HIV-related Merkel cell carcinoma: A report of three cases from the UK.

Merkel cell carcinoma (MCC) of the skin is a rare, aggressive and often fatal neuroendocrine skin cancer. The incidence of MCC has significantly increased in the last decades. Factors that have been associated with the development of MCC include infection with Merkel Cell polyomavirus (MCPyV), ultraviolet exposure, hematologic malignancies and immunosuppression.We present three cases of patients living with HIV who were diagnosed with MCC. HIV cases associated with MCC have been rarely reported and to our knowledge, not yet before in the UK.

Pseudoepitheliomatous keratotic and micaceous balanitis: a series of eight cases.

BACKGROUND: Pseudoepitheliomatous keratotic and micaceous balanitis (PEKMB) is a clinicopathological entity characterized clinically by micaceous scale on the glans, and histologically by acanthosis, hyperkeratosis and pseudoepitheliomatous hyperplasia. We present a series of eight cases of this rare condition, the first series of more than two cases to be reported. OBJECTIVES: To determine the clinical and histological characteristics of cases of PEKMB, and evaluate treatments used and clinical course. METHODS: This monocentric case series was conducted at the University College London Hospitals tertiary male genital dermatology clinic between April 2018 and August 2020. Eight patients with PEKMB were evaluated. Data were collected on demographics, clinical presentation, histological features, presence of human papilloma virus (HPV), history of lichen sclerosus, treatment of PEKMB and subsequent response, and presence or development of squamous cell carcinoma (SCC) or penile intraepithelial neoplasia (PeIN) during follow-up. RESULTS: Eight Caucasian males presented with clinical and histological evidence of PEKMB. Seven had a background of lichen sclerosus; two had failed treatment with superpotent topical steroids and four had symptoms for three or more years prior to circumcision. There was no clinical or histological relationship with HPV infection, and p16 staining was negative. HPV PCR, performed in two cases, was negative. Basal atypia, insufficient to amount to PeIN, was present in six patients. One patient progressed to PeIN during follow-up, and no patient progressed to invasive malignancy. Five patients were treated successfully with glans resurfacing and split skin graft reconstruction. CONCLUSIONS: Our observations demonstrate that PEKMB represents a form of chronic, undiagnosed or misdiagnosed, inadequately treated or treatment refractory, unstable lichen sclerosus. The significant potential for squamous carcinogenesis (differentiated PeIN and verrucous carcinoma) can be mitigated by timely diagnosis and treatment. Glans resurfacing and split skin graft reconstruction appears to be a successful treatment modality in patients with refractory disease.

Lichen sclerosus following genital piercing.

Male genital lichen sclerosus (MGLSc) is a chronic inflammatory dermatosis that predominantly affects uncircumcised males. Several aetiological factors have been described, with growing evidence supporting chronic urine exposure of susceptible epithelium being crucial to its pathogenesis. We describe a 45-year-old man with a 2-year history of MGLSc at the site of a urethrocutaneous fistula secondary to a penile piercing. To our knowledge, this is the first documented case of MGLSc occurring at the site of a genital piercing.

The management of penile intraepithelial neoplasia (PeIN): clinical and histological features and treatment of 345 patients and a review of the literature.

INTRODUCTION: Penile intraepithelial neoplasia (PeIN) is a histological term for precancerous penile lesions. PeIN is important due to the high morbidity and mortality associated with progression to penile squamous cell carcinoma (PSSC). But PeIN is rare, contributing to a limited evidence-base for the relative efficacy of available treatment options. OBJECTIVES & METHODS: To consolidate and expand knowledge about PeIN and its treatment, we describe the clinical and histological characteristics, treatments and outcomes of 345 patients with PeIN, managed by our multidisciplinary team. Our results are compared and contrasted with those in the literature, following comprehensive review. RESULTS: 8.7% of patients had concomitant, invasive PSCC, whilst 91.3% demonstrated PeIN alone. 84% had undifferentiated PeIN, and 10.7% differentiated PeIN (5.2%, not specified). Clinical or histological evidence of HPV alone was present in 58%; features of lichen sclerosus alone in 12%; features of both in 29.4%. Only 14.4% of patients could be treated solely with topical agents or cryotherapy, whereas the remaining 85.6% underwent some form of surgical intervention, circumcision being the mainstay. Just 2.6% progressed to PSCC. CONCLUSIONS: Clinical management of PeIN can be rationally optimized with excellent outcomes. Circumcision is important. Topical treatments alone are disappointing.

Male genital lichen sclerosus associated with urological interventions and microincontinence: a case series of 21 patients.

BACKGROUND: Male genital lichen sclerosus (MGLSc) is an acquired, chronic, inflammatory cutaneous disease associated with significant morbidity and squamous cell carcinoma of the penis. Consideration of all of the evidence suggests that chronic exposure of susceptible epithelium to urinary occlusion by the foreskin is the most likely pathomechanism. MGLSc never occurs in men who were circumcised at birth, and has been associated with trauma, instrumentation and anatomical abnormalities, e.g. frank hypospadia that results in microincontinence. AIM: To describe 21 patients who developed MGLSc following urological diagnoses and procedures. METHODS: We conducted a retrospective review of patients with a diagnosis of MGLSc whose symptoms related to urological procedures who attended or saw one of the authors (CBB) privately during the period June-October 2018. RESULTS: In total, 21 patients (mean age 59 years) were identified. The referrals came from the local urology departments, primary care or extramural dermatology services. Most of the patients were uncircumcised men. All had developed symptoms and signs of MGLSc within 5 years following their urological procedure; on examination, 30% of the patients were found to have damp penile skin due to microincontinence. Of the 21 patients, 10 had undergone radical prostatectomy for prostate cancer, 4 had a diagnosis of Peyronie disease, 4 had undergone multiple cystoscopies and urethroscopies, 2 had undergone surgery on the bladder neck and 1 had undergone implantation of a penile prosthesis to treat erectile dysfunction. CONCLUSION: This case series further strengthens the urinary occlusion hypothesis for the causation of MGLSc. It is important to recognize that urological interventions can create incompetence of the naviculomeatal valve post voiding. In uncircumcised men, this creates a risk factor for MGLSc that was not previously present. Occlusion, the phenomenon of koebnerization and currently unelucidated epithelial susceptibility factors lead to inflammation, sclerosis and cancer. Patients and urologists should be aware of these possibilities and preventative measures instituted, e.g. adaptive voiding habits and barrier protection.

Patient characteristics in tardive COVID-19 pseudoperniosis: a case series of 16 patients.

BACKGROUND: Acute pseudoperniosis (PP) has a recognized association with COVID-19 and tends to occur without cold precipitation in young, healthy patients, often without a clear history of COVID-19. These lesions usually resolve within 2 weeks and without long-term sequelae. In the early months of 2021, patients with delayed and protracted PP began to emerge. We have called this presentation 'tardive COVID-19 PP (TCPP)'. AIM: To consolidate and expand knowledge on TCPP, we describe the clinical characteristics, treatments and outcomes of 16 patients with TCPP who were reviewed by our outpatient dermatology service. RESULTS: The initial clinical manifestations were erythema, swelling and PP of the fingers in 56.2%, and of the toes in 31.2%, desquamation in 56.2% and acrocyanosis in 12.5%. Ten patients had eventual involvement of all acral sites. The median duration of symptoms was 191 days. Six patients reported close contact with a confirmed or suspected case of COVID-19, but only two had positive COVID-19 tests. Four patients experienced complete or almost complete resolution of symptoms, while the rest remain under active treatment. CONCLUSION: Unlike acute PP, TCPP has a protracted and delayed presentation that is typically associated with profound acrocyanosis. Patients with TCPP represent a new phenomenon that is part of the post-COVID-19 syndrome, with risk factors and pathophysiology that are not yet fully understood. Our data indicate that likely predisposing factors for developing TCPP include young age, a preceding history of cold intolerance and an arachnodactyloid phenotype. Anorexia, connective tissue disorders or sickle cell trait may also predispose to TCPP. In addition, low titre antinuclear antibody positivity, the presence of cryoglobulins, or low complement levels may represent further risk factors. Finally, prolonged low temperatures are also likely to be contributing to the symptoms.

Penile lymphoedema: approach to investigation and management.

BACKGROUND: Penile lymphoedema (with and without cellulitis) is a rare, often chronic, clinically heterogeneous entity with an uncertain pathogenesis and an important differential diagnosis. It creates significant physical and psychosexual morbidity, and presents considerable therapeutic challenges. The existing literature is limited. AIM: To describe and share our updated cumulative experience of a cohort of patients with penile lymphoedema. METHODS: This was a retrospective review of the case records of patients with chronic penile lymphoedema seen in two dedicated male genital dermatology clinics between January 2011 and July 2016. RESULTS: In total, 41 cases were identified. Over a third had Crohn disease (CD) (which was occult in one-third of these), and over a third had serological evidence of streptococcal infection. All patients responded to systemic antibiotics and specialized urological surgery circumcision and excision). CONCLUSIONS: Penile lymphoedema should be investigated to exclude underlying pathology especially CD and streptococcal infection. Treatment with antibiotics should be considered early and long term to try to preserve the foreskin: most patients are uncircumcised. Some patients may benefit from a course or courses of oral steroids. The development of gross dysfunction of the prepuce usually dictates circumcision and excision of lymphoedematous tissue once the situation is medically stabilized.

Localized genital bullous pemphigoid.

Genital bullous pemphigoid (GBP) is a rare localized subset of bullous pemphigoid (BP). BP is characterized by autoantibodies against hemidesmosomes, which are involved in the structural integrity of the epidermis, and this results in subepidermal blistering. Typically, GBP affects women and children. We report an adult male who presented with a scrotal rash and blisters that developed into erosions. Only two previous cases in men have been reported. Immunofluoresence and histopathology confirmed the diagnosis of BP. The patient responded to mycophenolate mofetil and doxycycline.

The diagnosis and management of male genital lichen sclerosus: a retrospective review of 301 patients.

INTRODUCTION: Male genital lichen sclerosus (MGLSc) is an acquired, chronic, inflammatory skin disease that is associated with significant morbidity and squamous cell carcinoma of the penis (PSCC). However, some clinical, diagnostic and management controversies endure, including the relationship with penile intraepithelial neoplasia (PeIN). OBJECTIVES: To clarify clinical presentations, diagnostic approaches, histological findings, response to treatment and the relationship with PeIN. METHODS: Retrospective review of patients with a diagnosis of MGLSc who attended a specialist male genital dermatoses clinic. RESULTS: 301 patients were identified: 260 had isolated MGLSc and 41 both MGLSc and PeIN. Referrals were made from the local Urology and Andrology departments (128), primary care (89), GUM (54), other dermatology departments (28) and other specialties (2). In isolated MGLSc, 94.6% were diagnosed clinically with 93.5% accuracy (based on data from subsequent circumcisions). In combined MGLSc/PeIN, 85.4% were diagnosed following diagnostic biopsy and 14.6% retrospectively after circumcision. In isolated MGLSc, 50% were treated topically, and 50% required surgery. In MGLSc/PeIN, 78% required surgical interventions. In isolated MGLSc, 92.2% achieved resolution of symptoms, 3.5% were awaiting procedures, and 4.8% were receiving ongoing topical therapy. In MGLSc/PeIN, 90.2% achieved clearance, 2.4% were waiting surgery, and 7.3% were treated topically. Only 2.7% reported ongoing symptoms, all in patients treated surgically. None progressed to PSCC. DISCUSSION: MGLSc is generally a disease of the uncircumcised; the majority of cases of MGLSc are accurately diagnosed clinically; suspected PeIN or PSCC requires histological confirmation; circumcision histology can be non-specific; most men are either cured by topical treatment with ultrapotent corticosteroid (53.1%) or by circumcision (46.9%); surgical intervention is required in most cases of concomitant MGLSc and PeIN; the majority of patients with MGLSc alone or with MGLSc and PeIN remit with this approach; effective management appears to negate the risk of malignant transformation to PSCC.