Intralymphatic Histiocytosis: An Unusual Presentation

Abstract

Intralymphatic histiocytosis (ILH) is a rare cutaneous condition initially described in 1994 by O’Grady et al. It often appears as a red to violaceous, livedoid patch or plaque usually on the extremities. We present a 71-year-old female with a history of psoriasis, 50 pack years smoking and recent Legionnaires disease who came to us complaining of a red to violaceous, blanching, edematous, mildly tender lesion covering the left lower lip and extending to the chin and anterior neck. After multiple biopsies, ILH was confirmed and the patient was initially started on tacrolimus 0.1% ointment b.i.d., but there was no response. Then, she was started on oral pentoxifylline and intermittent topical steroids, as well as continuing the topical tacrolimus. There was again no response, so now she is taking a TNF-ɑ inhibitor as it appears to be a granulomatous process. These ILH cases are very rare and there is limited literature that describes one treatment as a cure. Treatment of ILH is very difficult, but several different therapies have been reported with varying success. If the disease is secondary to an underlying inflammatory disease or malignancy, then treatment of the primary disorder can lead to resolution of the ILH.

Intralymphatic histiocytosis of the upper eyelid in a patient of Korean descent: A case report

“…[ 11 ] When the disease is associated with an underlying inflammatory condition or malignancy, the intralymphatic histiocytosis may resolve by treating the underlying disorder. [ 12 ] Possible treatments include electron beam therapy, cyclophosphamide, topical and systemic steroids, amoxicillin and acetylsalicylic acid, pentoxifylline, NSAIDs, excision, pressure bandages, topical tacrolimus, and methotrexate. [ 13 ] We observed partial symptom improvements after surgical debulking and a significant improvement in erythematous edema of the upper eyelid in this patient after intralesional triamcinolone injection.…”

Cutaneous Intravascular Hematolymphoid Entities: A Review

“…With regards to skin findings, presentations can be diverse and be present for years before biopsy [83,94]. Especially in the setting of rheumatoid arthritis, common findings include irregular, livedo-like patches of erythema proximal to swollen joints in patients with rheumatoid arthritis [79][80][81]83,94,95]; however, the trunk can also be involved with similar lesions [82]. Swelling and indurated plaques have also been reported, including on the limbs, trunk, eyelids, lips, and genital areas [76,81,83,94,95].…”

“…Especially in the setting of rheumatoid arthritis, common findings include irregular, livedo-like patches of erythema proximal to swollen joints in patients with rheumatoid arthritis [79][80][81]83,94,95]; however, the trunk can also be involved with similar lesions [82]. Swelling and indurated plaques have also been reported, including on the limbs, trunk, eyelids, lips, and genital areas [76,81,83,94,95]. Outside of rheumatoid arthritis, skin findings can present differently, as in the case of a patient who developed widespread red to violaceous patches after a breast implant [91] or a patient with a smoking history and Legionnaires disease who developed facial swelling eventually diagnosed as IH [95].…”

“…Swelling and indurated plaques have also been reported, including on the limbs, trunk, eyelids, lips, and genital areas [76,81,83,94,95]. Outside of rheumatoid arthritis, skin findings can present differently, as in the case of a patient who developed widespread red to violaceous patches after a breast implant [91] or a patient with a smoking history and Legionnaires disease who developed facial swelling eventually diagnosed as IH [95].…”