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Gynecologic Dermatology: Symptoms, Signs and Clinical Management
Gudula Kirtschig, Susan M Cooper
SECTION 1: GENERAL CONSIDERATIONS
CHAPTER 1:
Normal vulva
ETYMOLOGY
DEFINITION AND ANATOMY
HORMONE RECEPTOR EXPRESSION
DEVELOPMENT
Fetal period, neonate and childhood
Puberty
Adults and childbirth
Postmenopause
PHYSIOLOGIC DISCHARGE
NORMALITY VERSUS ‘SOCIETY'S IDEAL’
CHAPTER 2:
Normal histology of the vulva and anogenital area
THE VULVA IN POSTPUBERTAL AND PREMENOPAUSAL WOMEN
Hair-bearing skin of mons pubis, labia majora (Table 2.1)
‘Modified mucosa’ of interlabial sulci, labia minora, clitoris, clitoral prepuce
Glycogenated squamous mucosa of vestibulum including periurethral mucosa and introitus vagina
Specialized vestibular glands
Normal pigmentation
Innervation
Inflammatory infiltrate
THE VULVA IN PREPUBERTAL GIRLS AND POSTMENOPAUSAL WOMEN
CHAPTER 3:
Vulvovaginal care
GENERAL CARE
Patient history
Information and support
Self-examination
Sexual problems
CARE OF THE AGING VULVA/VAGINA
Estrogen hormone therapy
Topical vaginal estrogen therapy
Ospemifene
Non-hormonal vaginal moisturizers
Vaginal lubricants
CHAPTER 4:
Taking a vulvar history and examination of the vulva
HISTORY
Specific vulvar history
Gynecological history
Medical history
Dermatological history
Sexual history
EXAMINATION
General examination of skin and mucous membranes
Inspection of the vulva, perianal region and vagina
Evaluation of vulvar and vaginal pain
Equipment
Additional examinations
Vulvoscopy
Saline preparations
Staining with acetic acid
Microbiological scrapings, swabs for cultures, and viral tests
CHAPTER 5:
Scoring and recording of vulvar disease
CHAPTER 6:
How to take a vulvar biopsy and what to expect from a histopathological examination
HOW TO TAKE A VULVAR BIOPSY
Introduction
Prebiopsy medical history, written consent and information for the pathologist
Local anesthesia
Biopsy technique
Aftercare
WHAT TO EXPECT FROM A HISTOLOGICAL EXAMINATION
Introduction
WHAT TO EXPECT FROM A BIOPSY
SECTION 2: SYMPTOMS AND SIGNS OF DISEASE
CHAPTER 7:
General treatment considerations
INFORMATION ON THE DIAGNOSIS AND TREATMENT
TREATING VULVAR DISEASE AROUND OR AFTER THE MENOPAUSE
PSYCHOLOGICAL SUPPORT
CHAPTER 8:
Pruritus
INTRODUCTION
PATHOPHYSIOLOGY OF PRURITUS
CAUSES OF VULVAR PRURITUS
CHAPTER 9:
Pain
IS THE PAIN ACUTE OR CHRONIC?
IS THERE ANY VISIBLE CAUSE FOR PAIN?
WHAT IS THE QUALITY OF THE PAIN?
CAN THE PAIN BE LOCALIZED?
ARE THERE ANY PROVOKING FACTORS?
CHAPTER 10:
Red skin
INTRODUCTION
VASCULAR CHANGES
ERYTHROCYTE EXTRAVASATION
CHAPTER 11:
Broken skin
CARE OF BROKEN SKIN
CHAPTER 12:
White skin
IS THE SKIN TEXTURE NORMAL OR ABNORMAL?
IS THE PALLOR SYMMETRICAL OR ASYMMETRICAL?
ARE THE PALLOR LESIONS NUMEROUS AND WIDELY DISTRIBUTED OR LOCALIZED IN ONE PATCH?
IS PALLOR WELL DEMARCATED OR HAS IT DIFFUSE MARGINS?
CHAPTER 13:
Pigmented skin
CHAPTER 14:
Scarring
PREVENTION OF SCARRING
TREATMENT OF SCARRING
CHAPTER 15:
Swollen vulva
IS THE SWELLING ACUTE OR CHRONIC?
ARE THERE ANY ASSOCIATED SYMPTOMS?
DOES THE UNDERLYING SKIN APPEAR ABNORMAL?
ARE THERE ANY TRIGGERS?
SECTION 3: VULVAR DISEASES
CHAPTER 16:
Atopic eczema, lichen simplex, seborrheic dermatitis
ATOPIC ECZEMA
Synonyms
Introduction
Epidemiology
Pathogenesis
Immunology
Genetics
Environmental factors/triggers
Clinical presentation
Histopathology
Differential diagnoses
Principles of management
Treatment
Course and prognosis
LICHEN SIMPLEX
Synonyms
Introduction
Epidemiology
Pathogenesis
Environmental factors/triggers
Clinical presentation
Histopathology
Differential diagnoses
Principles of management
Treatment
Topical treatments
Systemic treatments
Surgical intervention
Course and prognosis
SEBORRHEIC DERMATITIS (SEBORRHEIC ECZEMA)
Introduction
Pathogenesis
Genetics
Environmental factors/triggers
Clinical presentation
Histopathology
Differential diagnoses
Principles of management
Topical treatments
Systemic treatments
Surgical intervention
Course and prognosis
CHAPTER 17:
Contact dermatitis (irritant and allergic), fixed drug eruption and immediate hypersensitivity reactions
CONTACT DERMATITIS OF THE VULVA
Synonyms
Introduction
Epidemiology
Pathophysiology
Etiology
Clinical presentation
Clinical features
Investigations
Histopathology
Patch testing
Diagnosis
Differential diagnoses
Treatment
FIXED DRUG ERUPTION
Introduction
Epidemiology
Pathophysiology
Etiology
Clinical presentation
Differential diagnoses
Investigations
Histopathology
Patch testing
Diagnosis
Treatment
IMMEDIATE TYPE I REACTION
CHAPTER 18:
Erythema multiforme majus, Stevens–Johnson syn rome/toxic epidermal necrolysis and Graft versus Host Disease
ERYTHEMA MULTIFORME MAJUS, STEVENS–JOHNSON SYNDROME/TOXIC EPIDERMAL NECROLYSIS
Synonyms
Introduction
Epidemiology
Pathogenesis
Etiology
Clinical presentation
EMM
SJS/TEN
Histopathology
Immunohistopathology
Differential diagnosis
Management
GRAFT VERSUS HOST DISEASE
Introduction
Epidemiology
Pathogenesis
Etiology
Clinical presentation
Investigations
Differential diagnosis
Histopathology
cGvHD
Management
Genital management
CHAPTER 19:
Lichen sclerosus
INTRODUCTION
EPIDEMIOLOGY
PATHOGENESIS
Immunology
T cells/Autoimmune
Disease associations
Genetics
Familial
HLA
Epigenetics
Cell kinetics
Environmental factors/triggers
Mechanical
Infectious triggers
Hormonal
Drugs
CLINICAL PRESENTATION
HISTOPATHOLOGY
DIFFERENTIAL DIAGNOSES
PRINCIPLES OF MANAGEMENT
TREATMENT
Topical treatments (Table 19.2)
Injections
Phototherapy
Systemic treatments
Surgical intervention
Psychological
ASSOCIATION WITH MALIGNANCY
COURSE AND PROGNOSIS
CHAPTER 20:
Lichen planus
INTRODUCTION
EPIDEMIOLOGY
PATHOGENESIS
Immunology
T cells/autoimmune
Disease associations
Genetics
Familial
HLA
Environmental factors/triggers
Mechanical factors
Infectious triggers
Drugs
Contact allergens
Psychological factors
CLINICAL PRESENTATION
Lichen planus affecting the anogenital site
Classical papulosquamous lichen planus
Hypertrophic lichen planus
Lichen planopilaris
‘Erosive’ mucosal lichen planus
HISTOPATHOLOGY
DIFFERENTIAL DIAGNOSES
PRINCIPLES OF MANAGEMENT
TREATMENT
Topical Treatments
Systemic treatments
Vaginal lichen planus
Surgical intervention
COURSE AND PROGNOSIS
CHAPTER 21:
Plasma cell vulvitis
INTRODUCTION
EPIDEMIOLOGY
PATHOGENESIS
CLINICAL FEATURES
INVESTIGATIONS
HISTOPATHOLOGY
DIFFERENTIAL DIAGNOSIS
MANAGEMENT AND TREATMENT
COURSE AND PROGNOSIS
CHAPTER 22:
Vulvar psoriasis
INTRODUCTION
EPIDEMIOLOGY
COMORBIDITIES
PATHOGENESIS
Genetics
Environmental factors/triggers
CLINICAL PRESENTATION
General psoriasis manifestations
Genital manifestations
INVESTIGATIONS
HISTOPATHOLOGY
DIFFERENTIAL DIAGNOSIS
MANAGEMENT AND TREATMENT
General care
Specific treatment
COURSE AND PROGNOSIS
CHAPTER 23:
Hidradenitis suppurativa
INTRODUCTION
EPIDEMIOLOGY
PATHOGENESIS
ETIOLOGY
CLINICAL PRESENTATION
Comorbidities
Inflammatory bowel disease
Pyoderma gangrenosum
Metabolic syndrome
Diseases of follicular occlusion
Cancer
Psychiatric disorders
Complications of disease
Risk factors
DIAGNOSIS
HISTOPATHOLOGY
DIFFERENTIAL DIAGNOSES
MANAGEMENT
Adjuvant therapy
Medical treatment
Antibiotics
Immunosuppressives/immunomodulating
Glucocorticosteroids
TNF-α inhibitors
Miscellaneous
Surgical treatment
Incision
Deroofing
Excision
Laser treatment
Other treatment options
PROGNOSIS
CHAPTER 24:
Granulomatous conditions of the vulva
INTRODUCTION
CROHN DISEASE – CUTANEOUS MANIFESTATIONS OF THE VULVA
Synonyms
Introduction
Epidemiology
Pathogenesis
Clinical manifestations and features of vulvar Crohn disease
Diagnostic criteria
Histopathology
Differential diagnosis
Management and treatment options for vulvar Crohn disease
Corticosteroids
Other systemic treatments
Biologics
Nutrition
Surgery
Course and prognosis
Concluding remarks
SARCOIDOSIS
Synonyms
Introduction
Epidemiology
Clinical features
Pathogenesis
Histopathology
Diagnosis
Differential diagnosis
Treatment
Prognosis
MELKERSSON–ROSENTHAL SYNDROME
Synonyms
Introduction
Epidemiology
Clinical features
Pathogenesis
Histopathology
Diagnostic criteria
Differential diagnosis
Treatment
Prognosis
IDIOPATHIC GRANULOMATOUS VULVITIS
Introduction
Etiology
Pathogenesis
Clinical features
Histopathology
Diagnosis
Differential diagnosis
Treatment
CHAPTER 25:
Scleroderma, systemic lupus erythematosus, and Sjögren syndrome
INTRODUCTION
SCLERODERMA
Synonyms
Introduction
Epidemiology
Pathogenesis
Clinical presentation and differential diagnosis
Morphea
Histopathology of scleroderma and morphea
Diagnosis
Management of vulvar complaints
SYSTEMIC LUPUS ERYTHEMATOSUS
Introduction
Epidemiology
Pathogenesis
Clinical presentation
Chronic cutaneous lesions
Other viral disease
Diagnosis
Management
Prevention
Therapeutic options
SJÖGREN SYNDROME
Introduction
Epidemiology
Pathogenesis
Clinical presentation
Diagnosis
Differential diagnosis
Principles of management
Therapeutic options
Course of disease
CHAPTER 26:
Autoimmune bullous diseases
INTRODUCTION
DIAGNOSIS OF AUTOIMMUNE BULLOUS DISEASES
Direct immunofluorescence
Indirect immunofluorescence
ELISA
Histopathology
SUBEPIDERMAL AUTOIMMUNE BULLOUS DISEASES
Bullous pemphigoid
Epidemiology
Pathogenesis
Clinical presentation
Histopathology
Diagnosis
Treatment
Course and prognosis
Childhood pemphigoid
Mucous membrane pemphigoid
Synonyms
Introduction
Epidemiology
Pathogenesis
Clinical features
Diagnosis
Treatment
Linear IgA disease
Synonym
Epidermolysis bullosa acquisita
Synonyms
Dermatitis herpetiformis
Synonyms
INTRAEPIDERMAL AUTOIMMUNE BULLOUS DISEASES
Pemphigus
Epidemiology
Pathology
Clinical features
Histopathology
Immunopathology
Diagnosis
Treatment
Paraneoplastic pemphigus
Clinical features
Histopathology
Immunopathology
Treatment and prognosis
DIFFERENTIAL DIAGNOSES OF AUTOIMMUNE BULLOUS DISEASES
CHAPTER 27:
Inherited bullous diseases
DARIER DISEASE
Synonyms
Introduction
Etiology
Clinical presentation
Histopathology
Differential diagnosis
Treatment
Surgery
HAILEY–HAILEY DISEASE
Synonyms
Introduction
Etiology
Clinical presentation
Histopathology
Differential diagnosis
Treatment
Prognosis
EPIDERMOLYSIS BULLOSA GROUP
Introduction
Etiology
Clinical presentation
Histopathology
Diagnosis
Differential diagnosis
Treatment
Supporting treatment
Treatment of complications
Emerging treatments
Prognosis
CHAPTER 28:
Vulvar ulcers
INTRODUCTION
HISTORY
INVESTIGATIONS
ACUTE ULCERS
Lipschütz ulcer
Synonyms
Pathogenesis
Clinical features
Diagnosis
Treatment and prognosis
Infectious non-sexually transmitted ulcer
CHRONIC ULCERS
Neutrophilic dermatoses
Sweet syndrome
Pyoderma gangrenosum
Solid malignant tumors
Langerhans cell histiocytosis
Medications
Vasculitis
Dermatitis artefacta
RECURRENT ULCERS
Recurrent herpes simplex infection
Recurrent idiopathic aphthosis
Pathogenesis
Clinical features
Diagnosis
Treatment
Behçet disease
Pathogenesis
Clinical features
Diagnosis
Histopathology
Treatment
CHAPTER 29:
Primary bacterial and fungal vulvar and vulvovaginal infections
PRIMARY BACTERIAL AND FUNGAL VULVAR INFECTIONS
Introduction
Impetigo
Bullous impetigo
Clinical presentation
Diagnosis
Treatment
Intertrigo
Clinical presentation
Diagnosis and differential diagnosis
Treatment
Erythrasma
Clinical presentation
Diagnosis and differential diagnosis
Treatment
Tinea cruris/inguinalis
Synonyms
Clinical presentation
Diagnosis
Treatment
Pityriasis versicolor
Clinical presentation
Diagnosis
Treatment
PRIMARY VAGINAL INFECTIONS
Introduction
Bacterial vaginosis
Definition
Clinical presentation
Diagnosis
Treatment
Candida infections
Introduction
Epidemiology
Promoting factors
Clinical presentation
Treatment
CHAPTER 30:
Viral diseases
INTRODUCTION
SEXUALLY TRANSMITTED INFECTIONS
MOLLUSCIPOXVIRUS INFECTIONS
Molluscum contagiosum
Introduction
Epidemiology/transmission
Pathogenesis
Clinical presentation and differential diagnosis
Histopathology
Diagnosis
Management of genital mollusca contagiosa
Course of disease
Therapeutic options
HUMAN PAPILLOMAVIRUS INFECTION
Anogenital warts
Synonym
Introduction
Epidemiology/transmission
Pathogenesis
Female pediatric population
Clinical presentation
Histopathology
Diagnosis
Differential diagnosis
Principles of management
Course of disease
HUMAN HERPES VIRUS INFECTIONS/α-SUBFAMILY
Genital herpes
Synonym
Introduction
Epidemiology/transmission
Pathogenesis
Histopathology
Pregnancy
Risk factors
Immunology
Clinical presentation
Recurrence
Diagnosis
Differential diagnosis
Management and treatment
Varicella zoster virus
Introduction
Epidemiology and transmission
Pathogenesis
Course of disease
Clinical presentation
Diagnosis
Differential diagnosis
Management and treatment
CHAPTER 31:
Sexually transmitted diseases
INTRODUCTION
CHLAMYDIA
Causative organism
Clinical presentation
Chlamydia trachomatis
Lymphogranuloma venereum
Dermatological signs
Chlamydia trachomatis
Lymphogranuloma venereum
Diagnosis
Management
Chlamydia trachomatis
LGV
GONORRHEA
Causative organism
Clinical presentation
Dermatological signs
Diagnosis
Management
SYPHILIS
Synonym
Causative organism
Clinical presentation
Dermatological signs
Primary syphilis
Secondary syphilis
Tertiary syphilis
Diagnosis
Management
CHANCROID
Causative organism
Clinical presentation
Dermatological signs
Diagnosis
Management
DONOVANOSIS
Causative organism
Clinical presentation
Dermatological signs
Diagnosis
Management
LICE
Causative organism
Clinical presentation
Dermatological signs
Diagnosis
Management
SCABIES
Causative organism
Clinical presentation
Dermatological signs
Diagnosis
Management
HIV INFECTION
Causative organism
Clinical presentation
Dermatological signs
Diagnosis
Management
REFERENCES FOR TREATMENT
CHAPTER 32:
Cysts and diseases of the glands
CYSTS
Developmental cysts
Bartholin gland cyst
Mucinous and ciliated cyst
Müllerian cyst
Mesonephric cyst
Mesothelial cyst
Periurethral cyst
Appendageal cysts
Epidermoid cyst
Steatocystoma
Pilonidal sinus
Management of cysts
GLANDULAR DISEASES
Benign mucinous metaplasia
CHAPTER 33:
Solid benign tumors
INTRODUCTION
SURFACE EPITHELIAL TUMORS
Seborrheic keratosis
Clinical presentation
Histopathology
Management
TUMORS SHOWING ADNEXAL DIFFERENTIATION (ECCRINE/APOCRINE TUMORS) AND GLANDULAR DIFFERENTIATION
Syringoma
Histopathology
Management
Nodular hidradenoma
Papillary hidradenoma (hidradenoma papilliferum)
BENIGN MIXED TUMORS
Follicular tumors
Trichoblastoma
SOFT TISSUE/MESENCHYMAL TUMORS
Leiomyomas
Angioleiomyomas
Aggressive angiomyxoma
Cellular angiofibroma
Angiomyofibroblastoma
Other
MALIGNANT COUNTERPARTS
CHAPTER 34:
Benign and malignant pigmented lesions of the vulva
INTRODUCTION
POST-INFLAMMATORY PIGMENTARY DISORDERS OF THE VULVA
Introduction
Pathogenesis and histopathology
Clinical features and management
Differential diagnosis
HYPERPIGMENTATION DUE TO INCREASED MELANIN PRODUCTION WITHOUT PROLIFERATION OF MELANOCYTES
Introduction
Pigmented epithelial neoplasms
Clinical features
Differential diagnoses
Melanosis
Pathogenesis and histopathology
Differential diagnosis
Management and prognosis
MELANOCYTIC PROLIFERATIONS
Introduction
Benign melanocytic proliferations
Lentigo simplex
Melanocytic nevi
Common acquired nevi
Malignant melanoma of the vulva
Introduction
Pathogenesis
Cutaneous malignant melanoma of the vulva
Superficial spreading malignant melanoma
Nodular melanoma
Lentiginous malignant melanoma
Mucosal malignant melanoma of the vulva
CHAPTER 35:
Malignant and premalignant nonpigmented tumors
INTRODUCTION
NOMENCLATURE
VULVAR SQUAMOUS CELL CARCINOMA
Treatment
Early stage disease
Advanced disease
Prognosis
HIGH-GRADE SQUAMOUS INTRAEPITHELIAL LESIONS OF THE VULVA
Epidemiology
Pathogenesis
Clinical presentation
Histopathology
Differential diagnosis
Principles of management Surgery
Medical
Prevention
Course and prognosis; malignant potential
Recurrence
Progression to invasive vulvar cancer
Spontaneous regression
Follow-up
DIFFERENTIATED VULVAR INTRAEPITHELIAL NEOPLASIA
Epidemiology
Pathogenesis
Clinical presentation
Histopathology
Differential diagnosis
Principles of management
Course and prognosis/malignant potential
CHAPTER 36:
Extramammary Paget disease of the vulva
INTRODUCTION
EPIDEMIOLOGY
CLASSIFICATION
Primary cutaneous origin: >75%
Secondary non-cutaneous origin: <25%
PATHOGENESIS
CLINICAL PRESENTATION
Association with malignancy
INVESTIGATIONS
Histopathology
Hematoxylin and eosin
Immunohistochemistry
SCREENING FOR MALIGNANCY
DIFFERENTIAL DIAGNOSIS
TREATMENT
Surgery
Mohs micrographic surgery
CO2 or Nd:YAG laser
Imiquimod
Photodynamic therapy
Topical 5-fluouracil
Systemic chemotherapy
Radiotherapy
COURSE AND PROGNOSIS
CHAPTER 37:
Vulvar vascular disease
VULVAR VARICOSE VEINS
Vulvar varices related to pelvic vein incompetence
Clinical findings
Diagnosis and management
Vulvar varices related to deep venous obstruction
Clinical findings
Diagnosis and management
VASCULAR MALFORMATIONS
Vulvar vascular malformations
Clinical findings
Diagnosis and management
VULVAR HEMANGIOMA OF INFANCY
Vulvar angiokeratomata
Vulvar lymphangioma circumscriptum
VULVAR LYMPHEDEMA
CHAPTER 38:
Vulvodynia
INTRODUCTION
EPIDEMIOLOGY
PAIN PATHOPHYSIOLOGY
CLINICAL PRESENTATION
History
Examination
VESTIBULODYNIA (PROVOKED PAIN)
Clinical features
UNPROVOKED VULVODYNIA
Clinical features
Treatment principles
Patient education and reassurance
Pain modification
Physical therapy
Psychological and psychosexual therapy and support
Surgery for provoked pain
CHAPTER 39:
Psychological and sexological aspects
INTRODUCTION
SEXUAL HISTORY
CLASSIFICATION OF SEXUAL DYSFUNCTION
Treatment
SEXUAL ABUSE
Signs and symptoms of child sexual abuse
Assessment and examination
Adult manifestations of childhood sexual abuse
Emotional sequelae
Physical sequelae
Sexual sequelae
Sexual history taking in cases of CSA
Management
FEMALE GENITAL MUTILATION
Complications
Sexological aspects
Recognition and management
Pregnancy and childbirth
CHAPTER 40:
Vulvar cosmetic surgery
INTRODUCTION
AESTHETIC VULVOVAGINAL SURGERY
Labia minora labioplasty (labia minora reduction surgery)
Free edge resection technique
Inferior pedicle resection and reconstruction with superior pedicle flap
Central wedge resection technique with 90° Z-plasty
CO2 laser technique
Complications
Labia majora labioplasty
Reduction vaginoplasty
THERAPEUTIC VULVAR SURGERY
Fissures of the navicular fossa
Vulvoperineoplasty for lichen sclerosus
Surgery for fourchette adhesions
Surgery for labia minora adhesions
Surgery for clitoral adhesions
PRACTICES NOT RECOMMENDED DUE TO LACK OF EVIDENCE FOR THEIR EFFECTIVENESS
G-spot amplification
Vulvar and/or anal bleaching
Reduction vaginoplasty
CONCLUSION
CHAPTER 41:
Vulvar body art
INTRODUCTION
PIERCING
Piercing retainers or ‘sleepers’
Bead closures
Procedure
Anatomical site and rationale for piercings
Side effects
Short-term side effects
Long-term side effects
Other complications
TATTOOING
Procedure
Side effects
OTHER BODY-MODIFYING PROCEDURES
CONCLUSION
ACKNOWLEDGMENTS
CHAPTER 42:
Vulvar disease in children
INTRODUCTION
VULVA IN INFANCY AND CHILDHOOD
PRINCIPLES OF PEDIATRIC VULVAR CARE
FUSED LABIA
Synonym
Treatment
DISCHARGE
Treatment
VULVAR DERMATITIS IN CHILDREN
Irritant vulvitis
Napkin dermatitis
Atopic vulvitis
Treatment
OTHER INFLAMMATORY DISEASES
Psoriasis
Treatment and prognosis
Lichen sclerosus
Synonyms
Treatment
Prognosis
VULVAR GROWTHS
COMMON VULVAR SKIN INFECTIONS IN CHILDREN
Candida infection
Bacterial infection
Scabies
Treatment
Genital warts
Molluscum contagiosum
CONCLUSION
INDEX
TOC
Index
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