Tender linear lesions of the fingers. Acrokeratoelastoidosis (acquired type) or degenerative collagenous plaques of the hands

Acrolocalized variant of lichen sclerosus initially manifesting as degenerative collagenous plaques of the hands

“…DCPH is a chronic and slowly progressive rare condition, clinically characterized by bilateral and symmetrical linear and firm plaques on the medial and lateral aspects of the hands at the juncture of the palmar and dorsal skin. The main histopathological features consist of numerous dense collagen bundles haphazardly mixed with elastic fibers and an amorphous basophilic material in the reticular dermis . On the basis of the clinical and histopathological findings of the early lesions of our patient, a diagnosis of DCPH was established.…”

Acquired disorders of elastic tissue: Part II. decreased elastic tissue

“…AKE is a rare disorder that commonly presents during childhood or adolescence, although presentation in adulthood (ages 54e77 years) has also been reported. 148,149 There appears to be no racial or ethnic predilection. 150 Etiology and pathogenesis.…”

“…146 In addition to presumptively acquired cases, both sporadic and autosomal dominant forms of AKE have been reported. 146,148,[151][152][153][154][155] There is some evidence to suggest that the pathogenesis of AKE may be due to a failure of elastic fiber synthesis rather than degeneration. Results of an ultrastructural study showed that fibroblasts of lesional skin contain dense granules at the periphery of their cytoplasm, while extracellular elastic fibers are deficient; such findings are consistent with a defect in the secretion of elastic material.…”

Acrokeratoelastoidosis

“…On the basis of its unique clinical and histologic characteristics, Costa named this distinct entity acrokeratoelastoidosis (AKE). Since his description of AKE, several cases have been reported with the typical clinical and histologic features (2–11).…”

“…The onset of AKE is often in infancy or adolescence (9). It is a disease with autosomal dominant transmission (6,7,9,12), although it can occur sporadically without identifiable causes (11). Herein we report sporadic AKE in a 13‐year‐old patient.…”