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Systemic sarcoidosis and cutaneous lymphoma: is the association fortuitous?
Author(s) -
Schmuth M,
Prior C,
Illersperger B,
Topar G,
Fritsch P,
Sepp N
Publication year - 1999
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1046/j.1365-2133.1999.02834.x
Subject(s) - sarcoidosis , medicine , mycosis fungoides , lymphoma , dermatology , pathology , systemic disease , cutaneous sarcoidosis , alpha interferon , cutaneous t cell lymphoma , immunology , interferon , immunopathology
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.