Intravascular histiocytosis: case report of a rare disease probably associated with silicone breast implant

Abstract Intravascular histiocytosis is a rare condition characterized by the aggregate of histiocytes within dilated dermal vessels. The diagnosis is mainly histophatological and immunohistochemical. We describe a case of a 55 year-old female patient presenting erythematous/purple patches on the breasts, back and limbs. She previously presented ductal carcinoma in the right breast in 2006 which was treated with mastectomy and proceeded to silicone breast implant in 2009. Clinical hypothesis was telangiectatic metastatic carcinoma. Histopathology showed vascular ectasia, thrombosis and recanalization of upper dermis small vessels. On immunohistochemistry, intravascular cells were CD 68+ and negative for estrogen and progesterone receptors, CK7, EMA and AE1/AE3 and endothelial cells were CD64+, leading to the diagnosis of intravascular histiocytosis.

Intravascular histiocytosis: case report of a rare disease probably associated with silicone breast implant.

Intravascular histiocytosis is a rare condition characterized by the aggregate of histiocytes within dilated dermal vessels. The diagnosis is mainly histophatological and immunohistochemical. We describe a case of a 55 year-old female patient presenting erythematous/purple patches on the breasts, back and limbs. She previously presented ductal carcinoma in the right breast in 2006 which was treated with mastectomy and proceeded to silicone breast implant in 2009. Clinical hypothesis was telangiectatic metastatic carcinoma. Histopathology showed vascular ectasia, thrombosis and recanalization of upper dermis small vessels. On immunohistochemistry, intravascular cells were CD 68+ and negative for estrogen and progesterone receptors, CK7, EMA and AE1/AE3 and endothelial cells were CD64+, leading to the diagnosis of intravascular histiocytosis.

Subcorneal pustular dermatosis in the pediatric age.

Subcorneal pustular dermatosis is a rare pustular eruption which occurs mainly in middle-aged women and rarely during childhood. We report a case of a 15-year-old female with a 4-year history of pustular lesions on the proximal region of the upper limbs with subsequent impairment of the trunk. Physical examination revealed small pustules distributed on the trunk and proximal region of the limbs. Histopathology showed a subcorneal pustule and direct immunofluorescence for IgA, IgM, IgG and fibrinogen was negative, confirming the diagnosis of subcorneal pustular dermatosis. The patient was treated with dapsone with good clinical response after one month. Subcorneal pustular dermatosis is a rare condition and there are only isolated cases reported in the literature in pediatric patients. Thus, we discuss the main clinical aspects and treatment response of this condition during childhood.

Subcorneal pustular dermatosis in the pediatric age

Abstract: Subcorneal pustular dermatosis is a rare pustular eruption which occurs mainly in middle-aged women and rarely during childhood. We report a case of a 15-year-old female with a 4-year history of pustular lesions on the proximal region of the upper limbs with subsequent impairment of the trunk. Physical examination revealed small pustules distributed on the trunk and proximal region of the limbs. Histopathology showed a subcorneal pustule and direct immunofluorescence for IgA, IgM, IgG and fibrinogen was negative, confirming the diagnosis of subcorneal pustular dermatosis. The patient was treated with dapsone with good clinical response after one month. Subcorneal pustular dermatosis is a rare condition and there are only isolated cases reported in the literature in pediatric patients. Thus, we discuss the main clinical aspects and treatment response of this condition during childhood.

Dermatofibrossarcoma atrófico: a importância da suspeita clínica / Atrophic Dermatofibrosarcoma: the importance of clinical suspicion

Dermatofibrossarcoma protuberante é tumor fibro-histiocítico de malignidade intermediária, relativamente incomum e recidivante. Sua variante mais rara é conhecida como atrófica, tipo morfeia ou dermatofibrossarcoma não protuberante, sendo de difícil diagnóstico clínico. Não há critérios dermatoscópicos bem estabelecidos, portanto o diagnóstico é histopatológico. Existem avanços nas áreas da imuno-histoquímica e da genética que auxiliam no diagnóstico e excisão cirúrgica completa. Relatamos um caso de dermatofibrossarcoma atrófico em paciente de 27 anos, revisado na literatura quanto aos aspectos clínicos, dermatoscópicos, histopatológicos e genéticos.

Dermatofibrosarcoma protuberans is an uncommon and relapsing fibrohistiocytic tumor of intermediate malignancy. Its rarest variant is known as atrophic, morphea type or dermatofibrosarcoma non protuberans and its clinical diagnose is difficult. There are not well established dermatoscopic criteria, so the diagnosis is histopathologic. There are advances in immunohistochemical and genetic that help the diagnosis and complete surgical excision. It is reported a case of atrophic dermatofibrosarcoma in a 27 years patient ant it is reviewed the clinical, dermoscopic, histological and genetic aspects.