Giant benign lymphangioendothelioma with positive expression of Wilms tumor 1: A case report.

Benign lymphangioendothelioma (BL, acquired progressive lymphangioma) is a rare, slow-growing lymphatic tumor, first described 40 years ago, with fewer than 50 published cases. Clinically, it presents as a skin-colored or erythematous patch. Definitive diagnosis requires histopathological examination. The immunohistochemical staining profile is still controversial regarding Wilms tumor 1 (WT1) expression, a marker of proliferative and neoplastic, rather than malformative nature. Here, we report a case of a 60-cm-long BL on the breast of an adult female. Biopsy revealed irregular vascular spaces dissecting the collagen bundles lined by swollen endothelial cells but without cellular atypia. Positivity for podoplanin (D2-40), CD31, and WT1 was observed, supporting the neoplastic nature of this lesion. Dermatologists and pathologists must be aware of this entity for early diagnosis and treatment.

Update on Atopic Dermatitis.

With an increasing prevalence during the past decades, atopic dermatitis has become a global health issue. A literature search following a targeted approach was undertaken to perform this non-systematic review, which intends to provide an overview of the epidemiology, pathophysiology, clinical features, comorbidities, and current therapies for the treatment of atopic dermatitis. In sum, this is a heterogeneous skin disorder associated with variable morphology, distribution, and disease course. Although not completely understood, its pathogenesis is complex and seems to result from a combination of genetic and environmental factors that induce skin barrier dysfunction, cutaneous and systemic immune dysregulation, skin microbiota dysbiosis, and a strong genetic influence. Diagnosis is based on specific criteria that consider patient and family history and clinical manifestations. Overall disease severity must be determined by evaluating both objective signs and subjective symptoms. Therapeutic goals require a multistep approach, focusing on reducing pruritus and establishing disease control. Patients should be advised on basic skin care and avoidance of triggers. Topical anti-inflammatory agents should be considered in disease flares or chronic/recurrent lesions. In case of inadequate response, phototherapy, systemic immunosuppressants and, more recently, dupilumab, should be added. Nevertheless, the treatment of moderate-to-severe atopic dermatitis remains challenging and novel, efficacious, safe and targeted treatments are urgently needed. In conclusion, although the last few years have seen important improvement in the understanding of the disease, future research in atopic dermatitis will continue exploring gene-environment interactions and how it affects pathophysiology, disease severity, and treatment outcomes.

Com uma prevalência crescente nas últimas décadas, a dermatite atópica tornou-se um problema de saúde global. Foi realizada uma revisão não sistemática com base numa pesquisa bibliográfica direcionada à epidemiologia, fisiopatologia, características clínicas, comorbilidades e tratamento da dermatite atópica. Em resumo, a dermatite atópica é uma patologia cutânea heterogénea associada a morfologia, distribuição e curso da doença variáveis. A sua patogénese é complexa, combinando fatores genéticos e ambientais que condicionam a disfunção da barreira epidérmica, a desregulação imune cutânea e sistémica e a disbiose do microbioma da pele. O diagnóstico baseia-se em critérios clínicos específicos, incluindo história pessoal e familiar de atopia, evolução da doença e manifestações clínicas. A gravidade da doença é determinada através da avaliação dos sinais objetivos e dos sintomas subjetivos. A sua abordagem deve ser progressiva, focada na redução do prurido e no controlo da doença. Os doentes devem ser aconselhados sobre os cuidados básicos a ter e evicção de agressores externos. Em situações de agudização ou lesões crónico-recidivantes, devem ser aplicados anti-inflamatórios tópicos. Na ausência de resposta ou controlo adequado no médio prazo, deve ponderar-se fototerapia, imunossupressores sistémicos ou, mais recentemente, dupilumab. Contudo, o tratamento da dermatite atópica moderada a grave permanece desafiador, sendo urgente o desenvolvimento de novas terapêuticas, eficazes, seguras e direcionadas. Concluindo, apesar de atualmente haver uma melhor compreensão e um maior conhecimento da doença, as investigações futuras deverão continuar a explorar a interação entre fatores genéticos e ambientais e seus efeitos na fisiopatologia e gravidade da doença, bem como nos resultados do tratamento.

Cutaneous Lymphangioma circumscriptum - dermoscopic features.

Lymphangiomas are congenital lymphatic malformations and cutaneous lymphangioma circumscriptum is the most common type. It is clinically characterized by clusters of translucent vesicles and the presence of dermoscopically yellow lacunae surrounded by pale septa, as well as reddish to bluish lacunae. In our case, the recently described hypopyon-like feature manifested, aiding in the sometimes difficult differential diagnosis of cutaneous lymphangioma circumscriptum with vascular lesions, further highlighting the importance of dermoscopy in what can be a diagnostic challenge.

Cutaneous Lymphangioma circumscriptum - dermoscopic features*

Lymphangiomas are congenital lymphatic malformations and cutaneous lymphangioma circumscriptum is the most common type. It is clinically characterized by clusters of translucent vesicles and the presence of dermoscopically yellow lacunae surrounded by pale septa, as well as reddish to bluish lacunae. In our case, the recently described hypopyon-like feature manifested, aiding in the sometimes difficult differential diagnosis of cutaneous lymphangioma circumscriptum with vascular lesions, further highlighting the importance of dermoscopy in what can be a diagnostic challenge.

Granuloma faciale of the scalp.

Granuloma faciale (GF) is an uncommon dermatosis with characteristic clinicopathological features. Extrafacial isolated GF is extremely rare. Pulsed dye laser (PDL) is a treatment option for GF to minimize the risk of scarring. We report a case of a 78-year-old male with an extensive GF of the scalp successfully treated with pulsed dye laser (PDL).

Epidermal nevus syndrome associated with hypophosphatemic rickets.

Epidermal Nevus Syndrome (ENS) is characterized by epidermal nevi associated with abnormalities involving the nervous, skeletal, and other systems. Rarely, hypophosphatemic rickets has been observed in association with epidermal nevi. A patient with ENS with right-sided serpiginous skin lesions, generalized weakness, and diffuse osteopenia associated with hypophosphatemic rickets is described. Medical management was enough to correct the clinical picture. The pathogenic mechanism involved in the onset of hypophosphatemic rickets in ENS is not fully clarified. Different studies suggest that phosphaturia, caused by circulating factor(s), called "phosphatonin(s)," may be secreted by an epidermal nevus. The nature of the phosphaturic factor(s) is not well understood, but elevated levels of circulating FGF-23 were recently reported in one patient with hypophosphatemic rickets. The authors suggest that serum FGF-23 measurement be included in the workup of this kind of rickets because there is growing evidence that in these situations the epidermal nevi produce a phosphaturic factor.

Eruptive keloids: spontaneous reactivation after 60 years.

An 84-year-old man with keloids since early adulthood, was referred to our clinic because of the recent appearance of erythematous, tumid plaques upon the old keloids and upon uninvolved skin. The plaques were located mainly on the trunk in a linear distribution. On the back there were round, nodular, erythematous nodules and plaques, with central crusts, that had just appeared on normal skin. A skin biopsy was performed on the plaques that appeared over the older lesions. The histological appearance was consistent with keloid. Similar histology was found in a biopsy taken from a plaque that had erupted on normal skin.