Intralymphatic Histiocytosis: An Unusual Presentation
A 71-year-old female initially presented complaining of a bright red, warm, and tender plaque on the left lower lip. Her past medical history was significant for 50 pack-years of smoking, psoriasis, and hospitalization 1 year prior from Legionnaire's disease. She was initially treated for a presumed infection without improvement. She was also referred to ENT, who recommended CT of the face and sinuses, which was negative for any significant findings.

Six months after initial presentation to a dermatologist, the patient presented with a 7-month history of a red to violaceous, blanching, edematous, mildly tender lesion covering the left lower lip and now extending to the chin and anterior neck. The intraoral exam was negative for any changes. On this visit, there was a concern for possible malignancy, and a 3-mm punch biopsy of involved skin on the left lower cutaneous lip was performed. This was interpreted as having “features most consistent with glomeruloid hemangioma.” In light of this pathology report, a workup for POEMS syndrome was initiated, although clinically the lesion did not appear to be consistent with this diagnosis.

Three weeks later, a repeat biopsy was performed and sent to a different laboratory for evaluation. The initial 3-mm punch biopsy was also sent for a second opinion. These two biopsies were found to be most consistent with intravascular/intralymphatic histiocytosis with features of granulomatous vasculitis. The patient was also sent for a second opinion, and a third and confirmatory biopsy of the left chin again showed intralymphatic histiocytosis. Initial bloodwork showed hemoglobin of 16.6 and hematocrit of 49.7, CEA of 6.4 (ref: 0–4.7), with ANA and ANCA negative. Age-appropriate cancer screening was also recommended and is still pending at this time. The patient had an MRI did which showed asymmetric prominence of the subcutaneous fat over the left face with no abnormal soft tissue mass or lymphadenopathy. As the diagnosis was not immediately evident, treatment was initiated with tacrolimus 0.1% ointment b.i.d. While this treatment did not shrink the lesion, it prevented the lesion from growing in size. Once the final diagnosis was made, it was decided to start oral pentoxyfylline and intermittent topical steroids, as well as continuing the topical tacrolimus. The patient was on this treatment regimen for 1 month with no improvement. As this appears to be a granulomatous process, our current plan is to pursue treatment with a TNF-? inhibitor.