DERMATOSI VULVA

La terminologia clinica della vulva della IFCPC del 2011 descrive la sede e la morfologia delle lesioni. Sempre nel 2011 la ISSVD ha redatto una classificazione clinica dei disordini dermatologici vulvari ( Lynch PJ, Moyal-Barracco M, Scurry J, Stockdale C. J Low Genit Tract Dis. 2012;16(4):339-44) che mette in relazione la terminologia clinica con la patologia dermatologica o la compatibilità morfologica con una causa scatenante. Si riporta lo schema in lingua originale:

2011 ISSVD CLINICAL CLASSIFICATION OF VULVAR DERMATOLOGICAL DISORDERS

1) SKIN-COLORED LESIONS

A. Skin-colored papules and nodules
1. Papillomatosis of the vestibule and medial labia minora (a normal finding; not a disease)
2. Molluscum contagiosum
3. Warts (HPV infection)
4. Scar
5. Vulvar intraepithelial neoplasia
6. Skin tag (acrochordon, fibroepithelial polyp)
7. Nevus (intradermal type)
8. Mucinous cysts of the vestibule and medial labia minora (may have yellow hue)
9. Epidermal cyst (Syn, epidermoid cyst; epithelial cyst)
10. Mammary-like gland tumor (hidradenoma papilliferum)
11. Bartholin gland cyst and tumor
12. Syringoma
13. Basal cell carcinoma

B. Skin-colored plaques
1. Lichen simplex chronicus (LSC) and other lichenified disease (see definitions in Part IV above)
2. Vulvar intraepithelial neoplasia

2) RED LESIONS: PATCHES AND PLAQUES

A. Eczematous & lichenified diseases (see definitions in Part IV above)
1. Allergic contact dermatitis
2. Irritant contact dermatitis
3. Atopic dermatitis (rarely seen as a vulvar presentation)
4. Eczematous changes superimposed on other vulvar disorders
5. Diseases clinically mimicking eczematous disease (candidiasis, Hailey-Hailey disease and extramammary Paget’s disease) 6. Lichen simplex chronicus (lichenification with no preceding skin lesions)
7. Lichenification superimposed on an underlying preceding pruriticdisease

B. Red patches & plaques (no epithelial disruption)
1. Candidiasis
2. Psoriasis
3. Vulvar intraepithelial neoplasia
4. Lichen planus
5. Plasma cell (Zoon’s) vulvitis
6. Bacterial soft-tissue infection (cellulitis and early necrotizing fasciitis)
7. Extramammary Paget’s disease

3) RED LESIONS: PAPULES AND NODULES

A. Red papules
1. Folliculitis
2. Wart (HPV infection)
3. Angiokeratoma
4. Molluscum contagiosum (inflamed)
5. Hidradenitis suppurativa (early lesions)
6. Hailey-Hailey disease

B. Red nodules
1. Furuncles (“boils”)
2. Wart (HPV infection)
3. Prurigo nodularis
4. Vulvar intraepithelial neoplasia
5. Molluscum contagiosum (inflamed)
6. Urethral caruncle and prolapse
7. Hidradenitis suppurativa
8. Mammary-like gland adenoma (hidradenoma papilliferum)
9. Inflamed epidermal cyst
10. Bartholin duct abscess
11. Squamous cell carcinoma
12. Melanoma (amelanotic type)

4) WHITE LESIONS

A. White papules and nodules
1. Fordyce spots (a normal finding; may sometimes have a yellow hue)
2. Molluscum contagiosum
3. Wart
4. Scar
5. Vulvar intraepithelial neoplasia
6. Squamous cell carcinoma
7. Milium (pl. milia)
8. Epidermal cyst
9. Hailey-Hailey disease

B. White patches and plaques
1. Vitiligo
2. Lichen sclerosus
3. Post-inflammatory hypopigmentation
4. Lichenified diseases (when the surface is moist—see definitions in Part IV above)
5. Lichen planus
6. Vulvar intraepithelial neoplasia
7. Squamous cell carcinoma

5) DARK COLORED (BROWN, BLUE, GRAY OR BLACK) LESIONS

A. Dark colored patches
1. Melanocytic nevus
2. Vulvar melanosis (vulvar lentiginosis)
3. Post-inflammatory hyperpigmentation
4. Lichen planus
5. Acanthosis nigricans
6. Melanoma-in-situ

B. Dark colored papules and nodules
1. Melanocytic nevus (includes those with clinical and/or histologic atypia)
2. Warts (HPV infection)
3. Vulvar intraepithelial neoplasia
4. Seborrheic keratosis
5. Angiokeratoma (capillary angioma, cherry angioma)
6. Mammary-like gland adenoma (hidradenoma papilliferum)
7. Melanoma

6) BLISTERS

A. Vesicles and bullae
1. Herpesvirus infections (herpes simplex, herpes zoster)
2. Acute eczema (see definitions In Part IV above
3. Bullous lichen sclerosus
4. Lymphangioma circumscriptum (lymphangiectasia)
5. Immune blistering disorders cicatricial pemphigoid, fixed drug eruption,
Steven-Johnson syndrome, pemphigus)

B. Pustules
1. Candidiasis (candidosis)
2. Folliculitis

7) EROSIONS AND ULCERS

A. Erosions
1. Excoriations (See the disorders in Group 2A above)
2. Erosive lichen planus
3. Fissures arising on normal tissue (idiopathic, intercourse related)
4. Fissures arising on abnormal tissue (candidiasis, lichen simplex
chronicus, psoriasis, Crohn’s disease, etc.)
5. Vulvar intraepithelial neoplasia, eroded variant
6. Ruptured vesicles, bullae and pustules (see all of the disorders listed
above in Group 6 “Blisters”)
7. Extramammary Paget’s disease

B. Ulcers
1. Excoriations (related to eczema, lichen simplex chronicus)
2. Aphthous ulcers; syn. Aphthous minor, aphthous major, Lipschütz ulcer
(occurring either as an idiopathic process or secondary to other diseases
such as Crohn’s, Behçet’s, various viral infections)
3. Crohn’s disease
4. Herpesvirus infection (particularly In immunosuppressed patients)
5. Ulcerated squamous cell carcinoma
6. Primary syphilis (chancre)

8) EDEMA (DIFFUSE GENITAL SWELLING)

A. Skin-colored edema
1. Crohn’s disease
2. Idiopathic lymphatic abnormality (congenital Milroy’s disease)
3. Post-radiation and post-surgical lymphatic obstruction
4. Post-infectious edema (esp. staphylococcal and streptococcal cellulitis)
5. Post-inflammatory edema (esp. hidradenitis suppurativa)

B. Pink or red edema
1. Venous obstruction (e.g., pregnancy, parturition)
2. Cellulitis (primary or superimposed on already existing edema)
3. Inflamed Bartholin duct cyst/abscess
4. Crohn’s disease
5. Mild vulvar edema may occur with any inflammatory vulvar disease

A seguire le definizioni della morfologia delle lesioni vulvari come previsto dalla 2011 IFCPC Clinical Terminology of the Vulva.

Dermatological Terminology / Definizione terminologia dermatologica

An understanding of basic dermatological terminology is essential to understand and describe morphologic characteristics in vulvar skin disease.

Lesion: a visible or palpable abnormality.

Rash: the word used colloquially to describe numerous or diffuse abnormalities. The lesions within a rash (exanthem) will resemble each other but may vary from being described as macular, papular, vesicular, urticarial, etc. The definitions below are more specific.

Patch: an extension of a macule in length and width, (>1.5 cm); a non-palpable area of color change.

Papule: a small, elevated, palpable lesion (< 1.5 cm in diameter).

Plaque: an enlargement of a papule in length and width, (>1.5 cm). Elevated, palpable and flat-topped.

Lichenification: thickening of the skin with increased prominence of skin markings, usually from scratching or rubbing; excoriations may be seen. Scale may or may not be present. Skin may be red, white or skin-colored; the white color is a result of moisture retention in the thickened outer epidermis. This lesion may occur on normal skin or be part of an underlying skin disease.

Nodule: a large papule, (>1.5 cm); often hemispherical or poorly marginated; may be located on the surface, within or below the skin; may be cystic or solid.

Cyst: a closed cavity lined by epithelium that contains fluid or semisolid material.

Vesicle: a small, fluid-filled blister (< 0.5 cm in diameter) in which the fluid is clear to slightly cloudy. When the roof of a vesicle has been removed or has disintegrated, an underlying erosion remains visible.

Pustule: a vesicle that is packed with neutrophils and appears to be opaquely white. The fluid in a pustule (pus) is white or yellow

Bulla: a large vesicle greater than 0.5 cm in diameter, containing clear fluid that is either located in a single compartment or is multiloculated, as a result of coalescence of multiple vesicles or bullae.

Crust: the irregular residue of exudates drying on the skin; implies disruption of the underlying epithelial barrier layer; may be thin or thick. The color will be yellow when made of dried serum, yellow or yellow-green when made of purulent material, or brown, dark red or brown when made of old blood.

Scale: a hyperproliferative response of the epidermis; grey, white, or silver in color, looking like fine dust but presenting with palpable roughness.

Erosion: a shallow defect affecting the epidermis down to the basement membrane; the dermis is intact.

Primary erosions: caused by trauma, usually scratching. These excoriations are linear or angular in shape, without “collarettes.”
Secondary erosions: caused by breakdown or removal of a blister roof. These erosions are round and have a collarette of scale encircling the defect.

Fissure: a thin, linear erosion of the skin surface.

Ulcer: a deeper defect affecting the epidermis and some or all of the dermis.

Nel 2006 fu redatta una classificazione istologica delle dermatosi vulvari e le correlazioni fra la stessa istologia correlazioni e la patologia clinica. A seguire la tabella originale e successivamente altra tabella simile modificata tratta da vulvovaginaldisorders.com.

2006 Histological Classification of Vulvar Dermatoses Pathological Subsets and Their Clinical Correlates - ISSVD

Spongiotic pattern:
Atopic dermatitis
Allergic contact dermatitis Irritant contact dermatitis

Acanthotic pattern (formerly squamous cell hyperplasia):
Psoriasis
Lichen simplex chronicus
Primary (idiopathic)
Secondary (superimposed on lichen sclerosus, lichen planus, or other vulvar disease)

Lichenoid pattern:
Lichen sclerosus
Lichen planus

Dermal homogenization/sclerosis pattern:
Lichen sclerosus

Vesiculobullous pattern:
Pemphigoid, cicatricial type
Linear IgA disease

Acantholytic pattern:
Hailey-Hailey disease
Darier disease
Papular genitocrural acantholysis

Granulomatous pattern:
Crohn disease
Melkersson-Rosenthal syndrome

Vasculopathic pattern:
Aphthous ulcers
Behcet disease
Plasma cell vulvitis

La successiva classificazione è un completamento della precedente in quanto al centro si descrive nei particolari il quadro istologico.

2006 ISSVD CLASSIFICATION OF VULVAR DERMATOSES: PATHOLOGIC SUBSETS AND THEIR CLINICAL CORRELATES (Table modified from the original) from: vulvovaginaldisorders.com
Name of finding	Definition	Common clinical correlates
Spongiosis	Intercellular edema between keratinocytes in the epidermis; keratinocytes may become elongated; often accompanied by exocytosis of lymphocytes and sometimes neutrophils or eosinophils.	Eczematous dermatitis (atopic, contact and allergic)
Acanthosis	Diffuse epidermal hyperplasia with increased thickness of the stratum spinosum.	Lichen simplex chronicus, Psoriasis, Reiter’s syndrome
Lichenoid pattern	A band-like infiltrate of inflammatory cells (usually lymphocytes) in the superficial dermis, parallel to the epidermis.	Lichen sclerosus, Lichen planus
Dermal sclerosis	Increased collagen with decreased numbers of fibroblasts causing thickening.	Lichen sclerosus
Vesiculobullous pattern	Immune deposits in skin may be bands of IgG or IgA at the basement membrane zone, of IgG deposits on the cell surface of keratinocytes.	Bullous pemphigoid, Cicatricial pemphigoid, Pemphigoid gestationis, Pemphigus vulgaris, Pemphigo vegetans
Acantholytic pattern	Loss of cohesion between keratinocytes because of dissolution of intercellular connections; may cause an intraepithelial vesicle. Keratinocytes are rounded rather than elongated and lymphocytes do not migrate into the epidermis.	Hailey-Hailey disease, Darier/s disease, Acantholytic dermatosis of the vulvocrural area
Granulomatous pattern	Granulomas are conglomerates of monocyte-derived histiocytes (macrophages); sometimes mixed with other inflammatory cells. Groups of these histiocytes may aggregate to form mulinucleated giant cells. Chronic granulomatous diseases are a genetically heterogeneous group of immunodeficiencies producing infectious and non-infectious disease in which phagocytic cells fail to kill organisms that they have engulfed because of defects in a system of enzymes that produce free radicals and other toxic small molecules.	Crohn disease, Melkersson-Rosenthal syndrome
Vasculopathic pattern	Non-inflammatory purpura: extravasation of RBCs into dermis. Narrowing or obliteration of small vessel lumina. Inflammation of blood vessel wall.	Aphthous ulcers, Behcet disease, Plasma cell vulvitis (vulvitis plasmacellularis)
References: Lynch PJ, Moyal-Barracho M, Scurry J, Stockdale C. 2011 ISSVD Terminology and Classification of Vulvar Dermatological Disorders: An Approach to Clinical Diagnosis. Journal of Lower Genital Tract Disease, Vol 16, 4, 2012, 339-344.

Queste sono le lesioni vulvari (lesion Type della classificazione 2011 IFCPC Clinical Terminology of the Vulva) misurabili in cm. Devo segnalare una perplessità sulla misurazione delle lesioni, in quanto nel lavoro originale : 2011 Terminology of the Vulva of the International Federation for Cervical Pathology and Colposcopy a pag.3 nella tab.2 Definition of primary lesion types si fa riferimento al lavoro: "2011 ISSVD Terminology and Classification of Vulvar Dermatological Disorders: An Approach to Clinical Diagnosis" di Peter J. Lynch, MD; Micheline Moyal-Barracco, MD; James Scurry, MD; Colleen Stockdale, MD, dove risulta una misurazione diversa (1 cm anzichè 1,5 cm).

Definitions of Primary Lesion Types
Term	Definition
Macule	Small (<1.5 cm) area of color change; no elevation and no substance on palpation
Patch	Large ( >1.5 cm) area of color change; no elevation and no substance on palpation
Papule	Small (<1.5 cm) elevated and palpable lesion
Plaque	Large ( >1.5 cm) elevated, palpable, and flat-topped lesion
Nodule	A large papule ( >1.5 cm); often hemispherical or poorly marginated; may be located on the surface, within, or below the skin;nodules may be cystic or solid
Vesicle	Small (<0.5 cm) fluid-filled blister; the fluid is clear (blister: a compartmentalized, fluid-filled elevation of the skin or mucosa)
Bulla	A large ( >0.5 cm) fluid-filled blister; the fluid is clear
Pustule	Pus-filled blister; the fluid is white or yellow
Jacob Bornstein, MD, MPA, Mario Sideri, MD, Silvio Tatti, MD, Patrick Walker, MD, Walter Prendiville, MD, and Hope K. Haefner, MD, For the Nomenclature Committee of the International Federation for Cervical Pathology and Colposcopy

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References: Lynch PJ, Moyal-Barracho M, Scurry J, Stockdale C. 2011 ISSVD Terminology and Classification of Vulvar Dermatological Disorders: An Approach to Clinical Diagnosis. Journal of Lower Genital Tract Disease, Vol 16, 4, 2012, 339-344.