Congenital “self‐healing” Langerhans cell histiocytosis (Hashimoto‐Pritzker disease): A report of two cases with the same cutaneous manifestations but different clinical course
2014
Congenital
self-healing
Langerhans cell histiocytosisor
Hashimoto-Pritzker diseaseis a rare condition present at birth or in the neonatal period characterized by small reddish-brown crusted papulonodular lesions. In most cases these lesions are not accompanied by systemic findings and tend to involute spontaneously within weeks or months, but in other cases there may be extracutaneous involvement and/or recurrence of the disease. This emphasizes that the clinical course is variable and a long-term follow-up is mandatory in order to reveal possible systemic involvement. We describe two cases of congenital
self-healing
Langerhans cell histiocytosiswith widespread and very similar cutaneous manifestations but different clinical course. The first patient had multisystemic disease (with lymph nodes, bones, liver and lungs affected) that required
systemic therapy. The second patient had cutaneous and bony lesions that resolved spontaneously. We think that the adjective “
self-healing” is misleading and should be abandoned. We stress the importance of a complete systemic evaluation and the necessity of a long-term follow-up.
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