Lichen sclerosus (LS) et atrophicus is a disease of unknown etiology, although hereditary, endocrine, and autoimmune factors are known to be involved. While the anal and genital regions are predominantly affected, only 2.5% of patients present with extragenital lesions, particularly of the trunk, neck, and upper limbs. The possible relationship between lichen sclerosus et atrophicus and both lichen planus (LP) and localized scleroderma (morphea) has not been clearly established, although in a number of cases, several of these conditions have been found simultaneously. We report the case of a 31-year-old woman with LS lesions affecting the neck, upper back, wrist and dorsum of the feet. The unusual character of this presentation is pointed out, along with its clinical similarity to LP.
This seemingly novel condition is characterized by the presence of histiocytic cells inside blood vessels, where they have not been described previously as an entity. The most reasonable explanation for this process is an origin from the non-classical subset of monocytes that 'patrol' the inner face of blood vessels acting as macrophages. The existence of this entity should be kept in mind to avoid overdiagnosis of malignancy.
Light chain deposition disease (LCDD) is a rare systemic disorder with deposition of mostly monoclonal amorphous nonamyloid light chains in multiple organs. Renal involvement with rapidly progressing renal failure presents the dominant manifestation of LCDD. Approximately 20%-30% of patients show symptomatic cardiac or liver involvement. Cutaneous manifestations are extremely rare with only a few published cases. We report 2 additional cases of cutaneous LCDD without detectable systemic disease.
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