Ridley's The Vulva
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Оглавление
Группа авторов. Ridley's The Vulva
Table of Contents
List of Tables
List of Illustrations
Guide
Pages
Ridley’s The Vulva
List of Contributors
Preface
Acknowledgements
About the Companion Website
1 Vulval Embryology and Developmental Abnormalities
CHAPTER MENU
Vulval embryology. Sexual determination and differentiation
Early female embryogenesis (weeks 1–8)
Carnegie stage 1–3
Carnegie stages 4–6
Carnegie stage 8
Carnegie stage 9
Carnegie stage 11
Carnegie stages 13 and 14
Carnegie stages 15 and 16
Carnegie stage 19
Carnegie stages 20–23
End of the female embryonic period and further development (week 8 onwards)
Development of the epithelia
Disorders of sexual development
Sex Chromosome DSD. Turner’s syndrome
Mosaicism. 45,X/46,XX mosaicism and X chromosome abnormality
45,X/46,XY MOSAICISM AND Y CHROMOSOME ABNORMALITY
46,XX DSD
Foetal androgen excess
Maternal androgen excess
Exogenous androgen excess
46,XY DSD
Disorders of gonadal development. Complete or partial gonadal dysgenesis
Ovotesticular disorder
Structural developmental defects. Vagina. Vaginal agenesis (Müllerian agenesis)
Vaginal atresia
Vaginal septa
Imperforate hymen
Vaginal cysts
External genitalia
Vulval and urinary system abnormalities. Kidney
Ureter
Bladder
Urethra
Vulval and intestinal abnormalities
Patient Support Groups
References
2 The Normal Vulva
CHAPTER MENU
Normal vulval anatomy
Mons pubis
Labia majora
Labia minora
Sebaceous glands (Fordyce spots)
The clitoris
The vestibule
Hart’s line
Bartholin’s glands
Minor vestibular glands
Vestibular papillomatosis
Hymen
The external urethral meatus and urethra
Associated structures. The vagina
The perineum
The urogenital triangle
The urogenital diaphragm
Deep perineal pouch
Superficial perineal pouch
Anal triangle
The pelvic floor
Ischiococcygeus muscle
Iliococcygeus muscle
Pubococcygeus muscle
The inguinofemoral region
Blood supply of the vulva
Internal pudendal artery
Femoral artery
Venous drainage
Microscopic anatomy
Lymphatic drainage of the vulva
The external iliac lymph nodes
Medial group
Anterior group
Lateral group
Microscopic anatomy
Nerve supply of the vulva
Somatic innervation
Autonomic (visceral) innervation
Pudendal nerve
Microscopic anatomy
Normal microscopic anatomy and histological features. Epithelia of the vulva
Mons pubis
Labia majora
AGMLGs
Labia minora
Clitoris
Vestibule
Bartholin’s glands
Minor vestibular glands
Skene’s glands (paraurethral glands)
Vestibular papillomatosis
Vagina
Other epithelial cell types
Melanocytes
Langerhans cells
Merkel cells
The basement membrane zone
The dermis
References
3 Vulval Physiology and Changes Through Life
CHAPTER MENU
Barrier function
Immune responsiveness
Changes through life
Neonate
Childhood
Puberty
Reproductive years
Sexual response
Pregnancy
Menopause
Resources
Patient support groups
References
4 Normal Microbiology of the Vulva
CHAPTER MENU
The vulval microbiome
Variations in site
Variations with physiological changes
Variations with age
The vaginal microbiome
Lactobacilli clusters
Pregnancy
Menopause
Other links
References
5 The Vulval Clinic, History, and Examination
CHAPTER MENU
The vulval clinic
The consultation. History
Examination
History and examination in children
Documentation
Management
Helpful websites for patient information
Dermatoses
Sexually transmitted infections
References
6 Symptoms and Signs in Vulval Disease
CHAPTER MENU
Symptoms in vulval disease
Vulval ulceration
Vulval oedema
Acute vulval oedema
Chronic vulval oedema
Signs in vulval disease
References
7 Investigations in Vulval Disease
CHAPTER MENU
Biopsy. Reasons for taking a biopsy
Site of biopsy
Pre‐biopsy
Types of biopsy. Punch biopsy
Incisional biopsy
Elliptical biopsy
Excisional biopsy
Shave biopsy
Local anaesthesia
Technique
Post‐biopsy instructions
Samples
Documentation
What do you need to tell the pathologist?
Cytology
Microbiological investigation. Swabs
Scrapings
Serology
Wet mount microscopy
Wood’s lamp examination
Patch testing
Resources
Prick testing
Blood tests
Imaging
Dermoscopy
Vulvoscopy
References
8 Topical and Non‐Surgical Treatments
CHAPTER MENU
Topical treatments
General vulval hygiene
Types of preparation
Emollients
Barriers
Topical steroids
Adverse effects
Local effects
Systemic effects
Calcineurin inhibitors
Adverse effects
Imiquimod
Adverse effects
Potassium permanganate
Lubricants
Non‐surgical treatments. Phototherapy and photochemotherapy
Photodynamic therapy (PDT)
Adverse effects
Resources
References
9 Bacterial Vaginosis
CHAPTER MENU
Pathophysiology
Clinical features
Diagnosis
Amsel’s criteria
Hay–Ison method
Nugent score
Differential diagnosis
Complications
Treatment
Prognosis and follow‐up
Resources
References
10 Vulvovaginal Candidiasis
CHAPTER MENU
Epidemiology
Pathophysiology
Clinical features
Diagnosis
Differential diagnosis
Complications. Recurrent vulvovaginal candidiasis
Obstetric complications
Associated disease
Treatment
Management of recurrent VVC
Prognosis and follow‐up
Resources. Guidelines
Patient information
References
11 Syphilis
CHAPTER MENU
Epidemiology
Pathophysiology
Stages of syphilis
Histological features
Clinical features
Differential diagnosis
Diagnosis
Complications
Treatment
Prognosis and follow‐up
Resources
References
12 Gonorrhoea
CHAPTER MENU
Epidemiology
Clinical features
Diagnosis
Differential diagnosis
Complications
Bartholin’s abscesses
Pelvic inflammatory disease
Pregnancy and neonatal infection
Conjunctival infection
Disseminated gonococcal infection
Treatment
Prognosis and follow‐up
Resources
References
13 Chlamydial Infection
CHAPTER MENU
Genital chlamydia. Epidemiology
Pathophysiology
Clinical features
Diagnosis
Complications
Treatment
Prognosis and follow‐up
Resources
Lymphogranuloma venereum
Pathophysiology
Clinical features
Diagnosis
Treatment
Resources
References
14 Trichomoniasis
CHAPTER MENU
Epidemiology
Clinical features
Diagnosis
Complications
Treatment
Treatment failure
Prognosis and follow‐up
Resources
Guidelines
References
15 Genital Herpes
CHAPTER MENU
Epidemiology
Pathophysiology
Clinical features. Initial primary and non‐primary episodes
Recurrent episodes
Diagnosis
Complications
Hypertrophic (pseudotumoral) herpes simplex
Herpes in pregnancy
Treatment. Primary herpes
Recurrent herpes. Infrequent recurrence
Suppressive treatment
Prognosis and follow‐up
Resources
References
16 Human Papillomavirus Infections
CHAPTER MENU
Epidemiology
Pathophysiology. Virology
Transmission
Immunology
Clinical features
Diagnosis
Treatment
Human papilloma virus vaccines
Prognosis and follow‐up
Resources
References
17 Other Sexually Transmitted Infections
CHAPTER MENU
Mycoplasma genitalium
Pathophysiology
Diagnosis
Treatment
Resources
Chancroid
Clinical features
Diagnosis
Treatment
Resources
Donovanosis
Clinical features
Diagnosis
Treatment
Molluscum contagiosum
Epidemiology
Pathophysiology
Histological features
Clinical features
Differential diagnosis
Diagnosis
Associated disease
Treatment
Prognosis
Scabies
Pubic lice
References
18 Tropical Infections
CHAPTER MENU
Introduction
Infection with nematodes, tapeworms, and flukes. Filariasis. Introduction
Pathophysiology
Histological features
Clinical features
Differential diagnosis
Treatment
Echinococcosis (Hydatid disease)
Onchocerciasis
Schistosomiasis (Bilharzia)
Epidemiology
Pathophysiology
Histological features
Clinical features
Investigations
Differential diagnosis
Complications
Associated disease
Treatment
Infection with protozoa. Leishmaniasis
Epidemiology
Pathophysiology
Histological features
Clinical features
Differential diagnosis
Investigations
Associated disease
Treatment
Amoebiasis
Epidemiology
Pathophysiology
Histological features
Clinical features
Differential diagnosis
Investigations
Complications
Treatment
Infection with fungi. Chromoblastomycosis (Chromomycosis)
Phycomycosis
Piedra (Trichosporis)
Mycobacterial infections. Tuberculosis
Epidemiology
Pathophysiology
Histological features
Clinical features
Differential diagnosis
Complications
Associated disease
Treatment
Infection with viruses. Chikungunya fever
Other infections
References
19 Non‐Sexually Transmitted Infections
CHAPTER MENU
Introduction
Infection with nematodes. Enterobiasis (threadworm/pinworm infection)
Epidemiology
Pathophysiology
Clinical features
Investigations
Differential diagnosis
Treatment
Infection with fungi. Tinea cruris
Pathophysiology
Clinical features
Investigations
Differential diagnosis
Complications
Treatment
Pityriasis versicolor
Epidemiology
Clinical features
Investigations
Differential diagnosis
Treatment
Infection with bacteria. Infection with gram‐positive cocci
Folliculitis and furunculosis
Pathophysiology
Clinical features
Investigations
Differential diagnosis
Complications
Treatment
Staphylococcal scalded skin syndrome
Epidemiology
Pathophysiology
Histological features
Clinical features
Investigations
Differential diagnosis
Complications
Treatment
Prognosis and follow‐up
Toxic shock syndrome (TSS)
Epidemiology
Pathophysiology
Clinical features
Associated disease
Treatment
Prognosis
Streptococcal vulvovaginitis
Perianal streptococcal dermatitis
Cellulitis
Pathophysiology
Clinical features
Differential diagnosis
Complications
Treatment
Necrotising fasciitis (NF)
Epidemiology
Pathophysiology
Clinical features
Differential diagnosis
Treatment
Prognosis
Vulval abscesses
Bartholin’s gland abscesses
Ecthyma gangrenosum
Corynebacterial infection. Erythrasma
Epidemiology/genetics
Pathophysiology
Clinical features
Investigations
Differential diagnosis
Associated disease
Treatment
Trichomycosis (trichobacteriosis)
Epidemiology
Pathophysiology
Clinical features
Differential diagnosis
Investigations
Treatment
Other bacterial infections. Actinomycosis
Histological features
Clinical features
Differential diagnosis
Treatment
Cat scratch disease
Infection with viruses. Varicella and herpes zoster
Clinical features
Differential diagnosis
Investigations
Histological features
Complications
Treatment
Resources
Cytomegalovirus
Pathophysiology
Histological features
Clinical features
Investigations
Treatment
Cowpox
Vaccinia (smallpox)
Parvovirus infection
Other infections
Acute genital ulcers (Lipschutz ulcers)
Epidemiology
Pathophysiology
Histological features
Clinical features
Investigations
Differential diagnosis
Treatment
Prognosis and follow‐up
References
20 HIV Infection and Vulval Disease
CHAPTER MENU
HIV and other infections
HIV and human papillomavirus (HPV)‐related disease
Immune reconstitution inflammatory syndrome (IRIS)
References
21 Vulval Psoriasis
CHAPTER MENU
Epidemiology
Genetics
Pathophysiology
Histological features
Clinical features
Differential diagnosis
Complications
Associated disease
Treatment
First‐line treatment
Second‐line treatment
Third‐line treatment
Future treatments
Prognosis and follow‐up
Resources
References
22 Eczema and Allergic Reactions
CHAPTER MENU
Endogenous eczema. Atopic eczema
Seborrhoeic eczema
Pathophysiology
Histological features
Clinical features
Differential diagnosis
Associated disease
Treatment
Prognosis and follow‐up
Lichen simplex
Epidemiology
Pathophysiology
Histological features
Clinical features
Investigations
Differential diagnosis
Associated disease
Treatment
Resources
Exogenous eczema
Irritant reactions
Epidemiology
Causes
Histological features
Clinical features
Differential diagnosis
Complications
Treatment
Allergic contact dermatitis
Epidemiology
Pathophysiology
Causes of allergic contact dermatitis on the vulva
Histological features
Clinical features
Investigations
Differential diagnosis
Complications
Treatment
Prognosis
Resources
Type I reactions
Epidemiology
Pathophysiology
Clinical features
Investigations
Differential diagnosis
Treatment
References
23 Lichen Sclerosus
CHAPTER MENU
Historical aspects
Epidemiology
Genetics. Family history
HLA antigens
Genome sequencing
Pathophysiology
Cytokines, T‐cell regulation, and microRNA 155
Autoantibodies
Collagen
Oxidative stress
Cell proliferation
Epigenetics
Trauma
Infection
Hormones
Others
Histological features
Clinical features. Symptoms
Signs
Impact on quality of life
Grading systems
Extra‐genital lesions
Dermoscopy
Differential diagnosis
Complications. Clitoral pseudocyst
Malignancy
Vulvodynia
Psychosexual factors
Associated disease. Autoimmune disease
Pigmented lesions
Others
Treatment. Historical aspects
Current treatment recommendations
Potent topical steroids – first line
Apparent treatment failure
Other topical steroids
Intralesional steroids
Hormone treatments
Topical retinoids
Calcineurin inhibitors
Systemic agents
Phototherapy
Photodynamic therapy
Laser treatment
PRP and fat transfer
Surgery
Other treatments
Management in special situation. Pregnancy
Scarring
Clitoral adhesions
Labia minora adhesions
Adhesions at the fourchette
Introital stenosis
Prognosis and follow‐up
Resources
References
24 Lichen Planus
CHAPTER MENU
Epidemiology
Genetics
Pathophysiology
Histological features
Clinical features
Classical (papulo‐squamous) LP
Hypertrophic LP
Erosive LP
Vulvovaginal‐gingival syndrome (VVG)
Scalp
External auditory meatus
Eye
Oesophagus
Urethra
Cervix
Other variants. Lichen planopilaris
LP pigmentosus
LS/LP overlap
LP pemphigoides
Investigations
Differential diagnosis
Complications
Malignancy
Associated disease
Treatment
Topical steroids
Calcineurin inhibitors
Systemic agents
Biologic agents
Photodynamic therapy
Surgery
Prognosis and follow‐up
Resources
References
25 Hidradenitis Suppurativa
CHAPTER MENU
Epidemiology
Genetics
Pathophysiology
Histological features
Clinical features
Staging systems
Differential diagnosis
Associated disease
Complications
Current therapeutic options for HS. General measures
Topical treatments
Oral antibiotics
Anti‐androgens
Retinoids
Dapsone
Biologic agents
Other treatments
Surgical management
Prognosis
Resources – Support groups and patient information
References
26 Immunobullous Disease
CHAPTER MENU
Diagnosis
Differential diagnosis of the immunobullous disorders
Principles of treatment
Bullous pemphigoid
Pathophysiology
Histological features
Clinical features
Treatment
Mucous membrane pemphigoid (cicatricial pemphigoid)
Histological features
Clinical features
Treatment
Pemphigus vulgaris
Pathophysiology
Histological features
Clinical features
Treatment
Paraneoplastic pemphigus
Clinical features
Treatment
Dermatitis herpetiformis
Clinical features
Treatment
Epidermolysis bullosa acquisita
Clinical features
Treatment
References
27 Other Inflammatory Dermatoses
CHAPTER MENU
Nodular prurigo
Intertrigo
Autoimmune progesterone dermatitis
Malacoplakia
Epidemiology
Pathophysiology
Histological features
Clinical features
Differential diagnosis
Complications
Associated disease
Treatment
Reiter’s syndrome (reactive arthritis)
Vulval aphthous ulcers
Epidemiology/genetics
Pathophysiology
Histological features
Clinical features
Differential diagnosis
Treatment
Prognosis and follow‐up
Behcet’s syndrome
Epidemiology
Pathophysiology
Histological features
Clinical features
Differential diagnosis
Complications
Treatment
Resources
Magic syndrome
Febrile ulcero‐necrotic Mucha‐Habermann’s disease (Pityriasis lichenoides et varioliformis acuta fulminans)
Angiolymphoid hyperplasia with eosinophilia
Amyloidosis
Histological features
Clinical features
Treatment
Genital papular acantholytic dyskeratosis
Genetics
Histological features
Clinical features
Differential diagnosis
Treatment
Acquired dyskeratotic leukoplakia
Vasculitis
Desquamative inflammatory vaginitis
Epidemiology
Pathophysiology
Histological features
Clinical features
Investigations
Differential diagnosis
Treatment
Prognosis
Plasma cell vulvitis (Zoon’s vulvitis)
Histological features
Clinical features
Investigations
Differential diagnosis
Associated disease
Treatment
Vulvovaginal adenosis
Epidemiology
Pathophysiology
Histological features
Clinical features
Differential diagnosis
Complications
Treatment
References
28 Genetic Conditions
CHAPTER MENU
Epidermolysis bullosa (EB)
Pathophysiology
Clinical features
Differential diagnosis
Complications
Treatment
Resources
Darier’s disease (keratosis follicularis)
Genetics
Pathophysiology
Histological features
Clinical features
Differential diagnosis
Complications
Treatment
Hailey‐Hailey disease (benign familial chronic pemphigus)
Genetics
Histological features
Clinical features
Differential diagnosis
Complications
Treatment
Prognosis
Resources
Goltz syndrome (focal dermal hypoplasia)
Genetics
Clinical features
Ehlers–Danlos syndrome
Genetics
Clinical features
Resources
Pseudoxanthoma elasticum
Genetics
Pathophysiology
Histological features
Clinical features
Complications
Congenital familial lipodystrophy
Neurofibromatosis
Ligneous disease
Genetics
Pathophysiology
Histological features
Clinical features
Treatment
Anderson‐Fabry disease
White sponge naevus
Genetics
Histological features
Clinical features
Differential diagnosis
Treatment
Cowden’s disease
Genetics
Clinical features
Associated disease
Treatment
Netherton’s syndrome (ichthyosis linearis circumflexa)
Incontinentia pigmenti
References
29 Disorders of Pigmentation
CHAPTER MENU
Hyperpigmentation. Post‐inflammatory pigmentation
Clinical features
Histological features
Differential diagnosis
Treatment
Prognosis and follow‐up
Vulval melanosis
Epidemiology
Histological features
Clinical features
Differential diagnosis
Treatment
Prognosis and follow‐up
Dowling‐Degos disease (reticular pigmented anomaly of the flexures)
Genetics
Histological features
Clinical features
Differential diagnosis
Treatment
Lentigines
Laugier–Hunziker syndrome
Confluent and reticulated papillomatosis (Gougerot–Carteaud syndrome)
Drug‐induced pigmentation
Hypopigmentation. Post‐inflammatory hypopigmentation
Vitiligo
Epidemiology/genetics
Pathophysiology
Histological features
Clinical features
Differential diagnosis
Associated disease
Treatment
Resources
References
30 Disorders of Skin Appendages and Hair
CHAPTER MENU
Disorders of sebaceous glands
Vulval acne
Epidemiology
Pathophysiology
Histological features
Clinical features
Differential diagnosis
Treatment
Sebaceous adenitis
Rosacea
Milia
Disorders of sweat glands
Eccrine miliaria (miliaria rubra)
Hyperhidrosis
Chromhidrosis
Fox‐Fordyce disease
Histological features
Clinical features
Treatment
Disorders of hair. Pseudo‐folliculitis
Pilonidal sinuses
Lichen planopilaris
Alopecia areata
Pathophysiology
Histological features
Clinical features
Treatment
Prognosis
Trichotillomania
References
31 Drug Reactions and the Vulva
CHAPTER MENU
Fixed drug eruption
Clinical features
Differential diagnosis
Histological features
Treatment
Stevens‐Johnson syndrome/toxic epidermal necrolysis (SJS/TEN syndrome)
Epidemiology
Genetics
Pathophysiology
Histological features
Clinical features
Differential diagnosis
Management
Complications
Acute generalised exanthematous pustulosis (AGEP)
Resources and patient information
References
32 Conditions Associated with Systemic Disease
CHAPTER MENU
Vulval involvement in connective tissue disease. Dermatomyositis
Lupus erythematosus
Sjögren’s syndrome
Epidemiology
Pathophysiology
Histological features
Clinical features
Complications
Associated disease
Treatment
Resources
Vulval disorders associated with metabolic disturbances. Necrolytic migratory erythema (Glucagonoma syndrome)
Pathophysiology
Histological features
Clinical features
Differential diagnosis
Associated disease
Treatment
Acrodermatitis enteropathica
Genetics
Pathophysiology
Histological features
Clinical features
Investigations
Differential diagnosis
Complications
Treatment
Acanthosis nigricans
Genetics
Pathophysiology
Histological features
Clinical features
Treatment
Other nutritional deficiencies
Calciphylaxis
Fish odour syndrome
Vulval disorders associated with inflammatory bowel disease
Ulcerative colitis
Crohn’s disease
Epidemiology
Pathophysiology
Histological features
Clinical features
Differential diagnosis
Complications
Associated disease
Treatment
Prognosis
Resources
Vulval disorders associated with neutrophilic disorders. Acute febrile neutrophilic dermatosis (Sweet’s syndrome)
Pathophysiology
Histological features
Clinical features
Differential diagnosis
Treatment
Prognosis and follow‐up
Pyoderma gangrenosum
Pathophysiology
Histological features
Clinical features
Differential diagnosis
Complications
Associated disease
Treatment
Vulval disorders associated with granulomatous disease. Sarcoidosis
Histological features
Clinical features
Differential diagnosis
Treatment
Anogenital granulomatosis
Vulval disorders associated with haematological disorders. Necrobiotic xanthogranuloma
Graft‐versus‐host disease
Epidemiology
Pathophysiology
Histological features
Clinical features
Differential diagnosis
Complications
Treatment
Prognosis
Langerhans cell histiocytosis
Histological features
Clinical features
Differential diagnosis
Associated disease
Treatment
Prognosis and follow‐up
References
33 Lymphatic Abnormalities and the Vulva
CHAPTER MENU
Epidemiology
Pathophysiology
Secondary Lymphoedema
Primary Lymphoedema
Histological features
Clinical features
Differential diagnosis
Complications
Treatment. First line
Second line. Surgical treatment
Psychological support
Prognosis and follow‐up
Resources
References
34 Vascular Abnormalities and the Vulva
CHAPTER MENU
Vascular malformations
Venous malformations
Arteriovenous malformations (AVM)
Syndromes with vascular anomalies
Vulval varicose veins
Epidemiology
Pathophysiology
Clinical features
Differential diagnosis
Complications
Associated disease
Treatment
References
35 Vulvodynia – History, Classification, and Terminology
CHAPTER MENU
History
Classification
References
36 Vulvodynia – Pathophysiology and Clinical Features
CHAPTER MENU
The physiology of acute and persistent pain
Peripheral modulation
Spinal modulation
Supraspinal modulation
Epidemiology of vulvodynia
Pain mechanisms in vulvodynia
Aetiology of vulvodynia
Co‐morbidities with other pain syndromes
Genetic factors
Hormonal factors
Inflammation and previous infections
Musculoskeletal factors
Neurologic mechanisms – central and peripheral
Psychosocial factors. Depression
Anxiety
Childhood sexual and physical abuse
Sexuality and relationship factors
Other psychosocial characteristics
Clinical features
Provoked vulvodynia (PVD)
Generalised unprovoked vulvodynia (GVD)
Patient history
Examination and differential diagnosis
Diagnosis
Resources
References
37 Vulvodynia – Management
CHAPTER MENU
Provoked vulvodynia (PVD)
Basic vulval care
Pelvic floor physical therapy
Psychosocial interventions
Medical management
Antinociceptive agents
Antidepressants
Anticonvulsants
Anti‐inflammatory agents
Hormonal treatments
Neurotoxic agents
Surgery
Combined treatments – multidisciplinary approach
Other treatments
Generalised unprovoked vulvodynia
Antidepressants
Anticonvulsants
Nerve blocks
Prognosis and predictors of treatment outcome
References
38 Benign Vulval Tumours
CHAPTER MENU
Benign cysts. Epidermoid (tricholemmal, sebaceous, keratinous, epidermal inclusion) cysts
Pathophysiology
Clinical features
Histological features
Complications
Management
Mucinous cysts
Clinical features
Histological features
Management
Anogenital gland cysts
Clinical features
Histological features
Cyst of canal of Nuck (Mesothelial cyst)
Clinical features
Histological features
Management
Benign cysts of probable adnexal origin
Benign tumours of the epithelium. Fibroepithelial polyps
Clinical features
Histological features
Management
Seborrhoeic keratoses
Clinical features
Differential diagnosis
Histological features
Management
Verruciform xanthoma
Clinical features
Histological features
Management
Epidermolytic acanthoma (epidermolytic hyperkeratosis)
Clinical features
Histological features
Management
Benign tumours of the anogenital mammary‐type glands
Fibroadenomas
Clinical features
Histological features
Management
Hidradenoma papilliferum
Clinical features
Histological features
Management
Benign lesions of the sweat glands. Syringoma
Clinical features
Differential diagnosis
Histological features
Management
Clear cell hidradenoma (eccrine acrospiroma)
Clinical features
Histological features
Management
Benign tumours of the hair follicle and pilosebaceous duct. Trichoepithelioma
Clinical features
Histological features
Management
Trichilemmoma (proliferating trichilemmal tumour, pilar tumour)
Clinical features
Histological features
Management
Steatocystoma multiplex
Genetics
Histological features
Clinical features
Differential diagnosis
Treatment
Benign tumours of smooth muscle. Leiomyoma
Pathophysiology
Clinical features
Differential diagnosis
Histological features
Management
Benign tumours of striated muscle. Rhabdomyoma
Epidemiology
Clinical features
Histological features
Management
Benign lesions of connective tissue. Prepubertal vulval fibroma
Clinical features
Histological features
Management
Myofibroblastic lesions. Postoperative spindle cell nodule
Genetics
Clinical features
Histological features
Management
Nodular fasciitis
Clinical features
Histological features
Management
Angiomyofibroblastoma
Clinical features
Histological features
Management
Cellular angiofibroma
Clinical features
Histological features
Differential diagnosis
Management
Benign lesions of fat. Lipoblastoma‐like tumour of the vulva
Clinical features
Histological features
Management
Spindle cell lipoma
Clinical features
Histological features
Management
Lipoma
Clinical features
Histological features
Management
Benign lesions of the lymphatic system. Lymphangioma circumscriptum
Clinical features
Histological features
Management
Cavernous lymphangioma
Clinical features
Histological features
Management
Benign lesions of perivascular cells. Glomus tumour
Clinical features
Histological features
Management
Benign vascular lesions. Pyogenic granuloma (lobular capillary haemangioma)
Pathophysiology
Clinical features
Histological features
Differential diagnosis
Treatment
Angiokeratoma
Epidemiology
Pathophysiology
Clinical features
Differential diagnosis
Histological features
Management
Benign neural lesions. Neurofibroma
Clinical features
Histological features
Management
Schwannoma (neurilemmoma)
Clinical features
Histological features
Management
Granular cell tumour
Clinical features
Histological features
Management
Miscellaneous benign proliferations and neoplasms. Endometriosis
Clinical features
Histological features
Management
Benign lesions of the Bartholin’s gland. Bartholin’s cysts
Clinical features
Histological features
Management
Bartholin’s gland hyperplasia
Clinical features
Histological features
Management
Vulval hamartomas
References
39 Benign Vulval Naevi
CHAPTER MENU
Benign melanocytic naevi
Atypical genital naevi (AGN)
Genetics
Clinical features
Histological features
Management
Blue naevi
Bulky naevocytoma of the perineum
Clinical features
Histological features
Management
Inflammatory linear verrucous epidermal naevus
Clinical features
Histological features
Management
References
40 High‐Grade Squamous Intraepithelial Lesions
CHAPTER MENU
Terminology
Epidemiology
Molecular genetics
Pathophysiology
Histological features
Special stains
Risk factors. Smoking
Immunosuppression
Herpes simplex infection
Multiple sexual partners
Clinical features
Investigations
Differential diagnosis
Complications
Associated disease
Treatment
Medical therapy. Imiquimod
Topical 5‐fluorouracil
Photodynamic therapy (PDT)
Surgical therapy
Therapeutic vaccine
Prognosis
Follow‐up
Resources
References
41 Differentiated Vulval Epithelial Neoplasia
Epidemiology
Pathophysiology
Histological features
p53
p16
Other immunohistochemical stains
Clinical features
Differential diagnosis
Basaloid dVIN
HSIL
DEVIL (differentiated exophytic vulval intraepithelial lesion)
VAAD (vulval acanthosis and altered differentiation)
VAM (vulval aberrant maturation)
Complications
Associated disease
Treatment
Prognosis
Follow‐up
References
42 Vulval Paget’s Disease
CHAPTER MENU
Epidemiology
Pathophysiology
Classification
Histological features
Clinical features
Differential diagnosis
Complications
Associated disease
Treatment
Surgical treatment
Topical 5% Imiquimod cream
Other treatment modalities
Prognosis
Follow‐up
Resources
References
43 Vulval Squamous Cell Carcinoma
CHAPTER MENU
Epidemiology
Pathophysiology
Clinical features
Histology
Variants of SCC. Verrucous carcinoma
Condylomatous carcinoma
Keratoacanthoma
Diagnosis
Treatment of primary disease. Surgery
Radiotherapy
Chemotherapy
Immunotherapy
Sentinel lymph node biopsy
Plastic surgical reconstruction
Treatment of recurrent disease
Complications
Follow‐up
Prognosis
Prevention
Resources
References
44 Malignant Melanoma of the Vulva
CHAPTER MENU
Epidemiology
Genetics
Pathophysiology
Histological features
Clinical features
Investigations
Differential diagnosis
Staging
Treatment
Surgery
Radiotherapy
Chemotherapy
c‐KIT inhibitors
Check point inhibitors
Others
Outcome predictors
Prognosis
Follow‐up
Resources
References
45 Other Malignant Vulval Tumours
CHAPTER MENU
Tumours of epithelium. Basal cell carcinoma
Clinical features
Histological features
Management
Tumours of anogenital mammary glands. Anogenital gland carcinoma
Clinical features
Histological features
Management
Tumours of smooth muscle. Leiomyosarcoma
Clinical features
Histological features
Management
Tumours of striated muscle. Rhabdomyosarcoma
Clinical features
Histological features
Management
Tumours of fibroblastic tissue. Desmoid tumour
Clinical features
Histological features
Management
Dermatofibrosarcoma protuberans
Clinical features
Histological features
Management
Aggressive angiomyxoma
Pathophysiology
Clinical features
Histological features
Management
Malignant tumours of the adipose cells. Liposarcoma
Clinical features
Histological features
Management
Tumours of vascular tissue. Kaposi sarcoma
Clinical features
Management
Epithelioid haemangioendothelioma
Clinical features
Histological features
Management
Angiosarcoma
Clinical features
Histological features
Management
Neural tumours. Malignant peripheral nerve sheath tumour (malignant schwannoma)
Paraganglioma
Clinical features
Histological features
Management
Ewing sarcoma
Clinical features
Histological features
Management
Mesenchymal tumours of uncertain origin. Epithelioid sarcoma
Clinical features
Histological features
Management
Alveolar soft‐part sarcoma
Clinical features
Histological features
Management
CIC rearranged sarcoma
Clinical features
Histological features
Management
Tumours of Bartholin’s glands. Bartholin’s gland carcinoma
Clinical features
Histological features
Management
Miscellaneous malignant tumours. Merkel cell carcinoma
Clinical features
Differential diagnosis
Histological features
Management
Metastatic carcinoma
Clinical features
Histological features
Management
Germ cell neoplasms. Yolk sac tumours (endodermal sinus tumours)
Clinical features
Histological features
Management
Lymphoma and leukaemia
References
46 Female Genital Mutilation
CHAPTER MENU
Types of Female Genital Mutilation/Cutting (FGM/C)
Human rights and FGM/C
Impact of FGM/C
Sexuality and FGM/C
The motivation for and realities of clitoral reconstruction
Benefits of the surgery
Clinical examination
Contraindications for surgery
Surgical technique of clitoral reconstruction
Post‐operative complications
Post‐operative care
Conclusion
References
47 Transgender Surgery and the Neovulva
CHAPTER MENU
Microbiology of the neovagina
Cytology of the neovagina
Problems in the neovagina. Early complications
Neovaginal prolapse
Neovaginal fistula
Later complications. Introital and vaginal stenosis
Vaginal hair growth
Neovaginal perforation
Diversion colitis
Inflammatory bowel disease
Inflammatory dermatoses
Urinary symptoms
Infections. HIV
Other STIs
Sexual function
Malignancy
Resources
References
48 Psychological Aspects of Vulval Disorders
CHAPTER MENU
The hypothalamic‐pituitary‐adrenal (HPA) axis
Psychological symptoms and the vulva
Psychiatric presentations. Depression
Questions to ask to help with a diagnosis of depression in association with physical illness
Questions to ask about alcohol consumption in the context of depression
Questions to ask about suicidal ideation
Anxiety
Psychotic illnesses, delirium, and drug‐induced states
Medically unexplained symptoms
Somatoform disorders
Conversion disorder (functional neurological disorder)
Factitious disorders and malingering
Psychological aspects of specific conditions or events. Vulval pain
Childhood sexual abuse
Sexual assault and rape
Surgical procedures. Vulvectomy
Gender reassignment surgery
Cosmetic procedures
Sexual dysfunction
Hypoactive sexual desire
Female sexual arousal disorders
Genital sexual arousal disorder
Priapism
Persistent genital arousal syndrome
Orgasmic dysfunction
Psychological assessment. Basic communication and clinical assessment
Brief psychological evaluation
The degree of concern
Negative feelings and thoughts
Symptoms of anxiety
Multiple secondary care consultations
General health and family history
Investigation of psychosexual problems
Psychological and psychiatric interventions
Individual psychological interventions
Couple therapy
Psychopharmacological treatments
Resources
References
49 Inflammatory Dermatoses of the Vulva in Children
CHAPTER MENU
Napkin (diaper) eruptions
General principles of management of napkin eruptions
Vulval eczema (dermatitis)
Epidemiology
Pathophysiology
Clinical features
Differential diagnosis
Treatment
Prognosis and follow‐up
Allergic contact dermatitis
Severe irritant reactions
Psoriasis
Epidemiology
Clinical features
Differential diagnosis
Complications
Treatment
Prognosis and follow‐up
Resources
Lichen sclerosus
Epidemiology
Genetics
Pathophysiology
Histological features
Clinical features
Differential diagnosis
Complications
Associated disease
Treatment
Prognosis and follow‐up
Resources
Immunobullous disease. Bullous pemphigoid
Clinical features
Investigations
Differential diagnosis
Treatment
Prognosis
Mucous membrane pemphigoid (MMP)
Linear IgA disease
Epidemiology
Genetics
Pathophysiology
Clinical features
Treatment
Prognosis and follow‐up
Dermatoses associated with systemic disease. Crohn’s disease
Acrodermatitis enteropathica
Kawasaki disease
Langerhans cell histiocytosis
Mastocytosis
Acne
Drug eruptions
Vulval pain in children
References
50 Vulval Infection in Children
CHAPTER MENU
Investigation of infection in children
Common vulval infections in children
Foreign bodies
Bacterial infections. Infective vulvovaginitis
Pathophysiology
Clinical features
Differential diagnosis
Complications
Treatment
Prognosis and follow‐up
Resources
Group A streptococcal dermatitis
Epidemiology
Pathophysiology
Clinical features
Differential diagnosis
Complications
Treatment
Ecthyma
Toxin‐mediated perineal erythema
Congenital syphilis
Viral infections. Molluscum contagiosum
Pathophysiology
Clinical features
Differential diagnosis
Complications
Treatment
Prognosis
Genital warts
Pathophysiology
Clinical features
Differential diagnosis
Complications
Associated disease
Treatment
Prognosis and follow‐up
Herpes simplex
Orf
Other infections. Tuberculosis
Other infections
Acute non‐sexually transmitted reactive genital ulcers (Lipschutz ulcers)
References
51 Anatomical Abnormalities in Children
CHAPTER MENU
Labial adhesions (labial agglutination)
Epidemiology
Pathophysiology
Clinical features
Differential diagnosis
Complications
Treatment
Prognosis
Infantile pyramidal perineal protrusion
Epidemiology
Pathophysiology
Histological features
Clinical features
Differential diagnosis
Associated disease
Treatment
Clitoromegaly in children
Childhood asymmetry labium majus enlargement (CALME)
Pathophysiology
Histological features
Clinical features
Investigations
Differential diagnosis
Treatment
Urethral prolapse
Epidemiology
Pathophysiology
Clinical features
Differential diagnosis
Treatment
Abnormalities of the hymen
Other developmental abnormalities. Accessory labial fold and perineal lipoma
Perineal groove
Resources
References
52 Vulval Trauma in Children
CHAPTER MENU
Non‐thermal vulval trauma
Causes
Clinical features
Differential diagnosis
Complications
Treatment
Thermal damage
Epidemiology
Causes
Clinical features
Complications
Treatment
Chemical damage
Hair tourniquet syndrome
Bites
Child sexual abuse
Resources
References
53 Vulval Lesions in Children
CHAPTER MENU
Clitoral cysts
Vulval naevi
Infantile haemangiomas
Clinical features
Differential diagnosis
Complications
Associated disease
Treatment
Vulval tumours
Other lesions
References
54 Vulval Trauma
CHAPTER MENU
Obstetric injury
Trauma with consensual coitus
Post‐coital fissures
Fossa navicularis fissures (granuloma fissuratum)
Hymenal fissures
Accidental injury
Epidemiology
Causes
Pathophysiology
Clinical features
Investigations
Differential diagnosis
Complications
Treatment
Thermal injury
Epidemiology
Causes
Clinical features
Complications
Treatment
Chemical injury
Radiation injury
Pathophysiology
Clinical features
Differential diagnosis
Complications
Treatment
Genital self‐mutilation
The vulva and sport
Cyclist’s vulva
Pathophysiology
Histological features
Clinical features
Investigations
Differential diagnosis
Treatment
Other sporting injuries
References
55 Cosmetic Procedures and Complications
CHAPTER MENU
Procedures on vulval structures. Labia minora
Labia majora
Mons pubis
Perianal bleaching
Pubic hair. Hypotrichosis
Pubic hair grooming
Shaving
Laser hair removal
Depilatory cream
Waxing
Piercing
Tattoos
Resources
References
Glossary of Terms
Index
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Отрывок из книги
Edited by
Fiona M. Lewis, MD, FRCP
.....
Atypical presentations may not be diagnosed until adolescence or adulthood, and features can overlap with those of polycystic ovarian syndrome. Patients with CAH are at increased risk of diabetes, thromboembolic disease, and thyroid disorders [33].
In rare instances, virilisation of the female foetus may occur if the mother is suffering from certain ovarian or adrenal tumours or if she has unrecognised CAH. The absence of virilisation in the mother does not exclude a maternal source of androgens since the level of androgen required to change the external genitalia of the early female foetus is much less than would be required to have the same effect on the adult female [34].
.....