Systemic sarcoidosis and cutaneous lymphoma: is the association fortuitous?

Abstract

The association of systemic sarcoidosis and malignant lymphoma is known as the 'sarcoidosis-lymphoma syndrome'. Cutaneous involvement is rare in this syndrome. We report a 52-year-old woman who was diagnosed as having tumour-stage mycosis fungoides. Complete remission was achieved by combination therapy consisting of isotretinoin, interferon (IFN) alpha, electron beam irradiation, photochemotherapy and topical corticosteroids. Three years later, the patient developed systemic sarcoidosis characterized by yellowish papules on the abdominal wall and the eyelids that histologically revealed non-caseating granulomas, multiple fine-nodular interstitial pulmonary infiltrates on chest X-ray, hilar lymphadenopathy, decreased vital capacity and increased lymphocyte count in bronchoalveloar lavage fluid. As opposed to most of the reported cases, in our patient the manifestation of cutaneous lymphoma preceded the diagnosis of systemic sarcoidosis. We review the cases reported in the literature and discuss a possible causal and temporal relationship as well as the role of IFN alpha in the development of sarcoidosis.

Cutaneous Granulomas and Malignant Lymphomas

“…fungi and mycobacteria among others) and that an infectious etiology should be ruled out by culture and/or special stainings and molecular techniques. The association between sarcoidosis or other granulomatous processes and different lymphomas is the most poignant example of the first ('nonspecific') pattern, and has been referred to in the past as 'sarcoidosis-lymphoma syndrome') [2,[4][5][6][7]. In most cases, cutaneous granulomatous lesions follow the course of the underlying lymphoproliferative disease and are considered as a possible, albeit nonspecific sign of the malignant lymphoma [5].…”

Mortimer′s Malady revisited: A case of polymorphic cutaneous and systemic sarcoidosis

“…[2] Our patient had specific sarcoidal lesions like papules and plaques with eyelid papules and nodules over palms and digital tips, which are uncommon lesions, reported in isolated cases previously. [5,6] The involvement of an old tattoo with infiltrated papules is another rare and notable finding. Following the first report by Madden, [7] tattoo sarcoid has been reported infrequently.…”

Cutaneous Sarcoidosis: An Uncommon Side Effect of Pegylated Interferon and Ribavirin Use for Chronic Hepatitis C

“…Sarcoidosis is a granulomatous disorder of unknown etiology and whose epidemiology suggest a genetic tendency face to infectious agents being supposed to result of immune system deregulation leading to non-caseating granulomas as an immune reaction to an unknown persistent antigenic stimulus [19]. Inflammatory mediators such as IFN and interleukin 2 are involved in granuloma formation.…”

“…IFN has been linked to pulmonary macrophage activation, a characteristic feature of sarcoidosis which has been assumed an exaggerated T helper 1 (Th-1) immune response to a variety of exogenous antigens. It seems very likely that a potent immunoregulatory protein for Th-1 response such as IFN may induce the disease [19]. In January 2003 the eighth case of IFN-related sarcoidosis was reported with a review of the literature [19].…”