ABSTRACT
Abstract The diagnosis of genital ulcers remains a challenge in clinical practice. Lipschütz ulcer is a non-sexually transmitted rare and, probably, underdiagnosed condition, characterized by the sudden onset of vulvar edema along with painful necrotic ulcerations. Despite its unknown incidence, this seems to be an uncommon entity, with sparse cases reported in the literature. We report the case of an 11-year-old girl who presented at the emergency department with vulvar ulcers. She denied any sexual intercourse. The investigation excluded sexually transmitted infections, so, knowledge of different etiologies of non-venereal ulcers became essential. The differential diagnoses are extensive and include inflammatory processes, drug reactions, trauma, and malignant tumors. Lipschütz ulcer is a diagnosis of exclusion. With the presentation of this case report, the authors aim to describe the etiology, clinical course, and outcomes of this rare disease, to allow differential diagnosis of genital ulceration.
Subject(s)
Humans , Female , Child , Ulcer/diagnosis , Vulvar Diseases/drug therapy , Dibucaine/therapeutic use , Anti-Infective Agents, Local/therapeutic use , Ulcer/drug therapy , Vulvar Diseases/pathology , Administration, Topical , Treatment Outcome , Epstein-Barr Virus Infections , Rare Diseases , Diagnosis, Differential , Dibucaine/administration & dosage , Anti-Infective Agents, Local/administration & dosageABSTRACT
RESUMEN El Pioderma Gangrenoso (PG) es una enfermedad inflamatoria necrotizante crónica, que pertenece al espectro de las dermatosis neutrofílicas. Histológicamente se caracteriza por mostrar un infiltrado inflamatorio denso de neutrófilos de origen no infeccioso. El PG suele asociarse a enfermedades sistémicas como la enfermedad inflamatoria intestinal, la artritis reumatoide o diversas enfermedades hematológicas. Presenta fenómeno de patergia y suele responder satisfactoriamente a tratamientos inmunosupresores. Su etiología no está bien definida. En la literatura se han publicado 15 casos de pioderma gangrenoso vulvar asociado al uso de rituximab. Nosotros presentamos un nuevo caso, que tuvo lugar en una mujer de 37 años en tratamiento de mantenimiento con rituximab por un linfoma no Hodgkin folicular. El rituximab (MabThera®) es un anticuerpo que reconoce la molécula CD20, que es una proteína no glucosilada que se expresa en la superficie de los linfocitos B. Este fármaco se ha utilizado para el tratamiento de diferentes enfermedades reumatológicas en los últimos años.
ABSTRACT Pyoderma Gangrenosum is a chronic necrotizing inflammatory disease that belongs to the spectrum of Neutrophilic Dermatoses. Histologically, it is characterized by a dense inflammatory infiltrate of non-infectious neutrophils. Etiology is not yet well defined. It is usually associated with systemic diseases such as inflammatory bowel disease, rheumatoid arthritis or hematological diseases. It presents pathergy phenomenon and usually respond satisfactorily to immunosuppressive treatments. There have been published only 15 cases of vulvar pyoderma gangrenosum associated with the use of rituximab. We present a new case, which occurred in a 37-year-old woman on maintenance treatment with rituximab for a follicular non-Hodgkin's lymphoma. Rituximab (MabThera®) is an antibody that recognizes the CD20 molecule, which is a non-glycosylated protein that is expressed on the surface of B lymphocytes. This drug has been used for the treatment of different rheumatic diseases in recent years.
Subject(s)
Humans , Female , Adult , Vulvar Diseases/drug therapy , Pyoderma Gangrenosum/diagnosis , Pyoderma Gangrenosum/drug therapy , Rituximab/adverse effects , Skin Diseases/pathology , Pyoderma Gangrenosum/epidemiology , Rituximab/administration & dosageABSTRACT
A 25-year-old Caucasian female with multiple genital warts involving the vulvar area was treated with imiquimod 5% cream. During follow-up the patient developed areas of hypopigmentation at the site of application of imiquimod cream and areas of hypomelanosis around multiple preexisting nevi of the trunk. At 18 months follow-up genital depigmentation persisted and halo nevi of the trunk were still present. Different mechanisms of imiquimod-induced depigmentation have been reported. Halo nevi are considered expression of an autoimmune response. In the case presented here, it might be conceivable that both vitiligo-like depigmentation at the site of application and halo of hypomelanosis around melanocytic nevi have been induced by the same immunologic mechanism elicited by topical application of imiquimod.
Subject(s)
Adult , Female , Humans , Adjuvants, Immunologic/adverse effects , Aminoquinolines/adverse effects , Condylomata Acuminata/drug therapy , Nevus, Halo/chemically induced , Vitiligo/chemically induced , Vulvar Diseases/drug therapy , Administration, Cutaneous , Nevus, Halo/immunology , Skin/drug effects , Skin/pathology , Treatment Outcome , Vitiligo/immunologySubject(s)
Humans , Adolescent , Female , Vulvar Diseases/drug therapy , Lidocaine/administration & dosage , Sucralfate/administration & dosage , Ulcer/drug therapy , Administration, Topical , Diagnosis, Differential , Drug Therapy, Combination , Vulvar Diseases/diagnosis , Ointments , Ulcer/diagnosisABSTRACT
Crohn's disease is a multisystem chronic granulomatous inflammatory disease that primarily affects the gastrointestinal tract. In the majority of the cases, the cutaneous manifestations follow the intestinal disease, but occasionally dermatological lesions are the inaugural event and may constitute the only sign of the disease. Vulvoperineal involvement is rare, may precede bowel symptoms by months to years and may go unrecognized. Due to the paucity of reports of Crohn's disease at this location and in the absence of randomized trials, there are no standard treatments for the cutaneous disease. We describe the case of a 47 year-old woman with vulvoperineal Crohn's disease without digestive involvement, that was successfully managed with metronidazole.
A doença de Crohn é uma doença granulomatosa multissistêmica inflamatória crónica que afecta primariamente o tracto gastrointestinal. Na maioria dos casos, as manifestações cutâneas sucedem a doença intestinal, mas, ocasionalmente, as lesões dermatológicas são o primeiro evento e podem constituir o único sinal da doença. O envolvimento vulvoperineal é raro, pode preceder os sintomas intestinais em meses ou anos, e pode passar despercebido. Devido à escassez de relatos de doença de Crohn com esta localização e na ausência de ensaios clínicos randomizados, não há nenhum tratamento padrão para a doença cutânea. Descrevemos um caso de uma mulher de 47 anos com doença de Crohn vulvoperineal sem envolvimento digestivo, que foi tratada com sucesso com metronidazol.
Subject(s)
Female , Humans , Male , Middle Aged , Anti-Infective Agents/therapeutic use , Crohn Disease/drug therapy , Metronidazole/therapeutic use , Perineum , Skin Diseases/drug therapy , Vulvar Diseases/drug therapy , Biopsy , Crohn Disease/pathology , Perineum/pathology , Skin Diseases/pathology , Treatment Outcome , Vulvar Diseases/pathologyABSTRACT
A histiocitose de células de Langerhans é representante de um raro grupo de síndromes histiocitárias, sendo caracterizada pela proliferação das células de Langerhans. Suas manifestações variam de lesão solitária a envolvimento multissistêmico, sendo o acometimento vulvar incomum. Segue-se o relato de caso refratário da doença limitada à pele, em mulher de 57 anos. A paciente apresentava história de pápulas eritematosas ulceradas em couro cabeludo, face, vulva, tronco e axila há seis anos. O diagnóstico da doença é difícil, sendo confirmado neste caso através de estudo imuno-histoquímico e se obteve resposta terapêutica e eficaz, com a administração de talidomida.
Langerhans cell histiocytosis is a member of a group of rare histiocytic syndromes and is characterized for the proliferation of histiocytes called Langerhans'cells. Its manifestations vary from a solitary injury to systemic involvement, and vulvar lesions are uncommon. We describe a refractory case of cutaneous limited disease in a 57-year-old woman. She presented with a 6-year history of an erythematous papular eruption of the scalp, face, vulva, trunk and axillae. The diagnosis is difficult and in this case it was confirmed through immunohistochemical study and clinical improvement was achieved with thalidomide.
Subject(s)
Female , Humans , Middle Aged , Histiocytosis, Langerhans-Cell/drug therapy , Immunosuppressive Agents/therapeutic use , Skin Diseases/drug therapy , Thalidomide/therapeutic use , Vulvar Diseases/drug therapy , Facial Dermatoses/drug therapy , Facial Dermatoses/pathology , Histiocytosis, Langerhans-Cell/pathology , Scalp Dermatoses/drug therapy , Scalp Dermatoses/pathology , Skin Diseases/pathology , Vulvar Diseases/pathologyABSTRACT
O líquen escleroso e atrófico é uma doença crônica da pele e mucosas, que, usualmente, afeta a área genital de mulheres. Poucos casos de líquen escleroso e atrófico, confinados nos pés e/ou mãos, têm sido relatados. Relatamos um caso de líquen escleroso e atrófico envolvendo as extremidades e,posteriormente, a área perigenital.
Lichen sclerosus et atrophicus is a chronic disorder of the skin and mucosal surfaces, most commonly affecting the female genitalia. Few cases of lichen sclerosus et atrophicus confined to the feet and/or hands have been reported. We report a case of lichen sclerosus et atrophicus involving first the extremities and then the perigenital area.
Subject(s)
Female , Humans , Middle Aged , Foot Dermatoses/diagnosis , Foot Dermatoses/pathology , Lichen Sclerosus et Atrophicus/pathology , Anti-Inflammatory Agents/therapeutic use , Chronic Disease , Clobetasol/therapeutic use , Foot Dermatoses/drug therapy , Immunosuppressive Agents/therapeutic use , Lichen Sclerosus et Atrophicus/drug therapy , Tacrolimus/therapeutic use , Vulvar Diseases/drug therapy , Vulvar Diseases/pathologyABSTRACT
A piodermatite-pioestomatite vegetante é uma rara dermatose inflamatória de etiologia desconhecida, com típico comprometimento mucocutâneo. Relatamos caso de paciente feminina com lesões pustulosas e vesiculosas em axilas, evoluindo com placas vegetantes e pústulas com agrupamento anular. Houve progressão com comprometimento vulvar, inguinal e mucosas oral, nasal e ocular. Proposto o diagnóstico, optou-se por iniciar prednisona 40mg ao dia, com remissão das lesões após um mês de uso da medicação. A associação com doença inflamatória intestinal ocorre em 70 por cento dos casos. A imunofluorescência é um fator que ajuda a caracterizar a doença, sendo tipicamente negativa. A rápida resposta à terapêutica com corticosteroides sistêmicos é esperada.
Pyodermatitis-pyostomatitis vegetans is a rare inflammatory dermatosis of unknown etiology, with a typical mucocutaneous involvement. We report the case of a woman with pustular and vesicular lesions in the axillae, evolving with vegetating plaques and pustules with annular grouping. The disease progressed with vulvar and inguinal involvement as well as involvement of the oral, nasal and ocular mucous membranes. She started the treatment with prednisone (40 mg/day), with remission of the lesions after one month of use of such medication. Association with inflammatory bowel disease occurs in 70 percent of the cases. Immunofluorescence, which is typically negative, helps to characterize the disease. A rapid response to systemic steroids is expected.
Subject(s)
Female , Humans , Middle Aged , Mucositis/pathology , Pyoderma/pathology , Vulvar Diseases/pathology , Anti-Infective Agents/therapeutic use , Axilla/pathology , Cephalothin/therapeutic use , Dapsone/therapeutic use , Eyelid Diseases/drug therapy , Eyelid Diseases/pathology , Mucositis/drug therapy , Prednisone/therapeutic use , Pyoderma/drug therapy , Stomatitis/drug therapy , Stomatitis/pathology , Vulvar Diseases/drug therapyABSTRACT
Una paciente de 22 años consultó por la presencia de pápulas de color blanquecino, pequeñas, de superficie plana, múltiples y levemente pruriginosas, localizadas en vulva, sin otras manifestaciones dermatológicas. Como antecedente familiar de relevancia refirió que su madre presentaba una dermatosis de similares características en pliegues inframamarios y axilas, recurrentes desde la adolescencia. El estudio histológico de la paciente correspondió a una dermatosis acantolítica con disqueratosis, vinculable a un Pénfigo Crónico Benigno Familiar (PCBF) o enfermedad de Hailey-Hailey. Se presentan las características clínicas e histológicas observadas y se analizan los diagnósticos diferenciales que se plantean ante una dermatosis acantolítica de localización exclusivamente genital.
A 22-year-old woman presented with multiple, small, whitish, domeshaped, itchy papules localized on the vulvar area whitout any other dermatologic lesions. Her family history disclosed that her mother had recurrent bullous lesions since her adolescence. The histopathologic examination demonstrated an acantholytic dermatosis with dyskeratosis, corresponding to familial bening pemphigus or Hailey-Hailey disease. Wepresent the clinical and histological characteristics observed in this case and analize the diff erential diagnosis of acantholytic dermatoses localized only on the vulvar area.
Subject(s)
Humans , Female , Adult , Pemphigus, Benign Familial/diagnosis , Pemphigus, Benign Familial/pathology , Pemphigus, Benign Familial/drug therapy , Vulvar Diseases/diagnosis , Vulvar Diseases/drug therapy , Skin/pathologyABSTRACT
Vulvodínia é um termo utilizado para descrever dor crônica em queimação na vulva, sem achados físicos objetivos que justifiquem os sintomas. Sua etiologia é desconhecida. Mulheres brancas, sexualmente ativas e na pré-menopausa são as mais acometidas. Entre os possíveis fatores envolvidos na gênese, estão anormalidades de desenvolvimento embrionário, aumento da excreção urinária de oxalato, fatores genéticos e imunológicos, fatores hormonais, anormalidades no assoalho pélvico e neuropatias. O exame clínico detalhado, com caracterização da dor, pesquisa de irritantes locais, inspeção cuidadosa da vulva e busca de pontos dolorosos, além de avaliação do assoalho pélvico, orienta o diagnóstico e o tratamento. Existem vários tratamentos citados, porém pouca evidência científica que comprove a sua eficácia. Deve-se descontinuar a utilização de produtos que possam funcionar como irritantes. O uso de citrato de cálcio pode diminuir a excreção de oxalato na urina. A fisioterapia pode ser empregada nas pacientes com instabilidade do assoalho pélvico. Os antidepressivos tricíclicos e anticonvulsivantes foram utilizados com boa resposta em vários estudos. Além destes, podem ser aplicados, topicamente ou por injeção local, substâncias como anestésicos, corticoides, estrogênios e interferon-alfa. A cirurgia fica reservada para casos extremos, sendo que os resultados na literatura são conflitantes.
Vulvodynia is a term used to describe chronic vulvar pain or discomfort, without signs that explain such symptom. Its etiology is unknown. It is more likely to occur in sexually active, white women in pre-menopausal period. Many factors may be involved in the onset of this condition (anomalies in embrionary development, increased urinary oxalate excretion, genetic and immune factors, hormonal factors, pelvic floor problems and neuropathies). A thorough clinical history must be done, with special attention to the pain characteristics and use of local irritants. Careful vulvar inspection, research of trigger points and pelvic floor evaluation will increase the likelihood of an accurate diagnosis. Many treatment options are described, but there is not enough evidence on their effectiveness. Local irritants should be discontinued. Calcium citrate may decrease urinary oxalate excretion. Physical therapy can be used to treat pelvic floor instability. Tricyclic antidepressants and anticonvulsants showed a good response in some studies. Besides systemic drugs, topic substances or local injections can be applied (anesthetics, estrogens, corticosteroids and alpha-interferon). Surgery should be reserved to unresponsive cases and its effectiveness has been inconsistent in the scientific literature.
Subject(s)
Female , Diagnosis, Differential , Vulvar Diseases/diagnosis , Vulvar Diseases/drug therapy , Vulvar Diseases/therapy , Pain/diagnosis , Pain/etiology , Calcium Oxalate , Patient Care Team , Vulvovaginitis/diagnosisABSTRACT
To compare the efficacy of aloe vera gel and placebo in the topical management of vulval lichen planus. Randomized, double-blind, placebo-controlled trial. Department of Dermatology, Isra University Hospital, Hyderabad, from January 2007 to January 2008. Thirty-four female patients were randomized into two groups to receive aloe vera gel or placebo for local application for 8 weeks. Clinical data and treatment response was graded according to Thongprasom criteria. Z-test was used for comparing response between the groups. Thirty-four consecutive patients participated in the study. We found erosive and ulcerative lesions in 83% and 17%, respectively. The most common site of vulval lichen planus was the labia minora. Fourteen [82%] out of 17 patients treated with aloe vera had a good response i.e. clinically improved by at least 50% after 8 weeks of treatment, while one [5%] of 17 placebo-treated patients had a similar response [p < 0.001]. Furthermore, one patient [5%] treated with aloe vera had a complete clinical remission. No side-effects were found in both groups. Aloe vera gel was a safe and effective treatment for patients with vulval lichen planus
Subject(s)
Humans , Female , Vulvar Diseases/drug therapy , Aloe , Disease Management , Double-Blind Method , Treatment Outcome , Administration, Topical , /pathology , PhytotherapySubject(s)
Humans , Female , Adult , Granuloma Inguinale/diagnosis , Vulva/pathology , Vulvar Diseases/diagnosis , Anti-Bacterial Agents/therapeutic use , Doxycycline/therapeutic use , Granuloma Inguinale/drug therapy , Granuloma Inguinale/surgery , Hypertrophy/diagnosis , Hypertrophy/drug therapy , Hypertrophy/surgery , Treatment Outcome , Vulvar Diseases/drug therapy , Vulvar Diseases/surgeryABSTRACT
Se reportan 21 pacientes portadoras de liquen escleroso vulval tratadas con testosterona al 2 por ciento o acetato de clostebol entre 1988 y 1995. Un 77,8 y 80 por ciento alcanzaron respuesta completa o parcial a los 3 meses de tratamiento con el uso de testosterona al 2 por ciento y acetato de clostebol, respectivamente. Un 53,3 por ciento abandonó el tratamiento y control antes del año. Los resultados obtenidos no avalan el uso de testosterona al 2 por ciento ni de acetato de clostebol. La revisión de la literatura muestra que el uso de propionato de clobetasol al 0,05 por ciento tendría una mayor tasa de éxito terapéutico y de control de la enfermedad a largo plazo que el uso de andrógenos tópicos
Subject(s)
Humans , Adult , Middle Aged , Female , Lichen Sclerosus et Atrophicus/drug therapy , Testosterone/pharmacology , Administration, Topical , Autoimmune Diseases/complications , Clobetasol/pharmacology , Lichen Sclerosus et Atrophicus/complications , Lichen Sclerosus et Atrophicus/diagnosis , Pruritus Vulvae/etiology , Refusal to Treat/statistics & numerical data , Testosterone/administration & dosage , Vulvar Diseases/drug therapyABSTRACT
O líquen escleroso (LS) é uma doença de etiologia desconhecida, com marcada predileçäo pela vulva, em torno da menopausa. Há relatos de involuçäo espontânea de casos infantis na época da puberdade. O tratamento com testoterona tópica controla os sintomas e melhora a lesäo cutânea. Sugeriu-se que uma deficiência da enzima 5-alfa-redutase pudesse ser responsável pela doença. O objetivo deste estudo foi investigar alterçöes nos esteróides sexuais plasmáticos em mulheres co LS vulvar. Realizamos um estudo transversal controlado. Andrógenos e estrógenos foram dosados no plasma de 67 mulheres, entre 21 e 81 anos de idade, com LS vulvar antes de iniciarem o tratamento com testoterona tópica, em 23 destas pacientes durante o tratamento e num grupo controle de 30 mulheres com LS apresentaram níveis plasmáticos de estradiol e de testoterona livre superiores aos dos controles e niveis de DHEA-SO e de estrona inferiores. Durante o tratamento com testoterona tópica houve elevaçäo significativa da testoterona total. A involuçäo de estruturas anatômicas andrógeno-dependentes, assim como a alteraçäo de componentes da pele, também influenciados pelos andrógenos, característicos do LS vulvar, e as alteraçöes encontradas nos níveis plasmáticos dos esteróides sexuais destas pacientes fortemente sugerem que um distúrbio do metabolismo androgênico participe da patogenia do LS
Subject(s)
Humans , Female , Adult , Middle Aged , Androgens/blood , Vulvar Diseases/blood , Estrogens/blood , Lichen Sclerosus et Atrophicus/blood , Testosterone/therapeutic use , Aged, 80 and over , Cross-Sectional Studies , Vulvar Diseases/drug therapy , Lichen Sclerosus et Atrophicus/drug therapyABSTRACT
A intençäo desta publicaçäo é rever aspectos vários das doenças sexualmente transmissíveis, sobretudo as de localizaçäo vulvar. Questöes relacionadas a conceitos, epidemiologia, clínica e tratamento säo abordadas
Subject(s)
Humans , Female , Sexually Transmitted Diseases , Vulvar Diseases , Vulvar Diseases/diagnosis , Vulvar Diseases/drug therapy , Sexually Transmitted Diseases/diagnosis , Sexually Transmitted Diseases/drug therapyABSTRACT
The authors related two cases of lichen sclerosus of the vulva in the childhood treated with medroxiprogesterone. By the presence of benefic effects and absence of toxic side effects of this drug, the authors suggest its use as the first choice in the therapeutic of this disease in the childhood and puberty
Subject(s)
Humans , Female , Child, Preschool , Child , Vulvar Diseases/diagnosis , Vulvar Diseases/drug therapy , Lichen Sclerosus et Atrophicus/diagnosis , Lichen Sclerosus et Atrophicus/drug therapy , Medroxyprogesterone Acetate/therapeutic use , Treatment OutcomeABSTRACT
Realizou-se estudo aberto e comparativo entre cloetebol/neomicina e clostebol no tratamento tópico exclusivo do líquen escleroso vulvar. Foram estudadas 33 pacientes, cuja idade variou de 27 a 72 (53,23) anos, divididas de forma homogênea em grupos A (clostebol/neomicina) e B (clostebol). Aspectos clínicos (prurido, eritema, edema e extensåo da lesåo branca) e a avaliaçåo clínica global (efeito terapêutico) foram analisados na visita inicial e aos 30, 60 e 90 dias de tratamento. Os resultados nos grupos A e B revelaram-se: excelente em 5 (29,5 por cento) e 2 (12,5 por cento), bom em 7 (41,2 por cento) e 6 (37,5 por cemto), regular em 1 (5,8 por cento) e 2 (12,5 por cento) e nulo em 4 (23,5 por cento) e 6 (37,5 por cento) casos, respectivamente. Concluiu-se que o clostebol/neomicina é interessante alternativa terapêutica no líquen escleroso vulvar, mas nåo de forma absoluta ou exclusiva