Nail and Scalp Lesions in a Man With Diabetes Insipidus

Langerhans cell histiocytosis with prominent nail involvement

“…To our knowledge, 21 previous cases of LCH with nail changes have been reported in the literature (Table 1). [4][5][6][9][10][11][12][13][14][15][16][17][18][19][20] As in other studies, 4,14,16,18,19 histopathologic differences between skin and nail lesions of LCH were not observed in our patient, but secondary change of acute inflammatory crusting was seen in the nail specimen.…”

“…Opposition to this theory was posed by Jain in 2000, who instead suggested that nail changes tend to occur in patients with multisystem disease and involvement of high‐risk organs, which is known to be significantly related to mortality. Therefore, nail changes did not independently indicate a poor prognosis, and in fact reported cases of nail changes not associated with multisystem disease showed excellent response to chemotherapy . Clearing of the nail abnormalities with chemotherapy is thought to depend on the extent of involvement of the nail matrix and seems to be distinct from response obtained to chemotherapy in other organs …”

“…Therefore, nail changes did not independently indicate a poor prognosis, 18 and in fact reported cases of nail changes not associated with multisystem disease showed excellent response to chemotherapy. 9,12,16 Clearing of the nail abnormalities with chemotherapy is thought to depend on the extent of involvement of the nail matrix 15 and seems to be distinct from response obtained to chemotherapy in other organs. 18 Recently, the pathogenesis of LCH has been linked to mutations within the Ras/Raf/MEK/ERK (MAPK) pathway.…”

Langerhans Cell Histiocytosis With Involvement of Nails and Lungs in an Adolescent

“…Besides these clinical features, erythematous and hemorrhagic macules, nodules in the proximal nail beds, paronychia, pus under the nails, longitudinal grooving, purpuric stria were also defined in nail LCH. 5,6 The initial systemic symptoms were dyspnea and sweating in our patient. Bilateral bullae and multiple cysts (honeycomb lungs) were identified by radiologic evaluation.…”

“…2 Nail involvement is rarely reported in the literature, both in children and adults, whereas pulmonary involvement is more frequent. [3][4][5][6][7][8][9][10] Herein, we present a patient diagnosed as LCH with both nail and pulmonary involvement which is an extremely rare occurrence. The clinicopathologic features of this rare association are discussed, including a brief review of the literature.…”