Penile lichen sclerosus: follow-up study

INTRODUCTION: The aim of this work was to study the long-term course of patients with penile lichen sclerosus and atrophicus. PATIENTS AND METHODS: We reviewed the files of 16 patients followed at our university center from 1982 to 1997 for pathologically proven penile lichen sclerosus and atrophicus. Age at disease onset, duration of the disease course, presence of a triggering factor, initial localization, signs and symptoms, treatment given and its efficacy were recorded. RESULTS: Mean age at diagnosis was 52 years. Inaugural prepuce localizations were observed in 6 cases, involving the distal part of the penis and the glan in 3 cases. Concomitant involvement of the glan and the balanopreputial groove were seen in 6 cases. The localization was limited to the glan in one case. Six patients underwent posthectomy and one had dorsal plasty of the prepuce. The 9 other patients were treated medically with topical corticosteroids and/or androgens. One patient had died prior to evaluation. Among the 9 patients given medical treatment, lichen sclerosus persisted in 7, one developed a squamous cell carcinoma of the penis and one was cured. Among the 6 cases of surgical treatment, 5 were cured and 1 had persistent lesions. Considering the initial localization, long-term course showed that 7 of the 8 patients with a prepuce localization (alone or in association with glan involvement) were cured while the 7 patients with a lesion of the balanopreputial groove (alone or in association with glan involvement) or a lesion of the glan alone still had lichen sclerosus and atrophicus. The non parametric Fischer test showed that the chances of cure were better for preputial localizations than for balanopreputial localizations (p = 0.001) and that surgical treatment was more effective than medical treatment. DISCUSSION: This study of a small number of patients highlighted two points: prepuce localizations of lichen sclerosus and atrophicus cure better than balanopreputial localizations, and that medical treatment is partially effective in balanopreputial localizations. One patient developed squamous cell carcinoma of the penis. As the level of the cancer risk remains unknown, it would be important to provide the most effective treatment in all cases of penis lichen sclerosus and atrophicus.     

Penile lichen sclerosus after allogeneic stem cell transplantation

Graft-versus-host disease (GVHD) often complicates allogeneic stem cell transplantation (SCT) and affects mainly the gut, liver, lung and skin. The microscopic morphological features of late-phase sclerodermatous chronic GVHD in the skin, namely epidermal atrophy, lymphoplasmacytic infiltration, dense dermal fibrosis and adnexal atrophy, are histologically indistinguishable from those in sporadic systemic sclerosis, morphoea and the related condition of lichen sclerosus. Mucosal orifices including those of the genitourinary system may be severely affected. We present three SCT recipients with chronic GVHD and severe posthitis leading to phimosis requiring surgery. The excised prepuces showed features of lichen sclerosus including epidermal atrophy and a subepidermal zone of eosinophilic, homogeneous and hyalinized collagen above a band-like lymphoplasmacytic infiltrate. These cases add further evidence to support the notion that penile lichen sclerosus should be included within the expanding sclerodermoid spectrum of late-stage cutaneous chronic GVHD.     

Penile lichen sclerosus: Correlation between histopathologic features and risk of cancer

The relationship between penile lichen sclerosus (LS) and cancer development has not been clearly assessed so far. In order to define these histological features of LS that may indicate or precede a malignant degeneration, 104 biopsy specimens from 86 patients with LS of the glans (90.5%) and from 9 patients with a penile malignancy (7 squamous cell carcinomas, 1 in situ carcinoma, and 1 verrucous carcinoma) arising on LS (9.5%) were reviewed. Three different histopathologic LS patterns were identified: pattern 1 with a prominent lichenoid inflammatory infiltrate in the dermis (9%), pattern 2 characterized by a band-like infiltrate separated from the epidermis by a band of dermal sclerosis (44%), and pattern 3 showing prominent sclerosis with minimal or absent inflammatory infiltrate (9%). These patterns have previously been described in vulvar LS, and have been considered typical of early, mature, and late LS, respectively. In our study, we also found a fourth pattern in 38% of cases, with overlapping features between the first and third pattern, occasionally showing areas of epidermal thickening, with loss of the normal keratinocyte cytoarchitectural differentiation, mitoses and apoptotic cells. In our opinion, the histological features observed in this last pattern may be interpreted as areas of disease reactivation within a chronic stage. Furthermore, 7 out of 9 cases of penile cancer from our series (78%) were associated with this pattern, suggesting that it may correlate with a malignant degeneration.     

UVA1 phototherapy for genital lichen sclerosus

BACKGROUND: Lichen sclerosus (LS) is characterized histologically by an inflammatory T-cell infiltrate, sclerosis and thickening of the dermis, and epidermal atrophy. Ultraviolet (UV) A1 therapy has been shown to be effective in the management of morphea and scleroderma, diseases that have some histological and clinical similarities with LS, and more recently in extragenital LS. AIM: To determine the effectiveness of UVA1 therapy for genital LS. METHODS: Seven women with severe genital LS uncontrolled by ultrapotent topical corticosteroids, with a median age of 62 years (range 48-78) and disease duration of 6-47 years, were treated with UVA1 therapy from a high output source. After completion of UVA1 therapy, a clinician and the patient graded the overall response of symptoms and physical signs. RESULTS: Five patients improved with therapy. Three obtained moderate improvement in overall disease severity and two had minimal improvement. Of these five, one relapsed within 3 months and another after a year. Both had a further course of UVA1 therapy, resulting in minimal improvement in one and moderate improvement in the other. In the remaining three, disease severity had improved to a point where intermittent use of topical corticosteroids resulted in acceptable control. DISCUSSION: UVA1 therapy may be of benefit in the management of vulval LS, a disease that is often poorly responsive to standard therapies. The therapy is well tolerated and could provide an acceptable therapeutic option for patients with severe disease.     

Risk factors for genital lichen sclerosus in men

Background Lichen sclerosus (LS) is an inflammatory disease of the skin and mucous membranes. Its aetiology is still unknown. Objectives To determine risk factors for genital LS in men. Methods In a case–control study, 73 patients with LS, consecutively diagnosed at the City Dispensary for Skin and Venereal Diseases in Belgrade, were compared with 219 male patients visiting the same institution because of tinea cruris. Univariate and multivariate logistic regression analyses were used for analysis of data collected. Results According to multivariate logistic regression analysis, risk factors for male LS were as follows: a personal history of genital injury [odds ratio (OR) 28·1, 95% confidence interval (CI) 5·2–150·8], vitiligo (OR 23·1, 95% CI 2·2–240·2), alopecia areata (OR 8·8, 95% CI 1·1–68·5) and hypercholesterolaemia (OR 3·1, 95% CI 1·1–8·2), and a family history of alopecia areata (OR 24·3, 95% CI 2·1–280·7), diseases of the thyroid gland (OR 9·1, 95% CI 2·3–36·2) and other autoimmune diseases (OR 8·6, 95% CI 1·3–58·6). Conclusions The results of the present study are in line with the hypothesis that trauma of the penis is a possible trigger of symptoms in genetically predisposed individuals and that personal and family histories of autoimmune disorders are risk factors for male LS.     

Angiokeratoma-like changes in extragenital and genital lichen sclerosus

Hemorrhagic blisters have rarely been described developing in the background of either genital or extragenital lichen sclerosus and have invariably been designated clinically as telangiectatic, hemorrhagic or bullous lichen sclerosus. We describe three patients with extragenital and genital lichen sclerosus, who presented clinically with hemorrhagic plaques and/or papules. In addition to the classical histology of lichen sclerosus, dilated, congested and focally thrombosed vascular channels lined by flat endothelium were seen within the sclerotic dermal collagen. They were in close proximity to and even in contact with the overlying epidermis and thus mimicked an angiokeratoma. Angiokeratoma-like changes in lichen sclerosus represent secondary features because of damage to the dermis by lichen sclerosus and are characterized histologically by ectatic thin-walled vascular spaces in the papillary dermis intimately associated with the epidermis. Increased venous pressure, local trauma, degenerative changes in the elastic tissue of the vessel wall and/or surrounding supportive tissue, as well as abnormalities in the extracellular protein network, appear to be implicated in their pathogenesis.     

Ultrapotent topical corticosteroid treatment of childhood genital lichen sclerosus.

OBJECTIVE: To observe the clinical effects of short-term application of ultrapotent topical corticosteroid on symptomatic genital lesions of lichen sclerosus in pediatric patients. DESIGN: Case series of 10 prepubertal girls with genital lichen sclerosus. Ultrapotent topical corticosteroids were applied twice daily for 6 to 8 weeks and patients were reexamined at completion of treatment. Long-term follow-up over 6 months to 3 years. SETTING: Pediatric dermatology clinic (referral center). PATIENTS: Ten prepubertal girls with typical clinical features of genital and/or perianal lichen sclerosus. INTERVENTION: Topical ultrapotent corticosteroid ointment was applied sparingly to affected areas for 6 to 8 weeks. MAIN OUTCOME MEASURE: Improvement of erythema, whitening erosions, and atrophy. Subjective improvement of symptoms. RESULTS: All patients showed partial or total subsistence of signs and symptoms of lichen sclerosus. Frequency and severity of recurrences varied, but patients responded within a few days to reapplication of ultrapotent topical corticosteroid. No significant adverse effects were noted after the initial 6- to 8-week course of therapy or during the 6-month to 3-year follow-up period. CONCLUSION: A 6- to 8-week course of ultrapotent topical corticosteroid is a safe and effective treatment for genital lichen sclerosus in pediatric patients.     

Male genital lichen sclerosus in recipients of bone marrow transplants

We describe two patients who received haematopoietic stem cell marrow transplantation, and developed male genital lichen sclerosus (MGLSc), one of whom also had squamous carcinoma in situ (Bowen disease). MGLSc has previously been associated with graft‐versus‐host disease. Various aetiological factors for LSc have been proposed, including a role for chronic occluded epithelial exposure to urine. A number of factors imply that the risk of malignant transformation in this bone marrow transplant group is likely to be higher than the overall figure of 2–9% cited for MGLSc. It is vital, therefore, that clinicians involved in the care of those with haematological malignancies are adequately prepared to examine the genitals of their patients, and to recognize and refer any suspect penile lesions.