[Congenital spontaneously regressive cutaneous Langerhans cell histiocytosis with bone involvement (eosinophilic granuloma)].
BACKGROUND
The classification of Langerhans' cell histiocytosis into 5 forms does not cover all types of clinical presentations. We observed a patient with inborn Langerhans' cell histiocytosis involving the skin and bone tissue. The clinical course was benign during the first two years of the patient's life.


METHODS
Skin lesions noted at birth resolved spontaneously but recurred twice in a more benign form. Histology examination showed "band" infiltration of the papillary derma composed of Langerhans' cells in direct contact with the basal epidermal layer and a few eosinophils. Immunolabelling was intensely positive for protein S100 confirming the diagnosis of Langerhans' cell histiocytosis. Extension was limited to a lytic lesion in the lower part of the left tibia which was treated by curettage.


CONCLUSIONS
In our opinion, Langerhans' cell histiocytosis covers a wide range of clinical presentations. Our case was remarkable because of the benign course despite its congenital nature and dissemination to skin and bone. Prolonged surveillance of Langerhans' cell histiocytosis is required for patients without any apparent prognosis factors.
García Muret
M P
MP
Dermatología, Clínica Quirón, Hospital de San Pablo, Barcelona.
Fernández-Figueras
M T
MT
González
M J
MJ
De Moragas
J M
JM
D001847:Bone Diseases / Q000151:congenital* / Q000000981:diagnostic imaging; D004803:Eosinophilic Granuloma / Q000151:congenital*; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000151:congenital* / Q000473:pathology; D006801:Humans; D007231:Infant, Newborn; D011859:Radiography; D012075:Remission, Spontaneous; D012871:Skin Diseases / Q000151:congenital* / Q000473:pathology; D013977:Tibia
Histiocytose langerhansienne congénitale cutanée auto-involutive avec atteinte osseuse (granulome éosinophile).