To evaluate the clinical course and outcome of infants with Langerhans cell histiocytosis (LCH) involving skin and to estimate the incidence of progression to multi-system (M-S) disease in those with isolated skin involvement.
A retrospective review was conducted on 22 LCH patients who were younger than 12 months at the onset of their skin eruption.
Twelve patients had isolated skin involvement at diagnosis and 10 were evaluable for progression. Four of the 10 (40%) evaluable patients progressed to multi-system (M-S) disease. Of the 10 patients with M-S disease at diagnosis, 5 had a history of a preceding skin eruption 2 to 13 months prior to diagnosis. Eleven of the 14 (79%) patients with M-S disease had risk organ involvement. The mortality rate of M-S disease was 50%.
It is important for primary caregivers to recognize that isolated cutaneous LCH in infants is not always a benign disorder. The diagnosis of self-healing cutaneous LCH should only be made in retrospect. Careful, albeit non-invasive, follow-up is recommended to monitor for disease progression and development of long-term complications.
"As the prognosis of isolated cutaneous involvement is favorable and as auto-resolution may be expected, it is recommended to start with the simplest treatment and progress to systemic or interventional therapy only if required. However, treatment is recommended as isolated cutaneous LCH in infants is not always a benign disorder and the evolution is not always predictable.10 "
[Show abstract][Hide abstract] ABSTRACT: Langerhans cell histiocytosis is a rare group of proliferative disorders. Beside cutaneous involvement, other internal organs can be affected. The treatment of cutaneous lesions is difficult and relies on topical corticosteroids, carmustine, nitrogen mustard, and photochemotherapy. Systemic steroids and vinblastine are used for recalcitrant skin lesions. However, some cases fail to respond. An 18-month old boy presented a CD1a+, S100a+ Langerhans cell histocytosis with cutaneous and severe scalp involvement. Topical corticosteroids and nitrogen mustard failed to improve the skin lesions. Systemic corticosteroids and vinblastine improved the truncal involvement but had no effect on the scalp lesions. Methylaminolevulinate (MAL) based photodynamic therapy (PDT) resulted in a significant regression of the scalp lesions. Control histology revealed an almost complete clearance of the tumor infiltrate. Clinical follow-up after six months showed no recurrence.
Although spontaneous regression of cutaneous Langerhans cell histiocytosis is observed, the rapid effect of photodynamic therapy after several failures of other treatment suggests that photodynamic therapy was successful. As far as we know this is the first report of photodynamic therapy for refractory skin lesions. Larger series are needed to determine whether photodynamic therapy deserves a place in the treatment of multiresistant cutaneous Langerhans cell histiocytosis.
[Show abstract][Hide abstract] ABSTRACT: Langerhans cell histiocytosis (LCH) is the most common type of childhood histiocytic disorder with an incidence of 0.2 to 1 per 100,000 children under the age of 15 years (1). The clinical picture of LCH varies from single system (S-S)bone or skin disease to multi-system disease (M-S) (2). We report a neonate with disseminated papulonodular eruption containing mononuclear CD1a and S100 positive histiocytic cells infiltration at epidermis and underlying dermis. The diagnosis of a congenital self healing Langerhans cell histiocytosis (CSHLCH) was made and follow up showed a complete recovery of the eruptions , leaving hypopigmented macules in the sites corresponding to the initial findings.
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