There are several benignBenignFibroadenoma vulvar diseases, but some of the most common are Bartholin cyst and abscessAbscessAccumulation of purulent material in tissues, organs, or circumscribed spaces, usually associated with signs of infection.Chronic Granulomatous Disease, lichen sclerosus, and lichen simplex chronicus. Bartholin cystsCystsAny fluid-filled closed cavity or sac that is lined by an epithelium. Cysts can be of normal, abnormal, non-neoplastic, or neoplastic tissues.Fibrocystic Change are formed due to an obstruction in the excretory duct that causes retention of their secretions (lubricating mucus). Bartholin cystsCystsAny fluid-filled closed cavity or sac that is lined by an epithelium. Cysts can be of normal, abnormal, non-neoplastic, or neoplastic tissues.Fibrocystic Change present as nontender fluctuantFluctuantDermatologic Examination masses at the 4 and/or 8 o'clock positions in the labia. If a Bartholin cyst becomes infected, it can develop into an extremely painful abscessAbscessAccumulation of purulent material in tissues, organs, or circumscribed spaces, usually associated with signs of infection.Chronic Granulomatous Disease. Lichen sclerosus is a chronic dermatologic condition that causes progressive thinning and fibrosisFibrosisAny pathological condition where fibrous connective tissue invades any organ, usually as a consequence of inflammation or other injury.Bronchiolitis Obliterans of the vulvar, perineal, and perianal skinSkinThe skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue.Skin: Structure and Functions, and presents classically with itching and white plaques. Lichen simplex chronicus is a thickening of the vulvar skinSkinThe skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue.Skin: Structure and Functions due to chronic itching or rubbing, which often occurs in the setting of atopic or contact dermatitisContact dermatitisA type of acute or chronic skin reaction in which sensitivity is manifested by reactivity to materials or substances coming in contact with the skin. It may involve allergic or non-allergic mechanisms.Male Genitourinary Examination.
Bartholin cystsCystsAny fluid-filled closed cavity or sac that is lined by an epithelium. Cysts can be of normal, abnormal, non-neoplastic, or neoplastic tissues.Fibrocystic Change are cystsCystsAny fluid-filled closed cavity or sac that is lined by an epithelium. Cysts can be of normal, abnormal, non-neoplastic, or neoplastic tissues.Fibrocystic Change within the Bartholin gland that result from the obstruction of their excretory duct and retention of their secretory fluid. If this fluid, or the gland itself, becomes infected, a Bartholin abscessAbscessAccumulation of purulent material in tissues, organs, or circumscribed spaces, usually associated with signs of infection.Chronic Granulomatous Disease is formed.
Paired glands located on both sides of the vestibuleVestibuleAn oval, bony chamber of the inner ear, part of the bony labyrinth. It is continuous with bony cochlea anteriorly, and semicircular canals posteriorly. The vestibule contains two communicating sacs (utricle and saccule) of the balancing apparatus. The oval window on its lateral wall is occupied by the base of the stapes of the middle ear.Ear: Anatomy (space between the labia minoraLabia minoraVagina, Vulva, and Pelvic Floor: Anatomy, which opens into the vaginaVaginaThe vagina is the female genital canal, extending from the vulva externally to the cervix uteri internally. The structures have sexual, reproductive, and urinary functions and a rich blood supply, mainly arising from the internal iliac artery.Vagina, Vulva, and Pelvic Floor: Anatomy)
Secrete lubricating mucus into the vestibuleVestibuleAn oval, bony chamber of the inner ear, part of the bony labyrinth. It is continuous with bony cochlea anteriorly, and semicircular canals posteriorly. The vestibule contains two communicating sacs (utricle and saccule) of the balancing apparatus. The oval window on its lateral wall is occupied by the base of the stapes of the middle ear.Ear: Anatomy during sexual arousal
Open into the vestibuleVestibuleAn oval, bony chamber of the inner ear, part of the bony labyrinth. It is continuous with bony cochlea anteriorly, and semicircular canals posteriorly. The vestibule contains two communicating sacs (utricle and saccule) of the balancing apparatus. The oval window on its lateral wall is occupied by the base of the stapes of the middle ear.Ear: Anatomy at 4 and 8 o’clock positions (when viewing the vaginal opening as the center of the clock face)
Location of the Bartholin glands and duct openings
Image: “The external female genitalia are referred to collectively as the vulva” by OpenStax College. License: CC BY 4.0
Bartholin gland cyst
Image by Lecturio.
Epidemiology
Most common vulvar massMassThree-dimensional lesion that occupies a space within the breastImaging of the Breast
Develop in approximately 2% of women of reproductive age
Risk factors:
ObesityObesityObesity is a condition associated with excess body weight, specifically with the deposition of excessive adipose tissue. Obesity is considered a global epidemic. Major influences come from the western diet and sedentary lifestyles, but the exact mechanisms likely include a mixture of genetic and environmental factors. Obesity
Poor hygiene
Shaving or waxing of pubic hair
ImmunocompromisedimmunocompromisedA human or animal whose immunologic mechanism is deficient because of an immunodeficiency disorder or other disease or as the result of the administration of immunosuppressive drugs or radiation.Gastroenteritis individuals
PregnancyPregnancyThe status during which female mammals carry their developing young (embryos or fetuses) in utero before birth, beginning from fertilization to birth.Pregnancy: Diagnosis, Physiology, and Care
Etiology and pathogenesis
Nonspecific inflammationInflammationInflammation is a complex set of responses to infection and injury involving leukocytes as the principal cellular mediators in the body’s defense against pathogenic organisms. Inflammation is also seen as a response to tissue injury in the process of wound healing. The 5 cardinal signs of inflammation are pain, heat, redness, swelling, and loss of function. Inflammation or trauma of the duct → duct occlusion → mucinous fluid accumulation within the gland (Bartholin cyst)
Bartholin abscessAbscessAccumulation of purulent material in tissues, organs, or circumscribed spaces, usually associated with signs of infection.Chronic Granulomatous Disease:
MRSAMRSAA strain of Staphylococcus aureus that is non-susceptible to the action of methicillin. The mechanism of resistance usually involves modification of normal or the presence of acquired penicillin binding proteins.Staphylococcus
Most common single pathogen: Escherichia coliEscherichia coliThe gram-negative bacterium Escherichia coli is a key component of the human gut microbiota. Most strains of E. coli are avirulent, but occasionally they escape the GI tract, infecting the urinary tract and other sites. Less common strains of E. coli are able to cause disease within the GI tract, most commonly presenting as abdominal pain and diarrhea. Escherichia coli
Less common, but also potentially due to STIsSTIsSexually transmitted infections (STIs) or sexually transmitted diseases (STDs) are infections that spread either by vaginal intercourse, anal sex, or oral sex. Symptoms and signs may include vaginal discharge, penile discharge, dysuria, skin lesions (e.g., warts, ulcers) on or around the genitals, and pelvic pain. Some infections can lead to infertility and chronic debilitating disease.Sexually Transmitted Infections (STIs) (e.g., Neisseria gonorrhoeaeNeisseria gonorrhoeaeA species of gram-negative, aerobic bacteria primarily found in purulent venereal discharges. It is the causative agent of gonorrhea.Neisseria, Chlamydia trachomatisChlamydia trachomatisType species of Chlamydia causing a variety of ocular and urogenital diseases.Chlamydia)
Clinical presentation
Signs and symptoms of Bartholin cystsCystsAny fluid-filled closed cavity or sac that is lined by an epithelium. Cysts can be of normal, abnormal, non-neoplastic, or neoplastic tissues.Fibrocystic Change:
Larger cystsCystsAny fluid-filled closed cavity or sac that is lined by an epithelium. Cysts can be of normal, abnormal, non-neoplastic, or neoplastic tissues.Fibrocystic Change may cause mild discomfort (especially during intercourse or movement).
Signs and symptoms of a Bartholin abscessAbscessAccumulation of purulent material in tissues, organs, or circumscribed spaces, usually associated with signs of infection.Chronic Granulomatous Disease:
Unilateral palpable massMassThree-dimensional lesion that occupies a space within the breastImaging of the Breast near the posterior vaginal introitus
May measure up to 4‒5 cm
Significant painPainAn unpleasant sensation induced by noxious stimuli which are detected by nerve endings of nociceptive neurons.Pain: Types and Pathways/tenderness in the vulvaVulvaThe vulva is the external genitalia of the female and includes the mons pubis, labia majora, labia minora, clitoris, vestibule, vestibular bulb, and greater vestibular glands. Vagina, Vulva, and Pelvic Floor: Anatomy:
Acute onset
Often difficult to walk and sit due to painPainAn unpleasant sensation induced by noxious stimuli which are detected by nerve endings of nociceptive neurons.Pain: Types and Pathways
DyspareuniaDyspareuniaRecurrent genital pain occurring during, before, or after sexual intercourse in either the male or the female.Primary Ovarian Insufficiency (painPainAn unpleasant sensation induced by noxious stimuli which are detected by nerve endings of nociceptive neurons.Pain: Types and Pathways with intercourse)
FeverFeverFever is defined as a measured body temperature of at least 38°C (100.4°F). Fever is caused by circulating endogenous and/or exogenous pyrogens that increase levels of prostaglandin E2 in the hypothalamus. Fever is commonly associated with chills, rigors, sweating, and flushing of the skin. Fever (possible, but uncommon)
Bartholin gland cyst: This cyst was recently injected with local anesthetic, resulting in the bleeding seen in the image.
Image: “Procedure with CO2 laser in the treatment of a Bartholin cyst” by Speck NM, Boechat KP, Santos GM, Ribalta JC. License: CC BY 4.0, cropped by Lecturio.
Diagnosis
Both cystsCystsAny fluid-filled closed cavity or sac that is lined by an epithelium. Cysts can be of normal, abnormal, non-neoplastic, or neoplastic tissues.Fibrocystic Change and abscesses are diagnosed clinically.
Culture: any drainage or fluid
Indications for biopsyBiopsyRemoval and pathologic examination of specimens from the living body.Ewing Sarcoma to rule out malignancyMalignancyHemothorax (rarely needed):
Individuals ≥ 40 years of age or postmenopausal women
If the massMassThree-dimensional lesion that occupies a space within the breastImaging of the Breast has solid components
If the massMassThree-dimensional lesion that occupies a space within the breastImaging of the Breast is fixed to surrounding tissue
Screen for STIsSTIsSexually transmitted infections (STIs) or sexually transmitted diseases (STDs) are infections that spread either by vaginal intercourse, anal sex, or oral sex. Symptoms and signs may include vaginal discharge, penile discharge, dysuria, skin lesions (e.g., warts, ulcers) on or around the genitals, and pelvic pain. Some infections can lead to infertility and chronic debilitating disease.Sexually Transmitted Infections (STIs) if the individual is at high risk.
To ↓ risk of recurrence, a word catheter is placed in the incision:
Allows continued drainage for up to 4 weeks
Allows the new tract to epithelialize and remain open, preventing reaccumulation of fluid
Management of a Bartholin gland abscess: A: Incision and drainage: A scalpel is used to make an incision in the abscess wall, allowing the puss to drain B: Word catheter placement: The catheter is placed in the abscess and provides a route for continued drainage by preventing the incision from closing C: Word catheter within the cyst cavity: A small balloon inflated inside the cyst wall keeps the catheter in place.
Image by Lecturio.
Advanced treatment: if the cyst/abscessAbscessAccumulation of purulent material in tissues, organs, or circumscribed spaces, usually associated with signs of infection.Chronic Granulomatous Disease continues to recur or never fully resolves
Marsupialization: Open the cyst and suture the cyst walls to the surrounding tissue to keep it open.
Gland excision:
Definitive treatment
Rarely performed due to ↑ risk of complications
Antibiotics:
Indications:
Culture-positive for MRSAMRSAA strain of Staphylococcus aureus that is non-susceptible to the action of methicillin. The mechanism of resistance usually involves modification of normal or the presence of acquired penicillin binding proteins.Staphylococcus
Surrounding cellulitisCellulitisCellulitis is a common infection caused by bacteria that affects the dermis and subcutaneous tissue of the skin. It is frequently caused by Staphylococcus aureus and Streptococcus pyogenes. The skin infection presents as an erythematous and edematous area with warmth and tenderness. Cellulitis
ImmunocompromisedimmunocompromisedA human or animal whose immunologic mechanism is deficient because of an immunodeficiency disorder or other disease or as the result of the administration of immunosuppressive drugs or radiation.Gastroenteritis individuals
Signs of systemic infection
NOT indicated for:
CystsCystsAny fluid-filled closed cavity or sac that is lined by an epithelium. Cysts can be of normal, abnormal, non-neoplastic, or neoplastic tissues.Fibrocystic Change
Regimens should cover anaerobesAnaerobesLincosamides and MRSAMRSAA strain of Staphylococcus aureus that is non-susceptible to the action of methicillin. The mechanism of resistance usually involves modification of normal or the presence of acquired penicillin binding proteins.Staphylococcus.
Recommended regimens include:
Trimethoprim-sulfamethoxazole +/- metronidazoleMetronidazoleA nitroimidazole used to treat amebiasis; vaginitis; trichomonas infections; giardiasis; anaerobic bacteria; and treponemal infections.Pyogenic Liver Abscess
Lichen sclerosus is a chronic, progressive, dermatologic condition of the vulvaVulvaThe vulva is the external genitalia of the female and includes the mons pubis, labia majora, labia minora, clitoris, vestibule, vestibular bulb, and greater vestibular glands. Vagina, Vulva, and Pelvic Floor: Anatomy characterized by inflammationInflammationInflammation is a complex set of responses to infection and injury involving leukocytes as the principal cellular mediators in the body’s defense against pathogenic organisms. Inflammation is also seen as a response to tissue injury in the process of wound healing. The 5 cardinal signs of inflammation are pain, heat, redness, swelling, and loss of function. Inflammation and epithelial thinning. As it progresses, scarringScarringInflammation can distort the anatomy. Lichen sclerosus itself is benignBenignFibroadenoma, but is associated with an increased risk for vulvar squamous cell carcinomaSquamous cell carcinomaCutaneous squamous cell carcinoma (cSCC) is caused by malignant proliferation of atypical keratinocytes. This condition is the 2nd most common skin malignancy and usually affects sun-exposed areas of fair-skinned patients. The cancer presents as a firm, erythematous, keratotic plaque or papule. Squamous Cell Carcinoma (SCC) (SCC).
IncidenceIncidenceThe number of new cases of a given disease during a given period in a specified population. It also is used for the rate at which new events occur in a defined population. It is differentiated from prevalence, which refers to all cases in the population at a given time.Measures of Disease Frequency:
Approximately 15 per 100,000 woman-years
IncidenceIncidenceThe number of new cases of a given disease during a given period in a specified population. It also is used for the rate at which new events occur in a defined population. It is differentiated from prevalence, which refers to all cases in the population at a given time.Measures of Disease Frequency is increasing.
Pathogenesis
Etiology is unknown.
Contributing factors likely include:
Genetic factors
Local factors (e.g., local irritation)
Hormonal factors
Immunologic abnormalities
Chronic, progressive process
InflammationInflammationInflammation is a complex set of responses to infection and injury involving leukocytes as the principal cellular mediators in the body’s defense against pathogenic organisms. Inflammation is also seen as a response to tissue injury in the process of wound healing. The 5 cardinal signs of inflammation are pain, heat, redness, swelling, and loss of function. Inflammation and altered fibroblast function lead to:
Thinning of the epidermisEpidermisThe external, nonvascular layer of the skin. It is made up, from within outward, of five layers of epithelium: (1) basal layer (stratum basale epidermidis); (2) spinous layer (stratum spinosum epidermidis); (3) granular layer (stratum granulosum epidermidis); (4) clear layer (stratum lucidum epidermidis); and (5) horny layer (stratum corneum epidermidis).Skin: Structure and Functions
Areas of atrophyAtrophyDecrease in the size of a cell, tissue, organ, or multiple organs, associated with a variety of pathological conditions such as abnormal cellular changes, ischemia, malnutrition, or hormonal changes.Cellular Adaptation
FibrosisFibrosisAny pathological condition where fibrous connective tissue invades any organ, usually as a consequence of inflammation or other injury.Bronchiolitis Obliterans in the upper dermisDermisA layer of vascularized connective tissue underneath the epidermis. The surface of the dermis contains innervated papillae. Embedded in or beneath the dermis are sweat glands; hair follicles; and sebaceous glands.Skin: Structure and Functions
Clinical presentation
Symptoms:
Vulvar pruritusPruritusAn intense itching sensation that produces the urge to rub or scratch the skin to obtain relief.Atopic Dermatitis (Eczema)
Vulvar irritation or soreness
DyspareuniaDyspareuniaRecurrent genital pain occurring during, before, or after sexual intercourse in either the male or the female.Primary Ovarian Insufficiency
Anal discomfort:
PruritusPruritusAn intense itching sensation that produces the urge to rub or scratch the skin to obtain relief.Atopic Dermatitis (Eczema)
“Figure of 8” pattern: white plaques around the labia, perineumPerineumThe body region lying between the genital area and the anus on the surface of the trunk, and to the shallow compartment lying deep to this area that is inferior to the pelvic diaphragm. The surface area is between the vulva and the anus in the female, and between the scrotum and the anus in the male.Vagina, Vulva, and Pelvic Floor: Anatomy, and anus
Lesions may also appear:
Hemorrhagic or purpuric
Eroded or ulcerated
Hyperkeratotic
Areas where fissures are common:
Posterior fourchette
Perianal region
Interlabial folds
Periclitoral region
ExcoriationsExcoriationsExcoriation is a linear abrasion produced by mechanical means (scratching, rubbing, or picking) that usually involves only the epidermis but can reach the papillary dermis.Secondary Skin Lesions, which may be associated with:
Mild lichenificationLichenificationAtopic Dermatitis (Eczema) (thickening of the epidermisEpidermisThe external, nonvascular layer of the skin. It is made up, from within outward, of five layers of epithelium: (1) basal layer (stratum basale epidermidis); (2) spinous layer (stratum spinosum epidermidis); (3) granular layer (stratum granulosum epidermidis); (4) clear layer (stratum lucidum epidermidis); and (5) horny layer (stratum corneum epidermidis).Skin: Structure and Functions)
EdemaEdemaEdema is a condition in which excess serous fluid accumulates in the body cavity or interstitial space of connective tissues. Edema is a symptom observed in several medical conditions. It can be categorized into 2 types, namely, peripheral (in the extremities) and internal (in an organ or body cavity). Edema of the labia minoraLabia minoraVagina, Vulva, and Pelvic Floor: Anatomy
Smaller introitus and perineumPerineumThe body region lying between the genital area and the anus on the surface of the trunk, and to the shallow compartment lying deep to this area that is inferior to the pelvic diaphragm. The surface area is between the vulva and the anus in the female, and between the scrotum and the anus in the male.Vagina, Vulva, and Pelvic Floor: Anatomy
The vaginaVaginaThe vagina is the female genital canal, extending from the vulva externally to the cervix uteri internally. The structures have sexual, reproductive, and urinary functions and a rich blood supply, mainly arising from the internal iliac artery.Vagina, Vulva, and Pelvic Floor: Anatomy is generally not involved.
Extragenital lichen sclerosus is possible, with white plaques most commonly seen on:
Thighs
BreastsBreastsThe breasts are found on the anterior thoracic wall and consist of mammary glands surrounded by connective tissue. The mammary glands are modified apocrine sweat glands that produce milk, which serves as nutrition for infants. Breasts are rudimentary and usually nonfunctioning in men. Breasts: Anatomy
Shoulders, neckNeckThe part of a human or animal body connecting the head to the rest of the body.Peritonsillar Abscess, and back
A 65-year-old woman with lichen sclerosus showing the characteristic “figure-of-8” distribution: Typical lesions include white, flat papules.
Image: “A 65-year-old woman with lichen sclerosus” by Fistarol SK. License: CC0 1.0
Diagnosis
The diagnosis can be made clinically, although biopsies are often preferred to confirm the diagnosis.
Vulvar biopsyBiopsyRemoval and pathologic examination of specimens from the living body.Ewing Sarcoma:
Gold standard for diagnosis (though not all cases require biopsyBiopsyRemoval and pathologic examination of specimens from the living body.Ewing Sarcoma)
Upper dermisDermisA layer of vascularized connective tissue underneath the epidermis. The surface of the dermis contains innervated papillae. Embedded in or beneath the dermis are sweat glands; hair follicles; and sebaceous glands.Skin: Structure and Functions: homogenization of collagenCollagenA polypeptide substance comprising about one third of the total protein in mammalian organisms. It is the main constituent of skin; connective tissue; and the organic substance of bones (bone and bones) and teeth (tooth).Connective Tissue: Histology with a bandA bandSkeletal Muscle Contraction of lymphocytesLymphocytesLymphocytes are heterogeneous WBCs involved in immune response. Lymphocytes develop from the bone marrow, starting from hematopoietic stem cells (HSCs) and progressing to common lymphoid progenitors (CLPs). B and T lymphocytes and natural killer (NK) cells arise from the lineage.Lymphocytes: Histology
Histologic section of a vulvar biopsy demonstrating the characteristic findings in lichen sclerosus: Note the thinned epidermal layer.
Image: “Lichen sclerosus, atrophic” by Ed Uthman. License: CC BY 2.0
ClobetasolClobetasolA derivative of prednisolone with high glucocorticoid activity and low mineralocorticoid activity. Absorbed through the skin faster than fluocinonide, it is used topically in treatment of psoriasis but may cause marked adrenocortical suppression.Glucocorticoids (topical):
Treatment of choice
Initial treatment: 6‒12 weeks
Maintenance treatment: may be lifelong
Good vulvar hygiene (1st line, along with clobetasolClobetasolA derivative of prednisolone with high glucocorticoid activity and low mineralocorticoid activity. Absorbed through the skin faster than fluocinonide, it is used topically in treatment of psoriasis but may cause marked adrenocortical suppression.Glucocorticoids):
Avoid harsh soaps, shampoos, and laundry detergents.
Avoid excessive washing/scrubbing.
Gently, but fully, rinse the area with water only.
Apply topical emollientsEmollientsOleaginous substances used topically to soothe, soften or protect skin or mucous membranes. They are used also as vehicles for other dermatologic agents.Pityriasis Rosea (e.g., vaseline, aquaphor) after rinsing (acts as a skinSkinThe skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue.Skin: Structure and Functions protectant to ↓ irritation).
White cotton or silk underwear (thongs, lace, or synthetic materials to be avoided)
Avoid tight-fitting pants.
Treatment resistanceResistancePhysiologically, the opposition to flow of air caused by the forces of friction. As a part of pulmonary function testing, it is the ratio of driving pressure to the rate of air flow.Ventilation: Mechanics of Breathing:
TriamcinoloneTriamcinoloneA glucocorticoid given, as the free alcohol or in esterified form, orally, intramuscularly, by local injection, by inhalation, or applied topically in the management of various disorders in which corticosteroids are indicated.Glucocorticoids (injected into the lesion)
TacrolimusTacrolimusA macrolide isolated from the culture broth of a strain of streptomyces tsukubaensis that has strong immunosuppressive activity in vivo and prevents the activation of T-lymphocytes in response to antigenic or mitogenic stimulation in vitro.Immunosuppressants
Rule out CandidaCandidaCandida is a genus of dimorphic, opportunistic fungi. Candida albicans is part of the normal human flora and is the most common cause of candidiasis. The clinical presentation varies and can include localized mucocutaneous infections (e.g., oropharyngeal, esophageal, intertriginous, and vulvovaginal candidiasis) and invasive disease (e.g., candidemia, intraabdominal abscess, pericarditis, and meningitis). Candida/Candidiasis infection or bacterial superinfection.
BiopsyBiopsyRemoval and pathologic examination of specimens from the living body.Ewing Sarcoma, if not yet done for:
PhototherapyPhototherapyTreatment of disease by exposure to light, especially by variously concentrated light rays or specific wavelengths.Hyperbilirubinemia of the Newborn (limited data)
Topical progesteroneProgesteroneThe major progestational steroid that is secreted primarily by the corpus luteum and the placenta. Progesterone acts on the uterus, the mammary glands and the brain. It is required in embryo implantation; pregnancy maintenance, and the development of mammary tissue for milk production. Progesterone, converted from pregnenolone, also serves as an intermediate in the biosynthesis of gonadal steroid hormones and adrenal corticosteroids.Gonadal Hormones (traditional treatment, although less effective than clobetasolClobetasolA derivative of prednisolone with high glucocorticoid activity and low mineralocorticoid activity. Absorbed through the skin faster than fluocinonide, it is used topically in treatment of psoriasis but may cause marked adrenocortical suppression.Glucocorticoids)
PrognosisPrognosisA prediction of the probable outcome of a disease based on a individual’s condition and the usual course of the disease as seen in similar situations.Non-Hodgkin Lymphomas
Increased risk of SCC of the vulvaVulvaThe vulva is the external genitalia of the female and includes the mons pubis, labia majora, labia minora, clitoris, vestibule, vestibular bulb, and greater vestibular glands. Vagina, Vulva, and Pelvic Floor: Anatomy → long-term follow-up is recommended
RemissionRemissionA spontaneous diminution or abatement of a disease over time, without formal treatment.Cluster Headaches less likely with age
Lichen simplex chronicus is a benignBenignFibroadenoma vulvar skinSkinThe skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue.Skin: Structure and Functions disorder characterized by hyperkeratosisHyperkeratosisIchthyosis Vulgaris (thickening of the skinSkinThe skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue.Skin: Structure and Functions) that occurs secondary to chronic vulvar irritation. Note: this condition can also occur on other areas of the body.
Epidemiology
The exact frequency is unknown.
More common in women than men (2:1)
Typically occurs in mid-to-late adulthood (30‒50 years of age)
Etiology
The exact pathophysiology is unknown, but anything that leads to chronic rubbing or itching of the vulvaVulvaThe vulva is the external genitalia of the female and includes the mons pubis, labia majora, labia minora, clitoris, vestibule, vestibular bulb, and greater vestibular glands. Vagina, Vulva, and Pelvic Floor: Anatomy can cause lichen simplex chronicus. Common factors include:
Vulvar eczemaEczemaAtopic dermatitis, also known as eczema, is a chronic, relapsing, pruritic, inflammatory skin disease that occurs more frequently in children, although adults can also be affected. The condition is often associated with elevated serum levels of IgE and a personal or family history of atopy. Skin dryness, erythema, oozing, crusting, and lichenification are present. Atopic Dermatitis (Eczema)
Insect bites
Psychological disorders including:
AnxietyAnxietyFeelings or emotions of dread, apprehension, and impending disaster but not disabling as with anxiety disorders.Generalized Anxiety Disorder/depression
OCDOCDObsessive-compulsive disorder (OCD) is a condition characterized by obsessions (recurring and intrusive thoughts, urges, or images) and/or compulsions (repetitive actions the person is compelled to perform) that are time-consuming and associated with functional impairment.Obsessive-compulsive Disorder (OCD)
Emotional stress
Clinical presentation
Signs and symptoms include:
Intense pruritusPruritusAn intense itching sensation that produces the urge to rub or scratch the skin to obtain relief.Atopic Dermatitis (Eczema):
Often worse at night or when still/quiet
Usually intermittent
May be described as a burning sensation
SkinSkinThe skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue.Skin: Structure and Functions lesions:
Well-demarcated, dry, patchy plaques
SkinSkinThe skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue.Skin: Structure and Functions is thick, scaly, firm, and/or rough.
Slightly erythematous
Accentuation of normal skinSkinThe skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue.Skin: Structure and Functions markings
Change in skinSkinThe skin, also referred to as the integumentary system, is the largest organ of the body. The skin is primarily composed of the epidermis (outer layer) and dermis (deep layer). The epidermis is primarily composed of keratinocytes that undergo rapid turnover, while the dermis contains dense layers of connective tissue.Skin: Structure and Functions pigmentation (typically hyperpigmentationHyperpigmentationExcessive pigmentation of the skin, usually as a result of increased epidermal or dermal melanin pigmentation, hypermelanosis. Hyperpigmentation can be localized or generalized. The condition may arise from exposure to light, chemicals or other substances, or from a primary metabolic imbalance.Malassezia Fungi)
ExcoriationsExcoriationsExcoriation is a linear abrasion produced by mechanical means (scratching, rubbing, or picking) that usually involves only the epidermis but can reach the papillary dermis.Secondary Skin Lesions
Extragenital lesions are possible:
Head, scalp, and neckNeckThe part of a human or animal body connecting the head to the rest of the body.Peritonsillar Abscess
Hands and arms
Legs and ankles
Lichen simplex chronicus of the hand
Image: “A plaque of lichen simplex chronicus” by kilbad. License: CC BY 3.0
Diagnosis
The diagnosis is primarily clinical; however, a biopsyBiopsyRemoval and pathologic examination of specimens from the living body.Ewing Sarcoma is frequently warranted to confirm the diagnosis and exclude malignancyMalignancyHemothorax.
BiopsyBiopsyRemoval and pathologic examination of specimens from the living body.Ewing Sarcoma findings include:
Hypertrophic epidermal layer
Prominent granular cell layer
Acanthosis
Spongiosis
Papillary dermal fibrosisFibrosisAny pathological condition where fibrous connective tissue invades any organ, usually as a consequence of inflammation or other injury.Bronchiolitis Obliterans
Perivascular and interstitial inflammationInflammationInflammation is a complex set of responses to infection and injury involving leukocytes as the principal cellular mediators in the body’s defense against pathogenic organisms. Inflammation is also seen as a response to tissue injury in the process of wound healing. The 5 cardinal signs of inflammation are pain, heat, redness, swelling, and loss of function. Inflammation
VulvovaginitisVulvovaginitisThe term vulvovaginitis is used to describe an acute inflammation of the vulva and vagina. Vulvovaginitis can be caused by several infectious and non-infectious etiologies, and results from disruption of the normal vaginal environment. Common signs and symptoms include pain, pruritus, erythema, edema, vaginal discharge and dyspareunia. Vulvovaginitis should be excluded by:
Wet mount (normal salineNormal salineA crystalloid solution that contains 9. 0g of sodium chloride per liter of water. It has a variety of uses, including: as a contact lens solution, in ophthalmic solutions and nasal lavage, in wound irrigation, and for fluid therapy.Intravenous Fluids)
Point-of-care tests for bacterial vaginosisBacterial vaginosisPolymicrobial, nonspecific vaginitis associated with positive cultures of gardnerella vaginalis and other anaerobic organisms and a decrease in lactobacilli. It remains unclear whether the initial pathogenic event is caused by the growth of anaerobes or a primary decrease in lactobacilli.Vulvovaginitis (BVBVPolymicrobial, nonspecific vaginitis associated with positive cultures of gardnerella vaginalis and other anaerobic organisms and a decrease in lactobacilli. It remains unclear whether the initial pathogenic event is caused by the growth of anaerobes or a primary decrease in lactobacilli.Vulvovaginitis)
ClobetasolClobetasolA derivative of prednisolone with high glucocorticoid activity and low mineralocorticoid activity. Absorbed through the skin faster than fluocinonide, it is used topically in treatment of psoriasis but may cause marked adrenocortical suppression.Glucocorticoids: for a shorter initial course
Lower potency steroidsSteroidsA group of polycyclic compounds closely related biochemically to terpenes. They include cholesterol, numerous hormones, precursors of certain vitamins, bile acids, alcohols (sterols), and certain natural drugs and poisons. Steroids have a common nucleus, a fused, reduced 17-carbon atom ring system, cyclopentanoperhydrophenanthrene. Most steroids also have two methyl groups and an aliphatic side-chain attached to the nucleus.Benign Liver Tumors may be used for a longer term as maintenance therapy.
Topical emollientsEmollientsOleaginous substances used topically to soothe, soften or protect skin or mucous membranes. They are used also as vehicles for other dermatologic agents.Pityriasis Rosea
Antibiotics, if cellulitisCellulitisCellulitis is a common infection caused by bacteria that affects the dermis and subcutaneous tissue of the skin. It is frequently caused by Staphylococcus aureus and Streptococcus pyogenes. The skin infection presents as an erythematous and edematous area with warmth and tenderness. Cellulitis is present
AntihistaminesAntihistaminesAntihistamines are drugs that target histamine receptors, particularly H1 and H2 receptors. H1 antagonists are competitive and reversible inhibitors of H1 receptors. First-generation antihistamines cross the blood-brain barrier and can cause sedation. Antihistamines for symptomatic treatment of pruritusPruritusAn intense itching sensation that produces the urge to rub or scratch the skin to obtain relief.Atopic Dermatitis (Eczema):
DiphenhydramineDiphenhydramineA histamine h1 antagonist used as an antiemetic, antitussive, for dermatoses and pruritus, for hypersensitivity reactions, as a hypnotic, an antiparkinson, and as an ingredient in common cold preparations. It has some undesired antimuscarinic and sedative effects.Antihistamines
HydroxyzineHydroxyzineA histamine h1 receptor antagonist that is effective in the treatment of chronic urticaria, dermatitis, and histamine-mediated pruritus. Unlike its major metabolite cetirizine, it does cause drowsiness. It is also effective as an antiemetic, for relief of anxiety and tension, and as a sedative.Antihistamines
Other treatment options:
PhototherapyPhototherapyTreatment of disease by exposure to light, especially by variously concentrated light rays or specific wavelengths.Hyperbilirubinemia of the Newborn (limited data)
PsychotherapyPsychotherapyPsychotherapy is interpersonal treatment based on the understanding of psychological principles and mechanisms of mental disease. The treatment approach is often individualized, depending on the psychiatric condition(s) or circumstance. Psychotherapy +/- antidepressants, as indicated
Surgical excision may be considered for small, localized lesions that do not respond to treatment.
PrognosisPrognosisA prediction of the probable outcome of a disease based on a individual’s condition and the usual course of the disease as seen in similar situations.Non-Hodgkin Lymphomas
Secondary infectionsInfectionsInvasion of the host organism by microorganisms or their toxins or by parasites that can cause pathological conditions or diseases.Chronic Granulomatous Disease
Malignant transformationTransformationChange brought about to an organism’s genetic composition by unidirectional transfer (transfection; transduction, genetic; conjugation, genetic, etc.) and incorporation of foreign DNA into prokaryotic or eukaryotic cells by recombination of part or all of that DNA into the cell’s genome.Bacteriology (rare)