Granulomatous slack skin : Report of three patients with an updated review of the literature

• Published in: Dermatology (Basel) Vol. 196; no. 4; pp. 382 - 391
• Main Authors: VAN HASELEN, C. W, TOONSTRA, J, VAN DER PUTTE, S. J. C, VAN DONGEN, J. J. M, VAN HEES, C. L. M, VAN VLOTEN, W. A
• Format: Conference Proceeding Journal Article
• Language: English
• Published: Basel Karger 01.01.1998; S. Karger AG
• Subjects: Adolescent; Adult; Aged; Biological and medical sciences; Dermatology; Female; Gene Rearrangement, beta-Chain T-Cell Antigen Receptor - genetics; Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor - genetics; Hematologic and hematopoietic diseases; Humans; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Lymphoma, T-Cell, Cutaneous - genetics; Lymphoma, T-Cell, Cutaneous - immunology; Lymphoma, T-Cell, Cutaneous - pathology; Male; Medical sciences; Middle Aged; Skin involvement in other diseases. Miscellaneous. General aspects; Skin Neoplasms - genetics; Skin Neoplasms - immunology; Skin Neoplasms - pathology; Human; Skin disease; Elastic tissue disease; Malignant hemopathy; Case study; Infiltrate; Association; Histopathology; Lymphoproliferative syndrome; Systemic disease; T-Lymphocyte; Cutis laxa; Localized; Bibliographic review
• ISSN: 1018-8665; 1421-9832
• DOI: 10.1159/000017929

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. The results of the patients with GSS from the Netherlands are compared with the cases reported in the world literature. 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 beta genes were found in the 2 female patients; the male patient could not be tested. 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.