Papular Elastorrhexis, a Distinctive Entity?

Abstract

Papular elastorrhexis is a rare entity of elastic tissue characterized by multiple white papules usually located on the trunk. We report a case of papular elastorrhexis in a 22-year-old man with sparse lesions and discuss the main controversial facts in this entity: its consideration as an independent disease or as an incomplete form of Buschke-Ollendorff syndrome and the confusion with and its possible inclusion as a variant of elastic tissue naevi.

Asymptomatic whitish papules over both arms in a 28-year-old woman

“…PE is a connective‐tissue naevus with relatively rare incidence 1 . Connective‐tissue naevi are hamartomas of dermal connective tissue, and are characterized by abnormal amounts and distribution of elastin, collagen and glycosaminoglycans 2 .…”

Papular Elastorrhexis: A Case and Differential Diagnosis

“…PE is regarded as a variant of elastic tissue nevi7,8; however, there is still controversy with regard to whether PE should be categorized as a variant of elastic tissue nevi or as a distinctive entity2,9. Several important disorders require both clinical and histological differentiation from PE (Table 1).…”

“…Nevus anelasticus is usually located on mammary areas as nonsymmetrical perifollicular papules and is characterized by prominent loss of elastic fibers rather than fragmentation9. These characteristics help to distinguish it from PE.…”

“…These characteristics help to distinguish it from PE. In addition, in some cases, unlike PE, the disorder has been reported to be congenital8,9. Schirren et al10 proposed that the disorder may be an abortive form of Buschke-Ollendorff syndrome (BOS) reporting familial PE.…”

“…BOS is a rare inherited autosomal-dominant syndrome characterized by two different types of connective tissue nevi with or without osteopoikilosis11. Juvenile elastoma is the cardinal elastic lesion showing asymmetrical distribution of yellowish nodules that tend to be grouped into plaques, which is very different from PE; histologically, it shows normal collagen fibrils and an increased number of elastic fibers9,12,13. Dermatofibrosis lenticularis disseminate (DLD), a less common type, shows clinically similar features to PE; however, histopathological study has demonstrated an increase in the number of abnormal collagen fibrils8,9,12,13.…”