Labial adhesions in postmenopausal women are caused by various inflammatory processes in the context of estrogen deficiency. Lichen sclerosus (LS) is a chronic, progressive condition characterized by idiopathic epithelial thinning of the anogenital region with symptoms of significant pruritus, discomfort, and dyspareunia. Early diagnosis and treatment of LS can prevent disease progression, leading to labial adhesions and malignancy. We present an 84-year-old woman with long-standing vulvar Hailey-Hailey disease who developed labial adhesions. Clinical examination with vulvar biopsy revealed histopathological findings consistent with LS. Surgical separation of the labia by blunt dissection was then performed, and clobetasol ointment and vaginal dilatators were postoperatively prescribed. This resulted in a significant improvement in her symptoms. This is the first reported case of LS in a patient with Hailey-Hailey disease.

This review investigates women suffering from chronic vulval pruritus who may suffer from lichen sclerosus (LS). LS is a chronic inflammatory skin condition involving the vulva and anogenital regions. Treatment may be initiated upon suspicion, although the diagnosis is predominantly clinical and at times a skin biopsy is needed to confirm the diagnosis. LS is treated with potent to very potent topical steroids initially for three months. Prophylactic lifelong treatment is recommended to avoid long term scarring, sexual dysfunction, improve quality of life and to prevent cancer. Mild cases can be managed in general practice.

Fox-Fordyce disease (FFD), an eponym for apocrine miliaria, is a rare chronic inflammatory skin disorder of the apocrine glands characterized by a pruritic papular eruption in the apocrine gland-rich parts of the body, including the axillae, areolae, anogenital region, among others. This disorder was first described in 1902 by George Henry Fox and John Addison Fordyce as pruritic papules in the axillae. Since then, it has been seen in the other aforementioned apocrine gland containing regions. FFD has a female predominance, and the mainstay of diagnosis is clinical evaluation. Treatment is challenging, as there is a paucity of research in this area, but it is often necessary secondary to prominent pruritus. There are multiple treatment options, including topical, systemic, and surgical, although topical medicines remain the initial treatment of choice.

Pruritus is the sensation of itching; it can be caused by dermatologic and systemic conditions. An exposure history may reveal symptom triggers. A thorough skin examination, including visualization of the finger webs, anogenital region, nails, and scalp, is essential. Primary skin lesions indicate diseased skin, and secondary lesions are reactive and result from skin manipulation, such as scratching. An initial evaluation for systemic causes may include a complete blood count with differential, creatinine and blood urea nitrogen levels, liver function tests, iron studies, fasting glucose or A1C level, and a thyroid-stimulating hormone test. Additional testing, including erythrocyte sedimentation rate, HIV screening, hepatitis serologies, and chest radiography, may also be appropriate based on the history and physical examination. In the absence of primary skin lesions, physicians should consider evaluation for malignancy in older patients with chronic generalized pruritus. General management includes trigger avoidance, liberal emollient use, limiting water exposure, and administration of oral antihistamines and topical corticosteroids. If the evaluation for multiple etiologies of pruritus is ambiguous, clinicians may consider psychogenic etiologies and consultation with a specialist.

This Clinical Opinion replaces the NASPAG Clinical Recommendation: Pediatric Lichen Sclerosus published in 2014. The objective of this document is to provide guidance in the diagnosis and management of vulvar lichen sclerosus (LS) in the pediatric and adolescent patient in order to treat patient symptoms and reduce long-term sequelae. LS is a chronic inflammatory condition affecting the anogenital region that may present in the prepubertal or adolescent patient. Clinical presentations include significant pruritus, loss of pigmentation and vulvar adhesions with loss of normal vulvar architecture. Management includes topical agents for induction and maintenance therapy, as well as long-term follow-up for identification and treatment of recurrence and sequelae. This document is intended for use by both primary and specialty pediatric and adolescent gynecology (PAG) providers, including specialists in pediatrics, gynecology, adolescent medicine, and dermatology.

The mechanisms underlying itch are not fully understood. Physicians usually encounter difficulty controlling itch in generalized pruritus. Since only a small percentage of patients with generalized pruritus respond to antihistamines (H1 receptor antagonists), a variety of itch mediators and mechanisms other than histaminergic signals are considered to be involved in itch for these non-responsive patients. In 2012, we created guidelines for generalized pruritus. Those guidelines have been updated and revised to make some of the definitions, diagnostic terms, and classifications more applicable to daily clinical practice. Cutaneous pruritus as designated in these guidelines is a disease characterized by itch without an observable rash. Generalized pruritus (without skin inflammation) is defined as the presence of itch over a wide area, and not localized to a specific part of the body. This entity includes idiopathic pruritus, pruritus in the elderly, symptomatic pruritus, pregnancy-associated pruritus, drug-induced pruritus, and psychogenic pruritus. Localized pruritus (without skin inflammation) represents fixed itch localized to a specific part of the body, and includes anogenital pruritus, scalp pruritus, notalgia paresthetica, and brachioradial pruritus. These guidelines outline the current concepts and specify the diagnostic methods/treatments for cutaneous pruritus.

Vulvar lichen sclerosus (VLS) is a chronic inflammatory condition affecting the anogenital region, which may present in a prepubertal or adolescent patient. The most popular theories are its autoimmune and genetic conditioning, although theories concerning hormonal and infectious etiology have also been raised. The most common presenting symptoms of VLS is vulva pruritus, discomfort, dysuria and constipation. In physical examination, a classic "Figure 8" pattern is described, involving the labia minora, clitoral hood, and perianal region. The lesions initially are white, flat-topped papules, thin plaques, or commonly atrophic patches. Purpura is a hallmark feature of VLS. The treatment includes topical anti-inflammatory agents and long-term follow-up, as there is a high risk of recurrence and an increased risk of vulvar cancer in adult women with a history of lichen sclerosus. This article reviews vulvar lichen sclerosus in children and provides evidence-based medicine principles for treatment in the pediatric population. A systematic search of the literature shows recurrence of VLS in children. Maintenance regimens deserve further consideration.

Epidermolytic acanthomas (EA) are rare benign tumors of unclear etiology that present as flat, sometimes slightly keratotic, pale or whitish papules that are usually asymptomatic. Not uncommonly, their clinical appearance in the anogenital area might lead to misdiagnosis as other lesions that commonly develop at this site, such as condylomata acuminata. Though mainly asymptomatic, there are also reports of EA presenting with persistent genital pruritus. We describe the first reported case of pruritic scrotal EA successfully treated with topical pimecrolimus.

Condyloma acuminata is an infectious disease caused by the human papilloma virus (HPV) and one of the most common sexually transmitted infections. It is manifested as warts that frequently cause pain, pruritus, burning, and occasional bleeding. Treatment (physical, chemical, or surgical) can result in erosion, scars, or ulcers, implying inflammatory processes causing pain. In this work, a biocompatible topical hydrogel containing 2% ketorolac tromethamine was developed to manage the painful inflammatory processes occurring upon the removal of anogenital condylomas. The hydrogel was physically, mechanically, and morphologically characterized: it showed adequate characteristics for a topical formulation. Up to 73% of ketorolac in the gel can be released following a one-phase exponential model. Upon application on human skin and vaginal mucosa, ketorolac can permeate through both of these and it can be retained within both tissues, particularly on vaginal mucosa. Another advantage is that no systemic side effects should be expected after application of the gel. The hydrogel showed itself to be well tolerated in vivo when applied on humans, and it did not cause any visible irritation. Finally, ketorolac hydrogel showed 53% anti-inflammatory activity, suggesting that it is a stable and suitable formulation for the treatment of inflammatory processes, such as those occurring upon chemical or surgical removal of anogenital warts.

Labial fusion in adolescence is uncommon and is usually secondary to other skin disorders or trauma of the vulvar area. In a five-year period, we treated five patients with labial fusion in our facility with a mean age of 16.4 years. Based on clinical presentation and biopsy of the vulvar skin, lichen sclerosus (LS) was the causative factor. Four out of five had urinary problems and one suffered from an inflamed inclusion cyst. All of them had a long history of pruritus. In all cases, blunt separation of the labia minora under general anaesthesia was performed, followed by local application of a potent glucocorticoid cream and an emollient agent. One patient received additionally oral and local antibiotics. One recurrence was noted, which resolved after re-separation and more meticulous treatment. Early identification and treatment of LS are crucial to prevent distortion of the vulvar anatomy.Impact StatementWhat is already known on this subject? Labial fusion is an uncommon problem in adolescence and an underlying cause should always be investigated. Lichen sclerosus typically affects the anogenital area and can lead to fusion of the labia minora.What do the results of this study add? Urinary symptoms may be the presenting feature of LS in adolescents.What are the implications of these findings for clinical practice and/or further research? Delay in diagnosis and appropriate treatment can result in irreversible changes to the vulva.

Less is known about gastrointestinal (GI) involvement of primary skin diseases due to the difference in embryology, histology, microbiology and physiology between integument and alimentary tract. Oesophagus, following the oropharyngeal mucosa, is the most common GI segment affected by primary skin diseases, especially by eosinophilic oesophagitis, lichen planus and autoimmune bullous dermatoses like pemphigus vulgaris, mucosal membrane pemphigoid and epidermolysis bullosa acquisita. Eosinophilic oesophagitis is an emerging chronic atopic disease with oesophageal dysfunction as the typical presentation, and oesophageal narrowing, rings and stricture as late complications. Oesophageal lichen planus mainly involves the proximal to mid-oesophagus in elderly aged women with long-term oral mucosal lesions. In acute attack of pemphigus vulgaris, oesophageal involvement is not uncommon but often neglected and may cause sloughing oesophagitis (oesophagitis dissecans superficialis) with acute GI bleeding in rare cases. GI manifestation of hereditary bradykininergic angio-oedema with colicky acute abdomen mostly affects small intestine, usually in the absence of pruritus or urticaria, and is more severe and long-lasting than the acquired histaminergic form. Strong evidence supports association between inflammatory bowel disease, especially Crohn disease, and hidradenitis suppurativa/acne inversa. Patients with vitiligo need surveillance of autoimmune liver disease, autoimmune atrophic gastritis or coeliac disease when corresponding symptoms become suspect. Melanoma is the most common primary tumour metastatic to the GI tract, with small intestine predominantly targeted. Gastrointestinal involvement is not uncommon in disseminated mycosis fungoides. Extramammary Paget's disease is an intraepidermal adenocarcinoma of controversial origin, and a high association between the anogenital occurrence and colorectal adenocarcinoma has been reported. As GI tract is the largest organ system with multidimensional functions, dermatologists in daily practice should be aware of the gastrointestinal morbidities related to primary skin diseases for an early diagnosis and treatment.

Extramammary vulvar Paget's disease is a very rare presentation of this disease, with few symptoms, whose initial complaint is the appearance of a slow-growing erythematous plaque in the anogenital region associated with pruritus. The evolution is chronic and the diagnosis is often late. Surgical excision is the main treatment and should be performed with wide margins, due to the high rate of local recurrence. Reconstruction is often complex, requiring the use of local or remote flaps. This manuscript presents the case report of a 65-year-old female patient, who had been suffering from pruritus in the vulvar region for 2 years and had a well-defined erythematous lesion, which showed no improvement with topical treatments and that was diagnosed as Paget's disease after biopsy. After surgical excision, reconstruction was performed using bilateral myofasciocutaneous flap of the gracilis muscle, with excellent aesthetic and functional results..

Lichen sclerosus (LS) is a chronic, inflammatory, mucocutaneous disorder of genital and extragenital skin (1). Simultaneous involvement of the oral mucosa is extremely rare, but it may be the only affected area (2). A 55-year-old woman was referred to the Department of Oral Medicine, School of Dental Medicine University of Zagreb due to whitish lesions on the right ventrolateral part of the tongue and buccal mucosa with desquamative gingivitis (Figure 1, a-c). The lesions were asymptomatic but indurated on palpation. Histology was conclusive for oral lichen sclerosus (OLS). The lesions on gingiva were successfully treated with betamethasone ointment, three times a day for two weeks. One year earlier, she had been referred to the Department of Dermatology and Venereology with progressive pruritus and dyspareunia, white patches, obliteration of the labia minora, and stenosis of the introitus (Figure 2). Histology was conclusive for vulvar LS (Figure 3, a and b). She was successfully treated for 5 months with clobetasol propionate 0.05% ointment. The patient was taking levothyroxine to treat hypothyroidism associated with Hashimoto's thyroiditis and was otherwise healthy. Oral LS is clinically characterized by the appearance of white macules, papules, or plaques mostly appearing on labial mucosa but also on buccal, palate mucosa and on the lower lip (2,3). On the genitals, it typically manifests as atrophic white plaques, which may be accompanied by purpura or fissuring (1). While vulvar LS is often associated with pruritus, dyspareunia, and dysuria, OLS is often asymptomatic, although pain, soreness, pruritus, and tightness when opening the mouth can be present (1,2). Oral manifestations of LS, as well as association of anogenital and oral LS, are rarely reported in the literature (4-6). Tomo et al. searched the Medline database for papers reporting oral LS cases with histological diagnosis confirmation from 1957 to 2016 and found only 34 cases of oral LS with histopathologic confirmation of the diagnosis (4). Kakko et al. reported 39 histologically proven cases of OLS (2). Attilli et al. (5) reviewed the clinical and histologic features of 72 cases of LS with oral/genital involvement. They reported that LS was diagnosed with exclusive genital lesions in 45, exclusive lip involvement in 20, and orogenital involvement in only 7 cases (5). Some believe that many cases of clinically diagnosed lichen planus may actually be LS and that isolated oral mucosal LS may not be as rare as is generally thought (2). While vulvar LS can occur at any age with increasing incidence with age, the median age of patients with OLS was 34 years and most of the patients were female (1,2,5). Due to the small number of patients in the literature, treatment recommendations for OLS are not available. In case of symptomatic oral lesions, topical or intralesional corticosteroids are considered to be the first-line treatment (2). First-line treatment for anogenital LS is a potent to very potent topical corticosteroid ointment, and second-line therapies include topical calcineurin inhibitors 1% pimecrolimus and 0.1% and 0.03% tacrolimus (1). For treatment-resistant genital LS, oral retinoids, methotrexate, and possibly local steroid injections for single lesions are mainly applicable for women (1). There is limited evidence for systemic treatments for both conditions. If it is not treated, genital LS is associated with a greater degree of scarring and an elevated risk of progression to squamous cell cancer; however, malignant transformation of OLS has not been reported (1-6). Due to the very rare presentation in the oral cavity, it is important to notice these lesions during a dental exam.

Lichen sclerosus et atrophicus is an acquired chronic inflammatory dermatosis commonly affecting the vulvar and perianal regions. It is associated with an increased risk of vulvar cancer even though it is not a premalignant condition itself. The true precursor of cancer associated with lichen sclerosus (LS) is vulvar intraepithelial neoplasia (VIN), differentiated type. The diagnosis is usually clinical, but in some cases, a biopsy can be performed, especially to exclude VIN or cancer. All females with anogenital LS can be offered clobetasol propionate 0.05% ointment on a regimen for 3 months (once a day for a month, followed by alternative days for a month, and then, twice weekly for a month), combined with a soap substitute and a barrier preparation.

Chronic anogenital pruritus can significantly impair affected patients' quality of life by disrupting their sleep, mood, sexual function, and personal relationships. Although a significant portion of these patients can be managed with hygiene measures, topical therapy, oral anti-pruritics, and allergen avoidance after patch testing, guidelines to treat patients who do not respond to standard therapy have yet to be established. We describe the therapeutic response of a case of anogenital pruritus recalcitrant to multiple topical and systemic therapies. Treatment of this patient with dupilumab, an interleukin-4 receptor alpha blocker, resulted in clinical remission at 1 year from the initiation of the therapy, without significant adverse effects.

Vulvar pruritus is a common complaint among young girls and women presenting to primary care physicians, gynecologists, and dermatologists. Female genital itch is especially disruptive because of its interference with sexual function and intimacy. Causes of vulvar itch are vast and may be inflammatory, environmental, neoplastic, or infectious, often with several causes coexisting simultaneously. Diagnosis may be difficult because of the unique anatomy and inherent properties of genital and perianal skin. Treatment is aimed at eliminating outside irritants, restoring epidermal barrier function, and suppressing inflammation.

Lichen sclerosus (LS) is a chronic inflammatory skin disease of unknown origin predominantly affecting the anogenital area that causes pruritus and pain and is associated with an increased risk of malignancy. In some cases, LS vanishes after application of imiquimod, raising the question whether human papillomavirus (HPV) may have an etiopathogenic role in anogenital LS. The databases MEDLINE and Embase were systematically searched using the PRISMA guidelines. Twenty-seven papers were included that reported the prevalence of HPV in LS and in LS associated with neoplasia. HPV was identified in 0-80% (median 22%) of all LS cases. The prevalence of HPV was higher among male patients with LS (median 29%) than among female patients (median 8%). HPV16 was the most prevalent genotype, but the distribution of genotypes indicates that even low-risk HPV can cause LS. The diverging detection rates are probably due to small sample sizes in the reviewed papers and different detection methods. Factors possibly underestimating the prevalence of HPV are a selective search for high-risk HPV, DNA destruction in fixed tissue, focally residing HPV, and possibly a clearing of HPV before the time of biopsy. Seventy-five percent of sexually active people acquire HPV during their lifetime, thus HPV alone is not a cause of LS. Genetic and immunological host factors and viral factors other than type are likely to contribute. Future studies should include patients with a short duration of symptoms, and biopsies should be multiple and fresh.

Lichen sclerosus (LS) is a benign, chronic, inflammatory skin disease with a predilection for the anogenital region in women. Although males can also be affected, the ratio of female to male incidence has been reported to be as high as 6-10:1 and possesses a bimodal age distribution of pre-pubertal girls and postmenopausal women [1, 2]. Affected skin usually demonstrates polygonal papules that coalesce into porcelain white plaques and can be associated with edema, telangiectasias, and comedo-like plug formation [3]. Lichen sclerosus can be debilitating for some patients causing significant pruritus, pain, dysuria, and dyspareunia [4]. Rarely, lichen sclerosus appears in various extragenital areas, although most cases are relatively asymptomatic [3]. Even more uncommonly, as displayed in this case report of a 69-year-old woman, LS can present extragenitally with a bullous or hemorrhagic appearance [5].

Itch management can be particularly complicated in some small areas like the scalp or the anogenital region for many reasons: the frequently poor diagnosis of the causes of itch in these areas, the dense innervation of these areas, and the symbolic value of these areas for the human psyche. The diagnosis of itchy scalp is easier than that of anogenital pruritus. Clinical examination and a careful inventory of all diseases of the patient and of the local environment are necessary. Localized treatments are frequently used at both sites, whereas specific pharmaceutical formulations are necessary for the pilose or the mucous environment. Nonetheless, systemic treatments or psychological interventions can be very useful.

Anogenital pruritus is defined as intense itching, acute or chronic, affecting the anal, perianal, perineal and genital skin, which is a dominant problem in the course of various cutaneous and systemic conditions. It is one of the common, extremely annoying symptom for which patients attend the Dermatology Outpatient Department (OPD). Anogenital skin is highly sensitive to soaps, perfumes, clothing and superficial trauma and it is more prone for itchy dermatoses as a result of warmth, friction, lack of aeration, sweating and occlusive inner garments. Anogenital pruritus is associated with a wide spectrum of diseases which includes localized infections, infestations, inflammatory dermatoses, allergic and irritant conditions, anorectal diseases, systemic causes, nutritional disorders, psychological and when the cause cannot be found out it is often termed idiopathic. Patients are highly reluctant in consulting the physician for anogenital itch in the early stage, they usually present at a later stage with either atypical manifestations or depigmentation and lichenification, secondary to constant scratching. They often resort to over the counter topical agents, particularly combination products which contain topical steroids. The irrational use of such products results in complications like skin atrophy, striae, incognito etc. A proper clinical history, clinical examination, investigations like scrapping for fungus and itch mite, skin biopsy, patch test and relevant blood investigations to rule out systemic conditions should be carried out, when needed, to arrive at an accurate diagnosis, before treating the patient.

Extramammary Paget's disease is a marginated plaque resembling Paget's disease but occurring in anogenital area, axilla or most commonly on the vulva. A 62-year-old postmenopausal woman presented with extremely pruritic plaque on the perineal skin which progressed gradually over 3 years and did not respond to any topical/systemic steroids, antibiotics, and antifungals. Examination revealed 7 × 8 cm. hypertrophic, verrucous plaque with erosions, and crusts at places. There was no evidence of visceral malignancy. Biopsy showed Paget's cells which were positive for Periodic Acid Schiff and alcian blue stain. Surgical excision was done considering the premalignant potential.

Dermatological complications in Turner syndrome are infrequent but occasionally cause significant morbidity. Lichen sclerosus (LS) is a chronic inflammatory mucocutaneous affection characterized by pruritus in the anogenital area. It is yet not clear its pathophysiology but it's linked with genetic factors and autoimmunity. This is a case report of a girl with Turner syndrome with growth hormone treatment that started with vulvar pruritus and was diagnosed as lichen sclerosus.

Epidermolytic acanthoma is an uncommon benign tumor with a predilection for the anogenital region, that is characterized histologically by epidermolytic hyperkeratosis. Most reported cases are asymptomatic while some are pruritic. Treatment of this condition is challenging and not well studied. We report a 55-year-old man with pruritic anogenital epidermolytic acanthomas that persisted despite various topical and ablative therapies, and whose pruritus was not relieved with emollients and antihistamines. He was given 0.1% tacrolimus ointment twice daily and experienced a marked improvement in his pruritus within 2 weeks. At 1 month, there was marked improvement in itch score from 8 to 3.5 (maximum of 10), itch frequency from 20 times a day to once every 4 days, sleep score from 5 to 1 (maximum of 5), and Dermatology Life Quality Index score from 15 to 5 (maximum of 30). Apart from a transient burning sensation, no other adverse effects were noted. To our knowledge, this is the first report of the effective use of a topical calcineurin inhibitor in relieving the intractable itch associated with epidermolytic acanthoma.