Nail involvement in Langerhans cell histiocytosis and its association with multisystem presentation and lung involvement

Onycholysis and purpuric nail striae in a child

“…4,5 Nail changes are most likely caused by direct infiltration of clonal Langerhans cells as was proven by nail biopsy in 10 of 40 reported cases. 4,5 The histopathological changes of the nails are similar to that of the skin in LCH with infiltration of the Langerhans cells in the nail matrix. 5 Nail involvement in LCH is often associated with multisystem involvement, with 37 of the 40 reported cases of LCH with nail changes having concurrent multisystem involvement.…”

“…One patient achieved spontaneous improvement during the 14‐month follow‐up 6 . One patient was treated symptomatically and remained stable during the 10‐month follow‐up 4 . The third patient was treated with vinblastine followed by cytarabine but still progressed 7 .…”

“…It has been shown that compared to BRAF wild‐type, having a BRAF mutation is associated with more severe disease, higher treatment resistance and higher reactivation rate 10 . However, data regarding the BRAF mutational status in LCH patients with nail involvement is scarce, with two patients reported and both were wild type 4,6 …”

“…6 One patient was treated symptomatically and remained stable during the 10-month follow-up. 4 The third patient was treated with vinblastine followed by cytarabine but still progressed. 7 The nail changes improved after adding NB-UVB to the treatment regimen.…”

“…The histopathological changes of the nails are similar to that of the skin in LCH with infiltration of the Langerhans cells in the nail matrix 5 . Nail involvement in LCH is often associated with multisystem involvement, with 37 of the 40 reported cases of LCH with nail changes having concurrent multisystem involvement 4,5 . For those with systemic involvement, systemic treatment is required 4,5 .…”