Deana Funaro

Lichen sclerosus: a review and practical approach

ABSTRACT:  Lichen sclerosus (LS) is a chronic dermatitis predominantly found in the anogenital area. It can be found in patients of any age group, sex, or race, but is most commonly present in Caucasian peri‐ or postmenopausal women. Although the etiology of LS remains uncertain, an autoimmune process is believed to underlie this condition. With many cases going unreported, its incidence is still unknown. There is no cure for LS, but treatment offers control of the condition. They are three reasons for treating LS: relief of symptoms and discomfort; prevention of any or further anatomical changes; and a theoretical prevention of malignant transformation. Although many treatments have been suggested to treat LS over the years, only potent or ultra‐potent corticosteroids remain as the treatment of choice. After initial therapy, some patients might only use corticosteroids as needed, while others may require a twice‐weekly maintenance therapy. There is no place for surgery in uncomplicated LS. Surgery should be limited exclusively to patients with malignancy and to correct scarring secondary to the disease. Lichen sclerosus is associated with a 4–6% risk of squamous cell carcinoma, making long‐term follow‐up essential in these patients.

Extramammary Paget’s Disease: Summary of Current Knowledge

Extramammary Pagets disease (EMPD) is a rare cutaneous malignancy accounting for approximately 1% of vulvar cancers. The rarity of this disease has caused difficulties in its characterization. Controversies exist in the literature regarding many aspects of this condition including the prevalence of concurrent vulvar adenocarcinoma or invasive EMPD, association with regional and distant cancers, and recurrence rates following surgical excision. This extensive review takes a closer look at what is known about EMPD and the conclusions that have been drawn from this information. This article also provides a practical approach to patients with EMPD.

Biopsychosocial Predictors of Postmenopausal Dyspareunia: The Role of Steroid Hormones, Vulvovaginal Atrophy, Cognitive-Emotional Factors, and Dyadic Adjustment

INTRODUCTION Although dyspareunia experienced after menopause is widely attributed to declining estrogen levels and vulvovaginal atrophy, critical reviews of the literature have suggested that these factors are incomplete as explanatory mechanisms. Little is known about psychosocial factors that may also be implicated in postmenopausal dyspareunic pain. AIM To determine the extent to which levels of estrogens and progesterone, vulvovaginal atrophy, cognitive-emotional factors, and dyadic adjustment are predictive of postmenopausal dyspareunic pain intensity. METHODS A total of 182 postmenopausal dyspareunia sufferers underwent a structured interview concerning sociodemographic status as well as medical and pain histories, gynecological examination, cytological evaluation, a blood draw, and answered a series of self-report questionnaires. Given the large number of genital and pelvic pain variables measured, a principal components analysis was undertaken to identify a smaller number of components representing meaningful dimensions of genital and pelvic pain. MAIN OUTCOME MEASURES Pain severity ratings during intercourse were obtained using the McGill Pain Questionnaire. Pain ratings were also obtained during gynecological assessment. Serum estrone, estradiol, and progesterone levels were measured via immunoassay. The Vaginal Atrophy Index and maturation value were used to determine vulvovaginal atrophy severity. Participants completed the Pain Catastrophizing Scale, State-Trait Anxiety Inventory, The Beck Depression Inventory-II, and Dyadic Adjustment Scale. RESULTS Hormone levels were not found to be consistent predictors of pain severity. Maturation value and cognitive-emotional variables (e.g., catastrophization, depression, anxiety) were significant predictors of vestibular pain, which affected over 90% of our sample. Relationship adjustment variables were inversely associated with pain severity within several genital locations. CONCLUSIONS Results suggest that the traditional hypoestrogen and vulvovaginal atrophy conceptualization of postmenopausal dyspareunia is an insufficient explanatory model, and that pain is also influenced by cognitive, affective, and dyadic factors.

Recurrent Group A Streptococcal Vulvovaginitis in Adult Women: Family Epidemiology

Group A beta-hemolytic streptococcal (GAS) vulvovaginitis has been reported in prepubertal girls. In adult women, a vaginal carrier state has been described, but vulvovaginitis is rarely reported. We describe 2 cases of recurrent GAS vulvovaginitis in women whose husbands were gastrointestinal carriers of GAS. Characterization of the isolated strains demonstrated that identical emm types of GAS were shared by partners. Treatment of both partners resulted in resolution of vaginitis. On the basis of negative vaginal culture results obtained after treatment of each individual episode of vaginitis, we believe that the female patients were reinfected as a result of exposure to their husbands, with shedding likely to have occurred in bed. These cases reiterate the necessity for adequate screening of the patients family and contacts in cases of recurrent GAS infection by culturing all potential areas of GAS carriage.

Challenging Atrophied Perspectives on Postmenopausal Dyspareunia: A Systematic Description and Synthesis of Clinical Pain Characteristics

This study investigated the clinical attributes of postmenopausal dyspareunia. The authors obtained a systematic description of pain symptomatology from 182 postmenopausal dyspareunia sufferers using a structured interview, quantitative sensory testing, a standardized pain measure, and gynecological examination. The authors conducted a cluster analysis to examine whether sufferers could be categorized using clinical pain and gynecological factors. The authors delineated 6 subgroups, each exhibiting distinct combinations of pain and gynecological characteristics. The results support the hypothesis that, similarly to premenopausal dyspareunia, postmenopausal dyspareunia is a heterogeneous condition.

Extramammary Paget disease: epidemiology and association to cancer in a Quebec-based population.

Objective This study aimed to further characterize the epidemiology, clinical manifestations, pathology, immunopathology, outcome from therapy, and associated underlying malignancy in extramammary Paget disease (EMPD). Materials and Methods We conducted a retrospective review of patients treated for EMPD in our tertiary care center during a 23-year period ranging from 1985 to 2008. Results Sixty-four cases of EMPD were diagnosed during this period. Mean age at diagnosis was 66.8 years. Of the patients, 79.7% were female. Tumors were mostly localized on the vulvoperineal region. Associated cancers were found in 30% of the patients and included breast cancer and urogenital cancers. Of the patients, 42% had a least 1 recurrence. The risk of recurrence could only be associated to tumor location on the vulvoperineal region. The limitations of this study include its retrospective nature and sample size. Conclusions Extramammary Paget disease is more commonly found on the vulva of older women and frequently recurs. Recurrence was not associated to margin status, which would support a more conservative therapeutic approach.

Rosai-Dorfman disease treated with methotrexate and low-dose prednisone: case report and review of the literature.

Background: Rosai-Dorfman disease is a rare condition characterized by an accumulation of histiocytes within lymph nodes and tissue. Treatment is indicated when the condition is highly symptomatic and when widespread involvement causes vital organ compression. Objective: To review the classic physical and histologic findings in Rosai-Dorfman disease and summarize the literature supporting the use of methotrexate in this condition. Methods and Results: We describe a case of Rosai-Dorfman disease that failed to respond to monotherapy with systemic corticosteroids but improved with the addition of methotrexate. Methotrexate alone or in combination with other agents has been described in at least 12 other cases in the literature and lead to a partial or complete resolution of disease in over half of these. Conclusion: The rapid improvement of clinical and physical symptoms with minimal side effects in our case and others reinforces the utility of antimetabolites in patients with Rosai-Dorfman disease who fail to respond to corticosteroids.

Vulvar sarcoidosis: case report and review of the literature.

Background: Sarcoidosis is a multisystemic disorder of unknown etiology that can affect multiple organs, including the lungs, skin, and eyes. Vulvar sarcoidosis has anecdotally been reported. Objective: The aim of this article is to describe a case of vulvar sarcoidosis and review the few cases that have been reported. Methods: We report the case of a 39-year-old woman who presented to the dermatologist with a 2-year history of vulvar pruritus. Results: Examination revealed infiltrated plaques on the vulva and perianal region. The biopsy demonstrated well-defined, nonnecrotizing granulomas in the dermis. Further investigation revealed hilar adenopathy consistent with sarcoidosis. The patient responded well to topical corticosteroids. Conclusion: In the presence of granulomatous lesions of the genital region, infectious causes, foreign body reaction, Crohn disease, and sarcoidosis should be part of the differential diagnosis.

Hodgkin lymphoma presenting as a vulvar mass in a patient with crohn disease: a case report and literature review.

Objective. A case of Hodgkin lymphoma of the vulva and perineum is presented along with a review of the literature. Materials and Methods. Medical chart and clinical images were reviewed. Results. A 45-year-old female patient with a longstanding history of Crohn disease presented with a large vulvar and perineal mass. Physical examination revealed a mass measuring approximately 20 × 20 cm involving primarily the labia majora, the labia minora, and the clitoris as well as the perineum. Incisional biopsy of the vulvar mass revealed histologic diagnosis and immunohistochemistry typical of classic Hodgkin lymphoma. Imaging revealed involvement of multiple lymph nodes as well as the liver. The patient was designated as having stage IV disseminated Hodgkin lymphoma, and chemotherapy with doxorubicin, bleomycin, vinblastine, and dacarbazine was instituted. A significant reduction of the size of the vulvar mass was observed following 8 cycles of chemotherapy. Conclusions. Lymphoma of the vulva is rare with the majority being of the non-Hodgkin lymphoma type. The most common subtypes of vulvar lymphoma reported are diffuse large B-cell lymphoma and follicular lymphoma. Perianal Hodgkin lymphoma is also very rare but has been reported in association with human immunodeficiency virus infection, Epstein-Barr virus infection, and Crohn disease. This is only the second reported case of Hodgkin lymphoma of the vulva and the second case of Hodgkin lymphoma involving the perianal area in a female patient. There is currently no evidence that Crohn disease is associated with an increased risk of Hodgkin lymphoma.