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.

Consensus on the treatment of autoimmune bullous dermatoses: bullous pemphigoid, mucous membrane pemphigoid and epidermolysis bullosa acquisita - Brazilian Society of Dermatology

Abstract: Bullous pemphigoid, mucous membrane pemphigoid and epidermolysis bullosa acquisita are subepidermal autoimmune blistering diseases whose antigenic target is located at the basement membrane zone. Mucous membrane pemphigoid and epidermolysis bullosa acquisita can evolve with cicatricial mucosal involvement, leading to respiratory, ocular and/or digestive sequelae with important morbidity. For each of these dermatoses, a literature review covering all therapeutic options was performed. A flowchart, based on the experience and joint discussion among the authors of this consensus, was constructed to provide treatment orientation for these diseases in Brazil. In summary, in the localized, low-risk or non-severe forms, drugs that have immunomodulatory action such as dapsone, doxycycline among others may be a therapeutic option. Topical treatment with corticosteroids or immunomodulators may also be used. Systemic corticosteroid therapy continues to be the treatment of choice for severe forms, especially those involving ocular, laryngeal-pharyngeal and/or esophageal mucosal involvement, as may occur in mucous membrane pemphigoid and epidermolysis bullosa acquisita. Several immunosuppressants are used as adjuvant alternatives. In severe and recalcitrant cases, intravenous immunoglobulin is an alternative that, while expensive, may be used. Immunobiological drugs such as rituximab are promising drugs in this area. Omalizumab has been used in bullous pemphigoid.

Granuloma annulare on the palms in an elderly patient

Abstract: Granuloma annulare is a relatively common, idiopathic, benign inflammatory dermatosis, with a varied clinical presentation that often makes diagnosis difficult. It mainly affects the extremities, such as the dorsa of the hands and feet, forearms and legs. Palmar and plantar regions are generally spared. It occurs mainly in young female patients. The presentation of the palmar variant in an elderly patient is a rarity.

Morbihan disease: a therapeutic challenge

Abstract: Morbihan disease is a rare condition characterized by chronic and persistent erythematous solid edema localized on the face. It is believed to be a complication of rosacea and may occur at any stage of the disease. Features of this condition include variable therapeutic response and great refractoriness. We report a case of a 61-year-old man with rosacea history diagnosed with Morbihan disease, who showed excellent therapeutic response with the combination of deflazacort and oral isotretinoin but developed recurrence after corticosteroid discontinuation. We believe that in severe cases of lymphedema of the face this combination is effective and corticosteroid suspension should be done slowly and gradually.

Pemphigus foliaceus with pustular presentation in a patient with psoriasis

Abstract Pemphigus foliaceus is a chronic autoimmune disease of the skin, clinically characterized by scaly and crusty cutaneous erosions involving the seborrheic areas. The patient can eventually become erythrodermic. There are reports of atypical cases of pemphigus foliaceus with pustules and neutrophils, and clinical differentiation from generalized pustular psoriasis of von Zumbusch is difficult. We report the case of a 55-year-old man with a history of psoriasis vulgaris that has developed pemphigus foliaceus with pustules, triggered by withdrawal of systemic corticosteroids. This is the first report associating this atypical form of pemphigus with psoriasis, suggesting that an overlap with generalized pustular psoriasis can occur.

Histiocytoid Sweet's syndrome presenting with annular erythematous plaques

Abstract Histiocytoid Sweet's Syndrome is a rare inflammatory disease described in 2005 as a variant of the classical Sweet's Syndrome (SS). Histopathologically, the dermal inflammatory infiltrate is composed mainly of mononuclear cells that have a histiocytic appearance and represent immature myeloid cells. We describe a case of Histiocytoid Sweet's Syndrome in an 18-year-old man. Although this patient had clinical manifestations compatible with SS, the cutaneous lesions consisted of erythematous annular plaques, which are not typical for this entity and have not been described in histiocytic form so far. The histiocytic subtype was confirmed by histopathological analysis that showed positivity for myeloperoxidase in multiple cells with histiocytic appearance.

Gianotti-Crosti syndrome: a case report of a teenager

Abstract Gianotti-Crosti syndrome is a rare disease characterized by acral papular eruption with symmetrical distribution. It is a benign and self-limited disease; the symptoms disappear after two to eight weeks, without recurrences or scars. Skin lesions are usually asymptomatic. Prodrome might occur, suggesting upper respiratory infection, or constitutional symptoms. Diagnosis is eminently clinical, and this disease is associated with viral infections. Due to its rarity and low occurrence in adolescents and adults, we report a case of Gianotti-Crosti syndrome of a teenager.

Lichen sclerosus associated with localized scleroderma: dermoscopy contribution

Abstract: Lichen sclerosus is an uncommon inflammatory dermatosis with preferential involvement of the urogenital region. The extragenital involvement is uncommon and is characterized by small rounded macules or papules, pearly white in color. The coexistence of lichen sclerosus and scleroderma plaques in most cases with extragenital location has been reported in the literature. We report a case of lichen sclerosus associated with scleroderma in children, highlighting the importance of dermoscopy in diagnosis.

Geographic tongue and psoriasis: clinical, histopathological, immunohistochemical and genetic correlation - a literature review

Abstract: Geographic tongue is a chronic, inflammatory, and immune-mediated oral lesion of unknown etiology. It is characterized by serpiginous white areas around the atrophic mucosa, which alternation between activity, remission and reactivation at various locations gave the names benign migratory glossitis and wandering rash of the tongue. Psoriasis is a chronic inflammatory disease with frequent cutaneous involvement and an immunogenetic basis of great importance in clinical practice. The association between geographic tongue and psoriasis has been demonstrated in various studies, based on observation of its fundamental lesions, microscopic similarity between the two conditions and the presence of a common genetic marker, human leukocyte antigen (HLA) HLA-C*06. The difficulty however in accepting the diagnosis of geographic tongue as oral psoriasis is the fact that not all patients with geographic tongue present psoriasis. Some authors believe that the prevalence of geographic tongue would be much greater if psoriatic patients underwent thorough oral examination. This study aimed to develop a literature review performed between 1980 and 2014, in which consultation of theses, dissertations and selected scientific articles were conducted through search in Scielo and Bireme databases, from Medline and Lilacs sources, relating the common characteristics between geographic tongue and psoriasis. We observed that the frequency of oral lesions is relatively common, but to establish a correct diagnosis of oral psoriasis, immunohistochemical and genetic histopathological analyzes are necessary, thus highlighting the importance of oral examination in psoriatic patients and cutaneous examination in patients with geographic tongue.

Clinical exuberance of classic Kaposi's sarcoma and response to radiotherapy

Abstract: Kaposi's sarcoma (KS) is a multicentric vascular neoplasm, with cutaneous and extracutaneous involvement. Different clinical and epidemiological variants have been identified. The classic form is manifested mainly in elderly men with indolent and long-term evolution, with lesions localized primarily in the lower extremities. We present two cases of classic Kaposi's sarcoma (CKS) in two female patients with extensive, exuberant skin involvement and rapid evolution, with good response to radiotherapy.

Dermatoscopic findings of urticaria pigmentosa / Achados dermatoscopicos na urticaria pigmentosa

Mastocytosis is a rare disease characterized by proliferation and accumulation of mast cells in various organs. The maculopapular cutaneus mastocytosis is divided into three subtypes: papular/plaque variant, urticaria pigmentosa and eruptive macular telangiectasia perstans. Dermoscopic may help to better characterize the different forms of cutaneus mastocytosis. We report a 55 year-old female with urticaria pigmentosa and its dermoscopy.

Mastocitose é uma doença rara caracterizada pela proliferação e acúmulo de mastócitos em vários órgãos. A mastocitose cutanea maculopapular divide-se em três subtipos: variante máculo-papular, urticária pigmentosa e telangiectasia macular eruptiva perstans. A dermatoscopia pode ajudar a caracterizar melhor as diferentes formas de mastocitose cutânea. Relatamos um caso de paciente feminina de 55 anos com urticária pigmentosa e sua dermatoscopia.

Systemic sarcoidosis induced by etanercept: first Brazilian case report / Sarcoidose sistemica induzida por etanercepte: primeiro relato de caso brasileiro

The antagonists of tumor necrosis factor alpha (TNF-α) are increasingly being used in the treatment of inflammatory and autoimmune diseases. Several adverse effects of these drugs have been reported, including the paradoxical development of sarcoidosis, especially with the use of etanercept. We present the first Brazilian case report of systemic sarcoidosis induced by etanercept and a literature review.

Os medicamentos antagonistas do fator de necrose tumoral alfa (TNF-α) estão sendo cada vez mais utilizados no tratamento de doenças inflamatórias e autoimunes. Efeitos adversos desses medicamentos vem sendo relatados, incluindo o desenvolvimento paradoxal de sarcoidose, principalmente com o uso do etanercepte. Apresentamos o primeiro relato de caso brasileiro de sarcoidose sistêmica induzida por etanercepte e uma revisão da literatura.

Diffuse systemic sclerosis with bullous lesions without systemic manifestations / Esclerose sistemica difusa bolhosa sem acometimentos sistemicos

Here, we describe an atypical case of systemic sclerosis in its diffuse cutaneous form with acute and rapid progression of the cutaneous condition, without any systemic manifestations and the infrequent formation of bullae, showing the importance of diagnosis and early treatment in such cases. This case also shows that special measures should be taken for bullous cutaneous lesions and ulcerations resulting from serious sclerosis, which are entry points and increase morbidity and risk of death. Other prognostic factors include age, ESR and renal and pulmonary involvement. Capillaroscopies can be useful predictors of greater severity of systemic scleroderma, revealing a greater link with systemic, rather than cutaneous, involvement.

Descrevemos um caso atípico de esclerose sistêmica em sua forma cutânea difusa com instalação aguda e rápida progressão do quadro cutâneo sem qualquer acometimento sistêmico e a infrequente formação de bolhas, demostrando a importância do diagnóstico e tratamento precoce frente a casos semelhantes e mostrando, com a experiência deste caso, que cuidados especiais devem ser tomados com as lesões cutâneas bolhosas e as ulcerações decorrentes da grave esclerose que são portas de entrada e aumentam a morbidade e risco de morte. Outros fatores prognósticos descritos são idade, VHS e envolvimento pulmonar e renal. A capilaroscopia pode ser preditor de maior gravidade da esclerodermia sistêmica, guardando maior relação com o envolvimento sistêmico do que cutâneo.

Case for diagnosis / Caso para diagnóstico

Lichen sclerosus is a chronic inflammatory mucocutaneous disorder of unknown etiology that most commonly affects the female genitalia. Cutaneous involvement with nonhaemorrhagic bullous is very unusual. We describe a case of bullous lichen sclerosus.

Líquen escleroso é uma doença mucocutânea inflamatória, crônica, de etiologia desconhecida, que afeta mais comumente a genitália feminina. O envolvimento cutâneo com bolhas não hemorrágicas é muito raro. Descrevemos um caso de líquen escleroso bolhoso.

Psoriasis, lymphoma and etanercept: is there a correlation? / Psoríase, linfoma e etanercepte: existe correlação?

Psoriasis is a chronic inflammatory disease that can affect skin and joints. Their treatment varies depending on the severity and includes topical and systemic. Among the latter are the immunobiological that target the T cell We report a case that demonstrates the close relationship between psoriasis, lymphoma and biologic therapies.

A psoríase é uma doença inflamatória crônica que pode afetar a pele e as articulações. Seu tratamento varia conforme a gravidade e inclui medicamentos tópicos e sistêmicos. Dentre os últimos estão os imunobiológicos, que têm como alvo a célula T. Relatamos um caso que demonstra a estreita relação entre a psoríase, o linfoma e os imunobiológicos.

Alterações cutâneas no Kwashiorkor: relato de caso de um homem adulto após cirurgia abdominal / Cutaneous manifestations of kwashiorkor: a case report of an adult man after abdominal surgery

Kwashiorkor é um tipo de desnutrição proteico-energética em que há deficiência dietética de proteína, embora a ingestão de calorias se mantenha adequada. As manifestações cutâneas incluem pele xerótica, com aspecto de esmalte descascado, típica coloração avermelhada a branco-acinzentada dos cabelos, o sinal da bandeira e edema mais evidente, nos membros inferiores e na face, dando aspecto de lua cheia. O presente artigo relata o caso de um paciente adulto, do sexo masculino, previamente submetido à duodenopancreatectomia para tratamento de pancreatite crônica associada ao pseudotumor em cabeça de pâncreas que evoluiu com alterações cutâneas de kwashiorkor após tuberculose pulmonar.

Kwashiorkor is a type of protein-energy malnutrition where diet protein deficit is found, in spite of appropriate caloric intake. Cutaneous manifestations include xerosis, with abnormally dry skin that has a flaking enamel paint aspect, a typical red to gray-white hair color, the "flag sign" and more evident edema in lower limbs and face, giving it a full moon appearance. This article reports a case of a male adult patient who had undergone Whipple surgery for treatment of chronic pancreatitis associated with pseudotumor of the pancreatic head that progressed to cutaneous manifestations of kwashiorkor after pulmonary tuberculosis.

Lipomatose simétrica benigna e pelagra, associadas ao alcoolismo / Benign symmetrical lipomatosis and pellagra associated with alcoholism

A 42-year-old male patient, alcoholic, presented showing signs of tumors in the neck and around the shoulders, scaly, erythematous-violaceous lesions and some bullous lesions in sun-exposed areas of upper and lower limbs. Based on clinical features, laboratory tests and imaging studies we have established the diagnosis of pellagra associated with benign symmetrical lipomatosis, both justified by chronic alcoholism. Treated with intravenous B-complex and oriented about the importance of alcohol withdrawal, the patient showed complete remission of skin lesions, but with no change in the lipomatosis.

Paciente masculino, 42 anos, etilista crônico, apresentando quadro de tumorações em região cervical e ao redor dos ombros, lesões eritemato-violáceas, descamativas e algumas lesões bolhosas, nas áreas fotoexpostas dos membros superiores e inferiores. Baseado no quadro clínico e nos exames complementares, foi estabelecido o diagnóstico de pelagra associada à lipomatose simétrica benigna, sendo ambos os quadros justificados pelo etilismo crônico. Tratado com reposição de complexo B intravenoso e orientado quanto à importância da abstinência alcoólica, apresentou remissão completa do quadro cutâneo, porém sem alteração da lipomatose.

Síndrome de Sweet associada a linfoma de Hodgkin: relato de caso / Sweet's Syndrome associated with Hodgkin's disease: case report

A síndrome de Sweet é enfermidade cutânea rara e de etiologia pouco esclarecida. Cerca de 20 por cento dos casos são associados a neoplasias hematológicas, sendo raros os casos relacionados à doença de Hodgkin. Relata-se caso de paciente masculino de 57 anos que desenvolveu a síndrome concomitantemente à neoplasia. As doenças foram controladas com o tratamento específico.

Sweet's syndrome is a rare cutaneous disease of unknown etiology. About 20 percent of the cases are associated with hematological neoplasms, and cases related with Hodgkin's disease are rare. We present the case of a 57-year old male patient who developed the syndrome concomitantly with the neoplasm. The diseases were controlled with specific treatment.

Associação de aciclovir oral e imiquimode no tratamento de herpes simples genital hipertrófico em paciente com sida: relato de dois casos / Association of oral acyclovir and imiquimod for the treatment of hypertrophic genital herpes simplex in HIV positive patients: report of two cases

Herpes simples crônico é doença capaz de gerar apresentações exuberantes, principalmente em pacientes com sida. O provável mecanismo da formação dessas lesões hiperplásicas consiste na invasão viral dos linfócitos T CD4 situados na epiderme. Diante das dificuldades terapêuticas e da grande taxa de insucesso do tratamento nesses pacientes, novas terapias têm sido citadas na literatura atualmente. O conhecimento da imunopatologia ratificou o mecanismo pelo qual o imiquimode poderia ser eficaz como terapia adjuvante aos antivirais. Relatamos aqui dois casos de pacientes tratados com aciclovir associado ao imiquimode tópico que obtiveram resposta clínica excepcional.

Chronic herpes simplex can present as exuberant clinical lesions, especially in HIV patients. The most probable mechanism of formation of these lesions is the invasion of the epidermal CD4 T cells by the herpes simplex virus. Due to the therapeutic difficulties and the high rates of treatment failure amongst these patients, new drugs are currently being discussed on the literature. Studies based on the immunophatology of these lesions have suggested that imiquimod might work as an adjuvant therapy to the antiviral drugs. Here we present two cases of excellent response to treatment with topical imiquimod as an adjuvant drug to acyclovir.