Granulomatous slack skin: a rare subtype of mycosis fungoides

Abstract

We report a case of granulomatous slack skin, a rare and indolent subtype of mycosis fungoides. It affects mainly men between the third and fourth decades. It is characterized by hardened and erithematous plaques that mainly affect flexural areas and become pedunculated after some years. Histological examination shows a dense infiltrate of small atypical lymphocytes involving the dermis (and sometimes the subcutaneous tissue) associated with histiocytic and multinucleated giant cells containing lymphocytes and elastic fibers (lymphophagocytosis and elastophagocytosis, respectively). Patients affected by this entity can develop secondary lymphomas. There are several but little effective therapeutic modalities described. Despite the indolent behavior of granulomatous slack skin, its early recognition and continuous monitoring by a dermatologist becomes essential for its management and prevention of an unfavorable outcome.

Elastophagocytosis and interstitial granulomatous infiltrate are more common in extragenital vs genital lichen sclerosus

“…Elastophagocytosis is a non‐specific histopathologic finding of macrophage engulfment of elastic fibers and is associated with a number of inflammatory, infectious, and neoplastic disorders 12,16 . Elastophagocytosis is usually appreciated in actinic granuloma/annular elastolytic granuloma, 17 mid dermal elastolysis, 18 and granulomatous slack skin, a variant of mycosis fungoides associated with elastic tissue loss 19,20 . Less commonly, it is a feature reported in sarcoidosis, 21 necrobiosis lipoidica, 22 or infections such as leprosy 23 and blastomycosis 24 .…”