Disseminated Xanthosiderohistiocytosis With Monoclonal Gammo
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In 1960, Halprin and Lorincz documented the first case of disseminated xanthosiderohistiocytosis (DXSH), a rare variant of xanthoma disseminatum (XD) with a distinct clinical and histopathologic presentation. To the knowledge, only 5 cases have been reported in the literature, with the most recent reported case dating back more than 25 years. Authors describe a 65-year-old man with DXSH in association with monoclonal gammopathy and ocular manifestations.

The patient first presented with bilateral conjunctival erythema and choroidal thickening. Initial choroidal biopsy results were interpreted as a granulomatous reaction with a lymphoplasmacytic infiltrate of undetermined origin. Medical history was notable for coronary artery disease, treated dyslipidemia, and hypertension. One year after the biopsy, the patient developed asymptomatic skin lesions on the lower limbs with trunk and upper limb progression.

A 3-week tapered course of oral prednisone (60 mg/d) was administered by the ophthalmology team, with neither ocular nor cutaneous improvement. One year later, he developed immunoglobulin G–K type monoclonal gammopathy. On the patient’s first visit to the dermatology department, 3 years after he had developed ophthalmic symptoms and 1 year following gammopathy onset, a physical examination revealed many flat, firm, brownish papules and plaques resembling keloids.

Some plaques were linear with an arborizing pattern. The lower limbs, trunk, and upper limbs were symmetrically involved, whereas the face, oral mucosa, and flexor regions were spared. Baseline blood test results were normal, including lipid and serum iron levels, although a hypocomplementemic state was noted.

Several skin biopsy specimens were obtained. The first was interpreted as granulomatous folliculitis with scarlike fibrosis. Several subsequent biopsies revealed diffuse fibrosis with scarlike morphologic features. In light of the clinical presentation of dense keloidal plaques arising in a cephalocaudal distribution, clinicopathologic correlation led to the diagnosis of DXSH. A 6-month IV immunoglobulin treatment regimen (1 g/kg) was started, but unfortunately the patient died of ventricular fibrillation a few months later. An autopsy was declined by the family.

Considered a rare variant of XD, DXSH is best classified within the spectrum of cutaneous non–Langerhans cell histiocytoses with a major systemic component.

Source: https://jamanetwork.com/journals/jamadermatology/fullarticle/2767072