Rowell's syndrome is believed to be a distinct and rare clinical entity originally described as lupus erythematosus associated with erythema multiforme-like lesions with immunological findings of speckled antinuclear antibodies, anti-La antibodies and a positive test for rheumatoid factor. We report two additional patients with Rowell's syndrome and review all the diagnostic criteria found in the literature. In view of the inconsistent findings of some of the diagnostic features, we propose that major and minor criteria be used to diagnose Rowell's syndrome.
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.
We highlight the novel use of a GnRH agonist as a neoadjuvant to surgery in aggressive angiomyxoma to shrink the tumor preoperatively and decrease surgical morbidity.
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 patient's family and contacts in cases of recurrent GAS infection by culturing all potential areas of GAS carriage.
Localized vulvar bullous pemphigoid of childhood is an excessively rare variant of bullous pemphigoid and affects almost exclusively young girls of 7-12 years of age. In contrast to adult-onset bullous pemphigoid, a prompt response to potent topical corticosteroids is observed in the majority of cases, with a favorable prognosis and rare relapses. We report the case of a 7-year-old girl who presented with this condition.Our case reinforces the recognition of this rare subtype of childhood bullous pemphigoid as a distinct entity and enlightens the importance of performing a cutaneous biopsy when clinically indicated. K E Y W O R D S bullous disease, bullous pemphigoid, immunobullous disease, mucous membrane disorders, vesiculobullous disorders, vulvar disorders How to cite this article: Belzile E, Funaro D, Powell J. Localized vulvar bullous pemphigoid of childhood: A rare cause of persistent vulvar erosions in children. Pediatr Dermatol.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.