Porokeratosis ptychotropica of the scrotum: dermoscopic evaluation of an atypical presentation.

Porokeratosis ptychotropica is a rare variant of porokeratosis that is classically located on the gluteal and perianal regions, seldom extending to the genitalia. The authors report an atypical presentation of porokeratosis ptychotropica and discuss the use of dermoscopy in evaluating this dermatosis. Dermoscopic findings, although not specific to this variant of porokeratosis, are helpful in the differential diagnosis of other genital disorders. Histopathology, through the visualization of multiple cornoid lamellae, prevails as the gold standard for the definite diagnosis of porokeratosis ptychotropica.

Porokeratosis ptychotropica of the scrotum: dermoscopic evaluation of an atypical presentation

Porokeratosis ptychotropica is a rare variant of porokeratosis that is classically located on the gluteal and perianal regions, seldom extending to the genitalia. The authors report an atypical presentation of porokeratosis ptychotropica and discuss the use of dermoscopy in evaluating this dermatosis. Dermoscopic findings, although not specific to this variant of porokeratosis, are helpful in the differential diagnosis of other genital disorders. Histopathology, through the visualization of multiple cornoid lamellae, prevails as the gold standard for the definite diagnosis of porokeratosis ptychotropica.

[The tip of the iceberg: cutaneous manifestations of paediatric diseases with neurological involvement]. / A Ponta do Iceberg: Manifestações Cutâneas de Patologia Pediátrica com Envolvimento Neurológico.

INTRODUCTION: Some cutaneous lesions might be the first clinical manifestation of diseases with systemic involvement. This article aims to review the relevant dermatological semiology for the concerning diseases with neurological involvement in childhood. MATERIAL AND METHODS: Review of the related literature indexed to MedLine published in the last twelve years. RESULTS: The main relevant nosologic groups include the genodermatosis (namely neurocutaneous syndromes), pigmentary, vascular and endocrine disorders, congenital enzymatic deficiencies and occult spinal dysraphisms. DISCUSSION: The recognition of specific cutaneous signs is important once may enable a much earlier diagnosis.

Introdução: Algumas alterações cutâneas podem ser as primeiras manifestações clínicas de diversas entidades nosológicas com atingimento sistémico. O presente trabalho tem como objectivo rever a semiologia dermatológica relevante no contexto das doenças com envolvimento neurológico na infância. Material e Métodos: Revisão dos artigos indexados à MedLine publicados nos últimos 12 anos e com relevância para o tema. Resultados: Os principais grupos nosológicos relevantes para o tema compreendem as genodermatoses (com destaque para as síndromes neurocutâneas), as alterações da pigmentação, as doenças vasculares, as endócrinas, os défices enzimáticos congénitos e os disrafismos espinhais ocultos. Discussão: O reconhecimento da semiologia cutânea específica é importante pois pode permitir um diagnóstico muito mais precoce.

Sweet's Syndrome and relapsing polychondritis signal myelodysplastic syndrome.

The emergence of certain skin conditions belonging to the group of mucocutaneous paraneoplastic syndromes may indicate the future appearance of a previously unknown malignancy. Sweet's Syndrome and relapsing polychondritis are included in this group. Sweet's Syndrome and relapsing polychondritis are very rarely found together in the same patient. This dual occurrence is more commonly found in cancer patients with associated hematological malignancies. We report the case of a 79 year-old male with Sweet's Syndrome and relapsing polychondritis, who was subsequently diagnosed with a myelodysplastic syndrome.

Effects of etanercept treatment on lipid profile in patients with moderate-to-severe chronic plaque psoriasis: a retrospective cohort study.

Treatment with tumor necrosis factor alpha (TNF-α) inhibitors may have favourable effects on the lipid profile. This is the first study to assess the impact of etanercept on the lipid profile in patients with moderate-to-severe plaque psoriasis. To investigate the effect of etanercept on the lipid profile after 24 weeks of treatment in patients with moderate-to-severe plaque psoriasis. We conducted a retrospective cohort study reviewing the medical records of 45 consecutive patients who were treated for psoriasis with etanercept between June 2006 and September 2009. Demographic and clinical data were collected. Levels of total cholesterol, LDL-C, HDL-C, triglycerides, fasting glucose and C-reactive protein were recorded at the start of etanercept and at week 24. Levels of total cholesterol, LDL-C and triglycerides increased after 24 weeks of treatment with etanercept, with mean differences of 7.1 mg/dL (p=0.505), 2.0 mg/dL (p=0.718) and 2.8 mg/dL (p=0.180), respectively. HDL-C decreased, with a mean difference of -1.4 mg/dL (p=0.995). None of these changes were statistically significant. We found no favourable changes on the lipid profile after 24 weeks of treatment with etanercept in responding patients with chronic plaque psoriasis.

Síndrome de Sweet e policondrite recidivante reveladores de síndrome mielodisplásica / Sweet's Syndrome and relapsing polychondritis signal myelodysplastic syndrome

Certas dermatoses, pertencentes ao grupo das síndromes paraneoplásicas mucocutâneas, podem ser o prenúncio de uma neoplasia previamente não conhecida. Tanto a síndrome de Sweet como a policondrite recidivante incluem-se neste grupo. A síndrome de Sweet e a PR são raramente encontradas em um mesmo paciente. A presença de policondrite recidivante e síndrome de Sweet em um mesmo paciente tem se revelado mais frequente em pacientes com neoplasias associadas, sobretudo hematológicas. Relata-se o caso de paciente do sexo masculino, 79 anos, com síndrome de Sweet e policondrite recidivante, em quem, subsequentemente, foi diagnosticada uma síndrome mielodisplásica.

The emergence of certain skin conditions belonging to the group of mucocutaneous paraneoplastic syndromes may indicate the future appearance of a previously unknown malignancy. Sweet's Syndrome and relapsing polychondritis are included in this group. Sweet's Syndrome and relapsing polychondritis are very rarely found together in the same patient. This dual occurrence is more commonly found in cancer patients with associated hematological malignancies. We report the case of a 79year-old male with Sweet's Syndrome and relapsing polychondritis, who was subsequently diagnosed with a myelodysplastic syndrome.

Incontinentia pigmenti in the neonatal period.

Incontinentia pigmenti (IP) is a rare multisystem disease, X linked dominant disorder. As all X linked dominant diseases, it is usually male-lethal. Female newborn admitted to the neonatal intensive care unit on the first day of life was diagnosed as having probable herpetic infection with vesicular skin lesions distributed on upper right limb and inferior limbs. Family history showed that her 22-year-old mother had hypopigmented lesions on the lower limbs and her 13-month-old sister had hyperpigmented lesions on the trunk and limbs. In newborns, herpes infection emerges as the principal diagnosis of vesicular rash, due to the importance of precocious diagnosis and treatment. Other hypothesis must be considered in a newborn with vesicobullous rash, such as IP.

[Future perspectives in the treatment of psoriasis: news in biological therapies]. / Perspectivas futuras no tratamento da psoríase: novidades em terapêutica biológica.

Psoriasis is an immune-mediated chronic, inflammatory disease. Due to the chronic nature of the disease, patients suffer from substantial psychological and financial burdens, thus adding to a significantly impaired quality of life. Current understanding of the pathophysiology of this condition has produced very encouraging new medical developments. The biologic therapies target precise elements of the immune cascade and their introduction to clinical practice was a significant advance in the management of refractory moderate-to-severe psoriasis. Biological therapies for the treatment of psoriasis and/or psoriatic arthritis are defined by their mode of action and are classified into the following categories: T-cell modulating agents, inhibitors of tumor necrosis factor-alpha (TNFα blockers) and inhibitors of interleukin (IL) 12 and IL-23. This article reviews the recent progress in the understanding of the molecular and immunologic basis of psoriasis and how this contributed to the introduction of new targeted therapies. It is provided an overview of the TNFα inhibitors golimumab and certolizumab, and also other agents such as ustekinumab, briacinumab, tasocitinib, sipilizumab and abatacept.