title: Congenital "self-healing" Langerhans cell histiocytosis (Hashimoto-Pritzker disease): a report of two cases with the same cutaneous manifestations but different clinical course.
abstract: Congenital self-healing Langerhans cell histiocytosis or Hashimoto-Pritzker disease is 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 histiocytosis with 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.
authors.affiliation: Department of Dermatology, University of Modena and Reggio Emilia, Modena, Italy.
authors.forename: Victor Desmond
authors.initials: VD
authors.lastname: Mandel
authors.forename: Chiara
authors.initials: C
authors.lastname: Ferrari
authors.forename: Anna Maria
authors.initials: AM
authors.lastname: Cesinaro
authors.forename: Giovanni
authors.initials: G
authors.lastname: Pellacani
authors.forename: Corrado
authors.initials: C
authors.lastname: Del Forno
mesh_terms: D006646:Histiocytosis, Langerhans-Cell / Q000151:congenital* / Q000473:pathology; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D012867:Skin / Q000473:pathology
keywords: Birbeck granules; Hashimoto-Pritzker disease; Langerhans cell histiocytosis; congenital self-healing Langerhans cell histiocytosis; histiocytes