Granulomatous slack skin: Cytogenetic and molecular analyses

Grammatico, Paola; Băluş, L.; Scarpa, Susanna; Varesco, Liliana; Rosa, C. Di; Grammatico, Barbara; Porto, G. Del

doi:10.1016/0165-4608(94)90122-8

Cited by 21 publications

(24 citation statements)

References 7 publications

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“…At this stage, the skin surface starts to wrinkle and softens [34]. In some patients ulceration may occur [10, 11, 13, 18]. When lesions become more atrophic, subcutaneous blood vessels may become visible [34].…”

Section: Discussionmentioning

confidence: 99%

“…Molecular clonality studies of skin samples have been reported in 8 patients [3, 8, 9, 10, 11, 12, 13]. These studies revealed clonal rearrangement of the T-cell receptor TCRβ or TCRγ gene.…”

Section: Introductionmentioning

confidence: 96%

“…The disappearance of elastic fibers results in lax skin. Follow-up data from all reported patients with GSS reveal a relationship between GSS and a preexistent or subsequent lymphoproliferative malignancy in about half of the cases [3, 4, 5, 6, 7, 8, 9, 10, 11]. …”

Section: Introductionmentioning

confidence: 99%

“…Genotypic studies in 2 cases revealed trisomy 8 in both of them [13, 14]. Therefore most authors suggest that GSS in fact is an indolent cutaneous T-cell lymphoma (CTCL) [3, 8, 9, 10, 11, 12, 13, 14, 15]. …”

Section: Introductionmentioning

confidence: 99%

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Granulomatous Slack Skin

et al. 1998

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Purpose: Granulomatous slack skin (GSS) is a rare cutaneous disorder characterized clinically by the evolution of circumscribed erythematous lax skin masses, especially in the body folds, and histologically by a granulomatous T-cell infiltrate and loss of elastic fibers. GSS is often associated with preceding or subsequent lymphoproliferative malignancies, especially mycosis fungoides (MF) and Hodgkin’s disease (HD). No effective treatment is known yet. Whether this entity is a benign disorder, a peculiar host reaction to a malignant lymphoma, a precursor of malignant lymphoma or an indolent cutaneous T-cell lymphoma (CTCL) in itself is still a matter of debate. Patients and Methods: The results of the patients with GSS from the Netherlands are compared with the cases reported in the world literature. Results: A female patient had had GSS for 8 years without developing a secondary malignancy. In a second female patient with a histologically confirmed diagnosis of MF, GSS developed 18 years later in the axillary and inguinal folds which had previously been affected by plaque-stage MF lesions. A third male patient with a 6-year history of erythematosquamous skin disease diagnosed as CTCL developed GSS. Moreover, granuloma formation was also found in a facial basal cell carcinoma, in a cervical lymph node and the spleen. Clonal rearrangements of the T-cell receptor β genes were found in the 2 female patients; the male patient could not be tested. Conclusion: GSS is a rare clinicopathological entity. Only 34 patients have been described so far. The development of GSS within plaque MF lesions has not been reported before. Our third case developed very extensive skin lesions and showed a strong propensity to develop granulomas as compared to cases reported before. The presence of a clonal T-cell population was demonstrated in all cases tested. Our cases support the idea that GSS is a very rare and rather indolent type of CTCL. Apparently, the disease is associated with a peculiar immune response, characterized by granuloma formation and disappearance of elastic fibers resulting in the lax skin. The relationship between GSS and other preexisting or subsequent lymphoproliferative diseases (diagnosed in approximately 50% of the cases) warrants a life-long follow-up.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 96%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Granulomatous Slack Skin

et al. 1998

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“…2,10 Essa alteração cromossômica está associada com a patogênese do linfoma de Hodgkin (LH), assim como dos linfomas não Hodgkin, leucemias e sarcoma de Ewing. 6,8 Rearranjos para os genes TCR β 6,9 e TCR δ 7 nas lesões cutâneas da CLG têm sido encontrados, confirmando a possibilidade de doença clonal T. A clonalidade não 3 O uso do interferon α nessa paciente apenas melhorou o quadro de hiperemia cutânea.…”

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