RESUMEN
BACKGROUND: Urticarial vasculitis (UV) is a rare form of leukocytoclastic vasculitis in which skin lesions resemble urticaria. UV comprises hypocomplementemic and normocomplementemic subtypes. To date, only 4 cases of UV associated with myeloproliferative disorders have been described, including 3 cases with essential thrombocythaemia (ET) and one case with polycythaemia vera. PATIENTS AND METHODS: We describe the case of a 59-year-old male patient with JAK2-positive TE and secondary myelofibrosis and who developed multiple urticarial papules persisting for more than 24hours. Skin biopsy showed perivascular neutrophilic infiltrate with margination of neutrophils in the lumen of vessels and some leukocytoclastic patterns, and with red cell extravasation consistent with UV. Treatment with ruxolitinib (a JAK2 inhibitor) induced transient and partial control of the haematological symptoms but did not prevent UV flare. Prednisolone 20mg once daily was added, with good clinical response. DISCUSSION AND CONCLUSION: To our knowledge, this is the fourth reported case of UV associated with ET and the first case associated with MF.
Asunto(s)
Médula Ósea/patología , Trastornos Mieloproliferativos/complicaciones , Urticaria/etiología , Vasculitis Leucocitoclástica Cutánea/etiología , Quimioterapia Combinada , Resultado Fatal , Humanos , Janus Quinasa 2/antagonistas & inhibidores , Masculino , Persona de Mediana Edad , Mutación Missense , Trastornos Mieloproliferativos/tratamiento farmacológico , Nitrilos , Prednisolona/uso terapéutico , Pirazoles/uso terapéutico , Pirimidinas , Piel/patología , Trombocitemia Esencial/complicaciones , Vasculitis Leucocitoclástica Cutánea/tratamiento farmacológicoRESUMEN
Pityriasis lichenoides et varioliformis acuta (PLEVA) is an inflammatory skin disease that is unknown to pediatricians. The ulceronecrotic febrile form is a rare and potentially lethal variant. We report the case of a 7-year-old boy with a papulovesicular eruption lasting for 4 weeks, secondarily associated with ulcers and necrotic crusts, fever, and systemic signs. After exploring infectious causes such as chickenpox, we discussed and confirmed PLEVA with histological analysis. Systemic steroids and methotrexate improved the symptoms. It is necessary to mention PLEVA in case of prolonged papulovesicular eruption. Prompt diagnosis allows appropriate treatment, although there is no consensus on therapeutic guidelines.