Scleredema diabeticorum in a patient: un uncommon etiology of restrictive lung pattern Escleredema diabeticorum en un paciente: una etiología infrecuente de patrón restrictivo pulmonar.

Scleredema adultorum of Buschke is a rare skin disease characterized by skin thickening and tightening typically at the neck and the upper part of the body. This thickening results from increased mucin deposition in the reticular dermis. Three variants are recognized. Scleredema diabeticorum is one subtype associated with diabetes mellitus. We report a man with a history of poorly controlled diabetes presenting with extensive scleredema adultorum of the trunk in a "cuirasse" pattern associated with restrictive lung disease. Cutaneous ultrasonography revealed a marked thickening of the dermis as well as various echogenic spots in the dermis. Cutaneous hardness secondary to scleredema may cause limited mobility. Therefore, respiratory manifestations may be associated and assessed, mainly in cases of extensive scleredema involving trunk and shoulders. Moreover, cutaneous ultrasonography may be useful to monitor the evolution of scleredema and confirm the diagnosis.

Osificación heterotópica de las piernas en un varón / Report of a man with heterotopic ossification of the legs

La osificación heterotópica es un trastorno infrecuente que consiste en el depósito de tejido óseo extraesquelético. En la piel, puede ser primario, en el contexto de síndromes genéticos, o secundario a trastornos muy diversos. Dentro de las formas secundarias, la osificación subcutánea de las piernas por insuficiencia venosa crónica es una complicación infrecuente y poco reportada. Presentamos un paciente con osificación subcutánea de las piernas secundaria a insuficiencia venosa y revisamos la literatura

Heterotopic ossification is an uncommon disorder that consists of deposition of ectopic bone outside the extraskeletal tissues. In the skin, it can be primary, in association with genetic syndromes, or be secondary to different disorders. The latter include subcutaneous ossification of the legs in chronic venousinsufficiency, an infrequent and unrecognized complication. We report the case of a patient with subcutaneous ossification of both legs secondary to venous insufficiency and review the literature

Persistent Facial Oedema and Erythema in a Woman, An Uncommon Manifestation of Chronic Cutaneous Lupus Erythematosus.

Manifestations of chronic cutaneous lupus erythematosus are variable. Periorbital and facial swelling occurs in dermatomyositis and systemic lupus, but it has been rarely reported as a manifestation of exclusively cutaneous lupus. A 48-year-old woman presented with a 16-year history of asymptomatic, bilateral swelling and erythema of her face with marked worsening after sun exposure. No systemic symptoms were associated. A complete evaluation did not reveal other findings. Cutaneous biopsy showed features of lupus erythematosus. She was treated with photoprotection, topical tacrolimus, hydroxychloroquine and azathioprine with a partial response. Facial swelling with erythema represents quite an unusual manifestation of chronic cutaneous lupus erythematosus. Dermatomyositis, systemic lupus and Morbihan disease are the main differential diagnoses. LEARNING POINTS: Periorbital and facial swelling with erythema are clinical manifestations of dermatomyositis and systemic lupus erythematosus. However, these manifestations represent quite an unusual presentation of chronic cutaneous lupus erythematosus.The periorbital area is most frequently affected, while extensive facial involvement is much more unusual.A complete evaluation and cutaneous biopsy are essential to make the diagnosis and to rule out other disorders such as dermatomyositis, systemic lupus erythematosus and Morbihan disease.

Report of a man with heterotopic ossification of the legs. / Osificación heterotópica de las piernas en un varón.

Heterotopic ossification is an uncommon disorder that consists of deposition of ectopic bone outside the extraskeletal tissues. In the skin, it can be primary, in association with genetic syndromes, or be secondary to different disorders. The latter include subcutaneous ossification of the legs in chronic venousinsufficiency, an infrequent and unrecognized complication. We report the case of a patient with subcutaneous ossification of both legs secondary to venous insufficiency and review the literature.

Neoplasia de celulas dendriticasblastica plasmocitoide.

Plasmacytoid blast dendritic cell neoplasia (NCDBP) is an uncommon malignant neoplasm, presenting clinically with cutaneous involvement and subsequent lymph node and bone marrow extension. It characteristically expresses the markers: CD56, CD4, and CD123. There is no optimal treatment, relapses are frequent, and the survival time is short. We present the case of an elderly patient with NCDBP who initially presented with cutaneous lesions, but experienced rapid systemic progression and did not, respond to treatment.

Pudendal Neuralgia as the Initial Manifestation of Infiltrative Sacrococcygeal Chordoma.

Sacrococcygeal chordoma is a malignant tumour originating from remnants of the notochord. Chordomas are slow-growing tumours whose symptoms develop insidiously. We present the case of a 72-year-old woman with a 6-month history of genital pain radiating to the perianal area and exacerbating when she was in a sitting position. MRI and PET studies revealed a large mass in the sacrococcygeal region causing bone destruction and invasion of neurovascular structures. The immunohistochemical study of the surgical specimen determined it to be chordoma. This is the first published case of pudendal neuralgia as a form of presentation of sacrococcygeal chordoma.

Mal de Pott: una causa infrecuente de dolor dorsolumbar en nuestros días / Pott's disease: an uncommon cause of thoracolumbar pain nowadays

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