Case for diagnosis. Radiotherapy-induced pemphigus vegetans

Abstract A 73-year-old male patient developed a poorly differentiated squamous cell carcinoma in the anal canal nine months ago. He was treated with two cycles of 5-fluorouracil and cisplatin and concomitant radiotherapy (6 MeV linear photon accelerator, total dose of 54 Gy), with complete remission. Since forty-five days he presentes a painful perianal and intergluteal erosion with circinate pustular borders. Light microscopy showed pseudoepitheliomatous hyperplasia of the epidermis with microabscesses of inflammatory cells (neutrophils and eosinophils) and acantholytic keratinocytes . Indirect immunofluorescence was positive for IgG, with an intercellular pattern, 1:80 titer. The diagnosis of radiotherapy-induced pemphigus vegetans was established and there was significant regression with oral prednisone (40 mg) and topical betamethasone.

Case for diagnosis. Penile lesion in HIV-negative patient

Abstract We present the case of an HIV-negative man with asymptomatic penile erythematoviolaceous papules associated with similar slightly verrucous papules in the interdigital space of the right foot. A biopsy of the penile lesion confirmed Kaposi's sarcoma. No other causes of immunosuppression were observed. Penile lesions of KS are rare in HIV-negative individuals but it should also be considered in the differential diagnosis. Careful follow-up is recommended.

Familial "benign" pemphigus? Erythroderma and fatal outcome

Abstract Hailey-Hailey disease, or familial benign pemphigus, is a rare bullous genodermatosis that usually presents with flaccid blisters, erosions, and maceration limited to flexural areas, resulting in increased morbidity and reduced quality of life for affected patients. The authors report an unusual case of generalized Hailey-Hailey disease with erythroderma and fatal outcome.

Verrucous hemangioma and histopathological differential diagnosis with angiokeratoma circumscriptum neviforme

Abstract: Verrucous hemangioma is a rare vascular skin disorder with an immune profile similar to vascular neoplasms, but with behavior and evolution of vascular malformations. Its main differential diagnosis is angiokeratoma circumscriptum neviforme, with an almost indistinguishable clinical presentation because both diseases appear as erythematous patches that evolve to violaceous plaques, becoming scaly and even verrucous, most commonly affecting the lower limbs. Histopathology is crucial for the correct diagnosis: while in angiokeratoma the vascular alterations are limited to the papillary dermis, verrucous hemangioma extends deep into the dermis, reaching the subcutaneous tissue.

Xantoma disseminado: relato de caso e revisão da literatura / Disseminated xanthoma: a case report and literature review

Xantoma disseminado é uma histiocitose não Langerhans, rara, não familiar, benigna, normolipídica, caracterizada pela deposição lipídica na pele e órgãos internos secundária à proliferação histiocitária. É caracterizado por lesões xantomatosas que geralmente afetam as regiões flexurais e, frequentemente, acomete mucosas. Tem curso em geral benigno, com resolução das lesões cutâneas em alguns anos, porém lesões em certas localizações podem resultar em morbidade e até óbito, sendo, nestes casos, o achado cutâneo uma importante pista diagnóstica. Devido à sua raridade, não há um consenso terapêutico, e as respostas às terapias têm sido insatisfatórias. Relatamos o caso de uma paciente com essa patologia, na qual não foi detectada patologia sistêmica (AU)

Disseminated xanthoma is a rare non-familial, benign, and normolipid form of non-Langerhans histiocytosis, characterized by lipid deposition in the skin and internal organs secondary to histiocytic proliferation. It is characterized by xanthomatous lesions that generally affect the flexural regions and often affects mucous membranes. Although it has a generally benign course, with resolution of cutaneous lesions in a few years, lesions in certain locations can result in morbidity and even death. In these cases, the cutaneous finding is an important diagnostic clue. Because of its rarity, there is no therapeutic consensus and responses to therapies have been unsatisfactory. Here we report the case of a patient with this disorder, in whom no systemic disease was detected (AU)

Zoon vulvitis

Abstract Zoon vulvitis or vulvitis chronica plasmacellularis is a rare, chronic benign inflammation of the vulvar mucosa, diagnosed histologically, with variable therapeutic responses. It is important to be diagnosed because it mimics the presentation of other genital conditions, such as lichen planus and squamous cell carcinoma, which require specific treatment. We report a case of a female patient with three asymptomatic shallow ulcers on the labia minora. Laboratory tests ruled out infectious diseases and the biopsy was consistent with Zoon Vulvitis.

Pênfigo crônico familiar benigno / Familial benign chronic pemphigus

O objetivo deste trabalho é relatar o caso de mulher, 44 anos, branca (fototipo 3), apresentando placas eritemato-violáceas com vesículas rompidas, maceração e odor intenso e desagradável em ambas as axilas e região inguinal, com início há dez anos. Foi diagnosticado Pênfigo Crônico Familiar Benigno ou doença de Hailey-Hailey, uma genodermatose autossômica dominante rara desencadeada por mutação no cromossomo 3q21-24 (AU)

The aim of this study is to report the case of a 44-year-old white (skin type 3) woman presenting with erythematous-violaceous plaques with ruptured vesicles, maceration, and intense and unpleasant odor in both armpits and groin, starting ten years ago. Benign familial chronic pemphigus, or Hailey-Hailey disease, a rare autosomal dominant genodermatosis triggered by a mutation on chromosome 3q21-24, was diagnosed (AU)

Eritema indurado de Bazin: uma manifestação de tuberculose cutânea / Erythema induratum of Bazin: a manifestation of cutaneous tuberculosis

O eritema indurado de Bazin caracteriza-se por nódulos nas extremidades inferiores, as quais podem apresentar úlceras e cicatrizes atrófi cas. Esse tipo de paniculite apresenta associação bem estabelecida com tuberculose, mas casos idiopáticos ou induzidos por outros agentes infecciosos podem ocorrer. Neste estudo, nosso objetivo foi relatar o caso de um paciente de 15 anos com derivado proteico purifi cado da cultura de Mycobacterium tuberculosis fortemente reator e apresentação clínica e histopatológica típica dessa forma de tuberculose cutânea.

The erythema induratum of Bazin is characterized by nodules in the lower extremities, which may have ulcers and atrophic scars. This type of panniculitis presents well-established association with tuberculosis, but idiopathic cases or induced by other infectious agents may happen. In this study, our aim was to report the case of 15-year-old patient with highly reactive purifi ed protein derivative of Mycobacterium tuberculosis culture and typical clinical and histopathological presentation of this form of cutaneous tuberculosis.

Papilite lingual transitória ? relato de caso / Transient lingual papillitis ? case report

com essa afecção.Descrição do caso: Um homem de 34 anos apresentava desde a infância surtos de lesões papulosas dolorosas na língua. O exame anatomopatológico de uma das lesões demonstrou papilite crônica.Conclusões: Papilite lingual transitória é uma doença inflamatória dolorosa das papilas fungiformes, que tem seu início na infância e evolui em surtos, podendo ser acompanhada de dor ou desconforto. Embora seja frequente, é um distúrbio pouco estudado.

Aims: To describe a case of transient lingual papillitis in order to familiarize health professionals with this condition.Case description: A man of 34 years of age had since childhood bouts of painful papular lesions on the tongue. The anatomopathological examination of one lesion showed chronic papillitis.Conclusions: Transient lingual papillitis is a painful inflammatory disease of the fungiform papillae, which has its onset in childhood, progresses in spurts, and may be accompanied by pain or discomfort. Although it is common, is an understudied condition.

Case for diagnosis / Caso para diagnóstico

Papular elastorrhexis is a rare acquired disease, first described in 1987 by Bordas, which has been very rarely reported in the literature. It is characterized by small asymptomatic non-follicular papules, mainly distributed in the trunk. Histology of the lesions shows homogenization of collagen and fragmentation of elastic fibers in the dermis. The rarity of this disease is probably due to the subtlety and benign nature of clinical and histopathological alterations, which can be easily confused with other pathologies. The authors report the case of a patient with exuberant clinical manifestations typical of elastorrhexis papular.

Elastorrexe papulosa é uma doença adquirida rara, descrita em1987 por Bordas e poucas vezes relatada na literatura. Caracteriza-se por pequenas pápulas, não foliculares, assintomáticas, distribuídas essencialmente no tronco. A histologia das lesões demonstra homogeneização do colágeno e fragmentação de fibras elásticas dérmicas. A raridade dessa entidade provavelmente se deve à sutileza e benignidade das alterações clínicas e histopatológicas, que podem facilmente ser confundidas com inúmeras outras afecções. Os autores relatam o caso de uma paciente com quadro clínico exuberante e característico de elastorrexe papulosa.

Facial miliary osteoma in HIV patient / Osteoma miliar da face em paciente portadora do HIV

Facial miliary osteoma is characterized by the presence of multiple normochromic papules, affecting mainly middle-aged women, with a previous history of acne. A 39-year-old HIV positive female patient presented hardened papules which had appeared 3 years before, located on the malar region, glabella and mentum. Optical microscopy showed spherical bone formations in the dermis. Computerized tomography of the face revealed small calcifications on the surface consistent with bone formation. Multiple facial osteomas are rare as well as its association with HIV virus infection.

Os osteomas miliares da face são caracterizados por múltiplas pápulas normocrômicas na face, afetando, geralmente, mulheres de meia idade, com história pregressa de acne. Uma paciente de 39 anos, portadora do HIV, apresentou pápulas endurecidas, com 3 anos de evolução, localizadas na região malar, glabela e mento. A microscopia óptica demonstrou formações ósseas esféricas na derme. A tomografia computadorizada da face observou pequenas calcificações na superfície, compatíveis com formação óssea. Os osteomas múltiplos da face são raros assim como sua associação com a infecção pelo HIV.