reverse ptosis. This is the first report of blepharoptosis correction in a child with BSS as a complication of FOAR.

Comparison of the effectiveness of fractional 1550-nm erbium fiber laser and 0.05% tretinoin cream in the treatment of acanthosis nigricans: a prospective, randomized, controlled trial.

Acanthosis nigricans is a common dermatological problem. There are currently limited clinical trials to determine the efficacy and safety of laser treatments. To compare the efficacy of fractional 1550-nm erbium fiber laser versus 0.05% tretinoin cream for the treatment of acanthosis nigricans at neck, a randomized, controlled, assessor-blinded study was conducted in 18 subjects with acanthosis nigricans at the neck. All patients were treated with both fractional 1550-nm erbium fiber laser and 0.05% tretinoin cream on each side of the neck. The laser side was treated with three treatment sessions, with a 4-week interval of 1550-nm fractional erbium laser. Another side was treated with 0.05% tretinoin cream daily at bedtime for 12 weeks. We evaluated at baseline, with a 4-week interval until 4 weeks after the last treatment. The efficacy was assessed by skin color ratio, melanin index, average roughness, photographic evaluation, patients' satisfaction, and the adverse effects. At the study endpoint, week 12, the mean Visiometer-average roughness showed greater reduction in laser-treated side (24.65%) than tretinoin side (22.94%) (p = 0.004). Laser-treated side also showed greater percentage of skin color ratio reduction, melanin index reduction, and better mean of photographic-based evaluation percentage change from the baseline than tretinoin side with no significant different (p = 0.331, p = 0.116, p = 0.327, respectively). The study showed one post-inflammatory hyperpigmentation in tretinoin side. Regarding to the average roughness, fractional 1550-nm erbium fiber laser was superior to 0.05% tretinoin cream for treatment of neck-acanthosis nigricans with less side effect. Fractional 1550-nm erbium fiber laser could be considered as an alternative treatment for acanthosis nigricans.

Posterior Distraction First or Fronto-Orbital Advancement First for Severe Syndromic Craniosynostosis.

PURPOSE: Posterior calvarial vault expansion using distraction osteogenesis is performed for syndromic craniosynostosis as the first choice. This procedure allows far greater intracranial volume than fronto-orbital advancement (FOA). This study aimed to determine the most suitable timing of posterior distraction or FOA to sufficiently increase the intracranial volume and remodel the skull shape. PATIENTS AND METHODS: From 2014 to 2017, the authors performed posterior distraction in 13 patients with syndromic craniosynostosis. Data on premature suture fusion, age at first visit, age at surgery, skull thickness, and complications were collected. RESULTS: Five patients underwent posterior distraction at approximately 12 months of age and had no complications, including cerebrospinal fluid leakage or gull wing deformity. However, during the waiting period for the operation, the skull deformity continues to extend upward (turribrachycephaly). To prevent progress of the skull deformity, the authors performed the operation at approximately 6 months of age in 7 patients. However, in 3 of 7 patients whose lambdoid sutures were opening, gull wing deformity occurred. From these results, in a patient with severe Beare-Stevenson syndrome, the authors performed FOA first at 5 months of age, followed by posterior distraction at 12 months of age, and achieved favorable results. CONCLUSIONS: Treatment patterns are patient specific and should be tailored to premature suture fusion, specific skull deformity, and required intracranial volume of each patient.

Laparoscopic sleeve gastrectomy improves body composition and alleviates insulin resistance in obesity related acanthosis nigricans.

BACKGROUND: Acanthosis nigricans (AN) has a close relationship with obesity. It is believed that obesity and AN have the common pathophysiological basis such as hyperinsulinism. This study is aimed to observe the effect of laparoscopic sleeve gastrectomy (LSG) on body composition and insulin resistance in Chinese obese patients with acanthosis nigricans. METHODS: A total of 37 obese patients who underwent LSG in our hospital were selected for analysis. They were divided into simple obesity (OB n = 14) and obesity with acanthosis nigricans (AN n = 23) group respectively. Body composition was measured by dual-energy X-ray absorptiometry (DEXA). Anthropometric measurements and glucolipid metabolism before and 3 months post LSG were collected for analysis. RESULTS: Patients with AN got noticeable improvement in skin condition and their AN score was significantly decreased (3.52 ± 0.79 vs. 1.48 ± 0.73, P < 0.001).Alleviated insulin resistance and more trunk fat loss than limbs' were observed in both groups (P value < 0.01). In AN group, preoperative android fat mass (FM) was positively correlated with fasting insulin and natural logarithm of HOMA-IR (LNIR) (r = 0.622, 0.608, respectively; all P < 0.01). Besides, changes in android FM and visceral adipose tissue (VAT) also showed significantly positive correlation with changes in LNIR (r = 0.588, r = 0.598, respectively; all P < 0.01). CONCLUSIONS: LSG had a positive impact on body composition and skin condition in Chinese obese patients with AN. Loss of android FM and VAT might result in the alleviation of insulin resistance in AN patients. Android fat distribution seems to be a potential indicator of postoperative metabolic benefits for obese patients with AN.

Acne-associated syndromes: models for better understanding of acne pathogenesis.

Acne, one of the most common skin disorders, is also a cardinal component of many systemic diseases or syndromes. Their association illustrates the nature of these diseases and is indicative of the pathogenesis of acne. Congenital adrenal hyperplasia (CAH) and seborrhoea-acne-hirsutism-androgenetic alopecia (SAHA) syndrome highlight the role of androgen steroids, while polycystic ovary (PCO) and hyperandrogenism-insulin resistance-acanthosis nigricans (HAIR-AN) syndromes indicate insulin resistance in acne. Apert syndrome with increased fibroblast growth factor receptor 2 (FGFR2) signalling results in follicular hyperkeratinization and sebaceous gland hypertrophy in acne. Synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) and pyogenic arthritis-pyoderma gangrenosum-acne (PAPA) syndromes highlight the attributes of inflammation to acne formation. Advances in the understanding of the manifestation and molecular mechanisms of these syndromes will help to clarify acne pathogenesis and develop novel therapeutic modalities.

Malignant acanthosis nigricans: an early diagnostic clue.

Acanthosis nigricans (AN) is characterized by velvety, hyperpigmented, verrucous, symmetric and occasionally pruritic plaques along with papillomatous lesions which have a special predilection for neck, axillae, groin, umbilicu and mucosal regions. Rarely, it presents as a paraneoplastic syndrome and prompts a thorough search for an internal malignancy. We present here a case of malignant acanthosis nigricans seen in an elderly patient who was found to have underlying adenocarcinoma of the lower end of esophagus. After diagnosing associated malignancy, he was referred for further evaluation and subsequent surgical resection of tumour.

Clearance of acanthosis nigricans associated with insulinoma following surgical resection.

Acanthosis nigricans is said to be a marker of insulin resistance. It is known to occur in patients with insulinoma where there is marked hyperinsulinaemia. We report a case wherein the acanthosis disappeared following surgical resection of insulinoma and this strengthens the hypothesis that hyperinsulinaemia is responsible for acanthosis.

A case of Beare-Stevenson cutis gyrata syndrome confirmed by mutation analysis of the fibroblast growth factor receptor 2 gene.

This paper reports a case of Beare-Stevenson cutis gyrata syndrome confirmed by DNA analysis of the patient's fibroblast growth factor receptor (FGFR) genes. At birth, the patient had ocular proptosis, a red nevus with skin tags on her forehead and an umbilical stump. She developed craniosynostosis, craniofacial dysmorphism and hydrocephalus. Her treatment included forehead and facial advancement and a ventriculoperitoneal shunt. Analysis of the FGFR genes revealed that she was heterozygous for a missense mutation in exon 10 for the FGFR2 protein, resulting in an amino acid substitution of cysteine for tyrosine at residue 375 (Tyr375Cys). This is the fourth case of Beare-Stevenson cutis gyrata syndrome confirmed by mutation analysis of the FGFR genes.

Clearance of acanthosis nigricans associated with the HAIR-AN syndrome after partial pancreatectomy: an 11-year follow-up.

We describe a woman with the syndrome characterised by hyperandrogenism, insulin resistance and acanthosis nigricans (the HAIR-AN syndrome), and an associated insulinoma (islet B-cell tumour), whose signs and symptoms cleared after partial pancreatectomy.

Malignant acanthosis nigricans. A case report.

PURPOSE: The authors present a case of malignant acanthosis nigricans that has two unusual aspects. The first is involvement of the lid margins with confluent papillomata, causing severe visual impairment, and the second is the association with lung malignancy. METHODS: A 65-year-old Portuguese man presented with decreased vision, papillomatous lid lesions, pruritus, and progressive thickening of the skin of his face, neck, axillae, and inguinal creases. A diagnosis of acanthosis nigricans was made after skin biopsy. Systemic investigations showed a suprahilar mass, and biopsy was positive for squamous cell carcinoma of the lung. RESULTS: The patient underwent excision of papillomata from all four lid margins, and this was repeated 1 year later. He also completed a course of palliative radiotherapy. CONCLUSION: Malignant acanthosis nigricans is rarely associated with lung neoplasms, being more commonly a manifestation of intra-abdominal malignancies. Of note in this case is the extensive ocular involvement and visual impairment.

Crouzon's syndrome associated with acanthosis nigricans: ramifications for the craniofacial surgeon.

Crouzon's syndrome is one of many disorders that have been associated with acanthosis nigricans. Previously reported cases documenting this association have been reviewed, and additional cases that have been treated at the Institute of Reconstructive Plastic Surgery at New York University Medical Center have been added. Recommendations for the surgical management of this unique group of patients are presented.