A D _ 0 2 3 _ _ _ MA Y 2 5 _ 1 2 . p d f Pa ge 2 3 1 6 / 5 / 1 2 , 2 : 1 1 PM HowtoTreat PULL-OUT SECTION www.australiandoctor.com.au inside COMPLETE HOW TO TREAT QUIZZES ONLINE (www.australiandoctor.com.au/cpd) to earn CPD or PDP points. Investigation Classification of male genital dermatoses Skin diseases affecting the genitals Authors’ case studies Male genital dermatoses The authors DR ADRIAN LIM dermatologist and phlebologist; board member of the Australasian College of Phlebology; director of training of the Australasian College of Dermatologists; consultant dermatologist, Royal North Shore Hospital, Sydney and in private practice in Sydney and Kogarah, NSW. Background GENITAL skin is an extension of the general skin surface and, therefore, shares the usual non-genital-specific skin diseases such as eczema, psoriasis and skin cancer. In addition, the distinct male genital anatomy makes the genitals susceptible to skin diseases not commonly encountered elsewhere. As a sexual organ, the penis is a conduit for many STIs. The psychosexual aspects of male genital skin disease should not be underestimated. It is a frequent source of great emotional and psychosexual morbidity. This article will focus on common skin diseases localised on the male genitalia, as well as less common, but important genital-specific skin conditions. Figure 1: Penis surface anatomy. pubis shaft coronal sulcus glans meatus scrotum Surface anatomy of the male genitalia For the purposes of this article, the term ‘male genitalia’ refers to the penis and scrotum. However, many conditions affecting the male genitalia may also affect the adjacent areas such as the pubic area (superior), groin, genitocrural folds (lateral), perineum and anus (inferior). The penis is the external and pendulous component of the male genitalia (figure 1). It starts from the pubic wall and comprises the body (shaft) that joins the head (glans) distally. The urethra opens as a slit (meatus) at the most distal point of the glans. The dorsal vein of the penis can sometimes be seen running along the dorsal surface. The ventral surface is contiguous with the scrotum. The coronal sulcus is the groove between the glans and the shaft of the penis. The prepuce (foreskin) covers the glans in the uncircumcised flaccid penis. In the circumcised penis, the glans is exposed, and consequently develops a reactive thickening (keratinisation) of the surface epithelium. Male circumcision is indicated as treatment for certain genital conditions such as foreskin stricture (phimosis) and a number of inflammatory conditions of the glans. Male circumcision is also performed for various social and religious indications. There is debate about the ethics and/or justification for the practice of non-medical circumcision. An argument against circumcision is the fact that surface keratinisation post circumcision makes the glans penis less sensitive and supplementary lubricants may be required for sexual intercourse and masturbation. In support of circumcision is evidence that circumcision is protective against a number of inflammatory, neoplastic and infectious penile diseases. cont’d page 25 ASSOCIATE PROFESSOR STEPHEN SHUMACK dermatologist; board member of the Australasian College of Phlebology, Skin and Cancer Foundation Australia, and Epiderm (Australian Dermatology Research and Education Foundation); consultant dermatologist, Royal North Shore Hospital, Sydney; medical director of the Skin and Cancer Foundation Australia; and in private practice in Sydney and Kogarah, NSW. Australian Doctor Online Education Earn 40 Category 1 CPD points by completing our new online interactive modules. Designed to deliver a comprehensive learning experience to help your day-to-day practice. COMPLETE THE LATEST MODULE Nutritional Challenges in General Practice Sponsored by www.australiandoctor.com.au/education www.australiandoctor.com.au 25 May 2012 | Australian Doctor | 23 A D _ 0 2 5 _ _ _ MA Y 2 5 _ 1 2 . p d f Pa ge 2 5 1 6 / 5 / 1 2 , 2 : 1 1 PM Investigation PATIENT history and examination, along with relevant investigations are the cornerstones of managing male genital dermatoses (table 1). Practitioners must show sensitivity and tact when dealing with genital disorders and recognise the frequent link between sexual dysfunction and psychological issues. The guiding principle when dealing with male genital dermatoses is to exclude STI, rule out malignancy and not to neglect the urological system, represented by the penile urethra. History The patient’s age is helpful in assessing likely male genital dermatoses. STIs are more common Table 1: Clinical approaches to male genital dermatoses History Age Circumcision Eczema/psoriasis/ allergies Presenting problem Sexual history Medications Examination Investigation General (non-genital) skin and mucous membrane Genital: penis and scrotum Perineum and anus in men in early adulthood; older males are more prone to skin cancer; and, foreskin-related problems (eg, phimosis) are more common in the paediatric and elderly age groups. Evidence of general skin diseases, such as eczema and psoria- Dermoscopy Swabs Skin scrapings Bloods Biopsy sis, should be sought and a thorough sexual history obtained where relevant. A detailed medication history including a timeline should be established in suspected drug-related genital eruptions. Urogenital symptoms such as dysuria, frequency and urethral discharge may indicate urological disease. ing pubic lice while a dermatoscope is helpful for pigmented lesions. Examination Investigation The genitalia need to be examined closely with particular focus on the penis, foreskin, scrotum, perineum and anus. Foreskin pathology such as phimosis should be confirmed. Any genital rash or eruption should be noted with reference to morphology, site and distribution. Non-genital skin including oral mucosa (blistering disorders), hair-bearing areas (lice) and nails (psoriasis, lichen planus) should also be examined. The perineum and anus should be examined for pathology such as lichen sclerosus. Visual aids such as a magnifying lens can assist greatly in visualis- Bacterial and viral swabs should be taken from any ulcers and discharge, and should then be processed for microscopy, culture and PCR that looks for herpes, chlamydia or gonorrhoea. Scrapings for microscopy can confirm fungal infections and can sometimes confirm scabies. Additional STI screening with serology for syphilis, viral hepatitis and HIV may be indicated. A skin biopsy may be necessary to establish the diagnosis, however, this is generally performed after specialist opinion, given the sensitive location. Classification of male genital dermatoses SKIN diseases of the male genitalia can be broadly grouped into the following categories (table 2): • Normal anatomical variants. • STIs. • Non-genital-specific skin diseases. • Skin diseases usually associated with the genital region. Normal anatomical variants Adolescent and young adults may become concerned about certain normal features of their genitals after having noticing them for the first time. Prominent normal anatomical structures such as veins, sebaceous glands and hair follicles are common reasons for presentation at a doctor’s clinic. Prominent sebaceous glands are known as Fordyce spots — commonly seen on the scrotum as translucent yellowish papules. Similar structures may be present on the labial (lip) mucosa. Confusingly similar sebaceous prominences on the glans penis are referred to as Tyson’s glands. Pearly penile papules are anatomical variants found on the coronal sulcus, and frequently mistaken for warts. In all these cases, reassurance and patient education is all that is required. Table 2: Classification of male genital dermatoses Normal anatomical STIs variants Non-genital-specific skin diseases Skin diseases usually associated with the genital region • Prominent veins • Pearly penile papules • Prominent hair follicles • Prominent sebaceous gland • Inflammatory - Eczema - Psoriasis - Seborrhoeic dermatitis - Lichen planus • Pigmentary - Vitiligo - Melanosis and lentiginosis • Infective/microbial - Candidiasis/tinea - Boils/folliculitis - Erythrasma • Neoplastic - Squamous cell carcinoma (Bowen’s disease/erythroplasia of Queyrat) Bowenoid papulosis - Extramammary Paget’s disease • Drug related - Fixed drug eruption - Stevens–Johnson syndrome • Viral - Warts/Bowenoid papulosis - Molluscum contagiosum - Herpes • Infestation - Scabies - Lice • Other venereal diseases • Inflammatory - Lichen sclerosus - Zoon’s balanitis - Genital ulcers (Behçet’s disease, pyoderma gangrenosum, cicatricial pemphigoid • Fibrosis/stricture - Phimosis - Paraphimosis - Peyronie’s disease • Tumour/cysts - Angiokeratomas - Scrotal calcinosis • Idiopathic - Chronic idiopathic oedema - Dysaesthesia/pruritus (ani) Figure 2: A: Genital scabies. B: Scabies in the digital cleft. A B STIs Skin-to-skin contact during sexual intercourse facilitates transmission of several well-known diseases such as viral warts, herpes, scabies (figure 2), pubic lice and nits (figure 3) and molluscum contagiosum. Although these infections can occur on non-genital sites, the sexually acquired subtypes often show phenotypic and genetic adaptation to the genital region. For example, the herpes simplex virus (HSV 2) and certain genital verrucal sub- types have a greater predilection for genital skin. Pubic lice (Pediculosis pubis) are also physically adapted to the pubic region with their crab-like body and prominent ‘claws’ to attach to the pubic hair shafts. By comparison, head lice have a Figure 3: Pubic lice and nits. more slender structure, better adapted to scalp hair. Despite these adaptations, transmission can also occur onto non-genital sites, for example, HSV 2 developing on the lip through orogenital sex, and pubic lice spreading to eyelashes and axillary hair. Scabies acquired non-sexually can often localise as genital scabetic nodules that are diagnostic of the condition. Other STIs affecting the genitals and genitourinary tract include syphilitic chancres, non-syphilitic chancroid (Haemophilus ducreyi), lymphogranuloma venereum (Chlamydia trachomatis), granuloma inguinale (Klebsiella granulomatis), gonorrhoea and chlamydia. Other causes of infective urethritis and proctitis may also present to the GP requiring appropriate investigation and management. Screening for sexually acquired blood-borne diseases such as HIV and viral hepatitis may be necessary, especially in the context of unprotected sex. Non-genital-specific skin diseases COMMON skin diseases such as eczema and psoriasis frequently affect the genitals. The morphology of the rash is generally recognisable, although scaly rashes, such as psoriasis, may appear less scaly, especially so over the moist glans in the uncircumcised penis. The presentation of a variety of skin diseases localised to the glans penis (especially in the uncircumcised) can appear non-specific, posing diagnostic challenges. Inflammatory conditions Inflammatory diseases are by far the most common group of disease seen on the male genitalia. This group includes eczema, psoriasis and seborrhoeic dermatitis. The management principles revolve around supportive measures with gentle cleansers and use of non-soap alternatives. The genital skin is more sensitive than non-genital skin and any specific treatment needs to take this into account. For example genital psoriasis is not usually treated with phototherapy because of the greater susceptibility of skin cancer in this area. www.australiandoctor.com.au The use of higher potency topical steroids on genital skin should also be monitored closely with the rapid development of topical steroid changes, such as striae, common in this area. Eczema As with eczema anywhere else on the body, the condition is made worse by over-cleaning, especially with harsh soaps. Parts of the genital region, such as the glans in the uncircumcised penis, are particularly sensitive to over-cleaning in fastidious individuals. In general, eczema severity tends to be milder on genital skin and more responsive to treatment than other body sites. Low-potency topical cont’d next page 25 May 2012 | Australian Doctor | 25 A D _ 0 2 6 _ _ _ MA Y 2 5 _ 1 2 . p d f Pa ge 2 6 1 6 / 5 / 1 2 , 2 : 1 1 PM HOW TO TREAT Male genital dermatoses from previous page steroids such as 1% hydrocortisone cream are often quite effective in this area. Mixed corticosteroid–anti-yeast preparation such as hydrocortisone with clotrimazole cream (eg, Hydrozole) can be used to limit any opportunistic secondary yeast overgrowth in the genital area. Contact dermatitis or eczema is also seen in the genital areas with the use of irritating topical agents such as antiseptics causing irritant contact dermatitis. Allergic contact dermatitis can also occur with the use of perfumed products or products containing allergens such as preservatives. Figure 4: Psoriasis. Figure 5: Seborrhoeic dermatitis affecting the glans penis and scrotum. Figure 6: Lichen planus affecting the glans penis. (Photo: Dr Ian Hamann). Figure 7: Vitiligo with focal pigment loss on the penile shaft. Figure 8: Penile melanosis on the penile glans and shaft. A lesion biopsy (glans) was performed to exclude malignancy. Figure 9: Erythrasma of the groin and scrotum. Figure 10: Bowen’s disease of the penile shaft. (Photo: Dr Ian Hamann). and candidiasis can also affect the male genitals — especially the uncircumcised glans penis — and can be considered the male equivalent of vaginal thrush. Candidal transmission between sexual partners can also occur. Treatment with topical antifungal creams can alleviate the problem. therapy may be viable options, but will need informed patient consent because of the increased possibility of local recurrence. Psoriasis This commonly affects the male anogenital region, especially the natal–gluteal cleft between the buttocks. Genital psoriasis can occur in isolation or as part of the skin–joint–nail complex of psoriasis (figure 4). The lesions can resemble psoriatic lesions elsewhere with well-circumscribed erythematous plaques topped with silvery scales. However, genital psoriasis may have less or no scaling especially under the foreskin. A psoriasiform rash can occur on the glans penis in association with reactive arthritis (Reiter syndrome) in genetically susceptible individuals after gastroenteritis or genitourinary infection (eg, chlamydia). Mild- to moderatepotency topical steroids are the mainstay of treatment of this condition. Seborrhoeic dermatitis This is a form of dermatitis occurring in conjunction with an overgrowth of commensal skin yeast. Apart from the typical areas of the scalp, eyebrows and nasolabial crease, groin and genital involvement can occur (figure 5). Candidiasis and tinea can occur in the setting of excessive sweating or dampness and poor personal hygiene. Management involves keeping the area dry with non-occlusive cotton underwear and use of 1% hydrocortisone with 1% clotrimazole topical preparations (eg, Hydrozole cream). Lichen planus This pruritic, papular, polygonal and purple-coloured skin eruption can affect the genitals (figure 6). It can also be associated with nail, and/or mucosal involvement with white lacy streaks in the inner buccal mucosa. The clinical appearance is usually diagnostic and can be confirmed histologically with the characteristic lichenoid inflammation at the junction between the epidermis and dermis. Itch may be severe and often requires moderate to high potent topical steroids such as betamethasone for adequate control. Pigmentary disorders As a sexual organ, the male genital is intimately linked with the person’s psychosexual identity and self-image. ‘Harmless’ pigmentary conditions — either increased or decreased pigmentation — can lead to significant emotional and psychosexual morbidity. Vitiligo and lentiginosis are the two more common pigmentary conditions encountered in clinical practice. Vitiligo and lentiginosis are the two more common pigmentary conditions encountered in clinical practice. tip areas (figure 7). Whether this pattern of distribution is as a result of trauma or pressure is still debated. The cosmetic impact of vitiligo is greater in darker-skinned individuals, and the exposed and visible areas such as the face have management priority. Individuals sufficiently bothered by their genital vitiligo can be carefully prescribed potent topical steroids for 2-3 months at a time that is usually the minimum time for pigment restoration. An alternative immunosuppressive agent is tacrolimus compounded as a 0.1% ointment. Patients with vitiligo may need to have screening blood test for associated autoantibody conditions such as thyroid disease, diabetes or pernicious anaemia. Lentiginosis Darker-skinned individuals are also more susceptible to hyperpigmented macules (melanosis and lentiginosis) that can affect the genitals (figure 8). Like hypopigmented spots, hyperpigmented spots can be just as cosmetically distressing for patients. The types of pigmented lesions include naevi (moles), lentigines (freckles) and post-inflammatory hyperpigmentation. It is important to ensure the pigmented lesion is not sinister (eg, melanoma), and this can usually be established with routine clinical and dermatoscopic examination. If there is any doubt about the diagnosis, a biopsy is required. Benign naevi or lentigines are best left alone. Surgical excision can be problematic in terms of scarring and subsequent functional restriction. Rarely, pigment-removing laser can be used to lighten benign pigmented lesions in self-conscious individuals. Vitiligo Depigmenting conditions such as vitiligo have a predilection for the genitals in the ‘lip-tip’ distribution involving perioral, genital and finger 26 | Australian Doctor | 25 May 2012 Infective/microbial Candidiasis/tinea Fungal infection can occur as tinea cruris (jock itch). Yeast overgrowth www.australiandoctor.com.au Boil/folliculitis Bacteria such as Staphylococcus aureus can cause troublesome folliculitis or boils (furuncles). Some individuals may be staph carriers with reservoirs in the axillary or nasopharyngeal areas. Individuals with repeat episodes of folliculitis and boils may need swabs of the nostrils, axillae and groin to exclude staphylococcal carriage. A 10-15 day course of topical application of mupirocin and systemic antibiotics, such as cephalosporin or flucloxacillin, may help in this situation. Erythrasma This is a micrococcal organism of the skin causing an erythematous rash of the groin (figure 9) or a superficial keratolytic change on the feet. Erythrasma is responsive to topical antibiotics such as erythromycin 2%, or topical 1% clindamycin. Topical antifungals are also helpful in treating any coexisting tinea. Neoplastic Skin cancers The most common genital skin cancer is squamous cell carcinoma (figure 10), also known as Bowen’s disease or erythroplasia of Queyrat. As with any skin cancer, the key is early detection followed by definitive treatment. Excision is generally preferred with clear margins on histology. With some cases of in situ SCC — where surgical morbidity may be significant — nonsurgical alternatives such as topical 5-fluorouracil cream, imiquimod cream or photodynamic Bowenoid papulosis This is an erythematous or slightly pigmented papule or plaque, secondary to viral warts. These lesions are premalignant and also know as penile intraepithelial neoplasia, a male version of the better-known vulval or cervical intraepithelial neoplasia. Although these lesions are recognised as premalignant, they often regress spontaneously. Treatment is similar to wart therapy and ranges from liquid nitrogen or cautery to the use of topical agents such as podophyllum and imiquimod. Extramammary Paget’s disease This condition can rarely present in the anogenital region. This is an adenocarcinoma that is analogous to Paget’s disease of the nipple. The diagnosis should prompt a search for associated adenocarcinoma in adjacent organs such as the bladder, genitourinary tract or gastrointestinal tract. Drug related Fixed drug eruption Some patients are susceptible to fixed drug eruptions that localise to the same body region with each exposure to the implicated drug (figure 11). The eruption tends to be erythematous and sharply demarcated with occasional blisters, accompanied by considerable burning and itching. Sulphonamides, tetracyclines, NSAIDs and codeine-containing drugs or mixtures are some of the more commonly associated medications that can trigger fixed drug eruption. The genitals are a common location for fixed drug eruption. The blisters usually heal without scarring, but may develop prolonged post-inflammatory hyperpigmentation. Identifying A D _ 0 2 7 _ _ _ MA Y 2 5 _ 1 2 . p d f Figure 11: Fixed drug eruption on the glans penis from sulfamethoxazole. Pa ge 2 7 1 6 / 5 / 1 2 , 2 : 1 1 PM affects mucosal surfaces such as the lips, eyes and genitals (figure 12), which may precede the skin rash. Most patients have involvement of several mucosal regions (oral, ocular, anogenital) and hospitalisation may be required for skin, eye, GI and respiratory care to minimise complications such as mucosal scarring and strictures. The role of immunosuppressants such as prednisone and cyclosporine, and immunomodulators such as IV immunoglobulin, is beyond the scope of this article, however, immediate cessation of the implicated drug is mandatory and can be life-saving. and stopping the implicated drug leads to resolution of the problem. Stevens–Johnson syndrome/toxic epidermal necrolysis This is a serious drug reaction with a mortality of up to 30%. Although drugs are the most common aetiologic factor, a minority of cases may result from preceding infections (eg, mycoplasma pneumonia, HSV). An initial maculopapular rash is followed by the characteristic targetlike lesions, which resemble blisters, each with a dusky central ‘bullseye’. Stevens–Johnson syndrome also Figure 12: Stevens–Johnson syndrome. Skin diseases usually associated with the genital region Inflammatory Lichen sclerosus ALSO known as balanitis xerotica obliterans, this is an uncommon, but important condition with a predilection for the genital region (figure 13). While lichen sclerosus is more common in females, it also occurs in males and children. Although the cause is unknown, there may be a link with autoimmune diseases such as vitiligo, thyroiditis, pernicious anaemia and diabetes. Lichen sclerosus may present initially as ivory-white papules and plaques that progressively become atrophic. The condition can be itchy and/or painful. The skin eventually becomes thinned and parchment-like with irregularly depressed areas. Purpura, erosions and lichenification (thickening of the skin from repeated scratching) may complicate the clinical appearance. The glans and foreskin are typically affected and lichen sclerosus is a leading cause of phimosis (stricture of foreskin) making it non-retractable and meatal stenosis. The first-line treatment is potent topical steroids applied until the condition stabilises and remits. Maintenance with mild- to moderate-potency topical steroids may be necessary for a period of time (ie, months). There is a low risk of SCC transformation with genital lichen sclerosus and long-term follow-up is recommended. Lichen sclerosus also occurs in non-genital sites, but these areas are not associated with malignant change. Circumcision is protective against lichen sclerosus and may be required in cases where there is secondary phimosis. Zoon’s balanitis Primary Zoon’s balanitis is an inflammatory condition of the glans penis of unknown cause, affecting middle-aged and older uncircumcised men (figure 14). The glans becomes irritated as a result of a dysfunctional foreskin leading to retained secretions, epithelium, urine and overgrowth of commensal organisms under the foreskin. The foreskin itself may be too tight or adherent, creating friction and further irritation to the underlying glans. The condition is often asymptomatic despite the appearance of shiny red patches occurring on the glans penis and inner surface of the foreskin. The condition affects mucosal genitalia only and spares the kera- Figure 13: Lichen sclerosus. A: Lichen sclerosus with ivory white plaques on the penile shaft. B: Lichen sclerosus with white plaques and erosions on the penile glans and ventral shaft. Figure 14: Zoon’s balanitis of the penile glans and shaft. (Photo: Dr Ian Hamann). yeast is the mainstay of treatment. The condition can be difficult to treat and there may be frequent relapses. With refractory Zoon’s balanitis, circumcision is usually curative. Fibrosis and stricture Phimosis and paraphimosis (Photos: Dr Ian Hamann). Genital ulcers A B required for confirmation followed by treatment with immunosuppressives such as systemic steroids and cyclosporine. Benign genital ulcers similar to oral aphthous ulcers can occur in response to acute systemic illness. They are more common in females than males. Unlike benign oral ulcers (aphthae) where this can generally be accepted as a clinical diagnosis, genital ulcers should not be labelled as benign until other possibilities — especially STIs — have been excluded. Rare, but distinctive causes of genital ulcers include Behcet’s disease, pyoderma gangrenosum, cicatricial pemphigoid and drug eruptions such as fixed drug eruption and Stevens–Johnson syndrome. Secondary Zoon’s balanitis may result from psoriasis, seborrhoeic dermatitis, lichen sclerosus, lichen planus, contact dermatitis and even skin cancers. Behçet’s disease. This is a very rare condition consisting of painful ulcers affecting the mouth and the anogenitalia. The cause is unknown, but probably due to abnormal cellular immunity resulting in recurrent ulcers. Behçet’s disease is also associated with arthritis, uveitis, meningitis and nerve palsies. The mucocutaneous disease is managed with topical and intralesional steroids while oral prednisone, azathioprine and other immunosuppressives are reserved for more severe systemic disease manifestations. Pyoderma gangrenosum. This is a destructive ulcerating genital condition that is often misdiagnosed as cancer or infection. The soft tissue breaks down, forming deep ulcers that can cause tissue destruction. The condition often worsens with physical injury or inadvertent surgical procedures such as aggressive debridement or attempted excision. The key to managing pyoderma gangrenosum is immunosuppressive therapy such as prednisone or cyclosporine, and investigation for associated disorders such as malignancy, inflammatory bowel disease, rheumatoid arthritis and viral hepatitis. tinised outer foreskin, shaft and scrotum. Zoon’s balanitis is also called plasma cell balanitis because of the presence of plasma cells on skin histology. Because of the non-specific nature of the presentation, it is necessary to exclude concurrent primary genital dermatoses that may be the cause of secondary Zoon’s balanitis. Secondary Zoon’s balanitis may result from psoriasis, seborrhoeic dermatitis, lichen sclerosus, lichen planus, contact dermatitis and even skin cancers (Bowen’s disease). In fact, any condition from the list of inflammatory, infective or neoplastic groups of genital dermatoses can cause secondary Zoon’s balanitis. The management of primary Zoon’s balanitis requires good genital and foreskin hygiene. Daily gentle cleaning with water and mild soap or cleanser is recommended. The use of topical steroids (initially potent) with topical anti- Cicatricial pemphigoid. This is an autoimmune blistering disorder that targets the oral, ocular and anogenital mucous membrane and frequently causes scarring, strictures and adhesions. A biopsy with direct immunofluorescence is www.australiandoctor.com.au The foreskin is non-retractable at birth (physiological phimosis), but becomes gradually retractable with age. Pathological phimosis occurs when a previously retractable foreskin becomes non-retractable or when the foreskin remains nonretractable after puberty. Pathological phimosis is usually a result of scarring of the foreskin from chronic inflammation and other disease processes such as lichen sclerosus. Paraphimosis refers to a retracted foreskin that becomes entrapped behind the coronal sulcus. Phimosis and paraphimosis are diseases of uncircumcised males. Many of the inflammatory genital diseases — especially lichen sclerosus — can lead to scarring and subsequent phimosis. Paraphimosis is less common and occurs in the setting of forceful retraction of the phimotic foreskin — after vigorous sexual activity or following acute swelling from contact dermatitis or urticaria or angioedema of the genitals. Paraphimosis is a medical emergency because the constriction around the coronal sulcus restricts blood supply to the glans penis that can lead to irreversible penile damage. Phimosis can be treated with potent topical steroids to reduce inflammation and swelling while paraphimosis can be managed with various manual reduction techniques. Circumcision should be considered when conservative measures have failed and is urgently indicated in strangulating paraphimosis. Peyronie’s disease This is a fibrotic condition of the penile shaft leading to distortion of the penis especially during erection. The erect penis with Peyronie’s disease typically curves, which can be painful and impact adversely on sexual function. The fibrous plaques are often palpable as lumps within the erectile tissue, and are more common on the dorsal penis. The cause is unknown, but there appears to be a link with other fibrotic conditions — such as Dupuytren’s concont’d next page 25 May 2012 | Australian Doctor | 27 A D _ 0 2 8 _ _ _ MA Y 2 5 _ 1 2 . p d f Pa ge 2 8 1 6 / 5 / 1 2 , 2 : 1 1 PM HOW TO TREAT Male genital dermatoses from previous page Summary tracture of the palms — in up to 10% of cases. Peyronie’s disease can resolve spontaneously, so waiting up to 1-2 years is advisable before considering surgery. In the meantime, intraregional steroids or collagenase may alleviate some of the symptoms, while not being curative. Male genital dermatoses can present as: • Normal anatomical variants • STIs • Non-genital-specific skin diseases • Skin diseases usually associated with the genital region Tumours/cysts Angiokeratomas These vascular lesions present as red to purple papules ranging from 1-5mm in diameter. Patients tend to present with multiple lesions, although solitary lesions are not uncommon, with greater numbers with increasing age. Older lesions are larger and more keratotic. They mainly occur on the scrotum, but can also be found on the penile shaft. Most are not associated with any underlying metabolic disorders and are benign capillary malformations commonly known as angiokeratoma of Fordyce. Fordyce angiokeratomas should be distinguished from angiokeratoma of Fabry — a rare genetic metabolic disorder secondary to alpha-galactosidase deficiency and impaired glycosphingolipid metabolism. Angiokeratoma of Fabry presents in childhood with multiple angiokeratomas, accompanied by organ dysfunction secondary to build-up of glycosphingolipids, especially in the skin, eye, kidney, heart and CNS. Patients with benign angiokeratomas have no symptoms, but may present for cosmetic treatment. Occasional bleeding may occur from a scratched or traumatised lesion. Genital angiokeratomas can be effectively treated with gentle hyfrecation or vascular lasers. Scrotal calcinosis This is a relatively common disorder presenting as firm whitish scrotal nodules that may be solitary or multiple (figure 15). It is uncertain whether the calcinosis is primary, or results from underlying disorders such as epidermoid cysts, sebaceous cysts, eccrine cysts, dartos muscle degeneration, foreign body irritation or trauma. The lesions can occasionally become inflamed or infected, secondary to trauma. If desired by the patient, treatment is by surgical excision, although additional lesions may continue to develop with time. Idiopathic Chronic idiopathic genital oedema This is a rare condition that can result in significant psychosexual and physical morbidity. The penile and foreskin swelling is thought to be the result of lymphoedema from either primary lymphatic hypoplasia or secondary lymphatic damage from recurrent infections. The lymphoedema is made worse by intercurrent episodes of cellulitis and lymphangitis that can complicate the clinical presentation. Phimosis can result from chronic cases. Chronic idiopathic genital oedema can be associated with Crohn’s disease. Patients with Crohn’s-associated genital oedema will often show a granulomatous histology on biopsy. Active exclusion of Crohn’s disease may be 28 | Australian Doctor | 25 May 2012 Psychological and sexual dysfunction may accompany the presentation Consider STIs in at-risk groups Rule out malignancy in the older age group Further reading Figure 15: Scrotal calcinosis arising from epidermoid cysts. warranted in persistent cases even in the absence of GI symptoms. Prophylactic antibiotics, such as erythromycin and trimethoprim, are commonly used to prevent secondary infection that can worsen underlying lymphatic obstruction and scarring. Short courses of systemic steroids can be helpful to reduce swelling in severe cases. Circumcision may be necessary in refractory cases. Genital dysaesthesia There is a strong link between genital dysaesthesia and psychosexual dysfunction. Genital dysaesthesia covers a diverse range of symptoms that include burning, itching and pain, with some individuals also complaining of redness. Some of the diagnostic labels include penodynia and scrotodynia for painful penis and scrotum, respectively. The complaints are typically persistent with repeated presentations to the same doctor or opinions sought from multiple practitioners. Because of the absence of outward signs, management is less straightforward and can contribute to a sense of frustration and despondency in both patient and practitioner. Not surprisingly, there is a strong link between genital dysaesthesia and psychosexual dysfunction. A discordance between societal expectation of accepted sexual behaviour vs the individual’s actual sexual behaviour can result in feelings of shame, guilt and low self-esteem. Examples include matters of sexual orientation, sexual acts such as oral and anal sex and non-monogamous sexual activity. For less liberal sociocultural groups where strict moral and social codes govern sexual behaviour and practice, there can be considerable psychological and emotional distress when personal sexual behaviour departs from the expected norm. Even in liberal societies unhampered by strict sexual mores, there is still a considerable stigma associated with STIs. It is difficult to estimate what proportion of genital dysaesthesia can be directly linked to underlying feelings of guilt or shame. For example, the occasional married patient anxiously repeatedly attending with genital itch or other forms of dysaesthesia without eviwww.australiandoctor.com.au dence of skin disease may be distressed by recent extramarital sexual contact. These patients are typically harbouring a sense of guilt and are also concerned they may have contracted an STI, or transmitted it to their spouse. Tactful yet unambiguous enquiry (eg, “Have you had sexual relations with anyone else?”) directed at the source of the psychological discomfort is often met with a sense of relief. For this group of patients, there can be significant symptom resolution as their covert fears and concerns are being openly addressed. Symptomatic treatment of this group may be required and medications such as doxepin or amitriptyline, 25-50mg at night for a period of 3-6 months may be required to alleviate symptoms. Neurogenic pain or neuropathy may be the cause of some of the complaints, but this is essentially a diagnosis of exclusion. The list of genital dermatoses as differential diagnoses should be actively considered and excluded. Postherpetic neuralgia and intercurrent HSV can account for a subset of neuropathic pain as can referred pain from the lumbosacral region. • Wolff K, et al. Fitzpatrick’s Dermatology in General Medicine. 7th edn. McGraw-Hill, New York, 2007. • Bolognia JL, et al. Dermatology. 2nd Edn. Elsevier Ltd, Edinburgh, 2008. Online resources • DermNet NZ — facts about the skin from the New Zealand Dermatological Society Inc: www.dermnetnz.org. Pruritus ani An itchy anus and related genital itch can range in severity from mild to severe or intractable. Most cases are idiopathic with no detectable cause. The challenge for the practitioner is to exclude and manage secondary causes including eczema, psoriasis, contact dermatitis, lichen sclerosus, candida, warts, pinworms, haemorrhoids and poor hygiene. Management involves treating the underlying cause and keeping in mind that overzealous cleaning will make the problem worse. With genital itch, common groin and genital rashes such as candida, tinea and dermatitis may occasionally be the cause. Scabies and pubic lice must be excluded. As discussed previously, scabies usually presents with diagnostic itchy, inflammatory penile or scrotal nodules while pubic lice (after careful search) can be clinically detected at the base of pubic hairs. cont’d page 30 A D _ 0 3 0 _ _ _ MA Y 2 5 _ 1 2 . p d f Pa ge 3 0 1 6 / 5 / 1 2 , 2 : 1 1 PM HOW TO TREAT Male genital dermatoses Authors’ case studies Case study 1 A 60-YEAR-old man presents with intermittent genital rash that is currently flaring (figure 5, page 26). This has been a recurring problem over the past decade with a fluctuating course. He has been using Dettol antiseptic washes daily. Clinically, there is significant erythema of the genital region with areas of scaling. There is associated scaling of the eyebrows and scalp dandruff. The likely primary diagnosis is seborrhoeic dermatitis also involving the scalp, eyebrows and genitalia. Seborrhoeic dermatitis results from commensal yeast overgrowth and typically follows a fluctuating course. The condition can be asymptomatic, although mild itching and irritation is not uncommon. The differential diagnoses include psoriasis and eczema. Management begins with general measures to reduce irritation. The use of Dettol antiseptic has exacerbated the problem by producing an irritant dermatitis on top of the seborrhoeic dermatitis. Swap the Dettol for a gentle soap-free cleanser such as QV cream or The use of antiseptic has exacerbated the problem by producing an irritant dermatitis on top of the seborrhoeic dermatitis. Cetaphil. Application of 1% hydrocortisone with 1% clotrimazole cream will settle the rash in a few days. The facial seborrhoeic dermatitis (eyebrows, nasolabial folds) can be similarly treated. Treatment of the scalp with antidandruff shampoos containing tar, ketoconazole or zinc pyrithione is recommended. Although the rash should respond well to treatment, the patient should be advised that recurrence, rather than cure, is the rule. Case study 2 A 45-year-old man presents with a six-month history of penile itch, pain and dyspareunia. Clinically, the penis shows ivory-white atrophic changes to the skin of the How to Treat Quiz 2. With respect to examination and investigation of the male genital skin which TWO statements are correct? a) Examination of non-genital skin is irrelevant b) Visual aids such as a magnifying lens can greatly assist in visualising pubic lice, while a dermatoscope is helpful for pigmented lesions c) Scrapings for microscopy can confirm fungal infections d) Skin biopsy by a GP is mandatory if there is a lesion of concern 3. With respect to normal anatomical variants of the male genital skin which TWO statements are correct? a) Prominent hair follicles on the penile skin are abnormal as this is hairless skin b) Prominent sebaceous glands in the penile skin are pre-malignant and require biopsy c) Pearly penile papules are anatomical variants found on the coronal sulcus and are frequently mistaken for warts d) Sebaceous prominences on the glans penis are referred to as Tyson’s glands INSTRUCTIONS Complete this quiz online and fill in the GP evaluation form to earn 2 CPD or PDP points. We no longer accept quizzes by post or fax. The mark required to obtain points is 80%. Please note that some questions have more than one correct answer. Male genital dermatoses — 25 May 2012 1. With respect to male genital dermatoses which TWO statements are correct? a) Foreskin-related problems (eg, phimosis) occur only in the paediatric age group b) STIs are more common in men in early adulthood c) Older males are more prone to skin cancer d) A history of eczema or psoriasis is not relevant in the assessment of genital skin glans penis extending along the shaft with focal areas of superficial erosion (figure 13B, page 27). The ivory-white skin changes and genital location are characteristic of genital lichen sclerosus. The diagnosis can be confirmed on biopsy or by a dermatologist. Check for lichen sclerosus in other sites as the condition can present on the torso, perineum and perianal regions. Look for possible autoimmune associations such as vitiligo. A blood test for thyroid autoantibodies and intrinsic factor antibodies (pernicious anaemia) can sometimes uncover occult associations. Start with potent topical steroids such as betamethasone. Review the patient after 4-6 weeks to assess treatment response. Review every 1-2 months until the condition stabilises and remits during which the frequency of application can be reduced to twice a week. Relapses should be managed by reversion to full potency topical steroids until control is re-established. Advise the patient to avoid harsh soaps and over-cleaning, which can exacerbate the symptoms. Inadequate treatment can lead to stricture of the urethral meatus, phimosis and irreversible sclerotic changes. There is also a rare, but recognised risk of SCC transformation with genital lichen sclerosus, and thus long-term follow-up is necessary. ONLINE ONLY www.australiandoctor.com.au/cpd/ for immediate feedback 4. With respect to sexually acquired disease which TWO statements are correct? a) Sexually acquired subtypes of infections such as herpes simplex virus (HSV 2) often show phenotypic and genetic adaptation to the genital region b) HSV 2 only occurs on genital skin c) Pubic lice have a more slender structure, better adapted to pubic hair, than do head lice d) Screening for sexually acquired blood-borne diseases, such as HIV and viral hepatitis, may be necessary, especially in the context of unprotected sex 5. With respect to non-genital-specific skin diseases which TWO statements are correct? a) Common skin diseases such as eczema infrequently affect the genitals b) Scaly rashes such as psoriasis may appear less scaly on the male genital skin, especially over the glans in the uncircumcised penis c) If it occurs, eczema tends to be milder on genital skin and more responsive to treatment than other body sites d) Genital psoriasis usually requires treatment with phototherapy 6. With respect to seborrhoeic dermatitis of the male genital skin which THREE statements are correct? a) Seborrhoeic dermatitis usually occurs in conjunction with an overgrowth of commensal skin yeast b) Seborrhoeic dermatitis can involve the groin and genital region c) Management involves keeping the area moist d) 1% hydrocortisone with 1% clotrimazole topical preparations (eg, Hydrozole) is effective in the management of seborrhoeic dermatitis of the male genital skin 7. With respect to vitiligo which THREE statements are correct? a) Depigmenting conditions such as vitiligo have a predilection for the genitals b) The cosmetic impact of vitiligo is greater in lighter-skinned individuals c) Vitiligo can be treated with potent topical steroids for 2-3 months at a time, which is usually the minimum time for pigment restoration d) Patients with vitiligo may need to have screening blood test for associated autoantibody conditions such as thyroid disease, diabetes or pernicious anaemia 8. With respect to skin cancers of the male genital skin which TWO statements are correct? a) The most common genital skin cancer is squamous cell carcinoma b) With some cases of in-situ SCC nonsurgical alternatives such as topical 5fluorouracil cream, imiquimod cream or photodynamic therapy may be viable options c) Bowenoid papulosis is not premalignant d) Bowenoid papuloses never regress spontaneously and always require excision 9. With respect to lichen sclerosus of the penis which TWO statements are correct? a) Lichen sclerosus has a predilection for the genital region b) While lichen sclerosus is more common in men, it also occurs in women and children c) There is a high risk of SCC transformation with genital lichen sclerosus d) Lichen sclerosus is a leading cause of phimosis/stricture of the foreskin 10. With respect to dyaesthesia of the male genital skin which THREE statements are correct? a) Genital dysaesthesia covers a diverse range of symptoms including burning, itching, and pain, with some individuals also complaining of redness b) These patients are commonly concerned they may have contracted an STI c) Often personal hygiene is at fault and symptoms resolve with more rigorous cleaning d) Tactful enquiry (eg, “Have you had sexual relations with anyone else?”) is often met with a sense of relief CPD QUIZ UPDATE The RACGP requires that a brief GP evaluation form be completed with every quiz to obtain category 2 CPD or PDP points for the 2011-13 triennium. You can complete this online along with the quiz at www.australiandoctor.com.au. Because this is a requirement, we are no longer able to accept the quiz by post or fax. However, we have included the quiz questions here for those who like to prepare the answers before completing the quiz online. HOW TO TREAT Editor: Dr Martine Walker Co-ordinator: Agilene De Villa Quiz: Dr Martine Walker NEXT WEEK For decades, warfarin has consistently been shown to be the most effective oral anticoagulant therapy. New oral anticoagulants such as dabigatran have been pushed into clinical development. Next week’s How to Treat discusses issues in the general use of newer agents as first-line therapy for patients with atrial fibrillation. The authors are Dr Stefan Buchholz, consultant cardiologist, cardiac services, Mackay Base Hospital, Mackay, Queensland; and Dr Vijay Solanki, head of cardiology, Hornsby Ku-ring-gai Hospital, NSW; VMO, echocardiography department, St Vincent’s Hospital, NSW. 30 | Australian Doctor | 25 May 2012 www.australiandoctor.com.au
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