Lung Tissue from:
1) open lung biopsy
2) Bronchoscopy with BAL Electron Microscopy is essential for diagnosis. X bodies with birbeck granules seen and confirmatory. Micro nodules or diffuse infiltrates noted. It is a proliferative disorder. LCH and Smoking: In adults, heavy smokers have a higher incidence of LCH. Tobacco is thought to act via glycoprotein as an immunostimulant. Cytokine IL-2 and lymphocytes are less proliferative with remission demonstrated in an adult treated with IL-2. Neonatal LCH: Many typical skin rashes are noted with LCH that are easily misdiagnosed. Skin lesions include: 1) Vecsicopustular(+/- crusting) 2) Eczema (seborrhea - like ) dermatitis
3) Mucosal lesions (erosions, granulomas, petechiae)
4) Erythema papules
5) Nodular ulceration lesions
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Cutis aplasia was a consideration in this case initially.
forms are another classification again including:
1) Congenital self healing reticulocytois (CSHRH)
2) Solitary congenital indeterminate histiocytosis (ICH)
3) Generalized eruptive histiocytosis (GEH)
Variable depending on presention and organs involved. Watchful waiting to steroid treatment +/- alkylating agents like Vinblastine, and Mecoptopurine(6MP). A pediatric oncologist should be involved in the chemotherapeutic regime as protocols now exist for chemo.
In summary pediatric histiocytosis can present with severe lung manifestations despite little clinical chest manifestations until extensive disease has occurred. Smoking is a known risk factor in adults, and should be strongly discouraged at all clinical follow-ups. Neonatal HCT is not a benign disease and can reoccur in other organs, so long term follow up is suggested. Long-term follow-up is recommended with PFT's, once children are old enough to perform these tests. Baseline CXR should be done on all LCH cases to document presence or absence of lung disease.
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