Maria Denise Fonseca Takahashi

Acquired localized cutis laxa confined to the face: case report and review of the literature

Background  Cutis laxa is an uncommon entity characterized by laxity of the skin, which hangs in loose folds, producing the appearance of premature aging. It can be subdivided into congenital and acquired. This latter variant is rare and the skin involvement varies from generalized to localized. We report a case of a localized acquired cutis laxa confined to the face, without preceding inflammatory lesions or systemic compromise. Four similar cases have been reported to date. The etiology remains unknown and there is no definitive treatment.

Generalized Pustular Psoriasis in Childhood

Abstract:  Generalized pustular psoriasis is a rare form of psoriasis consisting of a generalized eruption of sudden onset with erythema and sterile pustules. In children, generalized pustular psoriasis is even more uncommon and may present as a severe and potentially life‐threatening disorder. In this study, we present demographics, clinical aspects, treatment response, and follow‐up of seven children with generalized pustular psoriasis. Retrospective study reviewing the records of seven children with generalized pustular psoriasis including age, gender, age of onset, presence of scalp and nail involvement, family history, concomitant diseases, precipitating factors, treatment modalities, and outcome. Age of first symptoms ranged from 1 month to 11 years. All patients received systemic retinoids at one time of the follow‐up period. Other treatment modalities included immunosuppressive drugs, biologics, phototherapy, and sulfasalazine. Two patients presented with severe constitutional illness, secondary infection and septic shock, including one fatal outcome. All further cases have remained free of recurrences for a mean period of up to 3 years. In our study, generalized pustular psoriasis presented a wide clinical spectrum in children ranging from mild, asymptomatic outbreaks to more severe, life‐threatening episodes. One fatality was observed. Children generally responded well to systemic retinoids. Further studies and long‐term follow‐up periods are needed to define potential trigger factors, efficacy and safety of different treatment modalities in children with generalized pustular psoriasis.

Psoríase na infância e na adolescência

Psoriasis is a chronic, immunologically mediated, recurrent and universal inflammatory disorder. Approximately one third of adults refer onset before 16 years of age. The sooner the onset, the worse is the prognosis. In children, lesions may be physically disfiguring, leading to psychological impairment and evident loss of quality of life. Systemic therapy used in psoriasis, as well as phototherapy, has limited use in children due to accumulative effects of drugs, low acceptance, and risk of teratogenicity. In this section, we discuss the main clinical aspects of psoriasis in childhood and adolescence, differential diagnosis, therapeutic options, and prognosis.

Decrease in Mycobacterium tuberculosis specific immune responses in patients with untreated psoriasis living in a tuberculosis endemic area.

Tuberculosis has emerged as a major concern in patients with immuno-mediated diseases, including psoriasis, undergoing treatment with biologicals. However, it is not known whether the chronically activated immune system of psoriasis patients interferes with their Mycobacterium tuberculosis (Mtb)-specific immunity, especially in tuberculosis-endemic areas like Brazil. We evaluated T-cell responses to a Mtb lysate and to the recombinant Mtb proteins ESAT-6 and Ag85B of tuberculin skin test (TST) positive and TST negative patients with severe or mild/moderate, untreated psoriasis in three different assays: lymphocyte proliferation, enzyme immunoassay for interferon (IFN)-γ and interleukin (IL)-10 production by peripheral blood mononuclear cells and overnight enzyme immunospot (ELISpot) for enumerating IFN-γ-secreting cells. In our cohort, a low proportion (29%) of the severe psoriasis patients tested were TST-positive. IFN-γ and IL-10 secretion and T-cell proliferation to Mtb antigens were reduced in TST-negative but not in TST-positive patients with severe psoriasis when compared to healthy controls with the same TST status. Similarly, severe psoriasis patients had decreased cytokine secretion and proliferative response to phytohemagglutinin. However, most psoriasis patients and healthy controls showed detectable numbers of IFN-γ-secreting effector-memory T-cells in response to Mtb antigens by ELISpot. TST-negative, mild/moderate psoriasis patients had responses that were mostly intermediary between TST-negative controls and severe psoriasis patients. Thus, patients with severe psoriasis possess decreased anti-Mtb central memory T-cell responses, which may lead to false-negative results in the diagnosis of TB infection, but retain T-cell memory-effector activity against Mtb antigens. We hypothesize that the latter may confer some protection against tuberculosis reactivation.

Alpha-1-antitrypsin deficiency associated with panniculitis treated with plasma exchange therapy

Background  Alpha‐1‐antitrypsin is the principal serum protease inhibitor. In addition to the well‐recognized association with early‐onset emphysema and cirrhosis, alpha‐1‐antitrypsin deficiency may be associated with panniculitis. The treatment of this type of panniculitis presents a significant challenge. Previous attempts using immunosuppressive, anti‐inflammatory, and cytotoxic drugs have shown variable results.

Tinha favosa: relato de ocorrência familiar em Itapecerica da Serra (município da Grande São Paulo)

Four cases of Tinea favosa occurred in the same family are reported in a small city of Brazil (Itapecerica da Serra--State of São Paulo). Trichophyton schoenleinii was isolated from all four cases. Treatment consisted of oral griseofulvin (10 mg/kg/day) for at least three months.Four cases of Tinea favosa occurred in the same family are reported in a small city of Brazil (Itapecerica da Serra - State of Sao Paulo). Trichophyton schoenleinii was isolated from all four cases. Treatment consisted of oral griseofulvin (10 mg/kg/day) for at least three months.Os autores relatam 4 casos de tinha favosa por Trichophyton schoenleinii de ocorrencia familiar, identificados na cidade de Itapecerica da Serra, municipio da Grande Sao Paulo. Todos os casos foram tratados com griseofulvina por via oral na dose de 1.0 mg/kg/dia por no minimo 3 meses, tendo ocorrido cura clinica e laboratorial em 2 pacientes, e os dois restantes necessitaram de terapeutica mais prolongada.

Infliximab partially impairs the anti-Mycobacterium tuberculosis immune responses of severe psoriasis patients with positive tuberculin skin-test

Background  Infliximab and etarnecept are now widely used for treating severe psoriasis. However, these drugs, especially infliximab, increased the risk of tuberculosis reactivation. Surprisingly, epidemiological data suggest that the tuberculosis rate in patients taking infliximab in São Paulo State, Brazil, is similar to that of some developed, non‐endemic countries.

The many faces of tuberculosis of the oral mucosa - three cases with distinct pathomechanisms

mucormycosis in our patient. These findings reveal that cutaneous granuloma caused by R. oryzae can be a sign of CGD. Our case also highlights that early diagnosis and prophylactic treatment can significantly improve the prognosis for CGD. Supported by Beijing Natural Science Foundation (7174300), Beijing Municipal Administration of Hospitals Clinical Medicine Development of Special Funding (ZYLX201601), Capital Clinical Characteristics (Z161100000516070), National Science Foundation of China (81641118).

Tinea favosa. Familial occurrence in Itapecerica da Serra (São Paulo State).

Four cases of Tinea favosa occurred in the same family are reported in a small city of Brazil (Itapecerica da Serra--State of São Paulo). Trichophyton schoenleinii was isolated from all four cases. Treatment consisted of oral griseofulvin (10 mg/kg/day) for at least three months.Four cases of Tinea favosa occurred in the same family are reported in a small city of Brazil (Itapecerica da Serra - State of Sao Paulo). Trichophyton schoenleinii was isolated from all four cases. Treatment consisted of oral griseofulvin (10 mg/kg/day) for at least three months.Os autores relatam 4 casos de tinha favosa por Trichophyton schoenleinii de ocorrencia familiar, identificados na cidade de Itapecerica da Serra, municipio da Grande Sao Paulo. Todos os casos foram tratados com griseofulvina por via oral na dose de 1.0 mg/kg/dia por no minimo 3 meses, tendo ocorrido cura clinica e laboratorial em 2 pacientes, e os dois restantes necessitaram de terapeutica mais prolongada.

Tinea favosa: familiar occurence in Itapecerica da Serra (São Paulo State - Brazil)

Four cases of Tinea favosa occurred in the same family are reported in a small city of Brazil (Itapecerica da Serra--State of São Paulo). Trichophyton schoenleinii was isolated from all four cases. Treatment consisted of oral griseofulvin (10 mg/kg/day) for at least three months.Four cases of Tinea favosa occurred in the same family are reported in a small city of Brazil (Itapecerica da Serra - State of Sao Paulo). Trichophyton schoenleinii was isolated from all four cases. Treatment consisted of oral griseofulvin (10 mg/kg/day) for at least three months.Os autores relatam 4 casos de tinha favosa por Trichophyton schoenleinii de ocorrencia familiar, identificados na cidade de Itapecerica da Serra, municipio da Grande Sao Paulo. Todos os casos foram tratados com griseofulvina por via oral na dose de 1.0 mg/kg/dia por no minimo 3 meses, tendo ocorrido cura clinica e laboratorial em 2 pacientes, e os dois restantes necessitaram de terapeutica mais prolongada.