A case of mid-face toddler excoriation syndrome (MiTES).

The term mid-face toddler excoriation syndrome (MiTES) has been proposed to describe a rare condition that arises in infancy as is characterized by self-inflicted excoriations. It is considered to be a milder variant of hereditary sensory-autonomic neuropathy (HSAN) type VIII. We present an 8-year-old boy with lesions on the mid-face that were admittedly self-induced and associated with an abnormally high pain threshold. The diagnosis and management of MiTES is reviewed.

Infantile hemangioma with minimal or arrested growth as the skin manifestation of PHACE syndrome.

BACKGROUND: Infantile hemangiomas with minimal or arrested growth are vascular tumors with a proliferative component involving < 25% of their total surface area. They are commonly described as localized lesions and are mainly located on the lower body. Little has been described about segmental forms on the face and their associations with PHACE syndrome. METHODS: We carried out a multicenter, retrospective, case-series study involving 5 hospitals in Spain. Information was collected on cases of PHACE syndrome featuring infantile hemangiomas with minimal or arrested growth. RESULTS: The frontotemporal and maxillary areas were the most frequently involved sites in our series. The upper eyelid and upper lip were the 2 locations most frequently associated with proliferation and ulceration. Four patients experienced spontaneous resolution, and the rest had a very good cosmetic outcome with oral treatment. Cerebral and cervical arterial anomalies were the most frequent extracutaneous findings associated with PHACE, followed by cerebral and ocular anomalies. Some unique associated disorders were fructose intolerance and retinoblastoma. CONCLUSION: We present the largest case series of segmental facial infantile hemangiomas with minimal or arrested growth in PHACE syndrome and emphasize the importance of recognizing these lesions in early infancy, because they can indicate PHACE syndrome. The data presented suggest that infantile hemangiomas with minimal or arrested growth-associated PHACE syndrome does not seem to differ significantly from PHACE syndrome with classic infantile hemangiomas, and thus the same recommendations for diagnosis, management, and therapy should be followed. Future studies with more patients could contribute to enlighten this specific subset.

Pediatric dermatofibrosarcoma protuberans in Madrid, Spain: multi-institutional outcomes.

Little is known about the incidence and management of dermatofibrosarcoma protuberans (DFSP) in children. We conducted a retrospective review of all patients younger than 18 years of age treated for DFSP over a period of 11 years (2000-2011) in Madrid, Spain. The sample consisted of 13 children. The average annual incidence of DFSP in the pediatric population corresponded to 1.02 cases per million person-years (95% confidence interval 0.55, 1.73). Sites of involvement were diverse, with 15.3% of tumors found in acral locations. The median tumor size was 3.5 cm × 3 cm and the median time from apparent onset to diagnosis was 36 months. Histopathologic examination revealed conventional (77.0%), pigmented (15.4%), and myxoid (7.6%) variants. The mitotic index was consistently <5 per 10 high-power fields. All lesions were removed using surgical excision. One patient developed a local recurrence because of initial affected margins; none developed metastases. The median duration of clinical follow-up was 70.5 months. This study estimated the average annual incidence rate of DFSP in a population of patients younger than 18 years and reviewed the experience of several hospitals in the management of this tumor.

Inhibition of PI3K-AKT-mTOR signaling sensitizes melanoma cells to cisplatin and temozolomide.

In melanoma, the PI3K-AKT-mTOR (AKT) and RAF-MEK-ERK (MAPK) signaling pathways are constitutively activated and appear to play a role in chemoresistance. Herein, we investigated the effects of pharmacological AKT and MAPK pathway inhibitors on chemosensitivity of melanoma cells to cisplatin and temozolomide. Chemosensitivity was tested by examining effects on growth, cell cycle, survival, expression of antiapoptotic proteins, and invasive tumor growth of melanoma cells in monolayer and organotypic culture, respectively. MAPK pathway inhibitors did not significantly increase chemosensitivity. AKT pathway inhibitors consistently enhanced chemosensitivity yielding an absolute increase of cell growth inhibition up to 60% (P<0.05, combination therapy vs monotherapy with inhibitors or chemotherapeutics). Cotreatment of melanoma cells with AKT pathway inhibitors and chemotherapeutics led to a 2- to 3-fold increase of apoptosis (P<0.05, combination therapy vs monotherapy) and completely suppressed invasive tumor growth in organotypic culture. These effects were associated with suppression of the antiapoptotic Bcl-2 family protein Mcl-1. These data suggest that inhibition of the PI3K-AKT-mTOR pathway potently increases sensitivity of melanoma cells to chemotherapy.

Dermatitis de contacto por Agave americana / Contact dermatitis from Agave americana

Numerosas especies de plantas y sus derivados pueden causar reacciones cutáneas por diversos mecanismos: dermatitis irritativa de contacto, dermatitis alérgica de contacto, urticaria de contacto y fotodermatitis. Se presenta un caso de dermatitis irritativa de contacto tras la exposición a la savia de Agave americana. Representa un cuadro cutáneo descrito en escasas ocasiones y que, aunque habitualmente se presenta como una erupción papulovesiculosa, en el paciente descrito aparece con lesiones purpúricas en la zona de contacto

Numerous plant species and their derivatives can cause skin reactions through a variety of mechanisms: irritative contact dermatitis, allergic contact dermatitis, contact urticaria and photodermatitis. We present a case of irritative contact dermatitis after exposure to the sap of Agave americana. The skin symptoms in this case have only been described on rare occasions; although this condition usually presents with a papulovesicular rash, in this patient it appeared as purpuric lesions in the contact area

[Contact dermatitis from Agave americana]. / Dermatitis de contacto por Agave americana.

Numerous plant species and their derivatives can cause skin reactions through a variety of mechanisms: irritative contact dermatitis, allergic contact dermatitis, contact urticaria and photodermatitis. We present a case of irritative contact dermatitis after exposure to the sap of Agave americana. The skin symptoms in this case have only been described on rare occasions; although this condition usually presents with a papulovesicular rash, in this patient it appeared as purpuric lesions in the contact area.