Late-onset focal dermal elastosis is a rare disorder of elastic tissue, characterized by a local accumulation of elastic fibers in the mid and deep reticular dermis. This disorder occurs mainly in elderly people and consists of multiple yellow papules that are typically distributed on the sides of the neck and flexural areas, closely resembling pseudoxanthoma elasticum from the clinical point of view. We report 2 sisters who have presented with a 20-year history of multiple yellow papules, mainly located on the dorsum of their hands. They also had few lesions of similar morphology scattered on the anterior aspects of their wrists, thighs, and lower abdomen. Histopathologic study demonstrated a focal increase in thick, interlacing elastic fibers in the mid and deep reticular dermis. The most striking features of our cases were the early age of clinical presentation, the familial involvement, and the predominantly acral distribution of the lesions.
Lichen scrofulosorum is the most uncommon clinicopathologic variant of the tuberculids. Usually, the eruption appears in children and adolescents with strong immune sensitivity to Mycobacterium tuberculosis and consists of tiny follicular papules, closely resembling lichen nitidus. We report a case of lichen scrofulosorum in an adult male with active cervical scrofuloderma who developed lesions of lichen scrofulosorum mimicking clinically lichen planus. Histopathologic study demonstrated granulomas around the hair follicles, although acid-fast bacilli stains, immunohistochemical stain for mycobacteria, polymerase chain reaction investigations and cultures failed to demonstrate Mycobacterium tuberculosis in the cutaneous lesions. The most striking features of the reported case were the onset of the eruption in an adult patient and the clinical appearance of the lesions, resembling lichen planus.
Lipoatrophic panniculitis is the term used to describe those panniculitides in which atrophy of the subcutaneous fat is the main residual lesion. They can be classified as primary or secondary. Primary lipoatrophic panniculitis is idiopathic, whereas secondary lipoatrophic panniculitides include those because of infection, malignancy, pancreatic disorders or connective tissue diseases. Usually, these entities are characterized by the appearance of tender, erythematous nodules or plaques that resolve leaving one or several localized areas with loss of subcutaneous fat. We report a 76-year-old female who developed diffuse and symmetric lipoatrophy of lower limbs after an extensive inflammatory process affecting both extremities from thighs to ankles. Histopathologic assessment revealed a lobular panniculitis with a lymphohistiocytic infiltrate, foamy macrophages and lipophagic granuloma formation. The most striking feature in our patient was the clinical presentation as a symmetrical diffuse inflammatory process resulting in lipoatrophy of the lower limbs.
Silicone is one of the most widely used filler for facial cosmetic correction and soft tissue augmentation. Although initially it was considered to be a biologically inert material, many local and generalized adverse effects have been reported after silicone usage for cosmetic purposes. We present a previously healthy woman who developed progressive and persistent generalized livedo reticularis after cosmetic surgery for volume augmentation of buttocks. Histopathologic study demonstrated dermal presence of interstitial vacuoles and cystic spaces of different sizes between the collagen bundles, which corresponded to the silicone particles implanted years ago. These vacuoles were clustered around vascular spaces and surrounded by a few foamy macrophages. General examination and laboratory investigations failed to show any evidence of connective tissue disease or other systemic disorder. Therefore, we believe that the silicone implanted may have induced some kind of blood dermal perturbation resulting in the characteristic violet reticular discoloration of livedo reticularis.
Pseudoxanthoma elasticum (PXE)-like papillary dermal elastolysis is a rare disorder clinically characterized by multiple, asymptomatic nonfollicular papules, yellow or flesh colored, grouped to form plaques with cobblestone appearance. These plaques are arranged symmetrically on the neck and supraclavicular areas of adult or elderly women, closely resembling the lesions of PXE. Histopathologically, the lesions are characterized by a band-like area of elastolysis along the papillary dermis. We report 2 cases of PXE-like papillary dermal elastolysis, in which the anomalies of the elastic tissue were immunohistochemically investigated with an antibody to the amyloid P component. This immunostaining demonstrated a band-like loss of elastic tissue along the papillary dermis. No clumping, fragmentation, or calcification of the elastic tissue was seen. We also review the literature about this rare process and discuss the differential diagnosis with other elastic tissue disorders that may show similar clinical and/or histopathologic findings.
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