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A man in his 80s presented with an 18-month history of an irregularly linear, rusty-brown, atrophic, streaklike plaque extending from the tip of his right shoulder to the right biceps, where it ended in arborizing purple plaque with scattered, slightly indurated, irregular papules within it (Figure, A and B). There was no warmth, thrills, or pulsations across the affected area. The patient complained about a mild tingling sensation within the lesion. The patient could not think of any triggers or causes for his condition and denied any aggravating or alleviating factors as well as any treatment attempts. Complete review of systems yielded negative results except for easy bruising. He was taking finasteride, montelukast, enalapril, and metoprolol. His medical history was significant for right total reverse shoulder replacement 5 years prior. Apart from the previously described lesion, his physical examination was unremarkable, and he had full range of motion of his right shoulder. A punch biopsy of the slightly indurated papule from the involved area on the right biceps was completed (Figure, C and D).
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D. Intralymphatic histiocytosis
Microscopic findings from the punch biopsy showed a dense, nodular infiltrate of lymphocytes and histiocytes (highlighted by immunohistochemical stain for CD68 antigen) throughout the dermis and within thin-walled lymphatic vessels (highlighted by immunohistochemical stain for D2-40 antigen). These are diagnostic findings of intralymphatic histiocytosis.
Intralymphatic histiocytosis is a rare condition first described in 1994 as intravascular histiocytosis.1 It usually presents on limbs as ill-defined erythematous or reticulated plaques resembling livedo reticularis, and it may be associated with rheumatoid arthritis, malignant tumors (Merkel cell carcinoma, incidental finding at the periphery of the excision specimen2; breast carcinoma, in the mastectomy scar2; and lung adenocarcinoma, in the dermis overlying the soft tissue metastasis3), and orthopedic metal implants (such as in the present case).2- 5 However, it may also occur without any other associated conditions.2 The pathogenesis is unclear, but it is hypothesized to be a nonspecific reaction to an injury such as trauma, including surgery; inflammation; or infiltrative cancers, all of which may cause impaired lymphatic flow.6 There is an attempt to include intralymphatic histiocytosis with other peculiar histiocytic proliferations occurring in various body sites under a unifying diagnosis of histiocytosis with raisinoid nuclei. All of these proliferations are benign and, except for the skin lesions, asymptomatic conditions.6 Mazloom and colleagues7 make a case that intralymphatic histiocytosis is a distinct entity from intravascular histiocytosis, which they include in the group of reactive angiomatoses.
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Corresponding Author: Viseslav Tonkovic-Capin, MD, Dermatology Clinic, Golden Valley Memorial Hospital, 1602 N Second St, Clinton, MO 64735 (firstname.lastname@example.org).
Published Online: July 3, 2019. doi:10.1001/jamadermatol.2019.1643
Conflict of Interest Disclosures: None reported.
Additional Contributions: We thank the patient for granting permission to publish this information.
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