Acromegaly-related cutis verticis gyrata

Roque, João; Marques, Pedro

doi:10.1016/j.ghir.2022.101497

Cited by 8 publications

(9 citation statements)

References 93 publications

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“…Digital clubbing is the most specific sign of this condition, while joint manifestations typically include arthralgia, synovial effusions, periosteal changes, acroosteolysis and interosseous membranes ossification, affecting mainly the knees, ankles and wrists 1,2 . Skin involvement is characterized by facial coarsening, skin hypertrophy and pachydermia of the forehead, face, and scalp (cutis verticis gyrata) 1,3 . Other features of pachydermoperiostosis includes hyperhidrosis, seborrhoea, acne, blepharoptosis, hypoalbuminemia, chronic gastritis, peptic ulcer, episodes of watery diarrhoea, anaemia and myelofibrosis 1,2 .…”

Section: Discussionmentioning

confidence: 99%

“…1,2 The differential diagnosis of pseudoacromegaly is challenging due to the long list of disorders that may be associated with acromegalyrelated manifestations, including acromegaloid facial appearance, acral enlargement, overgrowth/tall stature, macroglossia, arthralgia, skin manifestations, hyperhidrosis, hypertension, diabetes, among others. 1,3 Such pseudoacromegaly conditions are rare and sometimes have overlapping features, which impose further challenges to the differential diagnosis, however some have distinctive features facilitating the establishment of the diagnosis of the underlying condition. 2,[4][5][6] Although the list of conditions associated with acromegaloid features but normal GH/IGF-1 axis may be extensive, not all pseudoacromegaly conditions mimic closely acromegaly or pituitary gigantism.…”

Section: Introductionmentioning

confidence: 99%

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Pseudoacromegaly—A challenging entity in the endocrine clinic: A systematic review

Marques,

Sapinho,

Korbonits

2024

Clinical Endocrinology

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ObjectivePseudoacromegaly encompasses conditions with features of acromegaly/gigantism, but no growth hormone (GH) or insulin‐like growth factor‐1 (IGF‐1) excess. We aimed to review published pseudoacromegaly cases evaluated due to clinical suspicion of acromegaly.Design/PatientsPubMed/Medline search was conducted to identify reported pseudoacromegaly cases, which were systematically reviewed to ensure they met eligibility criteria: (1) presentation suggestive of acromegaly; (2) acromegaly excluded based on normal GH, IGF‐1 and/or GH suppression on oral glucose tolerance test (OGTT‐GH); (3) diagnosis of the pseudoacromegaly condition was established. Data were retrieved from each case and analysed collectively.ResultsOf 76 cases, 47 were males, mean ages at presentation and at first acromegaloid symptoms were 28 ± 16 and 17 ± 10 years, respectively. Most common conditions were pachydermoperiostosis (47%) and insulin‐mediated pseudoacromegaly (IMP) (24%). Acromegaloid facies (75%) and acral enlargement (80%) were the most common features. Measurement of random GH was reported in 65%, IGF‐1 in 79%, OGTT‐GH in 51%. GH excess was more frequently excluded based on two tests (53%). Magnetic resonance imaging (MRI) was performed in 30 patients, with pituitary adenoma or hyperplasia being reported in eight and three patients, respectively. Investigations differed between cases managed by endocrine and non‐endocrine specialists, the former requesting more often IGF‐1, OGTT‐GH and pituitary MRI.ConclusionsPseudoacromegaly is a challenging entity that may be encountered by endocrinologists. Pachydermoperiostosis and IMP are the conditions most often mimicking acromegaly. Adequate assessment of GH/IGF‐1 is crucial to exclude acromegaly, which may be better performed by endocrinologists. Pituitary incidentalomas are common and require careful judgement to prevent unnecessary pituitary surgery.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Pseudoacromegaly—A challenging entity in the endocrine clinic: A systematic review

Marques,

Sapinho,

Korbonits

2024

Clinical Endocrinology

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“…Cells and appendant organs of the skin, including epidermal and follicular keratinocytes, papilla cells, fibroblasts, endothelial cells, melanocytes, sebocytes, and sweat glands, are all influenced by hormones ( 9 ). Naked-eye inspections of the cutaneous presentations of acromegaly include skin thickening, skin edema, skin wrinkling, face coarsening, neoplastic tags, excessive seborrhea, hyperhidrosis, and pigmentation ( 5 , 10 , 26 – 29 ). In this study, extremity enlargement, facial feature changes, hyperpigmentation, oily skin, and hyperhidrosis were detected in the majority of acromegaly patients while hypertrichosis and skin tags were detected in a small proportion of patients, in accordance with previous studies ( 9 , 29 – 31 ).…”

Section: Discussionmentioning

confidence: 99%

Sub-macroscopic skin presentation of acromegaly and effect of pituitary tumor surgery: A study using dermatoscopy and ultra-high-frequency ultrasound

et al. 2023

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ObjectiveExcessive growth hormone and insulin-like growth factor 1 contribute to cutaneous changes in acromegaly. We investigated the sub-macroscopic skin manifestation of acromegaly patients and explored its reversibility upon hormone reduction after pituitary adenoma surgery.DesignProspectively cohort study.MethodsWe enrolled 26 patients with acromegaly and 26 patients with non-functioning pituitary adenomas undergoing pituitary adenomectomy at Peking Union Medical College Hospital from July 2021 to March 2022. Skin presentations were evaluated by dermatoscopy and ultra-high-frequency ultrasound before and after surgery.ResultsSkin thickening, follicular plugs, perifollicular pigmentations, perifollicular orange haloes, red structureless areas, increased hair shafts, honeycomb-like pigmentations, widened dermatoglyphics, dilated appendage openings, excessive seborrhea, hyperhidrosis, enlarged pores, and acne-like lesions were commonly occurring in acromegaly patients, and their incidences were higher than the controls (P<0.05). At 3-month follow-up after surgery, the thickness of skin reduced (4.0 ± 0.4 to 3.7 ± 0.4, P=0.007), the incidences of hyperhidrosis (92.3% to 69.2%, P=0.035) and acne-like lesions (53.8% to 26.9%, P=0.048) declined, and the severity of multiple cutaneous lesions improved. Patients with surgical endocrine remission (53.8%) had greater declines in the thickness of skin than those without remission. Patients with improvement of >1 skin lesions were younger (P=0.028) and had higher baseline GH levels (P=0.021) than those with improvement of ≤1 skin lesion.ConclusionsDermatoscopy and ultra-high-frequency ultrasound provided augmented visual examination of the cutaneous changes in acromegaly. Some of the skin lesions could improve or reverse after pituitary surgery. Baseline GH levels, age, and endocrine remission were correlated with skin improvement at 3-month follow-up.

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“…CVG būdingi odos pakitimai gali pasireikšti ir kartu su specifiniais genetiniais sindromais, tokiais kaip Noonan ar Ternerio sindromas [10]. Mokslinėje literatūroje trūksta sutarimo, kaip tokius atvejus reikėtų klasifikuoti [10,11]. Pavyzdžiui, Ternerio sindromo atveju stebimi odos pakitimai gali būti sukelti tiek, kaip manoma, nykstančios Ternerio sindromą turintiems būdingos limfedemos (tokie CVG atvejai galėtų būti priskirti pirminiam tipui), tiek odos hamartomos [10,12].…”

Section: Tyrimo Medžiaga Ir Metodaiunclassified

Cutis Verticis Gyrata – Klinika, Diagnostika, Gydymas

Berškys

2024

Health Sciences

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Cutis verticis gyrata (CVG) – reta odos patologija, kuri dažniausiai pasireiškia galvos smegenų žievės vingius primenančių klosčių formavimusi skalpo odoje. Naujausioje klasifikacijoje CVG suskirstyta į 3 tipus, atsižvelgiant į jiems būdingą odos pakitimų morfologiją ir kitą patologiją. Šio darbo tikslas yra apžvelgti cutis verticis gyrata kliniką, diagnostiką ir gydymą, išanalizavus aktualią mokslinę literatūrą. Literatūros analizė atskleidė, jog pirminio esencialinio CVG klinika apsiriboja odos pakitimais, o cutis verticis gyrata protinės negalios tipui. Be odos pažeidimų, būdingi ir neuropsichiatriniai ar oftalmologiniai radiniai. Antrinio CVG klinikai būdingi odos pažeidimus sukėlusios patologijos požymiai. Diagnostikos tikslas yra nustatyti pakitimus sukėlusią priežastį ir konkretų atvejį priskirti vienam iš CVG tipų. Tam taikomas ištyrimas, galintis apimti anamnezės surinkimą, fizinį ištyrimą, odos biopsiją bei laboratorinius ir vaizdinius tyrimus. Gydymo taktikos pasirinkimas priklauso nuo ligos tipo, paciento norų bei pažeidimo laipsnio. Antrinio CVG atveju, gydymo pagrindas yra CVG sukėlusios priežasties korekcija. Visai atvejais būtina užtikrinti pažeistos odos higieną. Odos pažeidimus galima koreguoti chirurginėmis priemonėmis ar pasitelkiant mažiau invazyvias, vietines procedūras.

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Acromegaly-related cutis verticis gyrata

Cited by 8 publications

References 93 publications

Pseudoacromegaly—A challenging entity in the endocrine clinic: A systematic review

Pseudoacromegaly—A challenging entity in the endocrine clinic: A systematic review

Sub-macroscopic skin presentation of acromegaly and effect of pituitary tumor surgery: A study using dermatoscopy and ultra-high-frequency ultrasound

Cutis Verticis Gyrata – Klinika, Diagnostika, Gydymas

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