Histiocytosis X in two adults. Treatment with topical mechlorethamine

Abstract

Two adult patients with skin lesions typical of histiocytosis X were treated topically with mechlorethamine hydrochloride. In the patient whose disease was limited to the skin, a sustained remission was induced, but in the patient with extensive systemic disease, cutaneous remission lasted only six weeks.

Topical nitrogen mustard therapy in patients with Langerhans cell histiocytosis

“…Frequency and time to initial treatment response was similar to previous reports 7,8 . The duration of CR was short in patients with multisystem disease, which was similar to previously published case reports 9 …”

“…The frequency of contact dermatitis in the present cohort was higher than has been previously reported; however, the extent of cutaneous involvement in the present series was also greater, and therefore the dose of nitrogen mustard was larger 7,8 . In general, nitrogen mustard has not been used for extensive skin involvement by LCH, due to the risk of systemic involvement and possible development of secondary malignancies 1,7–9 . However, most of the studies showing secondary malignancies have looked at patients with cutaneous T‐cell lymphoma, who are in general older and have been treated with other therapies for prolonged durations 8,10,11 .…”

Adult Langerhans Cell Histiocytosis Limited to the Skin

“…One trial evaluating treatment of cutaneous manifestations in patients with multisystem LCH found that 14 out of 16 patients had complete lesional healing with topical mechlorethamine [39]. This treatment did not produce the same outcome in our patient although this finding has been supported by other reported cases [6, 22, 23, 24, 40]. Future studies are necessary to evaluate a possible uniform treatment for LCH.…”

Cutaneous Langerhans Cell Histiocytosis Responsive to Topical Nitrogen Mustard

“…Therapy was well-tolerated, with limited short-term side effects including hyperpigmentation and mild to moderate allergic contact dermatitis. [8][9][10][11] Our two cases support topical nitrogen mustard as a possible treatment for primary cutaneous LCH recalcitrant to other treatment modalities, inducing complete response of lesions in a rapid manner. In our two cases, patients experienced com-She attempted high-potency topical corticosteroids, topical tacrolimus, and a combination of amitriptyline, ketamine, and lidocaine patch 5% without relief.…”

“…1,5,7 The data on topical nitrogen mustard as treatment for primary cutaneous LCH in adults is limited to 5 known cases in the literature. [8][9][10][11] In these cases, patients had clearance within an average of 2 to 3 weeks. Therapy was well-tolerated, with limited short-term side effects including hyperpigmentation and mild to moderate allergic contact dermatitis.…”