Prurigo Pigmentosa-Report of 3 Cases From Brazil and Literature Review.

Prurigo pigmentosa (PP) is a rare inflammatory disease of the skin of uncertain etiology first reported in Japan. It is typified by recurrent eruptions of itching urticarial macules, papules, vesicopapules, and plaques with a reticular arrangement that quickly resolve leaving a net-like pigmentation. The disease presents specific histopathological features. Herein, 3 cases of PP in Brazilians with no Japanese ancestry are reported and a revision of all previous English-language case reports indexed on PubMed is provided. Two articles with original case reports not listed on PubMed were also included. Our patients are 2 women and 1 man at the ages of 39, 33, and 22 years, respectively. All 3 presented findings in consonance with previous cases of PP and were diagnosed based on clinicopathological correlation. They were successfully treated with oral minocycline or doxycycline. In our literature review, a total of 210 previously reported cases were included. Although PP seemed to be restricted to Japanese patients in the first years after its recognition, the geographic boundaries of the disease are continuously expanding. Korea responded for 83 previous cases and Japan for 53. The mean age was 24.4 years, with 84.3% of the cases occurring between 11 and 30 years of age. The female/male rate was 2.6 and the most affected anatomical sites were back, chest, and neck. We do believe that the rarity of case reports in western countries may represent lack of awareness about the disease by dermatologists and dermatopathologists in these regions.

Oral candidiasis in patients with psoriasis: correlation of oral examination and cytopathological evaluation with psoriasis disease severity and treatment.

BACKGROUND: Infections are known to trigger and exacerbate psoriasis. Although oral candidiasis is often clinically diagnosed, it is not always confirmed by laboratory tests such as oral cytopathology. OBJECTIVES: The aims of this study were to determine the prevalence of oral candidiasis in patients with psoriasis through clinical and cytopathological diagnosis and to investigate the association between oral candidiasis and psoriasis with regards to the severity of the clinical presentation and the type of treatment for psoriasis. METHODS: A total of 140 patients with psoriasis and 140 healthy control subjects received an oral examination. Scrapings of the tongue were also obtained for a cytopathological examination. RESULTS: The oral examination and the results of the cytopathological smear revealed 37 (26%) cases of candidiasis in the patients with psoriasis and no cases of candidiasis in the healthy control subjects. There was no correlation between the type of psoriasis treatment and the presence of oral candidiasis (P = .616). There was a statistically significant association (P = .033) between the clinical severity of psoriasis and the presence of Candida. LIMITATIONS: This study was limited by the small number of subjects and the lack of follow-up to determine the development of psoriasis after treatment for oral candidiasis. CONCLUSIONS: The presence of oral candidiasis is higher in patients with psoriasis and it is associated with disease severity. This increased presence of oral candidiasis was apparent despite any type of treatment for the psoriasis. Cytopathology to rule out oral candidiasis should be used in the routine medical workup of patients with psoriasis.

Comedonic lupus, a rare variant of chronic cutaneous lupus erythematosus (CCLE): case series and literature review.

BACKGROUND: Cutaneous lupus erythematosus (CLE) is an autoimmune disease with several clinical presentations. The chronic form predominantly presents as discoid rashes but may present with less common morphological findings that can sometimes make diagnosis difficult. Comedonic lupus is a rare and underdiagnosed variant, with unknown etiology and still poorly defined treatment. METHODS: The report illustrates a series of five cases of patients diagnosed with comedonic lupus, and it reviews 18 cases previously published in the literature. RESULTS: The clinical presentation is of comedonal lesions, mostly located on the face, making a differential diagnosis with other benign conditions such as acne vulgaris, Favre-Racouchot syndrome, and syringoma, emphasizing the importance of clinical practice and histopathology for diagnostic confirmation. CONCLUSIONS: There is scarcity in the literature regarding the condition and therapeutic possibilities for these cases of comedonic lupus.

Early intervention in psoriasis: Where do we go from here?

Patients with psoriasis often have comorbidities and are at increased risk of developing several complications compared with the general population. Knowledge on the role of immune mediators and systemic inflammation in psoriasis has led to the hypothesis that early intervention with systemic therapy has the potential to modify the course of the disease and reduce the risk of long-term adverse outcomes. In this article, we address some potential issues that need to be considered before early intervention can be implemented routinely. The first is determining what constitutes "early" intervention for psoriasis. A second point is whether the intervention should be considered for patients with early disease or for selected subsets based on risk stratification. A third important consideration is defining success for early intervention. Finally, adoption of early and effective intervention should be based on high-level evidence. Ideally, randomized trials would be the best strategy to compare early vs. late systemic treatment in patients with psoriasis, probably using the frequency of long-term outcomes as primary endpoint, with cutaneous and pharmacoeconomic outcomes assessed secondarily.

Skin manifestations associated with systemic diseases - Part I.

The skin demonstrates what is happening in the body in many diseases, as it reflects some internal processes on the surface. In this sense, skin as an organ, goes beyond its protective and barrier functions, as it provides clues for the identification of some systemic diseases. The dermatologist then raises diagnostic hypotheses for conditions related to all systems and refers them to the appropriate specialty. With easy access to examination by trained eyes and biopsies, the skin can present specific or non specific alterations on histopathology. In the first case this combination establishes the diagnosis of the disease itself. Non specific manifestations can occur in a variety of contexts and then histopathology is not specific of a particular disease. This article is divided into two parts that will cover large groups of diseases. In this first part, cutaneous manifestations of the main rheumatologic diseases are described, which are the ones with the greatest interface with dermatology. The authors also talk about vascular manifestations and granulomatous diseases. In the second part, endocrinological, hematological, oncological, cardiovascular, renal, gastrointestinal diseases, pruritus and its causes are discussed, and finally, the dermatological manifestations of SARS-CoV-2 coronavirus infection. The authors' intention is that, by using direct and easily accessible language, aim to provide practical material for consultation and improvement to all dermatologists who recognize the importance of a comprehensive assessment of their patients.

Semantic and psychometric validation of the Brazilian Portuguese version (PASE-P) of the Psoriatic Arthritis Screening and Evaluation questionnaire.

PASE (Psoriatic Arthritis Screening and Evaluation) was developed in the English language to screen for inflammatory arthritis among patients with psoriasis. It is 15 item self administered questionnaire with a score from 15 to 75. A higher score indicates a greater risk for inflammatory joint disease. The purpose of this study was to translate, adapt and validate this questionnaire into Brazilian Portuguese (PASE-P). METHODS: 465 patients diagnosed with psoriasis (158 with psoriatic arthritis confirmed by a rheumatologist according to the CASPAR criteria and 307 without) were evaluated in dermatology clinics. We performed the analysis of semantic equivalence in eight steps. For psychometric equivalence, we evaluated the data quality, reliability, construct validity, well-known groups and discriminant characteristics of the items, as well as a ROC curve to determine optimal PASE-P cutoff points in case identification and their sensitivity / specificity. The final version presented excellent reproducibility (CCI = 0.97) and reliability (Cronbach's alpha> 0.9). A cut-off point of 25 distinguished between patients with and without psoriatic arthritis, with sensitivity of 69.5 and specificity of 86.8. PASE-P proved to be culturally valid and reliable to screen for psoriatic arthritis in Brazilian patients with psoriasis.

Skin manifestations associated with systemic diseases - Part I

Abstract The skin demonstrates what is happening in the body in many diseases, as it reflects some internal processes on the surface. In this sense, skin as an organ, goes beyond its protective and barrier functions, as it provides clues for the identification of some systemic diseases. The dermatologist then raises diagnostic hypotheses for conditions related to all systems and refers them to the appropriate specialty. With easy access to examination by trained eyes and biopsies, the skin can present specific or non specific alterations on histopathology. In the first case this combination establishes the diagnosis of the disease itself. Non specific manifestations can occur in a variety of contexts and then histopathology is not specific of a particular disease. This article is divided into two parts that will cover large groups of diseases. In this first part, cutaneous manifestations of the main rheumatologic diseases are described, which are the ones with the greatest interface with dermatology. The authors also talk about vascular manifestations and granulomatous diseases. In the second part, endocrinological, hematological, oncological, cardiovascular, renal, gastrointestinal diseases, pruritus and its causes are discussed, and finally, the dermatological manifestations of SARS-CoV-2 coronavirus infection. The authors' intention is that, by using direct and easily accessible language, aim to provide practical material for consultation and improvement to all dermatologists who recognize the importance of a comprehensive assessment of their patients.

Evaluation of treatment with carboxymethylcellulose on chronic venous ulcers.

BACKGROUND: Among the chronic leg ulcers, venous ulcers are the most common and constitute a major burden to public health. Despite all technology available, some patients do not respond to established treatments. In our study, carboxymethylcellulose was tested in the treatment of refractory chronic venous ulcers. OBJECTIVE: To evaluate the efficacy of carboxymethylcellulose 20% on the healing of chronic venous ulcers refractory to conventional treatments. METHODS: This is an analytical, pre-experimental study. Thirty patients were included with refractory venous ulcers, and applied dressings with carboxymethylcellulose 20% for 20 weeks. The analysis was based on measurement of the area of ulcers, performed at the first visit and after the end of the treatment. RESULTS: There was a reduction of 3.9 cm2 of lesion area (p=0.0001), corresponding to 38.8% (p=0.0001). There was no interruption of treatment and no increase in lesion area in any patient. CONCLUSIONS: Carboxymethylcellulose 20% represents a low cost and effective therapeutic alternative for the treatment of refractory chronic venous ulcers. However, controlled studies are necessary to prove its efficacy.

Prevalence of psoriatic arthritis in a large cohort of Brazilian patients with psoriasis.

OBJECTIVE: To determine the prevalence of psoriatic arthritis (PsA) in a large cohort of Brazilian patients with psoriasis (PsO) being seen at dermatology centers. METHODS: A multicenter study was conducted in 4 university dermatology clinics. In each center, consecutive patients with confirmed diagnoses of PsO were evaluated by a rheumatologist. Individuals were classified as having PsA according to the ClASsification criteria for Psoriatic ARthritis (CASPAR). Laboratory tests and radiographs were performed, as needed, based on the clinical judgment of the rheumatologist. RESULTS: A total of 524 patients with PsO were evaluated. The mean age was 48.5 ± 14.5 years, 50% were women, and the mean PsO duration was 15.4 ± 11.7 years. A diagnosis of PsA was documented in 175 patients (33%), of whom 49% were newly identified by the rheumatologist. Most individuals with PsA (72%) had peripheral involvement, 11% had isolated axial involvement, and 17% had both peripheral and axial involvement. Dactylitis occurred in 20% and clinical enthesitis in 30% of the patients. Laboratory and/or radiograph tests were necessary for a definitive diagnosis of PsA in 42 of 175 individuals (24%). CONCLUSION: In our study, one-third of Brazilian patients with PsO, followed in dermatology settings, were diagnosed with PsA by a rheumatologist. Almost half of subjects with PsA had no previous diagnosis. A collaboration between dermatologists and rheumatologists is greatly needed to establish earlier PsA diagnoses and adequate multidisciplinary management.

Red face revisited: Endogenous dermatitis in the form of atopic dermatitis and seborrheic dermatitis.

Atopic dermatitis and seborrheic dermatitis are multifactorial dermatitides that are known collectively as endogenous dermatitis. Both conditions can affect the face, but they have clinical, epidemiological, and physiopathological peculiarities that distinguish them from each other. These two diseases are very common all around the world. Atopic dermatitis is associated with xerosis and increased susceptibility to irritants and proteins; patients with this condition have a tendency to develop asthma, allergic rhinitis, and systemic manifestations that are mediated by immunoglobulin E. Seborrheic dermatitis is a moderate chronic dermatitis that is restricted to regions with a high production of sebum and areas that have cutaneous folds. There are many studies about pathophysiology related to the immunology and genetics of atopic dermatitis, but little is known about the genetic and immunological markers of seborrheic dermatitis.

Geographic stomatitis: an oral manifestation of psoriasis?

BACKGROUND: Geographic stomatitis is an uncommon oral lesion that presents similar clinical, histopathological and genetic features as those of psoriasis. These findings suggest that this lesion may actually represent an oral manifestation of psoriasis. We report one case of geographic stomatitis and discuss a possible connection between this condition and psoriasis. MAIN OBSERVATIONS: A 37-year-old woman presented with red patches, surrounded by a white border on the labial mucosa and a positive family history of psoriasis. Histopathological examination, immunohistochemical analysis with antibodies against CD4, CD8, CD20, CD68, CD31, and Ki-67 and HLA-A*, -B*, -C*, -DRB1*, -DQA1* and -DQB1* genotyping were performed. Histopathological examination revealed parakeratosis, marked elongation of rete ridges with acanthosis and clubbing, exocytosis, Munro microabscesses, pustule of Kogoj, dilated tortuous vessels at the tip of dermal papillae, and predominant superficial and perivascular lymphocytic chronic inflammatory cell infiltrate. Immunohistochemistry analysis revealed a predominant T-cell subepithelial infiltrate. Based on the referred clinicopathological findings and in the absence of cutaneous lesions, the diagnosis of geographic stomatitiswas confirmed. CONCLUSIONS: This case and theoretical data indicate that geographic stomatitis may be an oral manifestation of psoriasis. Moreover, to improve our understanding, psoriatic patients should routinely undergo a detailed oral examination and patients with geographic stomatitis should routinely be submitted to a cutaneous routine examination.

Glomeruloid hemangioma in POEMS syndrome: a report on two cases and a review of the literature.

Glomeruloid hemangioma is characterized by coiled capillary vessels contained within enlarged vascular spaces displaying an architecture that resembles renal glomeruli. The condition is strongly associated with POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy and Skin changes). The present paper reports on two cases of glomeruloid hemangiomas associated with POEMS syndrome, and includes a review of the literature. Case one refers to a 63-year old female patient admitted to hospital with ascites, hepatosplenomegaly, walking difficulties and cutaneous hemangiomas. Histopathology revealed a diagnosis of glomeruloid hemangioma and served to guide the clinical work-up, which revealed sensorimotor polyneuropathy, a plasmacytoma in the L4 vertebra with tumor cells positive for kappa chain, and diabetes mellitus. These findings permitted a diagnosis of POEMS syndrome to be reached. The second case consisted of a 39-year old woman admitted to hospital with edema, ascites, pleural effusion, glomeruloid hemangiomas and lymphadenopathy (Castleman's disease). Additional findings included monoclonal IgG-lambda paraproteinemia, blastic lesions in the right iliac bone and L4 vertebra, and demyelinating sensorimotor neuropathy affecting all four limbs. The final diagnosis in this case was POEMS syndrome associated with Castleman's disease.

Seborrheic dermatitis.

Seborrheic dermatitis is a chronic relapsing erythematous scaly skin disease, the prevalence of which is around 1 to 3% of the general population in the United States. It has two incidence peaks, the first in the first three months of life and the second beginning at puberty and reaching its apex at 40 to 60 years of age. The prevalence of seborrheic dermatitis is higher in HIV-positive individuals and the condition tends to be more intense and refractory to treatment in these patients. Neurological disorders and other chronic diseases are also associated with the onset of seborrheic dermatitis. The currently accepted theory on the pathogenesis of this disease advocates that yeast of Malassezia spp., present on the skin surface of susceptible individuals, leads to a non-immunogenic irritation due to the production of unsaturated fatty acids deposited on the skin surface. This article provides a review of the literature on seborrheic dermatitis, focusing on immunogenetics, the clinical forms of the disease and its treatment.

Evaluation of treatment with carboxymethylcellulose on chronic venous ulcers

Abstract BACKGROUND: Among the chronic leg ulcers, venous ulcers are the most common and constitute a major burden to public health. Despite all technology available, some patients do not respond to established treatments. In our study, carboxymethylcellulose was tested in the treatment of refractory chronic venous ulcers. OBJECTIVE: To evaluate the efficacy of carboxymethylcellulose 20% on the healing of chronic venous ulcers refractory to conventional treatments. METHODS: This is an analytical, pre-experimental study. Thirty patients were included with refractory venous ulcers, and applied dressings with carboxymethylcellulose 20% for 20 weeks. The analysis was based on measurement of the area of ulcers, performed at the first visit and after the end of the treatment. RESULTS: There was a reduction of 3.9 cm2 of lesion area (p=0.0001), corresponding to 38.8% (p=0.0001). There was no interruption of treatment and no increase in lesion area in any patient. CONCLUSIONS: Carboxymethylcellulose 20% represents a low cost and effective therapeutic alternative for the treatment of refractory chronic venous ulcers. However, controlled studies are necessary to prove its efficacy.

[Sclerosing perineurioma: case report and literature review]. / Perineurioma esclerosante: relato de caso e revisão da literatura.

Sclerosing perineurioma is a rare benign neoplasm composed exclusively of perineural differentiation cells spread in a dense fibrous stroma. It affects primarily the skin of fingers and that of the palm of the hands. It appears as a hard papule or nodule, normochromic and asymptomatic. A case of sclerosing perineurioma in the left palm of a 16-year-old female is described, with detailed demonstration of clinical aspects, histopathology and literature review published in the English language.

Hemangioma glomeruloide e a síndrome POEMS: relato de dois casos e revisão da literatura / Glomeruloid hemangioma in POEMS syndrome: a report on two cases and a review of the literature

O hemangioma glomeruloide caracteriza-se por enovelados capilares contidos em espaços vasculares dilatados reminiscentes de glomérulos renais, sendo fortemente associado à síndrome POEMS (polineuropatia, organomegalia, endocrinopatia, gamopatia monoclonal e alterações cutâneas). Relatamse dois casos da síndrome associados a hemangiomas glomeruloides e faz-se uma revisão da literatura. O primeiro é uma paciente feminina, 63 anos, internada para investigação de ascite, hepatoesplenomegalia, dificuldade de deambulação e hemangiomas cutâneos. A histopatologia de uma dessas lesões estabeleceu o diagnóstico de hemangioma glomeruloide e direcionou a investigação, que revelou polineuropatia sensitivo-motora, plasmocitoma kappa-positivo em L4 e Diabetes mellitus, permitindo o diagnóstico da síndrome. O segundo caso é de uma paciente feminina, 39 anos, com edema, ascite, derrame pleural, hemangiomas glomeruloides e linfonodomegalias (doença de Castleman). Havia um componente monoclonal de IgG-lambda e lesões blásticas no ilíaco direito e em L4, assim como lesão desmielinizante sensitivo-motora nos quatro membros, compondo o diagnóstico de síndrome POEMS.

Glomeruloid hemangioma is characterized by coiled capillary vessels contained within enlarged vascular spaces displaying an architecture that resembles renal glomeruli. The condition is strongly associated with POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy and Skin changes). The present paper reports on two cases of glomeruloid hemangiomas associated with POEMS syndrome, and includes a review of the literature. Case one refers to a 63-year old female patient admitted to hospital with ascites, hepatosplenomegaly, walking difficulties and cutaneous hemangiomas. Histopathology revealed a diagnosis of glomeruloid hemangioma and served to guide the clinical work-up, which revealed sensorimotor polyneuropathy, a plasmacytoma in the L4 vertebra with tumor cells positive for kappa chain, and diabetes mellitus. These findings permitted a diagnosis of POEMS syndrome to be reached. The second case consisted of a 39-year old woman admitted to hospital with edema, ascites, pleural effusion, glomeruloid hemangiomas and lymphadenopathy (Castleman's disease). Additional findings included monoclonal IgG-lambda paraproteinemia, blastic lesions in the right iliac bone and L4 vertebra, and demyelinating sensorimotor neuropathy affecting all four limbs. The final diagnosis in this case was POEMS syndrome associated with Castleman's disease.

Dermatite seborreica / Seborrheic dermatitis

A dermatite seborreica é uma doença eritêmato-escamativa de caráter crônico-recidivante que acomete entre 1 e 3 por cento da população geral dos Estados Unidos. Possui dois picos de incidência - o primeiro, durante os três primeiros meses de vida, e o segundo, a partir da puberdade, atingindo seu ápice entre os 40 e 60 anos de idade. Os indivíduos HIV positivos têm maior prevalência da doença, que apresenta maior intensidade e tendência à refratariedade ao tratamento. Doenças neurológicas e outras doenças crônicas também estão associadas ao desenvolvimento da dermatite seborreica. Como mecanismo fisiopatogênico, reconhece-se que o fungo Malassezia sp., presente na pele de indivíduos suscetíveis, leve a uma irritação não-imunogênica a partir da produção de metabólitos à base de ácidos graxos insaturados deixados na superfície cutânea. Este artigo faz uma revisão da literatura sobre dermatite seborreica, com ênfase nos aspectos imunogenéticos, formas clínicas e tratamento.

Seborrheic dermatitis is a chronic relapsing erythematous scaly skin disease, the prevalence of which is around 1 to 3 percent of the general population in the United States. It has two incidence peaks, the first in the first three months of life and the second beginning at puberty and reaching its apex at 40 to 60 years of age. The prevalence of seborrheic dermatitis is higher in HIV-positive individuals and the condition tends to be more intense and refractory to treatment in these patients. Neurological disorders and other chronic diseases are also associated with the onset of seborrheic dermatitis. The currently accepted theory on the pathogenesis of this disease advocates that yeast of Malassezia spp., present on the skin surface of susceptible individuals, leads to a non-immunogenic irritation due to the production of unsaturated fatty acids deposited on the skin surface. This article provides a review of the literature on seborrheic dermatitis, focusing on immunogenetics, the clinical forms of the disease and its treatment.

Perineurioma esclerosante: relato de caso e revisão da literatura / Sclerosing perineurioma: case report and literature review

O perineurioma esclerosante é uma neoplasia benigna rara,constituída exclusivamente por células com diferenciação perineural, dispersas em meio a estroma fibroso denso. Acomete preferencialmente a pele dos quirodáctilos e das regiões palmares e se apresenta como pápula ou nódulo firme, normocrômico e assintomático. Apresentamos um caso de perineurioma esclerosante localizado na região palmar esquerda de uma paciente feminina de 16 anos de idade, com detalhada demonstração dos aspectos clínicos, histopatologia e revisão da literatura publicada em língua inglesa.

Sclerosing perineurioma is a rare benign neoplasm composed exclusively of perineural differentiation cells spread in a dense fibrous stroma. It affects primarily the skin of fingers and that of the palm of the hands. It appears as a hard papule or nodule, normochromic and asymptomatic. A case of sclerosing perineurioma in the left palm of a 16-year-old female is described, with detailed demonstration of clinical aspects, histopathology and literature review published in the English language.