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Mucous membrane pemphigoid
Published in Lionel Fry, Atlas of Bullous Diseases, 2020
Genital involvement in females presents with blisters and ulceration of the vulva. Introital narrowing may result from scarring, and in the end stage there is labial fusion. In the male, blisters and erosions of the penis occur, and scarring between the glans and foreskin.
The endocrine system
Published in C. Simon Herrington, Muir's Textbook of Pathology, 2020
This is a group of rare diseases, which are due to inherited mutations in the genes encoding the enzymes involved in steroidogenesis. The most common form is 21-hydroxylase deficiency, with a mean incidence of 1 in 14,000. There is inadequate cortisol production and secretion, which results in reduced negative feedback and consequently ACTH secretion is increased. This should cause an increase in cortisol, but due to the mutations present this does not happen, rather it causes an increase in androgens. The increased stimulation leads to massive adrenocortical hyperplasia. In female infants there is clitoromegaly and labial fusion. The internal reproductive tract is normal. Males present with precocious puberty. In two-thirds of cases, aldosterone synthesis may also impaired and these infants have features of salt loss with dehydration, vomiting, and hypotensive collapse.
Labial adhesions
Published in Alisa McQueen, S. Margaret Paik, Pediatric Emergency Medicine: Illustrated Clinical Cases, 2018
This child has labial adhesions (also called labial fusion, labial agglutination, and synechia vulvae). The cause is not known but may be associated with the low estrogen levels in pre-pubertal girls. Labial adhesions may be noted during a routine genital examination. Fusion can occur at the midline or below the labia minora. Presenting complaints include dysuria, recurrent urinary tract infections, vaginal pain, or discharge. Poor hygiene, candida vulvovaginitis, frequent diaper dermatitis, and genital trauma can lead to adhesions. Patients can also be asymptomatic. Topical estrogen cream is a conservative approach for symptomatic patients. Topical steroid cream can also be used. Manual or surgical separation of adhesions is indicated for patients with severe urinary flow obstruction and should be performed by an experienced physician.
Labial fusion in adolescence secondary to lichen sclerosus
Published in Journal of Obstetrics and Gynaecology, 2021
Andreas Kalampalikis, Sofia Ivanidou, Lina Michala
Labial fusion or agglutination results from adhesions between the labia minora leading to partial or complete obstruction of the vaginal orifice. The presence of labial fusion after menarche is unusual and its estimated prevalence in post-pubertal women is not known. The majority of cases occur during childhood, mostly between 3 months and 3 years, with an estimated incidence rate of 0.6–3%, however, the actual prevalence may be higher, since many of the patients are asymptomatic. In a low oestrogen environment, the skin that covers the genital area is thin and susceptible to infection and inflammation so that local irritants and trauma can lead to denudation of the vulva. Due to their proximity, labia minora can then fuse and when healing occurs avascular adhesions form between them. The condition is self-limiting and usually regresses without treatment, especially after menarche when circulating oestrogens rise. The presence of labial agglutination in adolescence should raise suspicion for genital trauma or other skin conditions, such as lichen sclerosus (LS) (Eyk et al. 2009; Michala and Creighton 2009; Topcuoglu et al. 2009).
Persistent Labial Minora Fusion in Reproductive Age Women: A Retrospective Case Series of Nine Patients and Review of Literature
Published in Organogenesis, 2021
Ze Liang, Juan Chen, Xin Yu, Lan Zhu
Labial fusion refers to partial or complete fusion of the labial minora (from the clitoris to the posterior commissure). This condition is most frequently referred to labial adhesion, which is related to a low estrogen state. Most labial adhesion patients are infants or postmenopausal women, and local use of estrogen can relieve most symptoms. Labial minora fusion either persisted into the reproductive age or discovered at reproductive age is an extremely rare type of labial fusion. Since the first report in 1972, only 17 sporadic case reports have been documented (Table 1),1–15 most of which were discovered at reproductive age. The main complaints of these patients were dyspareunia or difficult coitus. Physical examinations showed an otherwise normal woman with partial or complete fusion of the labial minora. In patients with complete labial fusion, a pinhole structure could be found, where urine and menstrual blood drained. Ultrasound or magnetic resonance imaging revealed normal urinary and genital systems. Surgical treatment could completely restore the normal appearance of the vulva, and all patients had a good prognosis in terms of both sexual and urinary function.