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Benign Vulvar Conditions

There are several benign Benign Fibroadenoma vulvar diseases, but some of the most common are Bartholin cyst and abscess Abscess Accumulation 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 cysts Cysts Any 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 cysts Cysts Any 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 fluctuant Fluctuant Dermatologic 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 abscess Abscess Accumulation 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 fibrosis Fibrosis Any 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 skin Skin The 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 skin Skin The 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 dermatitis Contact dermatitis A 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.

Last updated: Feb 1, 2022

Editorial responsibility: Stanley Oiseth, Lindsay Jones, Evelin Maza

Bartholin Cysts and Abscesses

Definition

Bartholin cysts Cysts Any 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 cysts Cysts Any 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 abscess Abscess Accumulation of purulent material in tissues, organs, or circumscribed spaces, usually associated with signs of infection. Chronic Granulomatous Disease is formed.

Anatomy review of the Bartholin glands Bartholin glands Mucus-secreting glands situated on the posterior and lateral aspect of the vestibule of the vagina. Vagina, Vulva, and Pelvic Floor: Anatomy

  • Also called the greater vestibular glands Greater vestibular glands Mucus-secreting glands situated on the posterior and lateral aspect of the vestibule of the vagina. Vagina, Vulva, and Pelvic Floor: Anatomy
  • Paired glands located on both sides of the vestibule Vestibule An 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 minora Labia minora Vagina, Vulva, and Pelvic Floor: Anatomy, which opens into the vagina Vagina The 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 vestibule Vestibule An 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
  • Normally, approximately 0.5 cm in diameter
  • Located deep to the posterior labia majora Labia majora Vagina, Vulva, and Pelvic Floor: Anatomy
  • Partially surrounded by vestibular bulbs Vestibular bulbs Vagina, Vulva, and Pelvic Floor: Anatomy and bulbospongiosus muscles
  • Ducts:
    • Approximately 2.5 cm in length
    • Open into the vestibule Vestibule An 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)

Epidemiology

  • Most common vulvar mass Mass Three-dimensional lesion that occupies a space within the breast Imaging of the Breast
  • Develop in approximately 2% of women of reproductive age
  • Risk factors:
    • Obesity Obesity Obesity 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
    • Immunocompromised immunocompromised A 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
    • Pregnancy Pregnancy The 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 inflammation Inflammation Inflammation 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 abscess Abscess Accumulation of purulent material in tissues, organs, or circumscribed spaces, usually associated with signs of infection. Chronic Granulomatous Disease:
  • Microbiology:
    • Usually polymicrobial, consisting of:
      • MRSA MRSA A 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
      • Enteric gram-negative aerobes
      • Lower genital tract anaerobes Anaerobes Lincosamides that are found in women
    • Most common single pathogen: Escherichia coli Escherichia coli The 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 STIs STIs Sexually 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 Neisseria Neisseria is a genus of bacteria commonly present on mucosal surfaces. Several species exist, but only 2 are pathogenic to humans: N. gonorrhoeae and N. meningitidis. Neisseria species are non-motile, gram-negative diplococci most commonly isolated on modified Thayer-Martin (MTM) agar. Neisseria gonorrhoeae, Chlamydia Chlamydia Chlamydiae are obligate intracellular gram-negative bacteria. They lack a peptidoglycan layer and are best visualized using Giemsa stain. The family of Chlamydiaceae comprises 3 pathogens that can infect humans: Chlamydia trachomatis, Chlamydia psittaci, and Chlamydia pneumoniae. Chlamydia trachomatis)

Clinical presentation Presentation The position or orientation of the fetus at near term or during obstetric labor, determined by its relation to the spine of the mother and the birth canal. The normal position is a vertical, cephalic presentation with the fetal vertex flexed on the neck. Normal and Abnormal Labor

Signs and symptoms of Bartholin cysts Cysts Any 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:

  • Typically present as a painless, unilateral, fluctuant Fluctuant Dermatologic Examination mass Mass Three-dimensional lesion that occupies a space within the breast Imaging of the Breast
  • Appear near the posterior vaginal introitus
  • Often 1‒3 cm in size
  • Larger cysts Cysts Any 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 abscess Abscess Accumulation of purulent material in tissues, organs, or circumscribed spaces, usually associated with signs of infection. Chronic Granulomatous Disease:

  • Unilateral palpable mass Mass Three-dimensional lesion that occupies a space within the breast Imaging of the Breast near the posterior vaginal introitus
  • May measure up to 4‒5 cm
  • Significant pain Pain An unpleasant sensation induced by noxious stimuli which are detected by nerve endings of nociceptive neurons. Pain: Types and Pathways/tenderness in the vulva Vulva The 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 pain Pain An unpleasant sensation induced by noxious stimuli which are detected by nerve endings of nociceptive neurons. Pain: Types and Pathways
    • Dyspareunia Dyspareunia Recurrent genital pain occurring during, before, or after sexual intercourse in either the male or the female. Primary Ovarian Insufficiency ( pain Pain An unpleasant sensation induced by noxious stimuli which are detected by nerve endings of nociceptive neurons. Pain: Types and Pathways with intercourse)
  • Fever Fever Fever 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)
Bleeding bartholin gland cyst

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 cysts Cysts Any 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 biopsy Biopsy Removal and pathologic examination of specimens from the living body. Ewing Sarcoma to rule out malignancy Malignancy Hemothorax (rarely needed):
    • Individuals ≥ 40 years of age or postmenopausal women
    • If the mass Mass Three-dimensional lesion that occupies a space within the breast Imaging of the Breast has solid components
    • If the mass Mass Three-dimensional lesion that occupies a space within the breast Imaging of the Breast is fixed to surrounding tissue
  • Screen for STIs STIs Sexually 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.

Management

Incision and drainage Incision And Drainage Chalazion:

  • Standard treatment
  • High risk of recurrence
  • 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
Bartholin gland cyst management

Management of a Bartholin gland abscess Abscess Accumulation of purulent material in tissues, organs, or circumscribed spaces, usually associated with signs of infection. Chronic Granulomatous Disease:
A: Incision and drainage Incision And Drainage Chalazion: A scalpel is used to make an incision in the abscess Abscess Accumulation of purulent material in tissues, organs, or circumscribed spaces, usually associated with signs of infection. Chronic Granulomatous Disease wall, allowing the puss to drain
B: Word catheter placement: The catheter is placed in the abscess Abscess Accumulation of purulent material in tissues, organs, or circumscribed spaces, usually associated with signs of infection. Chronic Granulomatous Disease 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/ abscess Abscess Accumulation 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 MRSA MRSA A 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 cellulitis Cellulitis Cellulitis 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
    • Immunocompromised immunocompromised A 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:
    • Cysts Cysts Any 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
    • Uncomplicated abscesses that can be treated with incision and drainage Incision And Drainage Chalazion
  • Regimens should cover anaerobes Anaerobes Lincosamides and MRSA MRSA A 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 +/- metronidazole Metronidazole A nitroimidazole used to treat amebiasis; vaginitis; trichomonas infections; giardiasis; anaerobic bacteria; and treponemal infections. Pyogenic Liver Abscess
    • Trimethoprim-sulfamethoxazole +/- amoxicillin-clavulanate
    • Doxycycline + metronidazole Metronidazole A nitroimidazole used to treat amebiasis; vaginitis; trichomonas infections; giardiasis; anaerobic bacteria; and treponemal infections. Pyogenic Liver Abscess

Lichen Sclerosus

Definition

Lichen sclerosus is a chronic, progressive, dermatologic condition of the vulva Vulva The 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 inflammation Inflammation Inflammation 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, scarring Scarring Inflammation can distort the anatomy. Lichen sclerosus itself is benign Benign Fibroadenoma, but is associated with an increased risk for vulvar squamous cell carcinoma Squamous cell carcinoma Cutaneous 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).

Epidemiology

  • Gender Gender Gender Dysphoria: women > men
  • Age: 2 peaks (both low-estrogen states):
    • Prepubertal girls
    • Perimenopausal or post-menopausal women
  • Incidence Incidence The 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
    • Incidence Incidence The 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
  • Inflammation Inflammation Inflammation 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 epidermis Epidermis The 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 atrophy Atrophy Decrease 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
    • Fibrosis Fibrosis Any pathological condition where fibrous connective tissue invades any organ, usually as a consequence of inflammation or other injury. Bronchiolitis Obliterans in the upper dermis Dermis A 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 Presentation The position or orientation of the fetus at near term or during obstetric labor, determined by its relation to the spine of the mother and the birth canal. The normal position is a vertical, cephalic presentation with the fetal vertex flexed on the neck. Normal and Abnormal Labor

Symptoms:

Signs:

  • Porcelain-white, “parchment-like” plaques (classic finding):
    • Most common on the labia
    • “Figure of 8” pattern: white plaques around the labia, perineum Perineum The 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
  • Excoriations Excoriations Excoriation 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 lichenification Lichenification Atopic Dermatitis (Eczema) (thickening of the epidermis Epidermis The 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)
    • Edema Edema Edema 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 minora Labia minora Vagina, Vulva, and Pelvic Floor: Anatomy
  • Scarring Scarring Inflammation → leads to “loss-of-vulvar architecture”:
    • Fusion of the labia
    • Fusion of the clitoral hood
    • Smaller introitus and perineum Perineum The 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 vagina Vagina The 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
    • Breasts Breasts The 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, neck Neck The 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

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 SK Seborrheic keratosis (sk) is the most common benign epithelial cutaneous neoplasm. The condition consists of immature keratinocytes. Seborrheic keratosis is the most common benign skin tumor in middle-aged and elderly adults and presents as a sharply demarcated, exophytic, skin lesion that may be tan or black and has a “stuck-on” appearance. Seborrheic Keratosis. License: CC0 1.0

Diagnosis

The diagnosis can be made clinically, although biopsies are often preferred to confirm the diagnosis.

Vulvar biopsy Biopsy Removal and pathologic examination of specimens from the living body. Ewing Sarcoma:

  • Gold standard for diagnosis (though not all cases require biopsy Biopsy Removal and pathologic examination of specimens from the living body. Ewing Sarcoma)
  • Indicated if:
    • Medical management fails.
    • Malignancy Malignancy Hemothorax is to be excluded.
    • Clinical diagnosis is uncertain.
  • Findings include:
    • Thinned epidermal layer
    • Areas of hyperkeratosis Hyperkeratosis Ichthyosis Vulgaris are possible.
    • Upper dermis Dermis A 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 collagen Collagen A 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 band A band Skeletal Muscle Contraction of lymphocytes Lymphocytes Lymphocytes 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

Histologic section of a vulvar biopsy Biopsy Removal and pathologic examination of specimens from the living body. Ewing Sarcoma demonstrating the characteristic findings in lichen sclerosus: Note the thinned epidermal layer.

Image: “Lichen sclerosus, atrophic” by Ed Uthman. License: CC BY 2.0

Management

The 1st-line treatment is medical management with high-potency topical corticosteroids Corticosteroids Chorioretinitis.

  • Clobetasol Clobetasol A 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 clobetasol Clobetasol A 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 emollients Emollients Oleaginous 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 skin Skin The 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 resistance Resistance Physiologically, 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:
    • Triamcinolone Triamcinolone A 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)
    • Topical calcineurin inhibitors Calcineurin Inhibitors Compounds that inhibit or block the phosphatase activity of calcineurin. Immunosuppressants:
      • Tacrolimus Tacrolimus A 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
      • Pimecrolimus Pimecrolimus Immunosuppressants
    • Rule out Candida Candida Candida 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.
    • Biopsy Biopsy Removal and pathologic examination of specimens from the living body. Ewing Sarcoma, if not yet done for:
  • Other treatment options:
    • Phototherapy Phototherapy Treatment of disease by exposure to light, especially by variously concentrated light rays or specific wavelengths. Hyperbilirubinemia of the Newborn (limited data)
    • Topical progesterone Progesterone The 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 clobetasol Clobetasol A 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)

Prognosis Prognosis A 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 vulva Vulva The 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
  • Remission Remission A spontaneous diminution or abatement of a disease over time, without formal treatment. Cluster Headaches less likely with age

Lichen Simplex Chronicus

Definition

Lichen simplex chronicus is a benign Benign Fibroadenoma vulvar skin Skin The 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 hyperkeratosis Hyperkeratosis Ichthyosis Vulgaris (thickening of the skin Skin The 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 vulva Vulva The 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:

  • Atopic dermatitis Atopic Dermatitis Atopic 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)
  • Contact (irritant) dermatitis Dermatitis Any inflammation of the skin. Atopic Dermatitis (Eczema)
  • Vulvar eczema Eczema Atopic 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:
    • Anxiety Anxiety Feelings or emotions of dread, apprehension, and impending disaster but not disabling as with anxiety disorders. Generalized Anxiety Disorder/depression
    • OCD OCD Obsessive-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 Presentation The position or orientation of the fetus at near term or during obstetric labor, determined by its relation to the spine of the mother and the birth canal. The normal position is a vertical, cephalic presentation with the fetal vertex flexed on the neck. Normal and Abnormal Labor

Signs and symptoms include:

  • Intense pruritus Pruritus An 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
  • Skin Skin The 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
    • Skin Skin The 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 skin Skin The 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 skin Skin The 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 hyperpigmentation Hyperpigmentation Excessive 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)
  • Excoriations Excoriations Excoriation 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 neck Neck The 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

Lichen simplex chronicus of the hand Hand The hand constitutes the distal part of the upper limb and provides the fine, precise movements needed in activities of daily living. It consists of 5 metacarpal bones and 14 phalanges, as well as numerous muscles innervated by the median and ulnar nerves. Hand: Anatomy

Image: “A plaque Plaque Primary Skin Lesions of lichen simplex chronicus” by kilbad. License: CC BY 3.0

Diagnosis

The diagnosis is primarily clinical; however, a biopsy Biopsy Removal and pathologic examination of specimens from the living body. Ewing Sarcoma is frequently warranted to confirm the diagnosis and exclude malignancy Malignancy Hemothorax.

  • Biopsy Biopsy Removal and pathologic examination of specimens from the living body. Ewing Sarcoma findings include:
    • Hypertrophic epidermal layer
    • Prominent granular cell layer
    • Acanthosis
    • Spongiosis
    • Papillary dermal fibrosis Fibrosis Any pathological condition where fibrous connective tissue invades any organ, usually as a consequence of inflammation or other injury. Bronchiolitis Obliterans
    • Perivascular and interstitial inflammation Inflammation Inflammation 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
  • Vulvovaginitis Vulvovaginitis The 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:
    • Microscopy of vaginal fluid:
    • Fungal cultures Fungal cultures Dermatophytes/Tinea Infections
    • Point-of-care tests for bacterial vaginosis Bacterial vaginosis Polymicrobial, 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 ( BV BV Polymicrobial, 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)

Management

  • Good vulvar hygiene
  • Medical management:
    • Topical corticosteroids Corticosteroids Chorioretinitis:
      • Clobetasol Clobetasol A 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 steroids Steroids A 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 emollients Emollients Oleaginous 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 cellulitis Cellulitis Cellulitis 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
    • Antihistamines Antihistamines Antihistamines 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 pruritus Pruritus An intense itching sensation that produces the urge to rub or scratch the skin to obtain relief. Atopic Dermatitis (Eczema):
      • Diphenhydramine Diphenhydramine A 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
      • Hydroxyzine Hydroxyzine A 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:
    • Phototherapy Phototherapy Treatment of disease by exposure to light, especially by variously concentrated light rays or specific wavelengths. Hyperbilirubinemia of the Newborn (limited data)
    • Psychotherapy Psychotherapy Psychotherapy 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.

Prognosis Prognosis A 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

  • Good with treatment
  • Lesions may resolve completely.
  • Possible complications:
    • Mild scarring Scarring Inflammation and pigment changes
    • Secondary infections Infections Invasion of the host organism by microorganisms or their toxins or by parasites that can cause pathological conditions or diseases. Chronic Granulomatous Disease
    • Malignant transformation Transformation Change 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)

References

  1. Quinn, A. (2017). Bartholin gland diseases. Medscape. Retrieved Sep 1, 2021, from https://emedicine.medscape.com/article/777112-overview 
  2. Chen, K.T. (2021). Bartholin gland masses: diagnosis and management. UpToDate. Retrieved Sep 1, 2021, from https://www.uptodate.com/contents/bartholin-gland-masses-diagnosis-and-management 
  3. Lazenby, G., Thurman, A., Soper, D.E. (2021). Vulvar abscess. UpToDate. Retrieved Sep 1, 2021, from https://www.uptodate.com/contents/vulvar-abscess 
  4. Cooper, S., Arnold, S. (2021). Vulvar lichen sclerosis. UpToDate. Retrieved Sep 1, 2021, from https://www.uptodate.com/contents/vulvar-lichen-sclerosus 
  5. Pappas-Taffer, L. (2020). Lichen sclerosus. Medscape. Retrieved Sep 1, 2021, from https://emedicine.medscape.com/article/1123316-overview#a4 
  6. Schoenfeld, J. (2020). Lichen simplex chronicus. Medscape. Retrieved Sep 1, 2021, from https://emedicine.medscape.com/article/1123423-overview 
  7. Charifa, A. (2021). Lichen simplex chronicus. StatPearls. Retrieved Sep 1, 2021, from https://www.statpearls.com/articlelibrary/viewarticle/24257/ 
  8. Larrabee, R., Kylander, D.J. (2001). Benign vulvar disorders. Identifying features, practical management of nonneoplastic conditions and tumors. Postgrad Med. 109, 151-4, 157-9, 163-4. https://pubmed.ncbi.nlm.nih.gov/11381665/
  9. Sinha, P., Sorinola, O., Luesley, D.M. (1999). Lichen sclerosus of the vulva. Long-term steroid maintenance therapy. J Reprod Med. 44, 621-4. https://pubmed.ncbi.nlm.nih.gov/10442326/
  10. Smith, Y.R., Haefner, H.K. Vulvar lichen sclerosus: Pathophysiology and treatment. Am J Clin Dermatol. 2004. 5, 105-25. https://link.springer.com/article/10.2165%2F00128071-200405020-00005

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