Biologic therapy for psoriasis - still searching for the best target.

Psoriasis is a chronic skin disease that results from the complex interaction between genetic and environmental factors. Over the last few decades, scientific evidence has redirected the focus of therapeutic studies to the immunologic pathways underlying its pathogenesis. This led to the biologic boom that we are currently experiencing, with the development and approval of targeted progressively more selective biological therapies and ongoing clinical trials of increasingly specific drugs, given their important implications for long-term efficacy and safety. Nevertheless, the search for the optimal biologic is still ongoing, and the best target has yet to be found.

Congenital multiple clustered dermatofibroma and multiple eruptive dermatofibromas--unusual presentations of a common entity.

Dermatofibroma is one of the most common entities seen in dermatology clinical practice. Several clinical subtypes have nevertheless been described, all of them of uncommon occurrence. The authors present two rare clinical variants of dermatofibromas: congenital multiple clustered dermatofibroma (the presented case is the 4th congenital case to be reported so far) and multiple eruptive dermatofibromas developing in the setting of a Sjögren's syndrome. Since the uncommon subtypes may not be clinically evident, dermatologists should familiarize themselves with their main features and we advise a high level of clinical suspicion in order to reach the correct diagnosis.

Congenital multiple clustered dermatofibroma and multiple eruptive dermatofibromas - unusual presentations of a common entity / Dermatofibroma multiplo agrupado congenito e dermatofibromas multiplos eruptivos - apresentacoes singulares de uma entidade comum

Dermatofibroma is one of the most common entities seen in dermatology clinical practice. Several clinical subtypes have nevertheless been described, all of them of uncommon occurrence. The authors present two rare clinical variants of dermatofibromas: congenital multiple clustered dermatofibroma (the presented case is the 4th congenital case to be reported so far) and multiple eruptive dermatofibromas developing in the setting of a Sjögren's syndrome. Since the uncommon subtypes may not be clinically evident, dermatologists should familiarize themselves with their main features and we advise a high level of clinical suspicion in order to reach the correct diagnosis.

O dermatofibroma é uma das entidades mais frequentemente observadas na prática clínica dermatológica. No entanto, além do dermatofibroma comum, vários subtipos clínicos de ocorrência incomum têm sido descritos na literatura. Os autores descrevem duas variantes clínicas raras de dermatofibromas: dermatofibroma múltiplo agrupado congênito (o caso apresentado é o quarto caso congênito reportado até hoje) e dermatofibromas eruptivos múltiplos no contexto de uma Síndrome de Sjögren. Estes diagnósticos menos comuns podem não ser clinicamente evidentes portanto os dermatologistas devem estar familiarizados com estas apresentações, sendo de suma importância um elevado índice de suspeita clínica.

Cutaneous lesions and finger clubbing uncovering hypocomplementemic urticarial vasculitis and hepatitis C with mixed cryoglobulinemia / Lesoes cutaneas e baqueteamento digital reveladores de vasculite urticariforme hipocomplementemica e hepatite C com crioglobulinemia mista

Urticarial vasculitis is a rare clinicopathologic entity characterized by urticarial lesions that persist for more than 24 hours and histologic features of leukocytoclastic vasculitis. Patients can be divided into normocomplementemic or hypocomplementemic. The authors report the case of a healthy 49-year-old woman with a 1-year history of highly pruritic generalized cutaneous lesions and finger clubbing. Laboratory tests together with histopathologic examination allowed the diagnosis of hypocomplementemic urticarial vasculitis, chronic hepatitis C and type II mixed cryoglobulinemia. The patient started symptomatic treatment and was referred to a gastroenterologist for management of the hepatitis C, with progressive improvement of the skin condition. The development of hypocomplementemic urticarial vasculitis in the context of chronic hepatitis C is exceedingly rare and possible pathogenic mechanisms are discussed.

A vasculite urticariforme é uma entidade clinico-patológica rara caracterizada por lesões urticariformes com duração superior a 24 horas e uma vasculite leucocitoclásica na histologia. É dividida em normo e hipocomplementêmica. Os autores relatam o caso de uma mulher saudável de 49 anos, com lesões cutâneas intensamente pruriginosas e baqueteamento digital com 1 ano de evolução. O estudo efectuado permitiu efectuar os diagnósticos de vasculite urticariforme hipocomplementêmica, hepatite C crônica e crioglobulinêmia mista tipo II. A doente iniciou tratamento sintomático e foi referenciada para a Gastroenterologia para orientação da hepatite, com melhoria progressiva das lesões cutâneas. O desenvolvimento de vasculite urticariforme hipocomplementêmica no contexto de hepatite C crónica é raro e os possíveis mecanismos patogênicos são discutidos.

Cutaneous manifestations of antiphospholipid syndrome: a review of the clinical features, diagnosis and management.

Antiphospholipid syndrome is a relatively recent systemic autoimmune disorder defined by thrombotic events and/or obstetric complications in the presence of persistent elevated antiphospholipid antibodies. It\'s characterized by a wide spectrum of clinical presentations and virtually any organ system or tissue may be affected by the consequences of vascular occlusion. Diagnosis is sometimes difficult and although classification criteria have been published and revised there remain ongoing issues regarding nomenclature, expanding clinical features, laboratory tests and management and much still has to be done. Cutaneous manifestations are common and frequently the first sign of the disease. Although extremely diverse it\'s important to know which dermatological findings should prompt consideration of antiphospholipid syndrome and the appropriate management for those patients. Much has been debated about when to consider antiphospholipid syndrome and consensus still does not exist, however in spite of being a diagnostic challenge clinicians should know when to look for antiphospholipid antibodies since an early diagnosis is important to prevent further and serious complications. In this article we focus on the cutaneous features that should raise suspicion on the presence of antiphospholipid syndrome and on the complex management of such patients. Many other dermatological signs related to this syndrome have been described in the literature but only occasionally and without consistency or statistic impact and therefore will not be considered here.

Unknown: Bluish-gray macules on the hands of a healthy 34 year-old man.

A 34 year-old man presented with asymptomatic bluish-gray macules on his hands that had developed over the previous 2 years. He was otherwise healthy and was on no regular medication. A detailed clinical history and histologic examination allowed the diagnosis.Histopathologic examination showed deposits of aggregated granules of black pigment in the dermis, localized preferentially around the sweat glands. This was consistent with the deposition of silver salts. Given the absence of systemic complaints or other signs and symptoms, a conservative approach was adopted. The lesions remain unchanged after one year of follow up.The wide range of uses for silver allows exposure to its compounds (metal, soluble and insoluble compounds) through different routes of entry, namely direct contact, ingestion, inhalation, and puncture. [1] This exposure is usually occupational, iatrogenic, or accidental. [1] Argyria is an exceedingly rare disease that became uncommon because medications containing silver are no longer used and occupational protection has evolved significantly. [2] It is caused by the deposition of silver grains in the skin and is further divided into localized and generalized forms, according to the route of entry. [1-4] Localized argyria is caused by direct contact with silver (the tiny particles penetrate the skin through the sweat glands) or puncture. [1, 3, 4] These deposits remain indefinitely in the skin and are characterized by a bluish gray color, more prominent in the photo-exposed areas. [3, 4] In the localized forms, patients usually don't have systemic symptoms and the problem is cosmetic. [1-4] Given the improvement in the safety of working conditions, occupational argyria cases are becoming quite uncommon. [1-4].

A dramatic case of Behçet disease successfully treated with infliximab.

Behçet disease is a chronic relapsing systemic disease with possible life-threatening presentations. Management of this disease can be challenging and reports of the off-label use of anti-TNFα agents for the treatment of severe manifestations are increasing, with good results. The authors report a case of Behçet disease with a sudden and severe multi-systemic onset successfully treated with infliximab.

Cutaneous lesions and finger clubbing uncovering hypocomplementemic urticarial vasculitis and hepatitis C with mixed cryoglobulinemia.

Urticarial vasculitis is a rare clinicopathologic entity characterized by urticarial lesions that persist for more than 24 hours and histologic features of leukocytoclastic vasculitis. Patients can be divided into normocomplementemic or hypocomplementemic. The authors report the case of a healthy 49-year-old woman with a 1-year history of highly pruritic generalized cutaneous lesions and finger clubbing. Laboratory tests together with histopathologic examination allowed the diagnosis of hypocomplementemic urticarial vasculitis, chronic hepatitis C and type II mixed cryoglobulinemia. The patient started symptomatic treatment and was referred to a gastroenterologist for management of the hepatitis C, with progressive improvement of the skin condition. The development of hypocomplementemic urticarial vasculitis in the context of chronic hepatitis C is exceedingly rare and possible pathogenic mechanisms are discussed.

Vesico-bullous subacute cutaneous lupus erythematosus--an uncommon entity successfully treated with dapsone and hydroxychloroquine.

Vesico-bullous subacute cutaneous lupus erythematosus is an uncommon and severe presentation. The authors report an exuberant case of vesico-bullous subacute cutaneous lupus erythematosus successfully treated with dapsone and hydroxychloroquine.

Exuberant cutaneous ulcers on the buttocks caused by multi-resistant Klebsiella pneumoniae.

Cutaneous infection develops because of environmental and local factors, host immunity, and organism adherence and virulence. The authors report a case of exuberant cutaneous ulcers on the buttocks of a diabetic patient. Microbiologic examination allowed the identification of Klebsiella pneumoniae and complete resolution was achieved with the appropriate antibiotic.