Childhood leprosy in Buenos Aires: four cases report. / Lepra infantil en Buenos Aires. Reporte de cuatro casos.

Childhood leprosy, which affects children up to 14 years old, is characterized by a delay in diagnosis since it is usually confused with other dermatoses. Its presence in a child is a relevant epidemiological indicator since it signals active disease transmission. We present 4 patients between 5 and 14 years old who attended a public hospital in Buenos Aires -two patients with borderline tuberculoid leprosy, one with lepromatous leprosy, and one with indeterminate leprosy.The World Health Organization provides therapy for people between 10 and 14 but does not consider children under 10. This difficulty implies adapting the dosage and pharmaceutical form to each patient under this age. Finally, it should be noted that the diagnosis of the patients led to the diagnosis and treatment of the disease in adult cohabitants.

La lepra infantil, aquella que afecta a niños de hasta 14 años, se caracteriza por la demora en su diagnóstico, ya que habitualmente es confundida con otras dermatosis. Su presencia en un niño es un indicador epidemiológico relevante, dado que señala la transmisión activa de la enfermedad. Presentamos 4 pacientes de entre 5 y 14 años asistidos en un hospital público de la Ciudad de Buenos Aires. Dos pacientes presentaron lepra borderline tuberculoide; uno, lepra lepromatosa; y otra, lepra indeterminada. La Organización Mundial de la Salud facilita la terapéutica para la población entre 10 y 14 años, pero no contempla a los menores de 10 años. Esta dificultad implica adecuar la dosis y la forma farmacéutica a cada paciente menor de la edad referida. Finalmente, es de destacar que el diagnóstico de los pacientes llevó al diagnóstico y tratamiento de la enfermedad en convivientes adultos.

CARD14-associated papulosquamous eruption (CAPE) in pediatric patients: Three additional cases and review of the literature.

CARD14-associated papulosquamous eruption (CAPE) is a proposed term that encompasses features ranging from psoriasis to pityriasis rubra pilaris (PRP) in association with CARD14 mutations. The early onset of the disease, prominent facial involvement, family history of an autosomal dominant trait, and poor response to conventional treatment are characteristics of CAPE that distinguish it from classical psoriasis and PRP. We describe the clinical features, family history, and response to therapy in three unrelated children with CAPE and compare these characteristics with those of previously described pediatric patients. Testing for CARD14 mutations in children with early onset of features of psoriasis or pityriasis rubra pilaris and resistance to conventional therapy should be considered.

Vasculitis crioglobulinémica con compromiso renal y síndrome de Sjögren / Cryoglobulinemic vasculitis with renal involment and Sjögren's syndrome

La vasculitis crioglobulinémica es una vasculitis de vasos pequeños que se da en presencia de crioglobulinas séricas. Las crioglobulinemias mixtas son las más frecuentes y se asocian a infecciones crónicas, típicamente al virus de la hepatitis C, así como a enfermedades linfoproliferativas y autoinmunes, más a menudo al síndrome de Sjögren. Las manifestaciones clínicas incluyen púrpura de los miembros inferiores, neuropatía periférica, artralgias y glomerulonefritis. La presencia de vasculitis crioglobulinémica en el contexto de un síndrome de Sjögren es marcadora de peor pronóstico. Presentamos el caso de una paciente con vasculitis crioglobulinémica con compromiso cutáneo y renal, que condujo al diagnóstico de un síndrome de Sjögren primario. (AU)

Cryoglobulinemic vasculitis is a small-sized vasculitis that occurs in the context of serum cryoglobulins. Mixed cryoglobulinemias are the most frequent and are associated with chronic infections, typically hepatitis C and autoimmune diseases, most commonly Sjögren's syndrome. Clinical manifestations include purpura of lower limbs, peripheral neuropathy, arthralgias and glomerulonephritis. The presence of cryoglobulinemic vasculitis in the context of Sjögren's syndrome is a marker of poor prognosis. We present the case of a patient with cryoglobulinemic vasculitis associated to cutaneous and renal involvement that led us to the diagnosis of primary Sjögren's syndrome. (AU)