Views: 1,451 | Downloads:
33
| Responses: 0
| XML | Respond to this article | Alert & updates | Request permissions | Email to a friend |
Case ReportOpen Access
Congenital Self-Healing Langerhans Cell Histiocytosis with Pulmonary Involvement : Spontaneous Regression
Chunharas A 
,
Pabunruang W ,
Hongeng S
,
Pabunruang W ,
Hongeng S Congenital self-healing Langerhans cell histiocytosis (CSHLCH) is a rare condition which
may present at birth or during the neonatal period. It is usually characterized by eruption of multiple,
discrete and red-brown papules and nodules which may increase in size and number during the first
few weeks of life with spontaneous regression. Systemic signs are usually absent except for occasional
mild hepatomegaly. The authors report a case of CSHLCH associated with multiple lung cysts with
spontaneous regression. A healthy full term male infant presented at birth with numerous diffuse
discrete dark-red papules sized 0.2-1.0 em scattered on the eyelids, temporal areas of the scalp, neck,
palms and soles. Histologic findings were mixed inflammatory infiltration of numerous histiocytes.
Immunochemistry findings were S-100 and COla positive consistent with CSHLCH. Hepatomegaly
and multiple lung cysts were detected at 1 month old. Since he was healthy, no medication was given
except for close monitoring. At 2 months of age, hepatomegaly was resolved but the liver had
sclerosing change. Skin lesions regressed completely at the age of 4 months. Lung cysts were
markedly improved at 7 months old and completely resolved at 1 year old. Spontaneous regression
in CSHLCH with lung involvement has never been reported in Thailand.
Key word : Congenital Self-Healing Histiocytosis, Langerhans Cell Histiocytosis, Lung Cyst
may present at birth or during the neonatal period. It is usually characterized by eruption of multiple,
discrete and red-brown papules and nodules which may increase in size and number during the first
few weeks of life with spontaneous regression. Systemic signs are usually absent except for occasional
mild hepatomegaly. The authors report a case of CSHLCH associated with multiple lung cysts with
spontaneous regression. A healthy full term male infant presented at birth with numerous diffuse
discrete dark-red papules sized 0.2-1.0 em scattered on the eyelids, temporal areas of the scalp, neck,
palms and soles. Histologic findings were mixed inflammatory infiltration of numerous histiocytes.
Immunochemistry findings were S-100 and COla positive consistent with CSHLCH. Hepatomegaly
and multiple lung cysts were detected at 1 month old. Since he was healthy, no medication was given
except for close monitoring. At 2 months of age, hepatomegaly was resolved but the liver had
sclerosing change. Skin lesions regressed completely at the age of 4 months. Lung cysts were
markedly improved at 7 months old and completely resolved at 1 year old. Spontaneous regression
in CSHLCH with lung involvement has never been reported in Thailand.
Key word : Congenital Self-Healing Histiocytosis, Langerhans Cell Histiocytosis, Lung Cyst
Download:
PDF