Congenital aggressive variant of Langerhans cells histiocytosis with CD56+/E-Cadherin- phenotype.
In children < 2 years of age, cutaneous involvement is the most frequent presentation of Langerhans cell histiocytosis (LCH). Cutaneous LCH can be localized or associated with dissemination and organ dysfunction. The clinical course is variable, ranging from spontaneous regression to a fatal outcome. We describe a female newborn presenting with congenital cutaneous lesions who rapidly developed pulmonary infiltrates and multiple osteolytic lesions. Skin biopsy showed a dermal infiltrate of medium to large cells morphologically and phenotypically consistent with LCH. The clinical course was rapidly fatal in spite of chemotherapy. No strict correlation between morphology and prognosis has been documented in LCH, but, in our case, distinct morphological and immunohistochemical features (CD56 expression and no E-Cadherin expression) may have contributed to an aggressive clinical course.
Lucioni
Marco
M
Anatomic Pathology Section, Department of Human Pathology, University of Pavia, Foundation IRCCS Policlinico San Matteo, Pavia, Italy.
Beluffi
Giampiero
G
Bandiera
Laura
L
Zecca
Marco
M
Inzani
Frediano
F
Fiandrino
Giacomo
G
Viglio
Alessandra
A
Stronati
Mauro
M
Necchi
Vittorio
V
Riboni
Roberta
R
Locatelli
Franco
F
Paulli
Marco
M
D019002:CD56 Antigen; D015820:Cadherins; D048430:Cell Shape; D017809:Fatal Outcome; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000151:congenital / Q000473:pathology*; D006801:Humans; D007231:Infant, Newborn; D010641:Phenotype; D011379:Prognosis
D019002:CD56 Antigen; D015820:Cadherins