Ultrasonography for the Diagnosis and Follow-Up of Extramammary Paget Disease (EMPD): A Review.

Extramammary Paget disease (EMPD) is a rare skin tumor that manifests as poorly delimited lesions located mainly in genital area. Prognosis correlates with thickness and dermal invasion. The gold standard for diagnosis is the histopathological study of a biopsy. However, this technique is invasive and only shows a small area of the tumor. A good correlation has been reported between ultrasounds (US) and histopathologic finding. Moreover, a US examination has the advantages of wide availability, noninvasiveness, low cost, ease of use, and real-time scanning. We review the ultrasound signs described for this disease.

Multiorgan histiocytosis after B-cell acute lymphoblastic leukemia.

The association of multiorgan histiocytosis after acute lymphoblastic leukemias is very rare as most cases are localized forms of Langerhans cell histiocytosis (LCH). We report on an 18-year-old man diagnosed with B-cell acute lymphoblastic leukemia (B-ALL) with p16 deletion (9p21). He was treated with induction chemotherapy using the Spanish PETHEMA group protocol and achieved complete remission. Three months after the diagnosis of B-ALL, he developed a severe multiorgan histiocytosis that is clinically suggestive of LCH but lacked typical immunohistochemical features of LCH and indeterminate cell histiocytosis: CD1a was strongly positive, CD68 and S-100 protein were moderately positive, and langerin was negative. The drugs of the first-line treatment recommended for LCH had been part of the chemotherapy of B-ALL that the patient had received. Therefore, we prescribed the second-line treatment for LCH (cytarabine and 2'-chlorodeoxyadenosine), and he achieved partial remission. The patient died during the aplasia induced by the third cycle of chemotherapy from pneumonia. We could not demonstrate the transdifferentiation of tumoral lymphocytes into histiocytes, using p16 deletion (9p21) as a marker, because these cells did not share the mutation. Neither could we study immunoglobulin-H rearrangement as we had exhausted all the tissue samples. In the medical literature, there are a few reported cases of T-cell acute lymphoblastic leukemia followed by disseminated LCH and just 1 case of B-ALL followed by localized LCH affecting the bones. Therefore, our patient may be the first published case of B-ALL followed by histiocytosis, which had 2 singularities: it was multiorgan and the immunohistochemistry was not typical of LCH.

Úlcera tropical / Tropical ulcer

Presentamos el caso de un varón de 8 años procedente de Guinea Ecuatorial que consultó por una úlcera en la pierna izquierda de 6 meses de evolución.Por las características clínicas y el país de origen del paciente, la lesión era compatible con una úlcera tropical. Se instauró tratamiento antibióticooral con amoxicilina y ácido clavulánico durante 2 semanas evolucionando a la curación en menos de 2 meses

A 8-year-old male coming from Equatorial Guinea who presented with an ulcer on the left leg of 6 month’s duration is reported. The clinical appearanceand his country of origin were highly suggestive of tropical ulcer. We prescribed an oral treatment with amoxiciline and clavulanic acid for 2 weeksand the ulcer health in less than 2 months

Reticulohistiocitosis congénita autoinvolutiva / Congenital self-healing reticulohistiocytosis

Presentamos una recién nacida con erosiones en flexuras, pápulas palmo-plantares y placas rosadas en la espalda sin afectación sistémica. La histologíamostraba un infiltrado histiocitario en dermis superior con epidermotropismo. La inmunohistoquímica resultó positiva para S100 y CD1a por loque se trataba de células de Langerhans. El estudio de extensión fue negativo. Las lesiones involucionaron espontáneamente en el plazo de 2 semanas,diagnosticándose de Reticulohistiocitosis congénita autoinvolutiva

A female newborn who presented erosions on folds, papules on palms and soles and red-brownish plaques on the back without involvement of internalorgans is reported. Light microscopy showed an histiocytic infiltrate in the papillary dermis with epidermotrophism. Inmunomarking with S100and CD1a was positive; therefore the histiocytes were Langerhans cells. The systemic evaluation excluded extracutaneous involvement. The cutaneouslesions resolved spontanously in two weeks so the case was diagnosed as Congenital self-healing reticulohistiocytosis