Claudin expression profile in flat wart and cutaneous squamous cell carcinoma in epidermodysplasia verruciformis.

Epidermodysplasia verruciformis (EV) is a genodermatosis related to human beta-papillomavirus (beta-HPV), with a high risk of cutaneous squamous cell carcinoma (cSCC). Claudins are transmembrane proteins expressed in epithelia and may be altered during carcinogenesis. For a better understanding of the role of beta-HPV in cutaneous carcinogenesis, this claudin expression study was conducted on lesions of patients with and without EV. In this study, claudins-1, -2, -3, -4, -5, -7 and -11 expressions were analyzed by applying the immunohistochemistry technique, in samples of 108 normal skin, 39 flat warts and 174 cSCC. The cSCC samples were organized in tissue microarrays. We found that claudin-1 and claudin-3 focal expressions were associated with cSCC (p < 0.001), and claudin-2 focal or negative expression with flat wart (p < 0.001), in EV and NEV (non-EV) groups. For claudin-5, EV group showed a lower chance of focal and negative expression (p < 0.001), and its negative expression was associated with flat wart (p < 0.001) and lower mean age (p < 0.001). Claudins-4, -7 and -11 showed a diffuse expression in almost all studied samples. Our findings suggest that claudin-5 increased expression observed on normal skin, flat wart and cSCC showed association with EV. Claudin-1 and -3 down expression were also observed, but they could not be related to beta-HPV infection.

Panniculitides of particular interest to the rheumatologist.

The panniculitides remain as one of the most challenging areas for clinicians, as they comprise a heterogeneous group of inflammatory diseases involving the subcutaneous fat with potentially-shared clinical and histopathological features. Clinically, most panniculitides present as red edematous nodules or plaques. Therefore, in addition to a detailed clinical history, a large scalpel biopsy of a recent-stage lesion with adequate representation of the subcutaneous tissue is essential to specific diagnosis and appropriate clinical management. Herein we review the panniculitides of particular interest to the rheumatologist.

Panniculitides of particular interest to the rheumatologist

Abstract The panniculitides remain as one of the most challenging areas for clinicians, as they comprise a heterogeneous group of inflammatory diseases involving the subcutaneous fat with potentially-shared clinical and histopathological features. Clinically, most panniculitides present as red edematous nodules or plaques. Therefore, in addition to a detailed clinical history, a large scalpel biopsy of a recent-stage lesion with adequate representation of the subcutaneous tissue is essential to specific diagnosis and appropriate clinical management. Herein we review the panniculitides of particular interest to the rheumatologist.

Nevus Lipomatosus Cutaneous Superficialis With Perifollicular Fibromas.

Nevus lipomatosus cutaneous superficialis (NLCS) is a rare cutaneous hamartoma characterized by groups of mature fat cells in the dermis. The authors report a case of a dark-skinnned, 6-year-old, Brazilian girl with 1 lesion in the right upper chest, extending to the neck, since 1 year of age. The dermatological examination consisted of skin-colored and yellowish follicular papules, isolated and grouped into a papillomatous plaque. The biopsy revealed ectopic mature adipose tissue in the dermis, compatible with NLCS, perifollicular well-circumscribed fibroblast and collagen proliferation, and decreased elastic fibers in the papillary and upper reticular dermis. The finding of perifollicular fibrosis in NLCS is not common, having been first described in 2003. The perifollicular fibrosis is characteristic of other rare hamartoma, the perifollicular fibroma, but is also present in fibrofolliculomas or trichodiscomas, angiofibromas, and fibrotic papules on the face of tuberous sclerosis and postinflammatory fibrosis. This case is very similar to the first described case of NLCS with perifollicular fibrosis, and the authors believe that this is the second case of this entity, but they propose to name it NCLS with perifollicular fibromas, which better describes the nature of this lesion.

Unilateral livedoid vasculopathy associated with involutional phase of cutaneous infantile hemangioma: the connection to coagulation disorders.

Livedoid vasculopathy is a bilateral painful and recurrent cutaneous ulcerative disorder of the legs that leads to atrophie blanche, atrophic white-porcelain scars, and is associated with disorders of fibrinolysis and/or coagulation. We present a young boy with an association between livedoid vasculopathy in the area of a previous involuted cutaneous hemangioma. We found 4 uncommon abnormalities associated with thrombo-occlusive events: heterozygous 20210 A→G genotype of prothrombin, reduced activity of anticoagulation proteins C and S, and elevated lipoprotein (a).

Cutaneous mucinosis in mixed connective tissue disease.

Cutaneous mucinosis is a group of conditions involving an accumulation of mucin or glycosaminoglycan in the skin and its annexes. It is described in some connective tissue diseases but never in association with mixed connective tissue disease. This report concerns two cases of cutaneous mucinosis in patients with mixed connective tissue disease in remission; one patient presented the papular form, and the other reticular erythematous mucinosis. These are the first cases of mucinosis described in mixed connective tissue disease. Both cases had skin lesions with no other clinical or laboratorial manifestations, with clinical response to azathioprine in one, and to an association of chloroquine and prednisone in the other.

Cutaneous mucinosis in mixed connective tissue disease* / Mucinose cutanea na doenca mista do tecido conjuntivo

Cutaneous mucinosis is a group of conditions involving an accumulation of mucin or glycosaminoglycan in the skin and its annexes. It is described in some connective tissue diseases but never in association with mixed connective tissue disease. This report concerns two cases of cutaneous mucinosis in patients with mixed connective tissue disease in remission; one patient presented the papular form, and the other reticular erythematous mucinosis. These are the first cases of mucinosis described in mixed connective tissue disease. Both cases had skin lesions with no other clinical or laboratorial manifestations, with clinical response to azathioprine in one, and to an association of chloroquine and prednisone in the other.

A mucinose cutânea é um grupo de condições em que há um acúmulo de mucina ou glicosaminoglicanos na pele e seus anexos. É descrita em algumas doenças do tecido conjuntivo, porem nunca em associação com doença mista do tecido conjuntivo. Relatamos dois casos de mucinose cutânea em pacientes com doença mista do tecido conjuntivo em remissão, um apresentava-se sob a forma papular e outro sob a forma reticular eritematosa de mucinose. Estes são os primeiros casos de mucinose descritos na doença mista do tecido conjuntivo. Ambos os casos apresentaram o quadro cutâneo de modo isolado, sem nenhuma outra manifestação clínico-laboratorial, havendo resposta à azatioprina em um e à cloroquina associada a prednisona no outro.

An unusual giant thrombus displaying percutaneous elimination.

We describe a female patient with a giant venous thrombus that exhibited transcutaneous elimination. This thrombus elimination is probably a unique case in the medical literature.

Leprosy and multiple endocrine neoplasia type i: a case report and review of literature

Context: Leprosy is an infectious disease in which progress to disease and clinical expressions are influenced by environmental factors and by the genetics of the host. The multiple endocrine neoplasia type 1 syndrome (MEN-1) is an inherited disorder that predisposes to endocrine and non-endocrine tumors...

Leprosy and multiple endocrine neoplasia type I: a case report and review of literature / Hanseníase e neoplasia endócrina múltipla tipo 1: relato de caso e revisão de literatura

Context: Leprosy is an infectious disease in which progress to disease and clinical expressions are influenced by environmental factors and by the genetics of the host. The multiple endocrine neoplasia type 1 syndrome (MEN-1) is an inherited disorder that predisposes to endocrine and non-endocrine tumors. Case report: A case of multi-bacillary leprosy in the 18-year-old female patient with MEN- 1, this association was probably an incidental case of an endemic disease in a susceptible individual with a rare syndrome. The investigation of comorbities, especially neglected or emergency disease, is very importance, because the lack of early diagnosis may result in serious complications as development of disabilities especially when there is a leprosy late diagnosis.

Contexto: A hanseníase é uma doença infecciosa cuja evolução e expressões clínicas são influenciadas por fatores ambientais e pela genética do hospedeiro. A síndrome neoplasia endócrina múltipla tipo 1 (NEM-1)é uma desordem hereditária que predispõe a tumores endócrinos e não-endócrinos . Relato de caso: Relata-se um caso de hanseníase multibacilar em paciente de 18 anos de idade com NEM- 1. Esta associação foi, provavelmente, um caso incidental de uma doença endêmica em um indivíduo suscetível com uma síndrome rara. A pesquisa de comorbidades, especialmente entre as doenças negligenciadas ou emergentes é muito importante, porque a falta de diagnóstico precoce pode resultar em complicações graves como o desenvolvimento de deficiências especialmente quando há um diagnóstico de hanseníase tardio.

[Pedroso and Gomes' verrucous Dermatitis (Chromoblastomycosis): 90 years on and still among us]. / Dermatite verrucosa de Pedroso e Gomes (Cromomicose): 90 anos depois, ainda entre nós.

In this report, the authors describe the clinical case of a woman seeking care at this dermatology outpatient clinic with a verrucous plaque on her left elbow.

Dermatite verrucosa de Pedroso e Gomes (Cromomicose): 90 anos depois, ainda entre nós / Pedroso and Gomes' verrucous Dermatitis (Chromoblastomycosis): 90 years on and still among us

Os autores relatam o caso clínico de uma doente que procurou atendimento dermatológico em decorrência da presença de lesão em placa papulosa de superfície verrucosa no cotovelo esquerdo.

In this report, the authors describe the clinical case of a woman seeking care at this dermatology outpatient clinic with a verrucous plaque on her left elbow.

Factor XIIIa+ dermal dendrocyte parasitism in American tegumentary leishmaniasis skin lesions.

Dendritic cells belong to a family of antigen-presenting cells that are localized at the entry sites, such as skin and mucosa. Dendritic cells are related to immune surveillance function. The role of Langerhans cells in the pathogenesis of skin infectious diseases is well studied; however, there are few articles addressing involvement of factor XIIIa-positive dermal dendrocytes (FXIIIa+ DD) in such processes. FXIIIa+ DDs are bone marrow-monocytic lineage-derived cells and members of the skin immune system. Due to their immune phenotype and functional characteristics, they are considered complementary cells to Langerhans cells in the process of antigen presentation and inducing immune response. To verify the interaction between FXIIIa+ DD and Leishmania amastigotes, 22 biopsies of American tegumentary leishmaniasis (ATL) skin lesions were subjected to double staining technique with anti-factor XIIIa and anti-Leishmania antibodies. FXIIIa+ DDs were hypertrophic and abundant in the cutaneous reaction of ATL. FXIIIa+ DDs harboring parasites were observed in 11 of 22 skin biopsies. The data obtained suggest that FXIIIa+ DD plays a role in the pathogenesis of ATL skin lesion as host cell, immune effector, and/or antigen-presenting cell.