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Cutaneous Diseases of the Vulva
Cutaneous Diseases of the Vulva JOHN F. WILSON, M.D. *
PROMPT diagnosis and treatment of cutaneous diseases of the vulva are of special importance, owing to the peculiar nature of the skin in this region. Conditions of heat, moisture and friction, together with diminished aeration, periodic changes in blood supply during menstruation and pregnancy, as well as more pronounced senile changes, serve to alter the ordinary aspect of various skin diseases, to prolong their course and to render them susceptible to unusual complications and malignant degeneration. A brief review of certain practical aspects of the problem and of recent therapeutic advances follows.
PYOGENIC INFECTIONS
The advent of the sulfonamides and the antibiotics has so reduced the incidence of carbuncle and erysipelas that they are now considered rare and much less formidable than they were some years ago, but the vulvar region is still a site of predilection for such conditions as impetigo, pyoderma, ecthyma and folliculitis (Fig. 260). Pyogenic granuloma appears as a smooth, pedunculated tumor of a pink to dark red color, which bleeds easily, leaving a fine brownish or blackish crust. Biopsy will exclude malignant transformation. In chronic, recurring hidroadenitis suppurativa (Fig. 261), the infection attacks the apocrine glands and red, tender, swollen nodules slowly erode to the surface. Differential diagnosis of the pyogenic infections of the vulvar region will include consideration of herpetic lesions, eczematous eruptions, drug eruptions, pemphigus and syphilitic lesions. At present the routine treatment recommended for pyogenic infections of the vulvar region is the application of neomycin ointment. It should be kept in mind, however, that the less expensive 3 per cent ammoniated mercury ointment suffices in many cases. Should ointment treatment lead to development of grease folliculitis, a neomycin lotion may be applied during the day, reserving antibiotic ointment therapy for night use. Hidroadenitis suppurativa, which formerly persisted for years, also
* Assistant Professor of Dermatology and Syphilology, JefJerson Medical College; Visiting Lecturer in Dermatology, Graduate School of Medicine, University of Pennsylvania; Chief Dermatologi8t, Misericordia and Presbyterian Hospitals, Philadelphia. 1741
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John F. Wilson
Fig. 260. Pustular folliculitis in obese patient.
Fig. 261. Chronic hidroadenitis suppurativa (note scar tissue with sinus tracts and comedones).
responds well to antibiotic treatment. Following isolation of:the offending organism, the sensitivity of the latter to antibiotics is established and the appropriate parenteral antibiotic may then be administered for several months.
Cutaneous Diseases of the Vulva FUNGOUS INFECTIONS
Numerous fungi flourish as saprophytes in the vulvar region, but heat, sweat and friction frequently transforms them into virulent pathogenic organisms. Superimposed acute intertrigo will activate dormant mycoses. The special properties of the offending fungus, the local and general predisposition of the skin in the individual patient, as well as lowered resistance due to other conditions, will determine the cutaneous manifestations and course of thE> infection. There is always a distinct tendency toward temporary disappearance followed by recurrence. Tinea cruri8 must be distinguished from other superficial dermatomycoses, seborrheic dermatitis and some circinate forms of psoriasis and syphilis, as well as from other fungous infections. A papulovesicular margin and a tendency to increase in size distinguish the lesions of tinea
Fig. 262. Erythrasma. Note superficial changes with very low-grade inflammatory reaction. A, Vulva; E, axilla of same patient.
cruris, while circular psoriasis presents a lamellar desquamation and a more reddish color. Psoriatic lesions are likely to appear also on other parts of the body. The circinate and annular syphilomas have an indurated border and a more brownish hue. In treatment of the dermatomycoses, various fungicides have been found of value. Ointments containing undecylenic acid, salicylic acid (3 per cent) or sulfur (5 per cent) are recommended. Once the lesions have disappeared, salicylic acid (3 per cent) and resorcinol (5 per cent) in aqueous or alcoholic solution may prevent otherwise frequent recurrences. In more superficial tinea, such as erythra8ma (Fig. 262), ointments containing 2 to 3 per cent salicylic acid are likewise recommended. Moniliasis is caused by the most common saprophyte of the normal .vagina and vulva, namely Candida (Monilia) albicans. Here, too, there is the problem of individual susceptibility, local predisposition, the effects of wet compresses, cold bandages or prolonged baths, as well as genera.l disposition due to lowered resistance owing to various affections includ-
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ing obesity and diabetes. Moniliasis may occur as a conjugal infection or from infection by douche nozzles and is very common in advanced stages of pregnancy or associated with diabetes. In moniliasis, it is important to direct proper treatment to a coexisting diabetes in order to prevent relapse of the eruption. One per cent aqueous gentian violet solution is of great value. PEDICULOSIS PUBIS
Pediculosis pubis (phthiriasis pubis) is caused by infestation of the pubic region with the crab louse. Itching followed by scratching results in excoriations and follicular dermatitis or pustular eruptions due to secondary infections. Also attempts at self-treatment with insecticides or mercury ointment may cause a dermatitis. Blue spots appear due to the bluish dye excreted by the parasite and help to distinguish pediculosis from the red syphilitic roseola. In treatment of pediculosis pubis, most antiscabetic drugs will prove effective, and, in particular, benzyl benzoate emulsion. VIRAL INFECTIONS
Cutaneous affections of the vulva due to filtrable viruses include common warts, molluscum contagiosum, herpetic eruptions and vaccinia. COIllIllon Warts
Common warts (condyloma acuminatum, Fig. 263) are benign, infectious tumors of viral origin. The vulvar region presents a site of predilection for their development. They are not of venereal origin, but may coincide with venereal disease. They vary in size from that of a pin head to that i)f a strawberry or fist, and coalescing lesions may resemble a cock's comb or cauliflower. When macerated owing to sweat, heat or friction, they emit a malodorous secretion, and rapid growth has been noted during pregnancy. They do not resemble the flat syphilitic condyloma in which the Treponema pallidum may be demonstrated, and which are associated with enlarged inguinal glands. In spite of a clinically and histologically benign appearance, warts may mask the onset of malignancy at a time when even biopsy is negative. Podophyllin is the treatment of choice for these warts, and podophyllin (25 per cent) in mineral oil has proved almost constantly effective after one or two applications. Podophyllin (25 per cent) in Sandrac varnish dries quickly and permits better control of medication, showing blanching of the lesions within a few hours, sloughing by the second or third day and complete disappearance in four days. When one wart disappears, others frequently follow suit. Herpetic Eruptions
These include herpes simplex, herpes progenitalis, herpes menstrualis and herpes zoster. The differentiation of herpes simplex from herpes zoster depends on the same criteria valid for other regions.
Cutaneous Diseases of the Vulva Herpes progenitalis is usually painless and is found most frequently on the labia majora, less frequently on the labia minora and clitoris. Healing occurs in about six days, leaving no scars. Injudicial treatment will occasionally give rise to secondary infection that may suggest venereal disease, and thus complicate the diagnostic problem. Early stages of lymphogranuloma venereum not infrequently suggest herpes progenitalis, but ca~ be differentiated within a short time, owing to their rapid spread to the lymph nodes and the appearance of the inguinal bubo. In herpes simplex, a whole series of vulvar skin affections must be excluded, such as eczematous eruptions, drug erup-
Fig. 263. Condylomata acuminata. Patient is 6 months pregnant. (Wharton: Gynecology, 2nd Ed.)
tions, ulcus vulvae acutum (Lipschutz) and aphthous ulcers. The distribution of the vesicles and rapid course of herpes simplex serve to distinguish the latter from venereal infections. In cases of uncomplicated herpes progenitalis, simple treatment with mild dusting powders such as boric acid powder, and interposition of a piece of lint as protection against friction in certain areas will suffice. One per cent hydrocortisone ointment is sometimes of value. In cases of persistently recurrent herpes simplex, some success has been achieved by administration of smallpox vaccine at intervals of six to twelve weeks. NEURODERMATITIS
The incidence of neurodermatitis is increasing with the strain and stress of modern living. Depletion of nerve energy beyond a given level
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results in irritation of the nerve endings in the skin, and the usual sequence of itching, scratching and inflammation may in the long run completely incapacitate the patient for her ordinary pursuits. With the superimposition of phobias related to malignancy, loss of position or of friends, or other wholly imaginary situations, a vicious circle is established, in which any nervous upset may act as a trigger mechanism resulting in a cutaneous reaction. Neurodermatitides occur not only in neurotically unstable individuals, but frequently in normal subjects.)This becomes more comprehensible when one takes into consideration that the blood vessels of the skin, the sweat glands and the pilomotor muscles
Fig. 264. Localized neurodermatitis. Note thickening of vulva.
are all supplied with sympathetic innervation, and that not only physical but psychogenic processes affect sweat secretion and blood supply of the genital area. Pruritus Vulvae
Although rather a symptom than a disease, itching of the vulva presents many problems from the etiologic, clinical; diagnostic and therapeutic point of view. For practical purposes it can be considered to be a form of localized neurodermatitis. In most instances, the skin of the vulva appears entirely normal. However, when changes in the skin occur there may be oozing and crusting in the acute stage, followed by the dry and scaly appearance in the subacute stage, and this, in turn, by the lichenification characteristic of the chronic stage. From the etiologic standpoint, innumerable psychosomatic conditions have been considered. Pruritus vulvae very rarely affects little girls, but is more common in older women, especially following the menopause. Where all other
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causes for itching in this region have been eliminated (contact dermatitis, seborrheic dermatitis, etc.), psychogenic irritation of the cutaneous nerve endings may be considered. Treatment includes removal of possible sources of irritation, antipruritic medication and psychotherapy, including mental and physical rest. During the acute stage of localized neurodermatitis, compresses of boric acid or Burow's solution may be alternated with application of mild lotions, such as calamine lotion. Calamine lotion and zinc paste are helpful during the subacute stage, and tar ointment is recommended for the chronic stage, but must not be applied in the hairy regions. Insomnia may be relieved by hypnotics. Tranquillizing medication (Reserpine or Thorazine) during the day is sometimes helpful. Rest, change of scene and change of usual occupations will often yield relief, but in some instances, psychotherapy is necessary. One of the newer remedies, hydrocortisone ointment (1 to 2.5 per cent) has proved successful in cases that have failed to respond to other treatments. Vulvectomy and nerve block, as "cures" for pruritus, are strongly contraindicated. ECZEMATOUS DERMATITIS
In this condition, the acute stage is characterized by erythema, edema, the appearance of vesicles, oozing and crusting. In the subacute stage erythema and edema are diminished, and the skin becomes dry and scaly. The chronic stage is distinguished by lichenification with papules and scaling, accentuation of the normal skin folds (shark-skin appearance) from thickening due to cellular infiltration. Eczematous dermatitis is of varied etiology and constitutCils the cutaneous reaction to irritants. It may represent a contact dermatitis, developing from contact with irritants oontained in clothing, toilet tissue, condoms, douches, contraceptives, nail polish, perfumes, deodorants or hygienic pads, the lacquer or plastie resins in toilet seats and many other things. A careful review of the patient's history will usually disclose the cause, and cure follows removal of the offending agent. Local soothing applications are indicated. INTERTRIGO
Intertrigo, due to irritation of apposing surfaces by heat, moisture and friction, presents a primary erythema, followed by maceration, erosion, exudation, itching and burning, particularly in obese persons, and as a result of walking, especially in hot weather (Fig. 265). Eventually, fissures and verrucous lesions may develop. Anamnesis will disclose the appearance in extravulvar locations, and in addition will exclude contact dermatitis and neurodermatitis. If intertrigo masks some previous eruption, diagnosis may be possible only after continued observation of the condition and its response to treatment. The latter consists in separation of the parts, dusting with zinc stearate and talc, and bathing with
John F. Wilson a lotion containing 5 per cent zinc oxide in liquor calcis. Adequate aeration of the region must be provided for with special garments to prevent friction when walking and the patient should be advised to reduce if obese. SEBORRHEIC DERMATITIS
This affection is of ~J1lmown etiology and is characterized by the development of greasy yellow scales, occasionally leading to lichenification and now and then showing exacerbations with constitutional conditions or emotional upsets. Diagnosis may prove difficult if the lesions happen to be located exclusively in the vulvar region, and if eczematization or intertrigo is present. Here, too, continued observation of the dermatitis and its response to treatment may offer diagnostic clues. In the acute stage, moist compresses and lotions will afford relief, and when the acute-
Fig. 265. Intertrigo in obese patient. A, Vulva; B, anal region; C, inframammary region.
ness has subsided somewhat, sulfur ointment in the form of a zinc paste containing 6 per cent precipitated sulfur is helpful in association with large doses of vitamin B complex, general tonic treatment and attention to possible concomitant constitutional disease. PSORIASIS
Whereas psoriasis (Figs. 266, 267) usually affects other parts of the body, it is occasionally limited to the vulvar region. However, friction, injudicious treatment, or the superimposition of other dermatoses may complicate diagnosis. Inflammation of the lesions as a result of irritation may lead to formation of fissures with whitish margins or to diffuse vulvitis. Superinfection with syphilis may convert the psoriatic patches into diffuse syphilitic infiltrations, or epithelioma may develop in these patches following prolonged arsenotherapy. If the lesions are exclusively vulvar, diagnosis may be difficult, as is also true in cases without much scaling or erythema. Finding of lesions elsewhere on the body, where conditions leS:d to a more typical development, are of diagnostic aid.
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Fig. 266. Psoriasis of seborrheic type.
Fig. 267. Psoriasis in patient with pruritus vulvae, showing Koebner's phenomenon with outbreak of psoriasis as a result of scratching. (From Fritz T. CaIJomon and John F. Wilson, The Nonvenereal Diseases of the Genitals, 1955. Courtesy of Charles C Thomas, Publisher, Springfield, Illinois.) KRAUROSIS AND LEUKOPLAKIA VULVAE
Kraurosis of the vulva develops in women after the menopause, or in younger women following ovariectomy. Kraurosis has been described as an exaggeration of normal postmenopausal atrophy. The labia and cli':
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Fig. 268. Chronic and atrophic leukoplakic vulvitis. (From Fritz T. Callomon and John F. Wilson, The N onvenereal Diseases of the Genitals, 1955. Courtesy of Charles C Thomas, Publisher, Springfield, Illinois.)
Fig. 269. Leukoplakia.
toris diminish in size and disappear. Sexual intercourse may lead to fissures and dyspareunia. The mucosa is dry and smooth, the affected areas appearing whitish or yellowish. The skin of the vulva becomes thinner and may be discolored with deposits of pigment. The pubic hair is sparse and the patient is afflicted with unbearable pruritus and often 'with painful micturition.
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Leukoplakia is characterized by the appearance of one or more circumscribed, slightly elevated, milky or bluish-white, later shiny, silvery patches that result from epithelial proliferation and keratinization and are frequently the source of erosions and ulcer formation. (See Figs. 268,269.) Kraurosis and leukoplakia are not infrequently associated (in about 50 per cent of cases). Malignant degeneration of leukoplakia is very common. It is stated that about 50 per cent of all cases of vulvar carcinoma have their origin in leukoplakia. Kraurosis, on the other hand, is not known to undergo malignant degeneration. Where kraurosis and leukoplakia exist together, it is the latter that provides the starting point for cancerous growths. Kraurosis is characterized by atrophic processes from the beginning, whereas in leukoplakia the tendency is toward hyperplasia and hypertrophy. Kraurosis bears some striking resemblances to lichen sclerosus et atrophicans. The secondary changes due to rubbing and scratching of neurodermatitis must be differentiated from leukoplakia. Whitish areas of localized neurodermatitis may disappear following application of 1 per cent hydrocortisone ointment and thus do away with the necessity for radical surgery necessary in true leukoplakia. In kraurosis vulvae, estrogen treatment, orally, parenterally or locally as an ointment, is definitely indicated, but is not always effective. Itching does tend to subside. Roentgen therapy for itching of the vulva that fails to respond to other treatment is often recommended, but is hardly to be prescribed for kraurosis. Also vulvectomy as a radical measure to relieve pruritus is not always completely successful and is most mutilating. In leukoplakia, on the other hand, the possibility of malignant transformation leads to a demand for early vulvectomy. However, when only small lesions are present, biopsy followed by electrocoagulation may suffice. Biopsy should be repeated at frequent intervals to be sure that malignant degeneration is not in process. BENIGN NEOPLASMS
Located on the labium majora, fibroma develop as subepidermal, firm, smooth nodules. They are at first hardly discernible but show a marked growth tendency during menstruation and pregnancy. Finally a pedicle is formed and the tumor may hang down between the thighs to be irritated by friction, sweat and urine, with final inflammation, erosion and ulceration. Cystic or myxomatous degeneration may eventually take place, and if not excised in time, malignant degeneration may result. Benign lipomas of the vulva grow very slowly, as a rule, to reach the size of an egg or a man's fist, but some lipomas show a marked growth tendency.
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Other benign tumors of the vulva include neurofibroma, lymphangioma, the tiny hemangiomas of hereditary telangiectasis, congenital cysts, follicular retention cysts, myomatous cysts, supernumerary mammary tissue and pigmented nevi. Melanotic nevi should be excised as early as possible to prevent malignant degeneration. Hemangiomas and lymphangiomas may be subjected to refrigeration with solid carbon dioxide or be injected with sclerosing solutions. Radium therapy is also recommended. MALIGNANT NEOPLASMS
In the present article, we are chiefly concerned with primary malignant neoplasms of the vulva and, in: particular, with the various forms of epithelioma. The initial lesion is usually a painless nodule or erosion and is rarely accorded attention. When chronic genital ulcers resist treatment, the fear of malignancy is engendered, as the laity is well informed as to the possibilities of malignant degeneration of chronic lesions. It must be emphasized that so-called precancerous lesions do not always become malignant. There are indefinite transition stages and clinical and histologic findings may clash. Many dermatologists refer to lesions that customarily undergo malignant transformation as malignant in fact. SUGh conditions include Bowen's and Paget's disease of the vulva, as well as leukoplakia. Cancer may also develop from chronic inflammatory lesions of most varied origin. Bowen's disease appears as solitary or multiple, irregular, red,hyperkeratotic patches. These lesions may become crusted or scaly and show no tendency to progress for long periods of time. Squamous cell carcinoma will develop in about 25 per cent of the cases, and immediate removal is recommended. This is true also in the case of Paget's disease. Removal of the regional glands is indicated only when they are definitely involved in the process. However, following removal of a single lesion of Bowen's disease, other unrelated lesions may appear elsewhere in the genital region. Basal cell epithelioma grows very slowly with a tendency toward central ulceration and formation of a firmly adherent crust. It has been estimated that about 20 per cent of all clinically diagnosed basal cell epitheliomas are transitional forms between basal and squamous cell epithelioma. Malignant melanoma of the genital region occurs more frequently in males than in females, in whom it develops around the age of 50 years. It is interesting that AlIen and Spitz, in a review of 934 cases of malignant melanoma, found the highest incidence of primary malignant melanoma on the soles of the feet, the mucosa of the female genitals and on the head and neck. It is calculated that about two-thirds of these neoplasms have their origin in pigmented nevi, and in many cases the
Cutaneous Diseases of the Vulva
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presence of such a lesion prior to malignant degeneration was probably overlooked by the patient. This tumor has been found to develop from embryonic nevus cells in the skin, remaining quiescent for years, only to undergo malignant transformation later in response to some stimulus. It is even possible that such tumors may arise from the normal skin, but in this area an accurate history is most difficult to obtain. In the absence of clinically noticed nevi, malignant melanoma begins as a solitary, blue-black or brownish black flat or raised nodule, and rapidly forms a solid black tumor mass. This tumor must be differentiated from benign pigmented nevus, verruca senilis and angiomatous
Fig. 270. Advanced carcinoma of vulva. (Novak: Gynecologic and Obstetric Pathology, 3rd Ed.) .
tumors. In the latter tumors, excision is imperative with the slightest sign of growth. In fact, any pigmented nevus in the genital region exposed to the irritating conditions of this area should be removed. Even following radical excision of the tumor, including the regional lymph glands, prognosis is poor. Squamous cell carcinoma of the vulva is very rare as compared to carcinoma of the uterus, the ratio being reported as probably 1: 35 to 40. Only 1 to 3 per cent of carcinomas of the female genitals affect the vulva. Vulvar carcinoma usually develops during the sixth or seventh decade of life and is seen much less frequently in other decades, although isolated cases in younger women and especially carcinoma of the clitoris in women under 40 years of age have been observed.
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John F. Wilson
This neoplasm usually begins in the mucosa of the vulva and not infrequently has its origin in a pre-existing pigmented nevus or in a patch of leukoplakia. The initial lesion appears on the inner aspect of the labia majora, and occasionally on the labia minora, or in the region of the urethral meatus where the squamous epithelium of the cutis and the cylindrical epithelium of the mucosa come together. The physician is not usually called until itching, soreness or the presence of a nodule has been observed by the patient. Upon examination, the physician finds a nodule, possibly of the size of a walnut, or a more diffuse nodular infiltration, movable under the skin or mucosa. Shortly, ulceration sets in with liberation of a purulent or bloody discharge. In other instances, a papillary growth may give rise to a cauliflower-like structure, suggesting condyloma acuminatum. Especially in younger women, vulvar carcinoma runs a rapid course. The clinical forms include a fungating and an infiltrating form. Carcinoma of the clitoris and urethral area spread very rapidly, owing to the rich blood and lymph supply of the region. The inguinal and subinguinal glands are involved early. In carcinoma of the clitoris, the patient rarely survives more than 15 to 18 months. Metastasis from vulvar carcinoma occurs early and the lymph glands may be involved, although they appear unchanged. Primary sarcoma of the vulvar region is very rare and usually is located on the labia majora, appearing first as a solid nodule beneath the movable skin. It grows rapidly and hemorrhages and necrosis soon transform its appearance. The primary nodule may remain stationary for a long period before growth suddenly ensues. Sarcoma may develop in pre-existing fibrolipoma or fibroma of the vulva and may be of any type or mixed. A ngioblastomas are rarely seen in this region. Metastatic tumors from primary tumors elsewhere in the body may involve the vulva. Metastatic chorionepithelioma of the vulva is a rare tumor giving a positive pregnancy test. It may appear as a single tumor or as several pea- to hazel-nut sized tumors of a dark, reddish blue to black color. These lesions may resemble a circumscribed hematoma or thrombotic varix. They show a marked hemorrhagic tendency with occasional fatal bleeding. They are highly malignant and disseminate rapidly. In the differential diagnosis of malignant tumors, it is well to remember that the initial nodules do not resemble syphilitic lesions, although syphilis has always to be considered in differential diagnosis. One must exclude syphilitic chancre, chancroid, secondary and tertiary syphilitic ulcers, lymphogranuloma venereum, tuberculosis and all ulcerative and indurative lesions of the vulva. Biopsy is imperative in the presence of a suspicious lesion. A careful inspection of the regional lymph glands is also required.
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Tenderness may be due to secondary infection of a malignant ulcer. Absence of glandular enlargement by no means indicates that the malignant process is necessarily still localized. Even in advanced carcinoma of the vulva, large compact masses of lymph glands may escape detection in cases in which autopsy has revealed metastatic dissemination. The recommended treatment for proven malignancy is radical surgery, i.e., vulvectomy with radical removal of all superficial and deep inguinal and subinguinal glands. In many cases, pelvic lymphadenectomy is also indicated. Roentgen or radium therapy will not in themselves suffice. Roentgenotherapy is recommended for postoperative or palliative treatment. REFERENCES AlIen, A. C. and Spitz, S.: Cancer 6: 1, 1953. CalIomon, F. T. and Wilson, J. F.: The Nonvenereal Diseases of the Genitals. Charles C Thomas, Springfield, Ill., 1955. 2013 Delancey Place Philadelphia 3, Pennsylvania
PROMPT diagnosis and treatment of cutaneous diseases of the vulva are of special importance, owing to the peculiar nature of the skin in this region. Conditions of heat, moisture and friction, together with diminished aeration, periodic changes in blood supply during menstruation and pregnancy, as well as more pronounced senile changes, serve to alter the ordinary aspect of various skin diseases, to prolong their course and to render them susceptible to unusual complications and malignant degeneration. A brief review of certain practical aspects of the problem and of recent therapeutic advances follows.
PYOGENIC INFECTIONS
The advent of the sulfonamides and the antibiotics has so reduced the incidence of carbuncle and erysipelas that they are now considered rare and much less formidable than they were some years ago, but the vulvar region is still a site of predilection for such conditions as impetigo, pyoderma, ecthyma and folliculitis (Fig. 260). Pyogenic granuloma appears as a smooth, pedunculated tumor of a pink to dark red color, which bleeds easily, leaving a fine brownish or blackish crust. Biopsy will exclude malignant transformation. In chronic, recurring hidroadenitis suppurativa (Fig. 261), the infection attacks the apocrine glands and red, tender, swollen nodules slowly erode to the surface. Differential diagnosis of the pyogenic infections of the vulvar region will include consideration of herpetic lesions, eczematous eruptions, drug eruptions, pemphigus and syphilitic lesions. At present the routine treatment recommended for pyogenic infections of the vulvar region is the application of neomycin ointment. It should be kept in mind, however, that the less expensive 3 per cent ammoniated mercury ointment suffices in many cases. Should ointment treatment lead to development of grease folliculitis, a neomycin lotion may be applied during the day, reserving antibiotic ointment therapy for night use. Hidroadenitis suppurativa, which formerly persisted for years, also
* Assistant Professor of Dermatology and Syphilology, JefJerson Medical College; Visiting Lecturer in Dermatology, Graduate School of Medicine, University of Pennsylvania; Chief Dermatologi8t, Misericordia and Presbyterian Hospitals, Philadelphia. 1741
1742
John F. Wilson
Fig. 260. Pustular folliculitis in obese patient.
Fig. 261. Chronic hidroadenitis suppurativa (note scar tissue with sinus tracts and comedones).
responds well to antibiotic treatment. Following isolation of:the offending organism, the sensitivity of the latter to antibiotics is established and the appropriate parenteral antibiotic may then be administered for several months.
Cutaneous Diseases of the Vulva FUNGOUS INFECTIONS
Numerous fungi flourish as saprophytes in the vulvar region, but heat, sweat and friction frequently transforms them into virulent pathogenic organisms. Superimposed acute intertrigo will activate dormant mycoses. The special properties of the offending fungus, the local and general predisposition of the skin in the individual patient, as well as lowered resistance due to other conditions, will determine the cutaneous manifestations and course of thE> infection. There is always a distinct tendency toward temporary disappearance followed by recurrence. Tinea cruri8 must be distinguished from other superficial dermatomycoses, seborrheic dermatitis and some circinate forms of psoriasis and syphilis, as well as from other fungous infections. A papulovesicular margin and a tendency to increase in size distinguish the lesions of tinea
Fig. 262. Erythrasma. Note superficial changes with very low-grade inflammatory reaction. A, Vulva; E, axilla of same patient.
cruris, while circular psoriasis presents a lamellar desquamation and a more reddish color. Psoriatic lesions are likely to appear also on other parts of the body. The circinate and annular syphilomas have an indurated border and a more brownish hue. In treatment of the dermatomycoses, various fungicides have been found of value. Ointments containing undecylenic acid, salicylic acid (3 per cent) or sulfur (5 per cent) are recommended. Once the lesions have disappeared, salicylic acid (3 per cent) and resorcinol (5 per cent) in aqueous or alcoholic solution may prevent otherwise frequent recurrences. In more superficial tinea, such as erythra8ma (Fig. 262), ointments containing 2 to 3 per cent salicylic acid are likewise recommended. Moniliasis is caused by the most common saprophyte of the normal .vagina and vulva, namely Candida (Monilia) albicans. Here, too, there is the problem of individual susceptibility, local predisposition, the effects of wet compresses, cold bandages or prolonged baths, as well as genera.l disposition due to lowered resistance owing to various affections includ-
John F. Wilson
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ing obesity and diabetes. Moniliasis may occur as a conjugal infection or from infection by douche nozzles and is very common in advanced stages of pregnancy or associated with diabetes. In moniliasis, it is important to direct proper treatment to a coexisting diabetes in order to prevent relapse of the eruption. One per cent aqueous gentian violet solution is of great value. PEDICULOSIS PUBIS
Pediculosis pubis (phthiriasis pubis) is caused by infestation of the pubic region with the crab louse. Itching followed by scratching results in excoriations and follicular dermatitis or pustular eruptions due to secondary infections. Also attempts at self-treatment with insecticides or mercury ointment may cause a dermatitis. Blue spots appear due to the bluish dye excreted by the parasite and help to distinguish pediculosis from the red syphilitic roseola. In treatment of pediculosis pubis, most antiscabetic drugs will prove effective, and, in particular, benzyl benzoate emulsion. VIRAL INFECTIONS
Cutaneous affections of the vulva due to filtrable viruses include common warts, molluscum contagiosum, herpetic eruptions and vaccinia. COIllIllon Warts
Common warts (condyloma acuminatum, Fig. 263) are benign, infectious tumors of viral origin. The vulvar region presents a site of predilection for their development. They are not of venereal origin, but may coincide with venereal disease. They vary in size from that of a pin head to that i)f a strawberry or fist, and coalescing lesions may resemble a cock's comb or cauliflower. When macerated owing to sweat, heat or friction, they emit a malodorous secretion, and rapid growth has been noted during pregnancy. They do not resemble the flat syphilitic condyloma in which the Treponema pallidum may be demonstrated, and which are associated with enlarged inguinal glands. In spite of a clinically and histologically benign appearance, warts may mask the onset of malignancy at a time when even biopsy is negative. Podophyllin is the treatment of choice for these warts, and podophyllin (25 per cent) in mineral oil has proved almost constantly effective after one or two applications. Podophyllin (25 per cent) in Sandrac varnish dries quickly and permits better control of medication, showing blanching of the lesions within a few hours, sloughing by the second or third day and complete disappearance in four days. When one wart disappears, others frequently follow suit. Herpetic Eruptions
These include herpes simplex, herpes progenitalis, herpes menstrualis and herpes zoster. The differentiation of herpes simplex from herpes zoster depends on the same criteria valid for other regions.
Cutaneous Diseases of the Vulva Herpes progenitalis is usually painless and is found most frequently on the labia majora, less frequently on the labia minora and clitoris. Healing occurs in about six days, leaving no scars. Injudicial treatment will occasionally give rise to secondary infection that may suggest venereal disease, and thus complicate the diagnostic problem. Early stages of lymphogranuloma venereum not infrequently suggest herpes progenitalis, but ca~ be differentiated within a short time, owing to their rapid spread to the lymph nodes and the appearance of the inguinal bubo. In herpes simplex, a whole series of vulvar skin affections must be excluded, such as eczematous eruptions, drug erup-
Fig. 263. Condylomata acuminata. Patient is 6 months pregnant. (Wharton: Gynecology, 2nd Ed.)
tions, ulcus vulvae acutum (Lipschutz) and aphthous ulcers. The distribution of the vesicles and rapid course of herpes simplex serve to distinguish the latter from venereal infections. In cases of uncomplicated herpes progenitalis, simple treatment with mild dusting powders such as boric acid powder, and interposition of a piece of lint as protection against friction in certain areas will suffice. One per cent hydrocortisone ointment is sometimes of value. In cases of persistently recurrent herpes simplex, some success has been achieved by administration of smallpox vaccine at intervals of six to twelve weeks. NEURODERMATITIS
The incidence of neurodermatitis is increasing with the strain and stress of modern living. Depletion of nerve energy beyond a given level
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results in irritation of the nerve endings in the skin, and the usual sequence of itching, scratching and inflammation may in the long run completely incapacitate the patient for her ordinary pursuits. With the superimposition of phobias related to malignancy, loss of position or of friends, or other wholly imaginary situations, a vicious circle is established, in which any nervous upset may act as a trigger mechanism resulting in a cutaneous reaction. Neurodermatitides occur not only in neurotically unstable individuals, but frequently in normal subjects.)This becomes more comprehensible when one takes into consideration that the blood vessels of the skin, the sweat glands and the pilomotor muscles
Fig. 264. Localized neurodermatitis. Note thickening of vulva.
are all supplied with sympathetic innervation, and that not only physical but psychogenic processes affect sweat secretion and blood supply of the genital area. Pruritus Vulvae
Although rather a symptom than a disease, itching of the vulva presents many problems from the etiologic, clinical; diagnostic and therapeutic point of view. For practical purposes it can be considered to be a form of localized neurodermatitis. In most instances, the skin of the vulva appears entirely normal. However, when changes in the skin occur there may be oozing and crusting in the acute stage, followed by the dry and scaly appearance in the subacute stage, and this, in turn, by the lichenification characteristic of the chronic stage. From the etiologic standpoint, innumerable psychosomatic conditions have been considered. Pruritus vulvae very rarely affects little girls, but is more common in older women, especially following the menopause. Where all other
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causes for itching in this region have been eliminated (contact dermatitis, seborrheic dermatitis, etc.), psychogenic irritation of the cutaneous nerve endings may be considered. Treatment includes removal of possible sources of irritation, antipruritic medication and psychotherapy, including mental and physical rest. During the acute stage of localized neurodermatitis, compresses of boric acid or Burow's solution may be alternated with application of mild lotions, such as calamine lotion. Calamine lotion and zinc paste are helpful during the subacute stage, and tar ointment is recommended for the chronic stage, but must not be applied in the hairy regions. Insomnia may be relieved by hypnotics. Tranquillizing medication (Reserpine or Thorazine) during the day is sometimes helpful. Rest, change of scene and change of usual occupations will often yield relief, but in some instances, psychotherapy is necessary. One of the newer remedies, hydrocortisone ointment (1 to 2.5 per cent) has proved successful in cases that have failed to respond to other treatments. Vulvectomy and nerve block, as "cures" for pruritus, are strongly contraindicated. ECZEMATOUS DERMATITIS
In this condition, the acute stage is characterized by erythema, edema, the appearance of vesicles, oozing and crusting. In the subacute stage erythema and edema are diminished, and the skin becomes dry and scaly. The chronic stage is distinguished by lichenification with papules and scaling, accentuation of the normal skin folds (shark-skin appearance) from thickening due to cellular infiltration. Eczematous dermatitis is of varied etiology and constitutCils the cutaneous reaction to irritants. It may represent a contact dermatitis, developing from contact with irritants oontained in clothing, toilet tissue, condoms, douches, contraceptives, nail polish, perfumes, deodorants or hygienic pads, the lacquer or plastie resins in toilet seats and many other things. A careful review of the patient's history will usually disclose the cause, and cure follows removal of the offending agent. Local soothing applications are indicated. INTERTRIGO
Intertrigo, due to irritation of apposing surfaces by heat, moisture and friction, presents a primary erythema, followed by maceration, erosion, exudation, itching and burning, particularly in obese persons, and as a result of walking, especially in hot weather (Fig. 265). Eventually, fissures and verrucous lesions may develop. Anamnesis will disclose the appearance in extravulvar locations, and in addition will exclude contact dermatitis and neurodermatitis. If intertrigo masks some previous eruption, diagnosis may be possible only after continued observation of the condition and its response to treatment. The latter consists in separation of the parts, dusting with zinc stearate and talc, and bathing with
John F. Wilson a lotion containing 5 per cent zinc oxide in liquor calcis. Adequate aeration of the region must be provided for with special garments to prevent friction when walking and the patient should be advised to reduce if obese. SEBORRHEIC DERMATITIS
This affection is of ~J1lmown etiology and is characterized by the development of greasy yellow scales, occasionally leading to lichenification and now and then showing exacerbations with constitutional conditions or emotional upsets. Diagnosis may prove difficult if the lesions happen to be located exclusively in the vulvar region, and if eczematization or intertrigo is present. Here, too, continued observation of the dermatitis and its response to treatment may offer diagnostic clues. In the acute stage, moist compresses and lotions will afford relief, and when the acute-
Fig. 265. Intertrigo in obese patient. A, Vulva; B, anal region; C, inframammary region.
ness has subsided somewhat, sulfur ointment in the form of a zinc paste containing 6 per cent precipitated sulfur is helpful in association with large doses of vitamin B complex, general tonic treatment and attention to possible concomitant constitutional disease. PSORIASIS
Whereas psoriasis (Figs. 266, 267) usually affects other parts of the body, it is occasionally limited to the vulvar region. However, friction, injudicious treatment, or the superimposition of other dermatoses may complicate diagnosis. Inflammation of the lesions as a result of irritation may lead to formation of fissures with whitish margins or to diffuse vulvitis. Superinfection with syphilis may convert the psoriatic patches into diffuse syphilitic infiltrations, or epithelioma may develop in these patches following prolonged arsenotherapy. If the lesions are exclusively vulvar, diagnosis may be difficult, as is also true in cases without much scaling or erythema. Finding of lesions elsewhere on the body, where conditions leS:d to a more typical development, are of diagnostic aid.
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Fig. 266. Psoriasis of seborrheic type.
Fig. 267. Psoriasis in patient with pruritus vulvae, showing Koebner's phenomenon with outbreak of psoriasis as a result of scratching. (From Fritz T. CaIJomon and John F. Wilson, The Nonvenereal Diseases of the Genitals, 1955. Courtesy of Charles C Thomas, Publisher, Springfield, Illinois.) KRAUROSIS AND LEUKOPLAKIA VULVAE
Kraurosis of the vulva develops in women after the menopause, or in younger women following ovariectomy. Kraurosis has been described as an exaggeration of normal postmenopausal atrophy. The labia and cli':
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Fig. 268. Chronic and atrophic leukoplakic vulvitis. (From Fritz T. Callomon and John F. Wilson, The N onvenereal Diseases of the Genitals, 1955. Courtesy of Charles C Thomas, Publisher, Springfield, Illinois.)
Fig. 269. Leukoplakia.
toris diminish in size and disappear. Sexual intercourse may lead to fissures and dyspareunia. The mucosa is dry and smooth, the affected areas appearing whitish or yellowish. The skin of the vulva becomes thinner and may be discolored with deposits of pigment. The pubic hair is sparse and the patient is afflicted with unbearable pruritus and often 'with painful micturition.
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Leukoplakia is characterized by the appearance of one or more circumscribed, slightly elevated, milky or bluish-white, later shiny, silvery patches that result from epithelial proliferation and keratinization and are frequently the source of erosions and ulcer formation. (See Figs. 268,269.) Kraurosis and leukoplakia are not infrequently associated (in about 50 per cent of cases). Malignant degeneration of leukoplakia is very common. It is stated that about 50 per cent of all cases of vulvar carcinoma have their origin in leukoplakia. Kraurosis, on the other hand, is not known to undergo malignant degeneration. Where kraurosis and leukoplakia exist together, it is the latter that provides the starting point for cancerous growths. Kraurosis is characterized by atrophic processes from the beginning, whereas in leukoplakia the tendency is toward hyperplasia and hypertrophy. Kraurosis bears some striking resemblances to lichen sclerosus et atrophicans. The secondary changes due to rubbing and scratching of neurodermatitis must be differentiated from leukoplakia. Whitish areas of localized neurodermatitis may disappear following application of 1 per cent hydrocortisone ointment and thus do away with the necessity for radical surgery necessary in true leukoplakia. In kraurosis vulvae, estrogen treatment, orally, parenterally or locally as an ointment, is definitely indicated, but is not always effective. Itching does tend to subside. Roentgen therapy for itching of the vulva that fails to respond to other treatment is often recommended, but is hardly to be prescribed for kraurosis. Also vulvectomy as a radical measure to relieve pruritus is not always completely successful and is most mutilating. In leukoplakia, on the other hand, the possibility of malignant transformation leads to a demand for early vulvectomy. However, when only small lesions are present, biopsy followed by electrocoagulation may suffice. Biopsy should be repeated at frequent intervals to be sure that malignant degeneration is not in process. BENIGN NEOPLASMS
Located on the labium majora, fibroma develop as subepidermal, firm, smooth nodules. They are at first hardly discernible but show a marked growth tendency during menstruation and pregnancy. Finally a pedicle is formed and the tumor may hang down between the thighs to be irritated by friction, sweat and urine, with final inflammation, erosion and ulceration. Cystic or myxomatous degeneration may eventually take place, and if not excised in time, malignant degeneration may result. Benign lipomas of the vulva grow very slowly, as a rule, to reach the size of an egg or a man's fist, but some lipomas show a marked growth tendency.
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Other benign tumors of the vulva include neurofibroma, lymphangioma, the tiny hemangiomas of hereditary telangiectasis, congenital cysts, follicular retention cysts, myomatous cysts, supernumerary mammary tissue and pigmented nevi. Melanotic nevi should be excised as early as possible to prevent malignant degeneration. Hemangiomas and lymphangiomas may be subjected to refrigeration with solid carbon dioxide or be injected with sclerosing solutions. Radium therapy is also recommended. MALIGNANT NEOPLASMS
In the present article, we are chiefly concerned with primary malignant neoplasms of the vulva and, in: particular, with the various forms of epithelioma. The initial lesion is usually a painless nodule or erosion and is rarely accorded attention. When chronic genital ulcers resist treatment, the fear of malignancy is engendered, as the laity is well informed as to the possibilities of malignant degeneration of chronic lesions. It must be emphasized that so-called precancerous lesions do not always become malignant. There are indefinite transition stages and clinical and histologic findings may clash. Many dermatologists refer to lesions that customarily undergo malignant transformation as malignant in fact. SUGh conditions include Bowen's and Paget's disease of the vulva, as well as leukoplakia. Cancer may also develop from chronic inflammatory lesions of most varied origin. Bowen's disease appears as solitary or multiple, irregular, red,hyperkeratotic patches. These lesions may become crusted or scaly and show no tendency to progress for long periods of time. Squamous cell carcinoma will develop in about 25 per cent of the cases, and immediate removal is recommended. This is true also in the case of Paget's disease. Removal of the regional glands is indicated only when they are definitely involved in the process. However, following removal of a single lesion of Bowen's disease, other unrelated lesions may appear elsewhere in the genital region. Basal cell epithelioma grows very slowly with a tendency toward central ulceration and formation of a firmly adherent crust. It has been estimated that about 20 per cent of all clinically diagnosed basal cell epitheliomas are transitional forms between basal and squamous cell epithelioma. Malignant melanoma of the genital region occurs more frequently in males than in females, in whom it develops around the age of 50 years. It is interesting that AlIen and Spitz, in a review of 934 cases of malignant melanoma, found the highest incidence of primary malignant melanoma on the soles of the feet, the mucosa of the female genitals and on the head and neck. It is calculated that about two-thirds of these neoplasms have their origin in pigmented nevi, and in many cases the
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presence of such a lesion prior to malignant degeneration was probably overlooked by the patient. This tumor has been found to develop from embryonic nevus cells in the skin, remaining quiescent for years, only to undergo malignant transformation later in response to some stimulus. It is even possible that such tumors may arise from the normal skin, but in this area an accurate history is most difficult to obtain. In the absence of clinically noticed nevi, malignant melanoma begins as a solitary, blue-black or brownish black flat or raised nodule, and rapidly forms a solid black tumor mass. This tumor must be differentiated from benign pigmented nevus, verruca senilis and angiomatous
Fig. 270. Advanced carcinoma of vulva. (Novak: Gynecologic and Obstetric Pathology, 3rd Ed.) .
tumors. In the latter tumors, excision is imperative with the slightest sign of growth. In fact, any pigmented nevus in the genital region exposed to the irritating conditions of this area should be removed. Even following radical excision of the tumor, including the regional lymph glands, prognosis is poor. Squamous cell carcinoma of the vulva is very rare as compared to carcinoma of the uterus, the ratio being reported as probably 1: 35 to 40. Only 1 to 3 per cent of carcinomas of the female genitals affect the vulva. Vulvar carcinoma usually develops during the sixth or seventh decade of life and is seen much less frequently in other decades, although isolated cases in younger women and especially carcinoma of the clitoris in women under 40 years of age have been observed.
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This neoplasm usually begins in the mucosa of the vulva and not infrequently has its origin in a pre-existing pigmented nevus or in a patch of leukoplakia. The initial lesion appears on the inner aspect of the labia majora, and occasionally on the labia minora, or in the region of the urethral meatus where the squamous epithelium of the cutis and the cylindrical epithelium of the mucosa come together. The physician is not usually called until itching, soreness or the presence of a nodule has been observed by the patient. Upon examination, the physician finds a nodule, possibly of the size of a walnut, or a more diffuse nodular infiltration, movable under the skin or mucosa. Shortly, ulceration sets in with liberation of a purulent or bloody discharge. In other instances, a papillary growth may give rise to a cauliflower-like structure, suggesting condyloma acuminatum. Especially in younger women, vulvar carcinoma runs a rapid course. The clinical forms include a fungating and an infiltrating form. Carcinoma of the clitoris and urethral area spread very rapidly, owing to the rich blood and lymph supply of the region. The inguinal and subinguinal glands are involved early. In carcinoma of the clitoris, the patient rarely survives more than 15 to 18 months. Metastasis from vulvar carcinoma occurs early and the lymph glands may be involved, although they appear unchanged. Primary sarcoma of the vulvar region is very rare and usually is located on the labia majora, appearing first as a solid nodule beneath the movable skin. It grows rapidly and hemorrhages and necrosis soon transform its appearance. The primary nodule may remain stationary for a long period before growth suddenly ensues. Sarcoma may develop in pre-existing fibrolipoma or fibroma of the vulva and may be of any type or mixed. A ngioblastomas are rarely seen in this region. Metastatic tumors from primary tumors elsewhere in the body may involve the vulva. Metastatic chorionepithelioma of the vulva is a rare tumor giving a positive pregnancy test. It may appear as a single tumor or as several pea- to hazel-nut sized tumors of a dark, reddish blue to black color. These lesions may resemble a circumscribed hematoma or thrombotic varix. They show a marked hemorrhagic tendency with occasional fatal bleeding. They are highly malignant and disseminate rapidly. In the differential diagnosis of malignant tumors, it is well to remember that the initial nodules do not resemble syphilitic lesions, although syphilis has always to be considered in differential diagnosis. One must exclude syphilitic chancre, chancroid, secondary and tertiary syphilitic ulcers, lymphogranuloma venereum, tuberculosis and all ulcerative and indurative lesions of the vulva. Biopsy is imperative in the presence of a suspicious lesion. A careful inspection of the regional lymph glands is also required.
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Tenderness may be due to secondary infection of a malignant ulcer. Absence of glandular enlargement by no means indicates that the malignant process is necessarily still localized. Even in advanced carcinoma of the vulva, large compact masses of lymph glands may escape detection in cases in which autopsy has revealed metastatic dissemination. The recommended treatment for proven malignancy is radical surgery, i.e., vulvectomy with radical removal of all superficial and deep inguinal and subinguinal glands. In many cases, pelvic lymphadenectomy is also indicated. Roentgen or radium therapy will not in themselves suffice. Roentgenotherapy is recommended for postoperative or palliative treatment. REFERENCES AlIen, A. C. and Spitz, S.: Cancer 6: 1, 1953. CalIomon, F. T. and Wilson, J. F.: The Nonvenereal Diseases of the Genitals. Charles C Thomas, Springfield, Ill., 1955. 2013 Delancey Place Philadelphia 3, Pennsylvania