Granulomatous slack skin

Very rare variant (about 50 cases in world literature) of a usually CD4-positive cutaneous T-cell lymphoma characterized by granulomatous tissue formations and loss of elastic fibers. Seen by some authors as a variant of Mycosis fungoides.

The elastolytic cutaneous T-cell lymphoma is clinically characterized by cutis laxa-like skin wrinkling typically in intertriginous areas. Just as in the case of the so-called granulomatous mycosis fungoides (entity is questionable), a loss of elastic fibers is observed in granulomatous slack skin which is often confirmed by elastophagocytosis. According to current knowledge, both forms of granulomatous T-cell lymphoma can only be reliably distinguished by the clinical findings described above.

Detection of often diffuse, dermal T-cell infiltrates (CD3+, CD4+ and CD8-) with granulomatous signs including giant histiocytic cells and occasionally multinucleated giant cells (nuclei arranged in a ring, sometimes > 40 nuclei). Epidermotropism of the lymphocytic infiltrate is not always observed, as would be expected in classical mycosis fungoides. Granuloma formation can also be so strong that the actual T-cell lymphoma is masked. It is also very difficult to differentiate granulomatous mycosis fungoides from other T-cell lymphomas with granulomatous reactions.

By detecting a monoclonal rearrangement of the T-cell receptor gene, granulomatous mycosis fungoides can be well distinguished from inflammatory granulomatous diseases (e.g. sarcoidosis, granuloma anulare).

In 20% of patients secondary occurrence of other lymphoproliferative diseases (non-Hodgkin's lymphomas, mycosis fungoides).

• S.u. Mycosis fungoides.
• The therapy of elastolytic cutaneous T-cell lymphoma does not differ significantly from the classical form of Mycosis fungoides (see there). However, it must be considered that interferons and retinoids can induce sarcoidal tissue reactions.

The prognosis of elastolytic cutaneous T-cell lymphoma is comparable to folliculotropic mycosis fungoides (see below mycosis fungoides, folliculotropic) and thus worse than the prognosis of classic mycosis fungoides.

Granuloma formation is only found in about 2% of cutaneous lymphomas. The most common form of granuloma is a granulomatous variant of mycosis fungoides. Granulomatous changes can occur in all lesions (patch, plaque, tumor, lymph nodes) of Mycosis fungoides and cannot be distinguished clinically from conventional clinical manifestations of Mycosis fungoides. Sometimes granulomatous lesions even occur before the classical skin lesions of Mycosis fungoides, which makes a correct diagnosis difficult.

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