Elastophagocytosis in extragenital lichen sclerosus

Abstract

Elastophagocytosis was observed in 20% of LS cases, all of which were extragenital. We hypothesize that elastophagocytosis in LS, especially in extragenital sites, may not be an epiphenomenon but rather represents a contributing factor to elastic fiber loss in the hyalinized papillary dermal collagen that typifies this disease.

Nodular secondary syphilis with associated granulomatous inflammation: case report and literature review

“…Elastophagocytosis in itself is a relatively non‐specific histopathologic finding that has been described in many diverse inflammatory and neoplastic processes on sun exposed and non‐sun exposed skin, which are characterized by granulomatous inflammation . These conditions include actinic granuloma, granuloma annulare, necrobiosis lipoidica, mid‐dermal elastolysis, upper dermal elastolysis, lichen sclerosus, resolving Sweet syndrome with granulomatous inflammation, sarcoidosis, eruptive vellus hair cysts with surrounding granulomatous inflammation, ruptured epidermoid cyst, morphea with occasional multinucleate giant cells, nicorandil‐associated perianal ulceration, a granulocyte colony‐stimulating associated granulomatous maculopapular eruption, North‐American blastomycosis, atrophic dermatofibroma, atypical fibroxanthoma, basal cell carcinoma and keratoacanthoma with surrounding granulomatous inflammation . Given the common feature of activated histiocytes in all of the above listed conditions, elastophagocytosis most probably represents an epiphenomenon related to the prominent granulomatous inflammation, as was seen in our biopsy specimen …”

Increase of elastic fibers in lichen sclerosus et atrophicus

“…3,6 This hypothesis is supported by the presence of prominent elastophagocytosis below the homogenized zone in 7 of 35 extragenital LSA patients. 14 Elastophagocytosis has also been observed in dermal elastolytic disorders such as annular elastolytic giant cell granuloma, 15 mid-dermal elastolysis 16,17 or upper dermal elastolysis. 18 Elastic tissue diseases are classified into two categories: diseases with diminished or increased elastic fibers.…”

Hypopigmented papules on the upper trunk and forearm of a young man

“…The pathogenicity of the anti‐ECM‐1 antibodies has not yet been substantiated. In addition, loss of elastic fibers has been proven to occur in the homogenized zone of LS, the underlying cause of which may be elastophagocytosis 4 …”

“…Histopathology usually shows epidermal atrophy, vacuolar degeneration of the basal cell layer, and homogenization of collagen in the papillary dermis with an underlying band‐like lymphohistiocytic inflammatory cell infiltrate. Loss of elastic fibers occurs in the homogenized papillary dermis 4 …”

“…In addition, loss of elastic fibers has been proven to occur in the homogenized zone of LS, the underlying cause of which may be elastophagocytosis. 4 Clinically, LS involves the genital area in 80% of patients and extragenital locations, most commonly the neck, upper trunk, shoulders, and arms, in 20%. 2,5 Oral and nail lesions have rarely been reported.…”