Papulosquamous Dermatoses

Papulosquamous Dermatoses Alberto Rosenblatt, Homero Gustavo de Campos Guidi, and Walter Belda Jr. 5 Contents 5.1 Introduction... 88 5.2 Psoriasis Common in Geriatric Group/Systemic Disease... 89 5.2.1 Overview... 89 5.2.2 Epidemiology... 89 5.2.3 Symptoms... 89 5.2.4 Clinical Findings... 89 5.2.5 Diagnosis... 91 5.2.6 Histology... 92 5.2.7 Differential Diagnosis... 92 5.2.8 Treatment... 92 5.3 Lichen Planus Systemic Disease... 93 5.3.1 Overview... 93 5.3.2 Epidemiology... 93 5.3.3 Symptoms... 93 5.3.4 Clinical Findings... 93 5.3.5 Diagnosis... 95 5.3.6 Histology... 95 5.3.7 Differential Diagnosis... 95 5.3.8 Treatment... 95 5.4 Lichen Nitidus Systemic Disease... 95 5.4.1 Overview... 95 5.4.2 Symptoms... 95 A. Rosenblatt, M.D., Ph.D. ( ) Albert Einstein Jewish Hospital (HIAE), Av. Albert Einstein 627/701, 05651-901, São Paulo, SP, Brazil e-mail: albrose1@gmail.com H.G. de Campos Guidi, M.D., Ph.D. University of São Paulo Medical School, Av. Eneas Carvalho de Aguiar, 255 - sl. 10.166 - São Paulo, SP, Brazil e-mail: hgcguidi@gmail.com W. Belda Jr. Department of Dermatology, University of São Paulo Medical School, Avenida Açocê 162, 04075020, São Paulo, SP, Brazil e-mail: walterbelda@uol.com.br 5.4.3 Clinical Findings... 95 5.4.4 Diagnosis... 96 5.4.5 Histology... 96 5.4.6 Differential Diagnosis... 96 5.4.7 Treatment... 96 5.5 Common in Geriatric Penile Lichen Sclerosus Group/Almost Exclusive to Uncircumcised Individuals... 96 5.5.1 Overview... 96 5.5.2 Epidemiology... 97 5.5.3 Symptoms... 97 5.5.4 Clinical Findings... 97 5.5.5 Diagnosis... 101 5.5.6 Histology... 102 5.5.7 Differential Diagnosis... 102 5.5.8 Treatment... 102 5.6 Seborrheic Dermatitis Systemic Disease... 103 5.6.1 Overview... 103 5.6.2 Epidemiology... 104 5.6.3 Symptoms... 104 5.6.4 Clinical Findings... 104 5.6.5 Diagnosis... 104 5.6.6 Differential Diagnosis... 104 5.6.7 Treatment... 105 5.7 Reactive Arthritis Systemic Disease... 105 5.7.1 Overview... 106 5.7.2 Epidemiology... 106 5.7.3 Symptoms... 106 5.7.4 Clinical Findings... 106 5.7.5 Diagnosis... 107 5.7.6 Histology... 107 5.7.7 Differential Diagnosis... 107 5.7.8 Treatment... 107 5.8 Chronic Exudative and Lichenoid Dermatosis... 108 5.8.1 Overview... 108 5.8.2 Symptoms... 109 5.8.3 Differential Diagnosis... 109 5.8.4 Treatment... 109 References... 109 A. Rosenblatt et al., Male Genital Lesions, DOI 10.1007/978-3-642-29017-6_5, Springer-Verlag Berlin Heidelberg 2013 87

88 5 Papulosquamous Dermatoses 5.1 Introduction The clinical presentation of scaly papules or plaques on the external male genitalia should direct the examiner to a more widespread papulosquamous condition. Therefore, a systematic examination of extragenital skin, oral mucosa, and nails may provide further clues that help guide the physician to the correct diagnosis of genital papulosquamous dermatoses. In this Chapter, only the noninfectious papulosquamous disorders affecting the male genital are discussed (Table 5.1 ). Table 5.1 Noninfectious papulosquamous lesions of the male genital Disease Symptoms Clinical findings Differential diagnosis Psoriasis Soreness, pruritus, and/or burning Well-demarcated, brightly erythematous (salmon-pink), thin plaques with Bowenoid papulosis, erythroplasia of Queyrat, Bowen s sensation with varying no or minimal scaling disease, plasma-cell balanitis, degrees of intensity Rhagades or fissures extramammary Paget s disease, Kaposi s sarcoma, lichen sclerosus, lichen planus, seborrheic dermatitis, scabies Lichen planus Usually not pruritic Raised, purpuric, fl at-topped, polygonal papules Soreness in eroded lesions Fine, lacy white streaks (Wickham striae) Annular erythematous plaques, multiple tiny papules or erosion, and/or ulceration of the glans penis Lichen nitidus Asymptomatic Discrete, slightly elevated, skin-colored or hypopigmented papules, with a fl at, shiny surface Lichen sclerosus Asymptomatic (early stage), pruritus, dyspareunia, dysuria Urethritis and voiding problems (meatal stenosis) Phimosis Seborrheic Mild pruritus dermatitis Mild soreness Reactive arthritis Fever, malaise, fatigue, anorexia, diarrhea, weight loss Dysuria, urgency, frequency, urethral discharge, balanitis Asymmetric joint pain Conjunctivitis, photophobia, pain Chronic exudative and lichenoid dermatosis Severe pruritus Single or multiple erythematous papules or macules Thin patches or plaques that involve the frenulum and foreskin Typical white sclerotic ring at the tip of the foreskin Mucosal fissures, petechiae, telangiectasias, and hemorrhagic lesions Intermittent penile rash, slight erythema, scaling of varying intensity Perifollicular or follicular involvement Keratoderma blennorrhagicum, painless oral ulcers, nail changes, circinate balanitis, urethritis, prostatitis, cystitis, and cervicitis Exsudative discoid lesions and lichenification involving resolving plaques Penile cancer, psoriasis, lichen sclerosus, secondary syphilis, plasma cell balanitis, erythroplasia of Queyrat, fi xed drug eruption, erosive balanitis, mucous membrane pemphigoid or pemphigus Pearly papules Penile carcinoma in situ, leukoplakia, scleroderma Balanoposthitis, psoriasis Psoriasis, gonococcal arthritis, Behçet s disease, balanoposthitis, lichen sclerosus, irritant and allergic contact dermatitis Nummular dermatitis, lymphoma, chronic eczema

Common in Geriatric Group/Systemic Disease 5.2 Psoriasis 89 5.2 PsoriasisCommon in Geriatric Group/Systemic Disease 5.2.1 Overview Psoriasis is a chronic, in fl ammatory epidermal skin disease. The condition may be associated with considerable morbidity and embarrassment and, according to a recent study performed by Meeuwis et al. ( 2011b ) in a large group of patients with genital psoriasis, it may impart the quality of life and psychosexual wellbeing of affected individuals. Psoriasis may be primary on the penis, but it is most often associated with typical lesions elsewhere on the body. Nonetheless, the disease is commonly observed on the genital skin. Psoriasis is currently considered an immunodysregulatory disorder, where an immune induced and ampli fi ed regeneration-like reaction of the skin occurs (Sabat et al. 2007 ). Fig. 5.1 Psoriasis involving trunk, limbs, and genital area 5.2.2 Epidemiology The prevalence of psoriasis in the general population is approximately 2% (Christophers 2001 ; Raychaudhuri and Farber 2001 ). Isolated presentation of psoriasis on genital skin occurs in only 2 5% of the psoriatic patients (van de Kerkhof 2003 ), but genital skin is involved in 29 40% of patients affected with the disease (Hellgren 1964 ; Farber et al. 1968 ; Farber and Nall 1974 ) (Figs. 5.1, 5.2, 5.3, and 5.4 ). Fig. 5.2 Psoriasis involving trunk, limbs, buttocks, and anogenital area (same patient as in Fig. 5.1 ) Inverse pattern psoriasis Psoriasis that affects intertriginous skin (axillae, gluteal folds, groins, and ventral side of the penis), as well as glans and preputial sac of uncircumcised individuals is termed inverse psoriasis and, according to Wang et al. ( 2005 ), the genital area is commonly involved. 5.2.3 Symptoms Soreness, pruritus, and/or burning sensation with varying degrees of intensity. Fig. 5.3 Psoriasis elbow (patient also manifested associated genital lesions see Fig. 5.5 ) 5.2.4 Clinical Findings Psoriasis of the genital region usually manifests as well-demarcated, brightly erythematous (salmon-pink), thin plaques with minimal scaling

90 5 Papulosquamous Dermatoses Fig. 5.4 Extragenital and genital psoriasis Fig. 5.6 Glans psoriasis well-demarcated, brightly erythematous (salmon-pink), thin plaques with minimal scaling Fig. 5.7 Glans psoriasis The glans penis is commonly affected (Fig. 5.7 ), but the shaft of the penis, prepuce, scrotum, inguinal folds, buttocks, and perianal area can also be involved (Figs. 5.8, 5.9, 5.10, 5.11a, b ). Fig. 5.5 Psoriasis salmon-pink lesions, thin plaques, and minimal scaling (occasionally observed on keratinized regions of the genital skin) (Coldiron and Jacobson 1988 ; Farber and Nall 1992 ) (Figs. 5.5 and 5.6 ), or even absence of scaling (Goldman 2000 ; Buechner 2002 ). However, when present, scales can be easily removed, which will lead to pinpoint bleeding areas. Rhagades or fi ssures can also be observed. In uncircumcised males, plaques are frequently observed under the prepuce and on the proximal glans. In circumcised individuals, lesions are usually present on the glans and corona and tend to be more desquamative (Coldiron and Jacobson 1988 ; Buechner 2002 ). Pustular psoriasis has been described in the male genital region, with numerous painless

Common in Geriatric Group/Systemic Disease 5.2 Psoriasis 91 Fig. 5.10 Psoriais involving pubis, shaft of the penis, scrotum, and inguinal folds Fig. 5.8 Psoriasis red plaque involving the scrotum (note also angiokeratomas) a b Fig. 5.9 Psoriasis salmon-pink thin plaque with minimal scaling (same patient as Fig. 5.8 ) white papules and pustules located on the penis (Quan and Ruben 1996 ). Genital psoriasis may be worsened by secondary fungal or bacterial infection, sexual intercourse, or tight- fi tting clothes (Koebner phenomenon) (Fiumara 1976 ). Scarring is usually absent. Potential triggers of psoriasis Drugs (beta-blockers, angiotensin converting enzyme (ACE), lithium, antimalarials, imiquimod (Fig. 5.12 )) HIV infection Genital herpes infection Fig. 5.11 ( a, b ) Psoriasis (same patient) 5.2.5 Diagnosis Clinical examination (including other body sites and nails) (Fig. 5.13 ). Biopsy to rule out premalignant penile diseases (particularly in uncircumcised individuals).

92 5 Papulosquamous Dermatoses Fig. 5.12 Imiquimod-induced psoriasis (Source: Patel et al. ( 2011 ). Reproduced with permission from Wiley) Fig. 5.14 Chronic eczematous dermatitis (compare with Fig. 5.9 and with Fig. 2.31 in Chap. 2 ) lichen planus, seborrheic dermatitis, eczematous dermatitis (Fig. 5.14 ), scabies. 5.2.8 Treatment Reassurance of benign condition. According to a recent systematic literature review for evidence-based data on genital psoriasis performed by Meeuwis et al. ( 2011a ), the treatment of genital psoriasis is often empirical (grade of recommendation: D). Mild emollients and soap substitutes may be useful in the management of genital lesions. Fig. 5.13 Psoriasis involving the umbilical area 5.2.6 Histology Immune cells in fi ltration of dermis and epidermis. Dilatation and an increase in the number of blood vessels in the upper dermis. Epidermal hyperproliferation with atypical keratinocyte differentiation. 5.2.7 Differential Diagnosis Bowenoid papulosis, erythroplasia de Queyrat, Bowen s disease (BD), plasma-cell balanitis (Zoon s balanitis), extramammary Paget s disease (EMPD), Kaposi s sarcoma, lichen sclerosus, 5.2.8.1 First-Line Treatment Topical corticosteroid isolated or combined with antibiotics or antifungal agents in case of concurrent bacterial or fungal infections, respectively. Less potent corticosteroids should be initially applied, and more potent formulas employed only in case of resistance to treatment. However, long-term use of potent corticosteroids should be avoided in view of their atrophic effects, particularly on genital skin. 5.2.8.2 Second-Line Treatment Vitamin D analogue (calcipotriol or calcipotriene) or weak tar-based treatment can reduce corticosteroids side-effects, but skin irritation may restrict their use. Strong tar preparations should be avoided on the genital skin because of increased cancer risk (i.e., scrotal).

Systemic Disease 5.3 Lichen Planus 93 Evidence for the use of immunomodulators on genital skin is scarce, and their use should be considered a third-line treatment option. Systemic treatment should be reserved for severe and/or extensive psoriasis and/or arthritis. Penile plaques resistant to usual therapy should be biopsied to rule out malignancy (Goldman 2000 ). Phototherapy for genital psoriasis is contraindicated because of the increased risk of anogenital cancer. 5.3 Lichen PlanusSystemic Disease 5.3.1 Overview Lichen planus (LP) is a systemic disease that usually affects mucous membranes, nails, the lower mid-back, acral sites, axillae, and the scalp (Porter and Bunker 2001 ). The glans penis is usually involved and, together with psoriasis, constitutes the most common papulosquamous disorder that affects this area. LP has been associated with chronic liver disease (35%) and hepatitis C virus infection (9.8 23%), but the exact pathogenic role of the infectious agent still remains to be elucidated (Nocente et al. 2003 ). Imiquimod, a topical immune response modi fi er used to treat genital warts, may induce severe genital LP unresponsive to steroid treatment (O Mahony et al. 2010 ). Fig. 5.15 Lichen planus polygonal papules and fi ne, lacy white streaks involving the penile shaft 5.3.3 Symptoms Pruritus is infrequent in LP, but soreness may occur in eroded lesions. 5.3.4 Clinical Findings Raised, purpuric, fl at-topped, polygonal papules and fi ne, lacy white streaks (Wickham striae) may appear on the surface of the lesions (Figs. 5.15, 5.16, 5.17, 5.18, and 5.19 ). Annular erythematous plaques, multiple tiny papules or erosion (Figs. 5.20 and 5.21 ), and/or ulceration of the glans penis (Alinovi et al. 1983 ) and foreskin may also occur. Phimosis as a result of lichen planus can be observed in both children and adults (Itin et al. 1992 ; Aste et al. 1997 ). 5.3.2 Epidemiology The genital region is affected in ~25% of the patients with lichen planus, and most individuals also manifest concurrent systemic manifestations (Altman and Perry 1961 ). However, the sole involvement of the glans penis is infrequently observed, and mucosal involvement is rare in children (Aste et al. 1997 ; Luis-Montoya et al. 2005 ). LP lesions in uncircumcised patients exhibit a lacy, white, reticulated pattern Koebner phenomenon may also be observed (particularly on the foreskin) and postin fl ammatory hyperpigmentation may persist for months or years. LP is mostly a self-limiting disease, but a relapsing course may occur in some patients. Erosive LP may persist for long periods.

94 5 Papulosquamous Dermatoses Fig. 5.18 Lichen planus involving the shaft of the penis and prepuce Fig. 5.16 Lichen planus Fig. 5.19 Lichen planus lacy white streaks on the scrotum and penis Fig. 5.17 Lichen planus subtle annular lesion at the glans penis ( arrow ) Fig. 5.20 Lichen planus polygonal papules, lacy white streaks, mild glans erosion

Systemic Disease 5.4 Lichen Nitidus 95 mucous membrane pemphigoid, or pemphigus (Porter and Bunker 2001 ). 5.3.8 Treatment Fig. 5.21 Lichen planus Topical corticosteroids are usually effective, but daily use of potent steroids should be avoided in view of their atrophic effects. Weekend use of ultrapotent preparations may be an ef fi cacious alternative. Circumcision for isolated penile lesions may be indicated when disease is unresponsive to medical treatment (Porter et al. 2001 ) or when phimosis is present (Itin et al. 1992 ; Aste et al. 1997 ). Malignant transformation of lichen planus into penile intraepithelial neoplasia (PIN), verrucous carcinoma of the penis (Bain and Geronemus 1989 ), and penile squamous cell carcinoma have been reported (Leal-Khouri and Hruza 1994 ; Hoshi et al. 2008 ). 5.3.5 Diagnosis Clinical examination. Biopsy and histology is commonly performed for diagnostic purposes, and also to rule out penile cancer in chronic, ulcerated, or indurated lesions (Rosen and Brown 1998 ). 5.3.6 Histology Mucosal involvement shows a thinned epidermis, mild degrees of epithelial dysplasia, parakeratosis, and plasma cells in the in fi ltrate. 5.3.7 Differential Diagnosis Penile cancer, psoriasis, lichen sclerosus, secondary syphilis, plasma cell balanitis, erythroplasia of Queyrat, fi xed drug eruption, erosive balanitis, 5.4 Lichen NitidusSystemic Disease 5.4.1 Overview Lichen nitidus is a chronic dermatosis of unknown origin, usually located on the penis, abdomen, and upper limbs (Davis et al. 1996 ; Kim and Shim 2006 ). However, a few cases of generalized lichen nitidus have been reported by Soroush et al. ( 1999 ), Arizaga et al. (2002 ), and Rallis et al. ( 2007 ). Although lichen nitidus is clinically and histologically distinct from LP (Smoller and Flynn 1992 ), the disease may be regarded as a micropapular variant of lichen planus (Shiohara and Kano 2008 ). Spontaneous resolution is common but, according to Davis et al. ( 1996 ), clinical course can be variable. 5.4.2 Symptoms Asymptomatic. 5.4.3 Clinical Findings Presence of discrete, slightly elevated, skin-colored or hypopigmented papules, with a fl at, shiny

96 5 Papulosquamous Dermatoses Fig. 5.22 Lichen nitidus slightly elevated, small hypopigmented papules surface, measuring approximately 1 mm (Figs. 5.22, 5.23, and 5.24 ). Fig. 5.23 papules Lichen nitidus slightly elevated, fl esh-colored 5.4.4 Diagnosis Clinical examination. 5.4.5 Histology Papules present a parakeratotic cap and epidermal atrophy is observed. Lymphocytes and histocytes in the papillary dermis have a ball-in-claw relation to the rete ridges of the epidermis. 5.4.6 Differential Diagnosis Pearly papules, herpes simplex virus. 5.4.7 Treatment Reassurance of benign condition. Corticosteroids, vitamin A analogues (calcipotriol or calcipotriene), cyclosporine, antifungal preparations (itraconazole), topical tacrolimus (Dobbs and Murphy 2004 ), and phototherapy (Kim and Shim 2006 ) can be used for cosmesis. However, according to Davis et al. ( 1996 ), laser therapy should be avoided because of scarring. Fig. 5.24 Lichen nitidus slightly elevated and tiny, flesh-colored papules (arrow) 5.5 Penile Lichen SclerosusCommon in Geriatric Group/Almost Exclusive to Uncircumcised Individuals Synonym Balanitis xerotica obliterans (BXO). 5.5.1 Overview Lichen sclerosus (LS) is a relatively common hypopigmented skin disorder, with a large incidence on genital skin. Penile LS is a chronic, progressive disease that may cause scarring, and which may result in significant voiding complications. The exact etiology of LS is unknown, but immune dysregulation is considered the factor most strongly associated with the disease (Regauer 2005 ). Moreover, there is a higher prevalence of infection with high-risk HPV types in

Common in Geriatric Group/Almost Exclusive to Uncircumcised Individuals 5.5 Penile Lichen Sclerosus 97 patients with penile LS (Nasca et al. 2006 ; Prowse et al. 2008 ). Together with low- and high-grade PIN and squamous hyperplasia, LS may be considered a precursor lesion of penile cancer (Powell et al. 2001 ; Cubilla et al. 2004 ; Velazquez and Cubilla 2007 ) with a 6% risk of progression to SCC. It has been hypothesized that infection with oncogenic HPV types may enhance the risk of penile cancer development in patients with genital LS (Nasca et al. 2006 ). In a recent clinicopathologic analysis performed by Cubilla et al. ( 2004 ), LS was speci fi cally related to well-differentiated SCC of the penis with pseudohyperplastic features. These multicentric tumors are usually present in older patients and preferentially involve the inner mucosal surface of the foreskin. However, malignant transformation may also be related to phimosis, a common complication of longstanding LS. Imiquimod use in the genital area has been associated with the development of fl orid penile LS unresponsive to steroid treatment (O Mahony et al. 2010 ). Fig. 5.25 Lichen sclerosus erythematous macules, postin fl ammatory hyper- and hypopigmentation (note associated perimeatal involvement) 5.5.2 Epidemiology The condition mostly affects uncircumcised middle-aged white men; however, LS has been described in 4-year-old boys (Rickwood et al. 1980 ) and, according to Chalmers et al. ( 1984 ), it affects the young male population more often than previously assumed. In this young group, the peak incidence of LS occurred between the ages of 9 and 11, and nearly all affected boys had secondary phimosis (Kiss et al. 2005 ). 5.5.3 Symptoms Asymptomatic at early stages Recurrent inflammatory symptoms (balanitis). Pruritus. Glans penis hypoesthesia. Dyspareunia. Dysuria. Fig. 5.26 Lichen sclerosus postinflammatory hyperand hypopigmentation Urethritis and voiding problems because of meatal stenosis. Dif fi culty or inability to retract the foreskin in uncircumcised individuals. 5.5.4 Clinical Findings Single or multiple erythematous papules or macules, that usually affect the glans penis and foreskin at the early stages of the disease (Figs. 5.25 and 5.26 ). The perianal region is not affected in male individuals. Presence of thin patches or plaques that involve the frenulum and foreskin (the penile shaft is rarely involved) (Figs. 5.27 and 5.28 ),

98 5 Papulosquamous Dermatoses Fig. 5.27 Lichenoid plaque involving the penile shaft Fig. 5.29 Lichen sclerosus phimosis and white sclerotic ring at the tip of the foreskin Fig. 5.28 Lichen sclerosus sclerotic plaque Fig. 5.30 Lichen sclerosus posthitis, waisting, and preputial atrophy as well as a typical white sclerotic ring that usually manifests at the tip of the foreskin (Fig. 5.29 ). Waisting may also occur (Figs. 5.30 and 5.31 ). Mucosal fi ssures, petechiae, telangiectasias, and hemorrhagic lesions may occur, leading to preputial adhesions to the glans penis. Frenular involvement may lead to destruction of this structure, and loss of anatomical de fi nition of the coronal sulcus at advanced stages. Fig. 5.31 Lichen sclerosus involvement of shaft, prepuce, and frenulum (note waisting)

Common in Geriatric Group/Almost Exclusive to Uncircumcised Individuals 5.5 Penile Lichen Sclerosus 99 Fig. 5.34 Lichen sclerosus with frenular sclerosis Fig. 5.32 Lichen sclerosus balanitis, perimeatal ecchymosis, and meatal slit Fig. 5.35 Lichen sclerosus leukoplastic plaque with ulcer from previous biopsy ( black arrow ) (note also scrotal seborrheic keratosis white arrow ) Fig. 5.33 Lichen sclerosus preputial and frenular involvement Balanitis xerotica obliterans (BXO) refers to a severe damage to the glans penis from chronic, undiagnosed, or even untreated LS (Figs. 5.40, 5.41, 5.42, 5.43, 5.44, 5.45, and 5.46 ). (Figs. 5.32, 5.33, 5.34, 5.35, and 5.36 ). Postinfl ammatory hyper- and hypopigmentation, atrophy, erosions, ulcerations, and phimosis may be observed (Figs. 5.30, 5.35, 5.36, 5.37, 5.38, and 5.39 ). The urethral meatus and fossa navicularis may be affected, resulting in urethral involvement in 20% of patients (Depasquale et al. 2000 ) (Figs. 5.41, 5.42, 5.43, 5.44, 5.45, and 5.46 ); urinary obstruction as a result of meatal stenosis

100 5 Papulosquamous Dermatoses a b Fig. 5.38 Lichen sclerosus balanitis xerotica, frenular destruction and preputial atrophy Fig. 5.36 Lichen sclerosus (a) Postinflammatory hypopigmentation, mucosal atrophy, super fi cial glans erosions. ( b ) Loss of architeture of coronal sulcus lateral view Fig. 5.39 Lichen sclerosus hypopigmentation involving prepuce and shaft of the penis Fig. 5.37 atrophy Lichen sclerosus phimosis and preputial was reported in 47% of the patients studied by Bainbridge et al. ( 1971 ). The urethra is initially affected at the level of the meatus ( pin hole ), but with long-standing disease the mucosal involvement and spongio fi brosis can progress proximally as far back as the posterior urethra.

Common in Geriatric Group/Almost Exclusive to Uncircumcised Individuals 5.5 Penile Lichen Sclerosus 101 Fig. 5.42 Lichen sclerosus telangiectasias and preputial adhesions (note perimeatal and meatal involvement) Fig. 5.40 Lichen sclerosus balanitis, glans erosion and perimeatal disease Fig. 5.43 Lichen sclerosus telangiectasias and preputial adhesions to the glans penis (same patient as in Fig. 5.42 ) Fig. 5.41 Lichen sclerosus balanitis xerotica, perimeatal involvement, and meatal stenosis 5.5.5 Diagnosis Clinical examination. Biopsy and histological examination. Urethroscopy and retrograde urethrography are mandatory to determine the proximal extent of disease if urethral involvement is suspected. Biopsy is recommended in all patients suspected of having LS (Pugliese et al. 2007 ); however, the biopsy of chronically hyperplastic or ulcerated lesions of LS is mandatory to rule out SCC.

102 5 Papulosquamous Dermatoses Fig. 5.46 Lichen sclerosus balanitis and meatal stenosis Fig. 5.44 Lichen sclerosus balanitis and meatal narrowing Dermal lymphocytic infiltration. Atrophic epidermis with loss of rete pegs. 5.5.7 Differential Diagnosis Penile carcinoma in situ (PIN), leukoplakia, scleroderma. 5.5.8 Treatment Fig. 5.45 Lichen sclerosus balanitis, perimeatal sclerosis, and ecchymosis 5.5.6 Histology (Fig. 5.47a, b ) Hyperkeratosis. Basal cells hydropic degeneration. Sclerosis of the subepithelial collagen. Collagen homogenization at the upper layer of the dermis. Corticosteroids recommended for mild disease (limited to the foreskin) in young individuals presenting minimal scar formation. According to Kiss et al. ( 2005 ), topical steroids may improve initial symptoms and slow disease progression. Clobetasol propionate (Temovate) 0.05% cream or ointment applied once or twice daily for 2 3 months with gradual dose reduction is the topical steroid most commonly used. The side effects are cutaneous atrophy, hypopigmentation, contact sensitivity, and adrenal suppression. Moreover, relapses may occur when treatment is discontinued. Clobetasol propionate is not FDA approved for this indication. Tacrolimus topical (Protopic) tacrolimus, a topical calcineurin inhibitor, is a highly selective immune modulator agent. Tacrolimus ointment is applied twice a day to the affected area, and it has shown lower relapse rates when compared with topical betamethasone (Ebert et al. 2007 ).

Systemic Disease 5.6 Seborrheic Dermatitis 103 Fig. 5.47 ( a ) Lichen sclerosus (LS). ( b ) LS exhibiting marked epithelial hyperplasia (increased risk of malignant progression) (Photograph courtesy of Filomena Marino Carvalho MD. Source : Rosenblatt and de Campos Guidi ( 2009 ) ) a b Circumcision recommended for early disease limited to the glans penis and foreskin (Depasquale et al. 2000 ). CO2 laser A recent study performed by Windahl (2006) has shown good long-term results (80% of patients asymptomatic and without visible lesions) after CO 2 laser treatment. Wide meatotomy (followed by long-term topical steroid or tacrolimus therapy), meatoplasty, and sometimes urethroplasty may be required for meatal stenosis or LS-related urethral strictures (Virasoro and Jordan 2008 ). Long-term follow-up is advised to detect possible progression to premalignant disease or SCC 5.6 Seborrheic DermatitisSystemic Disease 5.6.1 Overview Seborrheic dermatitis (SD) is a common dermatological disorder that frequently affects the genital region (Mallon et al. 2000 ). The penis and

104 5 Papulosquamous Dermatoses Fig. 5.48 Seborrheic dermatitis patient complained of moderate penile pruritus Fig. 5.50 Seborrheic dermatitis with fi ssures at the base of the penis 5.6.2 Epidemiology SD affects 1 3% of immunocompetent adults, and it is more prevalent in men than in women (Gupta and Bluhm 2004 ). In addition, a family history of the disease can be also found. Fig. 5.49 Seborrheic dermatitis involving the inguinoscrotal and pubic region (partially shown) scrotum may be the sole sites involved, but nongenital areas (scalp, face, ears, axillae, and trunk) commonly manifest the disease. The etiology of SD is poorly understood and includes a possible abnormal hypersensitivity to the cutaneous yeast Malassezia (previously known as Pityrosporum). SD may be associated with other skin diseases (rosacea, blepharitis, acne vulgaris), and some patients may also have a tendency to develop psoriasis ( sebopsoriasis or seboriasis ). SD symptoms and clinical manifestation varies in severity among individuals and with time, and it can be caused or exacerbated by certain conditions such as stress, fatigue, weather extremes, oily skin, and obesity. The disease is fairly common in those infected by human immunode fi ciency virus (HIV), and it may be severe in the fi nal stages of the HIV infection (Cedeno-Laurent et al. 2011 ). Moreover, in a recent prospective study, Lally et al. ( 2010 ) have shown that SD is also common in immunosuppressed renal transplant recipients (RTRs), but less frequent than in HIV immunosuppressed patients. 5.6.3 Symptoms Mild to moderate pruritus and mild local soreness. 5.6.4 Clinical Findings Nonspecific balanoposthitis (intermittent penile rash, slight erythema, scaling of varying intensity, fi ssures) (Figs. 5.48, 5.49, 5.50, 5.51, 5.52, and 5.53 ); perifollicular or follicular involvement may also occur (Fig. 5.54 ). 5.6.5 Diagnosis Clinical examination of nongenital sites may assist the diagnosis. Mycological test (to exclude Tinea). Biopsy and histology is not often required. 5.6.6 Differential Diagnosis Psoriasis, balanoposthitis.

Systemic Disease 5.7 Reactive Arthritis 105 Fig. 5.53 shaft Seborrheic dermatitis involving the penile Fig. 5.51 Seborrheic dermatitis involving upper thigh Fig. 5.54 Seborrheic dermatitis perifollicular and follicular involvement Fig. 5.52 areas Seborrheic dermatitis involving intertriginous Topical antifungals alone are effective and safe (without the side-effects of topical corticosteroids), but may be associated with mild to moderately potent corticosteroids. Immunomodulators (pimecrolimus and tacrolimus) may also be used when the disease is unresponsive to corticosteroids. Oral antifungals and/or antibiotics (tetracycline) may be indicated for severe cases associated with folliculitis or in immunosuppressed individuals. 5.6.7 Treatment Reassurance of a benign condition. Mild emollients and soap substitutes may be helpful. 5.7 Reactive ArthritisSystemic Disease Synonym Reiter s syndrome, Reiter s disease, Reiter s urethritis, circinate balanitis.

106 5 Papulosquamous Dermatoses 5.7.1 Overview Reactive arthritis (previously known as Reiter s syndrome) is a multisystem disease commonly triggered by a genitourinary or gastrointestinal tract infection. The exact pathophysiology is still unknown, although infectious and immune factors are likely involved. The disease is characterized by the classic triad of conjunctivitis, urethritis, and arthritis, and most cases usually develop after a short latent period (usually within 1 4 weeks). Agents most often implicated are the intestinal bacteria Shigella, Salmonella, Yersinia, and Campylobacter, as well as the sexually transmitted pathogens Chlamydia trachomatis and Ureaplasma urealyticum. Reactive arthritis is associated with the HLA- B27 genotype, and the condition has been reported in HIV/AIDS infection (Altman et al. 1994 ). Fig. 5.55 Reactive arthritis Keratoderma blennorrhagicum ( Source: Wu and Schwartz ( 2008 ). Reproduced with permission from Elsevier) 5.7.2 Epidemiology The condition usually affects adult men, although reactive arthritis cases have also been reported in women, children, and geriatric patients. 5.7.3 Symptoms Fever, malaise, fatigue, anorexia, diarrhea, weight loss. Genitourinary symptoms (dysuria, urgency, frequency, mucopurulent urethral discharge, vaginal discharge, balanitis). Asymmetric joint pain (especially of the knees, ankles and feet) and low back pain (spondylitis, sacroiliitis). Ocular symptoms (conjunctivitis, photophobia, pain). 5.7.4 Clinical Findings Dermatologic and urogenital manifestations are commonly observed and include keratoderma blennorrhagicum (pustular plantar lesions) (Figs. 5.55 Fig. 5.56 Reactive arthritis Keratoderma blennorrhagicum on the trunk may be clinically indistinguishable from pustular psoriasis ( Source: Wu and Schwartz ( 2008 ). Reproduced with permission from Elsevier) and 5.56 ), painless oral ulcers (Fig. 5.57 ), nail changes, circinate balanitis (psorasiform lesions on glans penis) (Figs. 5.58 and 5.59 ), ulcerative vulvitis, urethritis, prostatitis, cystitis, and cervicitis. Moreover, according to Quint et al. ( 2010 ), the dermatologic lesions of reactive arthritis are mainly induced by Chlamydia trachomatis. Pattern of circinate balanitis Uncircumcised males occasionally moist, well-demarcated shallow erosions with serpiginous, micropustular borders Circumcised males crusted and/or hyperkeratotic plaques

Systemic Disease 5.7 Reactive Arthritis 107 Fig. 5.57 Reactive arthritis tongue erosions ( Source : Wu and Schwartz ( 2008 ). Reproduced with permission from Elsevier) Fig. 5.59 Circinate balanitis (Source: Wiesner and Kaufman, Public Health Image Library, Center for Disease Control and Prevention, USA) 5.7.6 Histology Histopathology of penile lesions (balanitis circinata) resembles that of pustular psoriasis (Kanerva et al. 1982 ). 5.7.7 Differential Diagnosis Pustular psoriasis, gonococcal arthritis, Behçet s disease, balanoposthitis, lichen sclerosus, irritant, and allergic contact dermatitis. Fig. 5.58 Reactive arthritis circinate balanitis ( Source : Schwartz (2011). Reprinted with permission from Medscape.com. Available at: http://emedicine.medscape. com/article/1107206-overview ) 5.7.5 Diagnosis Screening of urine or synovium fl uid for Chlamydia trachomatis help distinguish between reactive arthritis and psoriatic arthritis. 5.7.8 Treatment Nonsteroidal anti-inflammatory drugs (NSAIDs) are recommended for the articular manifestations of the disease. Topical corticosteroids are used for mucocutaneous lesions. Topical pimecrolimus may be indicated for balanitis circinata erosiva (de Almeida and de Oliveira Filho 2005 ).

108 5 Papulosquamous Dermatoses a b Fig. 5.60 ( a, b ) Numular eczema the condition usually involves hands, extensor surfaces of the forearms, upper arms, legs, thighs, and feet ( Source : Halberg (2011). Reproduced with permission from Elsevier) Azithromycin 1,000 mg as a single oral dose is prescribed for genitourinary Chlamydia trachomatis infection (sexual partners should also be treated). A recent prospective, double-blind, triple-placebo trial performed by Carter et al. ( 2010 ) has shown that a 6-month course of combination antibiotics (doxycycline, rifampin, and azithromycin) is an effective treatment for chronic Chlamydia-induced reactive arthritis. 5.8 Chronic Exudative and Lichenoid Dermatosis Synonym Sulzberger-Garbe disease, oid oid disease. 5.8.1 Overview Sulzberger-Garbe disease (Sulzberger et al. 1959 ) preferably affects middle aged and elderly men, with a predilection for the Jewish race. The penis and scrotum are commonly involved, as well as neck, axillae, and fl exural arm surfaces. There is controversy in the literature regarding the justi fi cation of exudative discoid and lichenoid dermatosis as a clinical entity (Rongioletti et al. 1989 ). Stevens and Ackerman ( 1984 ) have even reported that the condition is a manifestation of nummular dermatitis (Figs. 5.60a, b ), although the penis and scrotum are infrequently involved with the latter disorder (Braun-Falco 2000 ). However, a histopathological study of 13 cases performed by Sachs and Kirsch ( 1947 ) has suggested that there was

References suf fi cient evidence in the microscopic picture to establish a diag nosis of exudative discoid and lichenoid dermatosis. 5.8.2 Symptoms Intense pruritus (Sachs and Kirsch 1947 ). Clinical Findings. Chronic course of exsudative discoid lesions and licheni fi cation involving resolving plaques (Braun-Falco 2000 ). Recurrences are frequently observed. 5.8.3 Differential Diagnosis Nummular dermatitis, lymphoma, chronic eczema. 5.8.4 Treatment Short course of systemic corticosteroids, as the disease tends to be resistant to topical preparations (Braun-Falco 2000 ). Azathioprine may be used when lesions fail to respond to oral prednisolone (Freeman et al. 1984 ). References Alinovi A, Barella PA, Benoldi D (1983) Erosive lichen planus involving the glans penis alone. Int J Dermatol 22:37 38 Altman J, Perry HO (1961) The variations and course of lichen planus. Arch Dermatol 84:179 191 Altman EM, Centeno LV, Mahal M, Bielory L (1994) AIDS-associated Reiter s syndrome. Ann Allergy 72:307 316 Arizaga AT, Gaughan MD, Bang RH (2002) Generalized lichen nitidus. Clin Exp Dermatol 27:115 117 Aste N, Pau M, Ferreli C, Biggio P (1997) Lichen planus in a child requiring circumcision. Pediatr Dermatol 14:129 130 Bain L, Geronemus R (1989) The association of lichen planus of the penis with squamous cell carcinoma in situ and with verrucous squamous carcinoma. J Dermatol Surg Oncol 15:413 417 Bainbridge DR, Whitaker RH, Shepheard BG (1971) Balanitis xerotica obliterans and urinary obstruction. Br J Urol 43:487 491 109 Braun-Falco O (2000) Dermatology. Springer, Heidelberg Buechner SA (2002) Common skin disorders of the penis. BJU Int 90:498 506 Carter JD, Espinoza LR, Inman RD, Sneed KB, Ricca LR, Vasey FB, Valeriano J, Stanich JA, Oszust C, Gerard HC, Hudson AP (2010) Combination antibiotics as a treatment for chronic Chlamydia-induced reactive arthritis: a double-blind, placebo-controlled, prospective trial. Arthritis Rheum 62:1298 1307 Cedeno-Laurent F, Gomez-Flores M, Mendez N, Ancer- Rodriguez J, Bryant JL, Gaspari AA, Trujillo JR (2011) New insights into HIV-1-primary skin disorders. J Int AIDS Soc 14:5 Chalmers RJ, Burton PA, Bennett RF, Goring CC, Smith PJ (1984) Lichen sclerosus et atrophicus. A common and distinctive cause of phimosis in boys. Arch Dermatol 120:1025 1027 Christophers E (2001) Psoriasis epidemiology and clinical spectrum. Clin Exp Dermatol 26:314 320 Coldiron BM, Jacobson C (1988) Common penile lesions. Urol Clin North Am 15:671 685 Cubilla AL, Velazquez EF, Young RH (2004) Pseudohyperplastic squamous cell carcinoma of the penis associated with lichen sclerosus. An extremely well-differentiated, nonverruciform neoplasm that preferentially affects the foreskin and is frequently misdiagnosed: a report of 10 cases of a distinctive clinicopathologic entity. Am J Surg Pathol 28:895 900 Davis DA, Skidmore RA, Woosley JT (1996) Lichen nitidus. Urology 47:573 de Almeida HL Jr, de Oliveira Filho UL (2005) Topical pimecrolimus is an effective treatment for balanitis circinata erosiva. Int J Dermatol 44:888 889 Depasquale I, Park AJ, Bracka A (2000) The treatment of balanitis xerotica obliterans. BJU Int 86:459 465 Dobbs CR, Murphy SJ (2004) Lichen nitidus treated with topical tacrolimus. J Drugs Dermatol 3:683 684 Ebert AK, Vogt T, Rosch WH (2007) Topical therapy of balanitis xerotica obliterans in childhood. Long-term clinical results and an overview. Urologe A 46: 1682 1686 Farber EM, Nall ML (1974) The natural history of psoriasis in 5,600 patients. Dermatologica 148:1 18 Farber EM, Nall L (1992) Genital psoriasis. Cutis 50:263 266 Farber EM, Bright RD, Nall ML (1968) Psoriasis. A questionnaire survey of 2,144 patients. Arch Dermatol 98:248 259 Fiumara NJ (1976) Psoriasis of the penis: Koebner reaction. Following oral genital exposure. J Am Vener Dis Assoc 3:59 60 Freeman K, Hewitt M, Warin AP (1984) Two cases of distinctive exudative discoid and lichenoid chronic dermatosis of Sulzberger and Garbe responding to azathioprine. Br J Dermatol 111:215 220 Goldman BD (2000) Common dermatoses of the male genitalia. Recognition of differences in genital rashes and lesions is essential and attainable. Postgrad Med 108(89 91):89 91, 95 96

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