Mycosis Fungoides: A Dermatological Masquerader

D. Nashan; D. Faulhaber; S. Ständer; T.A. Luger; R. Stadler

Disclosures

The British Journal of Dermatology. 2007;156(1):1-10. 

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Granulomatous Mycosis Fungoides and Granulomatous Slack Skin

Ackerman and Flaxman[61] proposed the term 'granulomatous MF' in 1970. In 1978 Ackerman[62] added the term 'granulomatous slack skin disease'. In 1968 Bazex et al.[63] described a clinical form similar to cutis laxa as 'Besnier-Boeck-Schaumann disease'. In 1997 and 2005 'granulomatous slack skin' was included in the EORTC[64] and then the WHO-EORTC classification[6] as a provisional entity. So far up to 42 cases have been reported. Granulomatous T-cell infiltrates and loss of elastic fibres lead to asymptomatic skin wrinkles, mainly in the flexures (Fig. 5).[65] Sometimes pruritus and erythema are described. Granulomatous MF may be preceded by erythematous scaling patches or macules, and may coexist with classical MF lesions. Benign as well as progressive courses were described. In 50% of cases coexistence with Hodgkin disease or nodal non-Hodgkin disease is observed.

Mycosis fungoides with granulomatous slack skin.

From a histological point of view it is discussed whether granulomatous MF and granulomatous slack skin belong together.[66,67] The pathogenesis of granuloma formation in lymphoma is unknown and its occurrence is not specific for MF. Similar histological formations have been described in pleomorphic T-cell lymphoma, panniculitis-like T-cell lymphoma and in B-cell lymphomas.[68] A case report of a CD30+ lymphoma with granulomatous slack skin exists, so not even granulomatous slack skin is a specific subform of MF.[69] Therefore clinically and histologically granulomatous MF variantshave to be differentiated from other cutaneous T-cell lymphomas and from sarcoidosis, granuloma annulare and infectious granulomas ( Table 8 ).[70,71] The so-called 'sarcoidosis-lymphoma syndrome' includes the association of sarcoidosis and lymphoma, among other entities described in association with MF.[72]

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