Special Issues in Genital Dermatology: Psychosexual Matters, Concerns of Immunosuppression, and Aging
Peter Lynch
Libby Edwards
PSYCHOLOGICAL ISSUES
Although the situation is improving slowly, discussion about genital disorders and their role in the patient’s psychological, social, and sexual function has historically been indirect, played down, or even avoided completely. In order to maximize the quality of care offered, these aspects need to be explored with virtually every patient presenting with a genital problem. There are two ways of doing so. One is to offer patients a chance to express these concerns indirectly by way of a computerized questionnaire filled out privately by the patient prior to a face-to-face interaction with the clinician. The other is for the clinician to take the initiative in bringing up this discussion at the time of the examination. Rarely, with individuals, who by culture or temperament are very shy, it may be appropriate to defer this discussion until the second or third visit. Because of space limitations only a few aspects or psychosocial and sexual function related to genital disease can be covered in this chapter.
Psychological factors obviously can play some role in the pathophysiology of all diseases and the degree to which they do so probably exists on a spectrum. Unfortunately, for most diseases, there is no consensus as to what these factors are and how important a role they play in the etiology, extent, severity, and duration of the problem. In addition, there is always controversy over the degree to which psychological factors cause the disease versus the degree to which the disease causes the psychological factors. Essentially this presents a “chicken and egg” situation that, while it can be discussed, can never be resolved to the satisfaction of all participants. With this in mind, I have divided this chapter into three segments: (1) psychosocial and sexual dysfunction may cause the disease, (2) psychosocial and sexual dysfunction may influence the course of the disease, and (3) psychosocial and sexual dysfunction may occur as a result of the disease.
PSYCHOSOCIAL DYSFUNCTION MAY CAUSE DISEASE
The major genital disorders for which I believe psychosocial and sexual dysfunction plays a significant etiologic role are (1) chronic idiopathic genital pain, (2) chronic itching in the absence of a recognizable disease, (3) chronic scratching or gouging occurring in the absence of a recognizable disease, (4) fixed ideation that some aspect of the genitalia, although normal on clinical examination, is in fact abnormal (body dysmorphic disorder), and (5) intentional or unintentional self-mutilation (dermatitis artefacta).
Chronic Idiopathic Genital Pain
Mucocutaneous pain can arise secondary to an underlying cutaneous or neurologic disorder or occur as an idiopathic problem. The main idiopathic mucocutaneus pain disorders include unexplained pain involving the head (tongue, lips, face, and scalp) and the anogenital area (vulva, penis, scrotum, and anus). Idiopathic pain that occurs at these latter four sites is generally termed vulvodynia, penodynia, scrotodynia, and anodynia. Of these, only vulvodynia has been studied sufficiently to warrant discussion here. Nevertheless, one of us (PJL) believes that the information regarding vulvodynia, as cited below, can be generalized to pain occurring at the three other sites of involvement.
Nearly all patients with vulvodynia have psychological, social, and sexual dysfunction, although the degree to which this occurs is quite variable (1,2,3 and 4). The major question, of course, is whether the presence of pain causes this dysfunction or whether the dysfunction causes the pain. While the majority of clinicians currently favors the former explanation, the minority including one of us (PJL) favors the latter. Support for this latter point of view is perhaps best established by examination of the data suggesting that psychosocial and/or sexual dysfunction precedes the development of the pain. In that regard, there is arguably
acceptable evidence that significant depression, anxiety, somatization, relationship dysfunction, and painful physical, sexual, or psychological trauma precede the development of vulvar pain (4). Depending on the degree to which these data are correct and acceptable, vulvodynia might then be considered as a somatoform disorder (4).
acceptable evidence that significant depression, anxiety, somatization, relationship dysfunction, and painful physical, sexual, or psychological trauma precede the development of vulvar pain (4). Depending on the degree to which these data are correct and acceptable, vulvodynia might then be considered as a somatoform disorder (4).
Regardless of whether psychosocial and sexual dysfunction either causes the pain or arises from it, all agree that vulvodynia is a very debilitating condition and that it has a dramatic adverse effect on quality of life (QOL) (5). In fact, it appears that women with this condition have significantly poorer QOL than do women with most other general dermatologic diseases and also worse than women with other vulvar disorders (6). Finally, while both medical and procedural therapy can lead to good results for most patients with vulvodynia, it is clear that these individuals can also benefit from a variety of psychological approaches to therapy (7).
Chronic Itching in the Absence of Recognizable Disease
As is true for cutaneous pain, pruritus can arise either as an idiopathic process or develop secondary to an underlying systemic, cutaneous, or neurologic (neuropathic) disorder. Idiopathic pruritus, often termed psychogenic pruritus or pruritus sine materia, is associated with a variety of psychogenic problems, notably anxiety and depression (8,9). It, also, like chronic idiopathic pain, can be classified as a somatoform disorder (10). The fact that psychogenic pruritus responds very well to psychotropic medications such as the tricyclics, benzodiazepines, SSRIs (selective serotonin reuptake inhibitors), and antipsychotic agents supports the supposition that this form of itching is primarily caused by psychological dysfunction (11).
Chronic Scratching and Gouging in the Absence of Recognizable Disease
Psychogenic excoriation (“neurotic excoriation”) is the term used for those patients who chronically scratch, gouge or pick at skin that is otherwise visibly normal. This condition differs from the scratching and rubbing that occurs in pruritic skin disorders such as atopic dermatitis and lichen simplex chronicus in several ways. First, there is no atopy or other recognizable underlying disorder. Second, the excoriations are appreciably deeper and thus usually appear as ulcers rather than erosions. Third, individual gouge marks are separated one from another by areas of intervening normal skin. And, fourth, the scratching and picking is often not preceded by a sensation of itching but rather by the perception that there is something in the skin and that its presence requires removal by the patient.
Essentially all patients with the milder forms of psychogenic excoriation have underlying psychological dysfunction, primarily related to depression or bipolar disorder (12). In addition, most of these patients meet the criteria for impulse-control disorder or obsessive-compulsive disorder (12). This type of psychogenic excoriation is one of the commonest forms of psychocutaneous disorders (13) and often involves the genitalia. These patients can be considered to have a somatoform disorder.
On the other hand, patients with the more severe form of psychogenic excoriation, those who believe that their skin contains “bugs” (“delusions of parasitosis”) or fibers (“Morgellons disease”) are almost always delusional (14,15). These latter two conditions (which are essentially identical from a psychological viewpoint) only occur on the genitalia when other parts of the body are also involved.
All forms of psychogenic excoriation may be associated with the use of recreational drugs, and evidence for this possibility should be sought before ascribing the problems solely to a psychiatric etiology (12). Psychotropic agents, to include the SSRIs and antipsychotics, are useful for the milder forms and are required for the more severe forms of psychogenic excoriation (14,15 and 16). Additional information on this subject can be found in the section on self-induced ulcers in Chapter 10.
Body Dysmorphic Disorder
Body dysmorphic disorder (BDD) is defined as preoccupation with some slight, or nonexistent, defect in appearance that causes significant distress resulting in psychosocial or sexual dysfunction. BDD is quite common with an overall prevalence of 1% to 2% of the general population and about 5% of the patients seen in dermatology practices (17). BDD is classified with the somatoform disorders, but in those instances where no defect whatsoever is present, patients may be delusional. The severity of preoccupation with the perceived defect is generally at the level of that found in patients with obsessive-compulsive disorder and, in fact, these two conditions frequently coexist (18).
Not surprisingly, preoccupation with minor or imagined defects most often involves the face, head, and hair but it can also involve the genitalia (17). The level of preoccupation may be relatively mild and only obsessive in nature or it may be more severe, representing fully developed BDD. Preoccupation regarding the genitalia most often revolves around size or color.
In terms of preoccupation with size, men not surprisingly, most often focus on the penis perceiving that their normal size penis is too small (“small penis syndrome”) (19,20). On the other hand, in women it is usually the misperception that their labia minora are too large or too asymmetric (21,22). Penile concerns in men have resulted in a huge industry involving the use of nonprescription medications advertised as “guaranteed” to result in penile enlargement. Similarly, misperception about labia size or asymmetry has resulted in a booming business for female genital cosmetic surgery (23).
There is very little published about preoccupation with genital color. However, a search of the Internet reveals a considerable level of concern on the part of women regarding the perception that their external genitalia (primarily the labia majora) or anal area are too darkly pigmented. Because of this concern, many products and services are offered for genital and anal bleaching. In addition, many women who develop vestibular pain examine the vulvar vestibule and perceive that the color is abnormally red. This perception may be reinforced by clinicians who on examination confirm the presence of “excessive” redness. This “excessive” redness is then perceived as an inflammatory process responsible for the occurrence of vestibular pain. This led to use of the term “vulvar vestibulitis.” However, several studies have demonstrated that normal, asymptomatic women, with entirely normal biopsies, frequently have a similar degree of vestibular redness. This suggests that the red color is a normal finding unrelated to inflammation and the development of pain (24). In recognition of this information, the ISSVD has recommended that the term “vestibulodynia” replace the term “vestibulitis” (24).
A similar situation also occurs in men. A small number of men develop idiopathic scrotal skin pain and on selfexamination, they perceive that excessive redness is present. They then believe that the redness is abnormal and that it is directly related to the development of their pain. This association may be supported by clinicians who are unfamiliar with genital skin color. The severity of preoccupation with this redness often reaches the level of BDD. However, invariably examination by experienced clinicians reveals that the redness is within the normal variability of scrotal wall color and that there is no local pathology present. Very little has been written about this “red scrotum syndrome” (25), but we have seen at least 20 such patients suggesting that it is appreciably more common than the medical literature would suggest.
Self-mutilation (Dermatitis Artefacta, Factitial Dermatitis)
Self-mutilation involving the skin is an uncommon condition in which individuals knowingly and repeatedly damage the skin through burning, cutting, abrasion, chemical application, or other similar behaviors (13,26,27). We exclude those conditions that occur on a one-time basis such as tattooing and skin piercing. Self-mutilation can occur at any age, but some forms, especially cutting, occur most often in adolescents and young adults (27,28). Characteristically, patients strenuously deny that they are doing anything injurious to the skin. Surprisingly often these patients have a background of work in health care (26).
Self-mutilation occurs in two major settings: with malingering, where secondary gain is the driving factor, and in those individuals with moderate to severe psychological impairment, where the behavior is carried out to satisfy an internal, unknown, and unrecognized emotional need. The underlying psychiatric abnormalities present in these individuals are variable but include anxiety, depressive, bipolar, and personality disorders (13).
These traumatically self-induced lesions are fairly easy to recognize although it is extremely difficult to prove that they occur directly as a result of self-induction. A major clue is the observation that they develop only where the patient can reach. The most common sites are the face, arms, and legs, but the genitalia may be involved in a small percentage of cases (27). There are very few publications in the medical literature about genital self-mutilation, but a short search on the Internet suggests that the problem is much more common than physicians believe. Based mostly on anecdotal information, one of us (PJL) has concluded that major forms of mutilation (such as penile self-amputation) are more common in men, whereas less damaging behaviors such as genital cutting are more likely to occur in young women. A dramatic and disturbing example of this latter problem figured prominently in a recent movie, The Piano Teacher.
PSYCHOSOCIAL DYSFUNCTION INFLUENCES THE COURSE OF DISEASE
Psychosocial problems play an important, but not causative, role for many genital disorders. Since this is true of numerous genital diseases, this section will focus on only two examples, atopic dermatitis and psoriasis, wherein psychological factors may play an important role regarding the time of onset, extent, severity, and duration of the disease. The published literature on these two disorders relates almost entirely to generalized forms of the disease, but it is reasonable to expect that when the genital area is involved, there will be even greater psychosocial dysfunction than is described for generalized involvement (29).
Atopic Dermatitis and Lichen Simplex Chronicus
As indicated in Chapter 4, we believe that lichen simplex chronicus represents the localized form of atopic dermatitis, and these two conditions will be treated as a single entity in this section. Atopic dermatitis develops in only a portion of patients who are biologically predisposed to develop the disease through defects (such as filaggrin mutations) in the differentiation of epithelial keratinocytes. Psychological dysfunction appears to be one of the major factors influencing which of these predisposed individuals develop the disease. Often these psychological aspects are present very early in life and frequently occur as a result of a dysfunctional mother-child relationship (30). Other sorts of psychosocial dysfunction associated with atopic dermatitis may develop in childhood and young adult life (31).
For adults, several studies of patients with atopic dermatitis reveal increased levels of both state and trait anxiety (32,33). This heightened anxiety leads to increased itching that in turn leads to incessant scratching. This then
is responsible for the development of the “itch-scratch cycle,” which characterizes the disorder. In addition, there is evidence that patients with atopic dermatitis have increased levels of depression, somatization, obsession-compulsion, interpersonal sensitivity problems, angerhostility, and dissociation (29,34). In more psychoanalytic terms, atopic dermatitis patients are often described as irritable, resentful, guilt-ridden, and hostile (31). In what might be called a “proof of concept” observation, the significant improvement in lesional severity, itch intensity, and scratch activity that can be obtained by way of psychological intervention demonstrates the high level of importance that psychological factors play in the development of atopic dermatitis and lichen simplex chronicus (35).
is responsible for the development of the “itch-scratch cycle,” which characterizes the disorder. In addition, there is evidence that patients with atopic dermatitis have increased levels of depression, somatization, obsession-compulsion, interpersonal sensitivity problems, angerhostility, and dissociation (29,34). In more psychoanalytic terms, atopic dermatitis patients are often described as irritable, resentful, guilt-ridden, and hostile (31). In what might be called a “proof of concept” observation, the significant improvement in lesional severity, itch intensity, and scratch activity that can be obtained by way of psychological intervention demonstrates the high level of importance that psychological factors play in the development of atopic dermatitis and lichen simplex chronicus (35).
Psoriasis
It is clear that there is a genetic, biologic predisposition for the development of psoriasis. But, as for atopic dermatitis, psychological factors appear to influence the time of lesion development, severity of disease, and response to therapy. There is some consensus that high stress levels frequently precede the development and exacerbation of psoriasis (31,36,37). Patients with psoriasis have significantly higher levels of anxiety and depression than control populations (36,38,39), and it is reasonable to believe that heightened stress plays a role in the development of the abnormal levels of anxiety and depression that are so regularly found in patients with psoriasis (38). Moreover, men with psoriasis consume more alcohol, and patients of both genders are more likely to smoke than do controls (40,41 and 42). These behavioral events are also likely to be associated with the noted anxiety and depression (43).
Patients with psoriasis are also appreciably more likely to exhibit alexithymia (an inability to understand, process, or describe emotions), and it is possible that this personality trait plays a role in the development of their disease (36,44). Lastly, psoriatic patients are found to have higher than normal levels of stigmatization and social avoidance/attachment and tend to perceive that social support is lacking (36,37). These findings however probably occur as a result, rather than as a cause, for their disease.
PSYCHOSOCIAL DYSFUNCTION OCCURS AS A RESULT OF DISEASE
Poor health always has a detrimental effect on patients’ QOL. The magnitude of this detrimental effect has been primarily determined through the use of questionnaire surveys. These have been carried out for a very large number of dermatologic diseases (45,46). Four aspects regarding the results of these QOL studies are worth exploring.
First, dermatologic disorders, compared to significant systemic medical disease, appear to have a disproportionably large detrimental effect on overall QOL. Two studies suggest that the impact of psoriasis on QOL is greater than that for patients with arthritis, cancer, diabetes hypertension, and heart disease (47). For patients with severe psoriasis, more than 7% reported suicidal ideation; this rate was higher than the approximately 3% of general medical patients (47).
Second, skin disease, again when compared with significant medical disease, has a disproportionably large effect on the sexual function aspect of QOL. This is probably self-evident based on the realization that visible, palpable clearly abnormal, skin problems (when compared to “invisible problems such as diabetes and hypertension”) will likely have a very detrimental effect on the patient’s self-image and on the partner’s response in intimate situations. In a recent study, 35% to 70% of patients with psoriasis were reported to have experienced sexual problems because of their psoriasis (48,49). This problem is probably accentuated in patients who have lesions in the genital area (30). Similarly, more than 50% of patients with atopic dermatitis and 30% of their partners indicated that the disease had an adverse effect on their sex lives (48,50).
Third, dermatologic problems occurring on face and in the anogenital area have more impact on QOL than that same disease occurring at other body sites (29,30). As regards disease at a genital site, some of this effect on QOL is directly due to symptoms of pain such as that occuring with genital lichen sclerosus and erosive lichen planus. But, in other instances it is due to high levels of concern by patients and partners about intimacy and sexual function. And, in still other situations, there will be concern about potential contagiousness. This may, of course, be entirely appropriate when conditions like genital warts are present or may be related to ignorance about what is transmissible and what is not. For instance in one study, 15% of the sexual partners of patients with atopic dermatitis, a clearly noncontagious disease, thought that the disease was potentially contagious (50).
Fourth, the assessment of disease severity and its impact on QOL is quite different between patients and clinicians (51). Clinicians very frequently believe that the decrement in QOL due to a given disease is much less than that perceived by patients. This is largely related to the fact that clinicians base their estimate of disease effect on QOL almost entirely on their observation of the extent and severity of the disease without taking into account what even trivial disease may mean to individual patients. The use of patient-completed QOL questionnaires would alleviate this discrepancy between the viewpoint of the patient and the clinician, but, unfortunately, such surveys have almost only been used in a clinical research setting rather than for individual patient encounters. This needs to change as such underestimation of a patient’s perception of disease effect on QOL is demeaning to the patient and will inevitably have an adverse effect on the clinician-patient relationship and possibly also on the patient’s response to therapy.
IMMUNOSUPPRESSION
Patients who are immunosuppressed are more likely not only to develop infections but also to exhibit atypical morphology, more severe disease, and unusual infections. Some malignancies are more common, and malignant tumors invade and metastasize more quickly than in immunocompetent patients. In addition, patients who are immunosuppressed by virtue of infection with the human immunodeficiency virus (HIV) are at risk of developing several inflammatory but noninfectious diseases, most notably psoriasis, Reiter disease, and aphthae.
INFECTIONS
Herpes Simplex Virus Infection (see also Chapter 9)
Genital ulcers in an immunosuppressed patient are most often the result of chronic herpes simplex virus (HSV) infection.
Clinical Manifestations
Chronic HSV can occur in any immunosuppressed patient who has been exposed to this virus. However, this occurs more often in those who are immunosuppressed due to HIV. This is because HSV is more common in individuals with a history of intravenous drug use and with multiple sexual partners, which are also risk factors for HIV. With the availability of highly active antiretroviral therapy, the frequency of chronic HSV ulcerations has decreased remarkably.
Like immunocompetent patients, individuals with chronic HSV describe pain, and careful questioning usually reveals prior, more typical recurrent HSV infection outbreaks. HSV infections in these patients begin as the typical grouped vesicles on an erythematous base, disintegrating almost immediately into well-demarcated, discrete erosions. Unlike immunocompetent patients, immunosuppressed patients often experience nonhealing erosions that coalesce and deepen to form large, well-demarcated, painful, chronic ulcers (Figs. 16-1, 16-2, 16-3 and 16-4). The ulcers may be superficial initially but may become deeper and more poorly demarcated if untreated or secondarily infected. Coinfection with Candida or human papillomavirus (HPV) is common and can change the clinical appearance (Fig. 16-5).
Men most often experience chronic HSV ulcers in a perianal and gluteal cleft distribution as well as on the penis, scrotum, or groin. In the female patient, ulcers may involve the mucous membrane portion of the vulva and may extend to the labia majora, lateral labia minora and even to the crural creases or inner thighs. As in the male patient, HSV infection also often extends to a perianal location and gluteal cleft. Any chronic nonhealing ulcer located on or near a mucocutaneous surface in an immunosuppressed patient is statistically an HSV infection until proven otherwise. Herpes simplex ulcers occasionally are exophytic rather than ulcerative, with prominent elevation above the skin because of prominent fibrin debris and exudate on the surface, and sometimes due to thickened tissue producing a pseudotumor (Fig. 16-6) (52).