Oral lichen planus: case series and experience in a tertiary dermatology service in Brazil

Abstract Background: Lichen planus is an inflammatory disease that can affect both the skin and mucous membranes, including the oral mucosa. There is very little original Brazilian dermatology literature about oral lichen planus. Objective: To describe the clinical, pathological, and treatment data of 201 patients diagnosed with oral lichen planus followed at the Stomatology Outpatient Clinic of Hospital das Clínicas, Universidade de São Paulo, from 2003 to 2021. Method: The patients demographic profile, the morpho-topographic features of the lesions, the treatment employed, and the possible presence of squamous cell carcinoma were analyzed. Results: The disease was more common in women over 50 years of age, tending to be chronic, with a large number of cases showing cicatricial sequelae in the mucosa. Topical treatment with potent corticosteroids was shown to be effective in the vast majority of cases. Squamous cell carcinoma in oral lichen planus cicatricial sequelae was observed in eight cases. Study limitations: Retrospective study of medical records, with gaps regarding the filling out of data; unequal observation time among the studied cases. Conclusions: This is the largest Brazilian dermatology series on oral lichen planus. The response to topical corticoid therapy was excellent in the vast majority of cases. The high prevalence of atrophic lesions, demonstrating the chronicity and tissue destruction potential of this disease, may explain the large number of cases of squamous cell carcinoma.

Bullous lesions following phototherapy in a newborn

ABSTRACT A male infant presented with progressive jaundice immediately after birth. Fecal acholia and choluria associated with extensive bullous skin lesions in his trunk, abdomen, and upper and lower limbs developed during phototherapy. Several diagnostic hypotheses were presented, including neonatal porphyria, hemochromatosis, Alagille syndrome, and neonatal lupus. A 24-hour urine sample for the dosage of urinary porphyrins was collected, showing high results (1823.6µg in 100mL). At 50 days of life, fluorescence spectroscopy using a Wood's lamp revealed simultaneous bright red fluorescence of urine-stained diapers and sample blood. A definitive diagnosis of congenital erythropoietic porphyria was made following identification of a mutation of the uroporphyrinogen synthetases III gene on genetic testing. The patient was subsequently maintained in a low light environment since then, resulting in improvement of the lesions. Congenital erythropoietic porphyria is a disease of the group of porphyrias that presents shortly after birth with blistering occurring in regions exposed to the sun or other ultraviolet light. Atrophic scars, mutilated fingers, and bright red fluorescence of the urine and teeth may also be observed. There is no specific treatment, and prophylaxis comprising a total avoidance of sunlight is generally recommended. A high degree of suspicion is required for diagnosis. An early diagnosis can lead to less damage. Here, we present the case of a newborn with congenital erythropoietic porphyria diagnosed after presenting with bullous lesions secondary to phototherapy.

CHILD syndrome: successful treatment of skin lesions with topical lovastatin and cholesterol lotion

Abstract: CHILD syndrome (Congenital Hemidysplasia, Ichthyosiform erythroderma, Limb Defects) is a rare X-linked dominant disease. The authors report a 2-month-old patient presenting with typical features of CHILD syndrome that was treated with a topical solution containing cholesterol and lovastatin, with complete clearance of her CHILD nevus. The changes in skin lipid metabolism that explain the CHILD ichthyosiform nevus and their correction through topical application of cholesterol and lovastatin are discussed.

Oral mucosal diseases in children - casuistics from the Department of Dermatology - University of São Paulo - Brazil

Abstract There are no studies about pediatric oral mucosal diseases performed by dermatologists in Brazil. This study presents the casuistics of oral mucosal diseases in children examined at the Oral Diseases Clinic at the Department of Dermatology - University of São Paulo - Brazil. Cases were retrospectively studied from the hospital records from 2003 to 2015. A hundredsix children have been examined. Commoner lesions examined included mucoceles and aphthae. Rare and difficult cases were also seen and have been published; this clinic is based in a tertiary hospital center that deals mostly with complex cases.

Circumscribed palmoplantar hypokeratosis: report of two brazilian cases / Hipoqueratose circunscrita palmoplantar: relato de 2 casos brasileiros

Circumscribed palmoplantar hypokeratosis is a recently recognized dermatosis and rarely reported. It was first described in 2002 and is characterized by localized loss of the horny layer in the palmoplantar area. This dermatosis is clinically presented with a sharply circumscribed, reddish and asymptomatic plaque with slightly depressed surface localized on the palms or the soles. The clinical differential diagnosis includes mainly porokeratosis and Bowen's disease. Its pathogenesis remains unknown, but studies have proposed a human papillomavirus induced disease or a localized keratinization disorder in the palmoplantar area. We report herein two cases of patients with lesions clinically and microscopically compatible with the diagnosis of circumscribed palmoplantar hypokeratosis. We also present a brief literature review of the etiopathogenic hyphoteses of this dermatosis.

A hipoqueratose palmar circunscrita é uma condição rara e pouco conhecida que foi caracterizada apenas em 2002. Caracteriza-se por uma perda circunscrita e focal da camada córnea das regiões palmares ou plantares. Clinicamente, observam-se lesões avermelhadas, circunscritas, arredondadas, discretamente deprimidas e assintomáticas, nas palmas ou, mais raramente, nas plantas. A diagnose diferencial clínica é feita principalmente com a poroqueratose palmoplantar e com a disqueratose de Bowen. Sua patogênese é desconhecida, havendo especulações sobre possível origem viral ou de alteração localizada da expressão de citoqueratinas das regiões palmoplantares. Relatamos os casos clínicos de duas doentes femininas com lesões clínicas e alterações histopatológicas características de hipoqueratose palmar circunscrita de evolução crônica, além de uma breve revisão da literatura sobre as hipóteses etiopatogênicas desta dermatose.

Líquen plano oral / Oral lichen planus

O líquen plano da mucosa oral (LPO) é afecção relativamente comum, que pode aparecer isolado ou associado ao líquen plano cutâneo, havendo, no entanto, significantes diferenças clínicoevolutivas: o LPO tende a ser crônico, recidivante e de difícil tratamento, levando a importante morbidade, principalmente em sua forma erosiva. Novas formas clínicas agressivas têm sido salientadas na literatura, como a forma gingivo-vulvar. Este artigo revisa a etiopatogenia, as formas clínicas, a diagnose diferencial e laboratorial, a prognose e o tratamento do LPO, além de mencionar, brevemente, a experiência dos autores com esta enfermidade, vivida no Ambulatório de Estomatologia da Divisão de Dermatologia do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo.

Oral lichen planus (OLP) is a relatively common mucosal disease that can present isolated or associated with cutaneous lichen planus. Contrarily to its cutaneous counterpart, though, OLP tends to be chronic, relapsing, and difficult to treat. Severe morbidity is related to erosive forms, and more aggressive presentations have been described, such as the "gingivo-vulvar syndrome". This article reviews the current knowledge about the pathogenesis, clinical picture, differential and laboratorial diagnosis, prognosis, and treatment of OLP.

Síndrome carcinóide: relato de caso / Carcinoid syndrome: case report

Os tumores carcinóides são raros, com incidência aproximada de um a dois casos por 100.000 habitantes. Estima-se que a síndrome carcinóide - que se caracteriza por flushing, diarréia, dor abdominal e, em menor freqüência, pelagra, broncoespasmo e doença valvar cardíaca - ocorra em menos de 10% dos doentes. Entretanto, nos casos avançados a incidência varia de 40 a 50%. Estudos retrospectivos e de série de casos mostram que as manifestações cutâneas são freqüentes nessa entidade, embora sejam raramente relatadas. Apresenta-se um caso de síndrome carcinóide diagnosticadoa partir das manifestações dermatológicas.

Carcinoid tumors are rare neoplasms with an estimated incidence of 1 to 2 cases per 100,000 inhabitants. The malignant carcinoid syndrome, characterized by flushing, diarrhea, abdominal pain and, less frequently, pellagra, bronchospasm and valvular disease, occurs in less than 10% of patients. Nevertheless, in advanced stages of the disease, the incidence is about 40 to 50%. Retrospective studies and descriptive case series have shown that cutaneous involvement is relatively common but has rarely been reported. We present a case of carcinoid syndrome diagnosed with basis on skin findings on clinical examination.

Angioma em tufos: relato de um caso tratado com laser corante pulsado / Tufted angioma: a case report treated with pulsed-dye laser

O angioma em tufos é quadro raro, benigno, de proliferação cutânea angiomatosa. Acomete mais crianças e clinicamente caracteriza-se por lesões em placas, vermelho-violáceas, dolorosas e mais freqüentes no tronco. Quando não regride espontaneamente, os tratamentos propostos (cirurgia convencional, corticoterapia, interferon e laser) alcançam pouco sucesso. Descreve-se o caso de uma menina de 11 anos, com angioma em tufos no tronco, tratada com laser corante pulsado que mostrou alívio importante da dor local e redução discreta do tamanho da lesão. A raridade de casos relatados no Brasil e a tentativa terapêutica com laser corante pulsado motivaram a divulgação do caso.

Tufted angioma is a rare, benign, cutaneous angiomatous proliferation. It is more common in children and is clinically characterized by red-purple painfull plaques, more common in trunk. When spontaneous regression does not occur, proposed treatments (conventional surgery, steroid therapy, interferon and laser) achieve little success. We describe a case of a 11-year-old girl with a tufted angioma located in the trunk treated with pulsed-dye laser, showing important relief of local pain, and small reduction in lesion size. We have been motivated to describe this case due to the small number of such reports in Brazil and by the attempt to treat it with pulsed-dye laser.

Queilite actínica: estudo comparativo entre os achados histopatológicos da biópsia e dos cortes seriados provenientes da vermelhonectomia; estudo de 20 casos / Actinic cheilitis: comparision of microscopic findings on biopsy and on sections of vermilionectomy; a 20 cases study

Vinte casos de queilite actínica com alterações clínicas difusas foram inicialmente submetidos a uma biopsia e posteriormente `a vermelhonectomia. As peças cirúrgicas foram secionadas a cada 3 mm e os achados histopatológicos da biopsia foram comparados aos dos cortes seriados. As alterações epiteliais não ocorreram uniformemente ao longo do vermelhão; a biopsia freqüentemente não representou a área de maior gravidade histológica. Foram detectados focos de carcinoma superficialmente invasivo em áreas descontínuas ao longo do vermelhão em 4 casos. Os achados sugerem que casos de queilite actínica com alterações clínicas difusas possam se tratar de casos mais graves à histopatologia. / Twenty cases of actinic cheilitis with diffuse clinical changes were initially biopsied; a vermilionectomy was then performed. Surgical specimens were sectioned every 3mm; histopathological findings from biopsies were compared with the sections from vermilionectomies. Epithelial changes were not uniform along the vermilion; biopsies did not reveal the most severe area in many cases. Foci of non-continuous areas of superficially invasive carcinoma were detected in 4 cases. These findings suggest that cases of actinic cheilitis presenting with diffuse and poorly demarcated clinical lesions...

Sarcoma de Kaposi clássico disseminado associado a infecção por herpes-vírus humano tipo 8: relato de um caso e revisão de literatura / Disseminated Classic Kaposi's Sarcoma associated with human Herpesvirus 8 infection: case report and review of the Literature

O herpes-vírus humano tipo 8 foi isolado em lesões de Kaposi pela primeira vez em 1994. Desde então, o vírus tem sido relacionado a todas as formas de sarcoma de Kaposi estudadas, incluindo o sarcoma de Kaposi clássico. Seqüências de DNA viral e testes sorológicos têm sido usados para demonstrar essa relação. Os autores relatam o caso de um homem brasileiro de 68 anos com lesões extensas de sarcoma de Kaposi e comprovada infecção por herpes-vírus humano tipo 8. Apresentam breve revisão de literatura e discutem aspectos terapêuticos

Manifestacoes ungueais em indivíduos com a doenca pelo HIV / Nail changes in HIV disease

Alteraçoes ungueais sâo comumente observadas em doentes com doença pelo HIV. Neste trabalho apresentamos as mais importantes e propomos uma classificaçâo prática em: 1) onicoses devidas à doença debilitante; 2) onicoses medicamentosas; 3) onicoses infecciosas; 4) onicoses relacionadas a dermatoses diversas; 5) tumores acometendo o aparato ungueal