A diagnostic challenge: chronic cutaneous ulcers as an isolated clinical finding in metastatic Crohn's disease.

Metastasic Crohn disease (MCD) is the most uncommon cutaneous manifestation of Crohn disease. The actual incidence is not clear. There are fewer than 200 cases described in the literature. We report a case of 21 years-old girl that came to our emergency care with large exudative ulcers in the inguinal folds, the vulva, the perianal region and the popliteal fossae. Histopathological examination of the ulcers revealed a non-caseating granulomatous inflammation with abundant multinucleated giant cells and intense lymphocytic infiltrate. Colonoscopy, contrast study of the small bowel and video capsule endoscopy were carried out without evidence of digestive disease. High potency topical steroids (betamethasone 0,5mg/gr twice daily) and 300mg intravenous Infliximab were initiated as the initial line therapy and the ulcers began to heal. We report this case to highlight the presence of cutaneous ulcers without intestinal disease in a young non-pediatric woman as the unique manifestation of the disease.

Angiokeratoma circumscriptum.

Angiokeratoma circumscriptum is the rarest variant of angiokeratoma. It usually affects females and it is characterized by dark-red to blue-black confluent papules or nodules on lower limbs in a segmental and unilateral distribution. We describe the clinical and histopathological findings in a patient with angiokeratoma circumscriptum and discuss the etiology, associations, diagnosis, differential diagnosis, and treatment.

Subcutaneous fat necrosis of the newborn: clinical and histopathological review and use of cutaneous ultrasound Necrosis grasa subcutánea del recién nacido: revisión clínica e histopatológica y utilidad de la ecografía cutánea.

Subcutaneous fat necrosis of the newborn is a rare lobular panniculitis. It is characteristic of term or post-term neonates with a history of perinatal compromise or maternal gestational pathology. The cutaneous manifestations consist of erythematous and indurated subcutaneous plaques and nodules located over the dorsal region and the shoulders. Diagnosis is clinical and pathological. Histopathological findings include lobular panniculitis with a lymphohistiocytic inflammatory infiltrate with few neutrophils, fatty necrosis, deposition of radial needle-shaped crystals in the adipocytes, and possible calcification and hemorrhage. The cutaneous ultrasound shows hyperechoic and avascular subcutaneous cellular tissue and acoustic shadows may appear corresponding to calcifications. The clinical differential diagnosis includes sclerema neonatorum and post-corticosteroid panniculitis. Histologically crystal-forming panniculitis conditions are in the differential diagnosis. The disease is usually self-limited but complications such as hypercalcemia, hypoglycemia, hypertriglyceridemia, thrombocytopenia, and anemia may occur. Complications should be ruled out and treated at diagnosis and during follow-up. The most important complication is hypercalcemia.La necrosis grasa subcutánea neonatal es una paniculitis lobulillar infrecuente. Es característica de neonatos a término o postérmino con antecedentes de sufrimiento perinatal o patología gestacional materna. Las manifestaciones cutáneas consisten en placas y nódulos subcutáneos eritematosos e indurados localizados preferentemente en la región dorsal y los hombros. El diagnóstico es clínicopatológico. Los hallazgos histopatológicos comprenden una paniculitis lobulillar con un infiltrado inflamatorio linfohistiocitario con escasos neutrófilos, necrosis grasa, depósito de cristales radiados en los adipocitos y posibles focos de calcificación y hemorragia. En la ecografía cutánea se observa hiperecogenicidad y avascularización del tejido celular subcutáneo y pueden aparecer sombras acústicas posteriores que se correspondencon calcificaciones. El diagnóstico diferencial clínico se debe realizar con el escleredema neonatorum y la paniculitis postesteroidea, e histológicamente con las paniculitis con formación de cristales. La enfermedad suele ser autolimitada pero pueden aparecer complicaciones como la hipercalcemia, la hipoglucemia, la hipertrigliceridemia, latrombocitopenia y la anemia. Las complicaciones deben ser descartadas y tratadas en el diagnóstico y durante el seguimiento. La complicación másimportante es la hipercalcemia.

Type V aplasia cutis congenita in a preterm newborn successfully resolved.

Aplasia cutis congenita (ACC) associated with fetus papyraceus is a rare subtype of aplasia cutis categorized as type V in Frieden's classification. It is characterized by stellate lesions in a symmetrical distribution over the trunk and proximal extremities. Conservative treatment is recommended, but there is not a well-defined therapeutic protocol. We report the case of a type V ACC in a preterm male newborn with lesions on the trunk and scalp successfully treated with topical 1% silver sulfadiazine and petrolatum gauze with an excellent evolution. This case associates a severe affectation of the scalp which represents a rare variant of type V ACC.