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.

Congenital “self-healing” Langerhans cell histiocytosis (Hashimoto-Pritzker disease): A report of two cases with the same cutaneous manifestations but different clinical course / Mandel, Victor Desmond; Ferrari, Chiara; Cesinaro, Anna Maria; Pellacani, Giovanni; Del Forno, Corrado. - In: JOURNAL OF DERMATOLOGY. - ISSN 0385-2407. - 41:12(2014), pp. 1098-1101. [10.1111/1346-8138.12670]

Congenital “self-healing” Langerhans cell histiocytosis (Hashimoto-Pritzker disease): A report of two cases with the same cutaneous manifestations but different clinical course

PELLACANI, Giovanni;
2014

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.
2014
Birbeck granules; Congenital self-healing Langerhans cell histiocytosis; Hashimoto-Pritzker disease; Histiocytes; Langerhans cell histiocytosis.
01 Pubblicazione su rivista::01a Articolo in rivista
Congenital “self-healing” Langerhans cell histiocytosis (Hashimoto-Pritzker disease): A report of two cases with the same cutaneous manifestations but different clinical course / Mandel, Victor Desmond; Ferrari, Chiara; Cesinaro, Anna Maria; Pellacani, Giovanni; Del Forno, Corrado. - In: JOURNAL OF DERMATOLOGY. - ISSN 0385-2407. - 41:12(2014), pp. 1098-1101. [10.1111/1346-8138.12670]
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/1483809
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