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.

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.

Abstract Tattooing one’s body is currently a common practice worldwide; however, it is not risk-free. This is a case of a patient who tattooed himself motivated by his passion for motorcycles and then developed an exuberant lichenoid reaction to the red pigment used in the tattoo, with the appearance of verrucous lesions. Despite the lack of response to treatment, he states that he would tattoo his own skin again.

Abstract Background: Sarcoidosis is a multisystem disease of unknown cause that is characterized by the presence of granulomas in various organs. Cutaneous involvement is common and the reported incidence has varied from 9% to 37%. Studies on cutaneous sarcoidosis in Brazil are lacking. Objectives: To describe the clinical and epidemiological aspects of patients with cutaneous sarcoidosis diagnosed at the Department of Dermatology of the University of São Paulo, from May 1994 to March 2018. Methods: Clinical data of patients with confirmed cutaneous sarcoidosis were retrospectively reviewed and classified according to gender, ethnicity, age at diagnosis, cutaneous presentation, systemic involvement and treatment. Results: Cutaneous sarcoidosis was diagnosed in 72 patients with a female predominance (74%). The mean age at diagnosis was 49.6 years and most of the patients were white (61%). Papules and plaques were the most common lesions. Systemic sarcoidosis was detected in 81% of patients, affecting mainly the lungs and thoracic lymph nodes (97%). Typically, cutaneous lesions were the first manifestation (74%). Systemic therapy was necessary for 72% of patients; the dermatologist managed many of these cases. Oral glucocorticoids were the most commonly used systemic medication (92%). The mean number of systemic drugs used was 1.98 per patient. Limitations: Insufficient data in medical records. Conclusions: This series highlights the dermatologist role in recognizing and diagnosing cutaneous sarcoidosis, evaluating patients for systemic disease involvement and treating the skin manifestations. Cutaneous sarcoidosis was once considered exceedingly infrequent in Brazil in comparison to infectious granulomatous diseases; however, the present series seems to suggest that the disease is not so rare in this region.

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.

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.

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.

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.

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

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.

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.

É descrito um caso de entomoftoromicose rinofacial por Conidiobolus coronatus, em paciente procedente de São Paulo (Capital), tratado com sucesso pelo fluconazol, por via oral, através terapêutica prolongada. Dados histopatológicos e micológicos confirmaram o diagnóstico.

A case of rhinofacial entomophthoromycosis caused by Conidiobolus coronatus is reported in a 30 years old male from São Paulo, Brazil. The patient was successfully treated with oral fluconazole in a prolonged regimen. The diagnosis was confirmed by histopathological and mycological data.