[Down syndrome: another cause of immunosuppression associated with multiple eruptive dermatofibromas?]. / Síndrome de Down: ¿otra causa de inmunosupresión asociada con dermatofibromas eruptivos múltiples?

The term "multiple eruptive dermatofibromas" usually refers to a clinical situation characterized by the development of between five and eight dermatofibromas during a period of up to four months. It is usually linked to immunodeficiency associated conditions as autoimmune disorders, hematologic malignancies, HIV infection, and transplants. We report three patients with Down syndrome. One patient had psoriatic arthritis under treatment with methotrexate, one had Graves-Basedow disease, and one had hypercholesterolemia. All three patients developed multiple eruptive dermatofibromas. We suggest that the immunologic disturbances associated with Down syndrome, together with other underlying conditions present in these patients, could trigger the development of cutaneous lesions.

Biocompatibility of agarose gel as a dermal filler: histologic evaluation of subcutaneous implants.

BACKGROUND: The search for safe and effective tissue fillers has been an ongoing effort in plastic and cosmetic surgery over recent decades. Biocompatibility is a prerequisite for any substance to be used as an implant material, and potential biomaterials need to be characterized by histologic evaluation of tissue responses. Collagen is a well-known tissue filler. Agarose gel is widely used in bioengineering. Both products are considered biocompatible. The purpose of this study was to evaluate the bioactivity of agarose gel as a dermal filler compared with collagen. METHODS: Tissue responses to agarose gel and collagen were evaluated in a rat in vivo model (n = 96). Four groups were evaluated: group 1 (n = 24), rats with agarose gel implants; group 2 (n = 24), rats with collagen implants; group 3, a placebo group (n = 24); and group 4, a control group (n = 24). Responses and biocompatibility were assessed by histopathologic and histomorphometric evaluation at 1 week to 8 months after implantation. RESULTS: Agarose gel showed marked bioactivity and biodegradation, although the implants integrated well into tissues: newly formed collagen bands were observed inside the implants and no granulomas were detected. Collagen implants showed low cell infiltration and a significant loss of product over time. CONCLUSIONS: Agarose gel is a biocompatible product that can be considered for use as a tissue filler. Further investigation is required to assess its long-term efficacy and safety.

Blepharophimosis-ptosis-epicanthus inversus syndrome (BPES).

Blepharophimosis-ptosis-epicanthus inversus syndrome (BPES) is a rare inherited condition that includes characteristic eyelid malformations and sometimes reduced fertility in females. Genetic studies have implicated mutations in the forkhead transcription factor FOXL2 as responsible for BPES. We report a female and her father with BPES type I, who presented the 1092-1108dup17 mutation in the FOXL2 gene. Molecular studies and the typical clinical features of BPES should allow the dermatologist to reach an early diagnosis and permit the treatment of eyelid alterations and the investigation of infertility.

Reticular erythematous mucinosis (REM) with telangiectasias associated with essential thrombocytosis and lung carcinoma.

The cutaneous mucinoses are a heterogeneous group of diseases in which mucin accumulates in the skin. Reticular erythematous mucinosis (REM) is an infrequent variant. We present a 48-year-old man with essential thrombocytosis and REM lesions with atypical telangiectasias on his chest, who developed a non-small cell lung carcinoma. We discuss the unusual clinical finding of telangiectasias over REM lesions and the association with essential thrombocytosis and lung carcinoma.

Poroqueratosis lineal / Linear Porokeratosis

La poroqueratosis lineal (PL) es una infrecuente variante de poroqueratosis, que aparece típicamente en la infancia. Clínicamente presenta lesiones de poroqueratosis de distribución lineal siguiendo las líneas de Blaschko. Presentamos un caso de PL en una joven de 21 años, localizado en el miembro inferior izquierdo. Debido a la falta de una terapia efectiva y la posibilidad de degeneración maligna de las lesiones cutáneas, la PL debería incluirse siempre en el diagnóstico diferencial de las dermatosis lineales de la infancia (AU)

Penfigoide ampolloso en paciente hemipléjica / Bullous pemohigoid in hemiplegic patient

Presentamos el caso de una paciente hemipléjica que desarrolló un cuadro de penfigoide ampolloso con manifestaciones cutáneas circunscritas al lado paralítico. Recientemente se ha descrito la posible asociación entre enfermedades neurológicas y penfigoide ampolloso (AU)