 |
|
|
|
|
|
|
|
|
|
 |
 |
 |
 |
 |
 |
 |
 |
|
Background
This rare and unusual skin disease was once thought to be an unusual autoimmune disorder. Dermatopathologists first elucidated the cause as a rare variant of an indolent cutaneous T-cell lymphoma. It is characterized by pendulous folds of erythematous skin especially prominent in flexural areas.
SYNONYMS GSS
DISEASE ASSOCIATIONS CHARACTERIZATION HODGKIN'S DISEASE Granulomatous slack skin: report of a case associated with Hodgkin's disease and a review of the literature.
Noto G, Pravata G, Miceli S, Arico M.
Instituto di Dermatologia Sperimentale, University of Palermo, Policlinico P. Giaccone, Italy.
Br J Dermatol 1994 Aug;131(2):275-9 Abstract quote
We report a case of granulomatous slack skin (GSS) associated with Hodgkin's disease, and review the literature on this entity.
GSS, a variant of cutaneous T-cell lymphoma, clinically presents with erythematous patches in the flexures, which gradually transform into bulky, pendulous areas of skin. Histology shows an elastolytic granulomatous infiltrate, with atypical lymphoid cells, and occasional epidermotropism. As far as we are aware, 10 cases of GSS, including our patient, have been reported in detail. The male:female ratio of these cases is 9:1, and the age range 15-51 years.
Five cases were associated with Hodgkin's disease, one with small lymphocytic lymphoma, and one developed cutaneous T-cell lymphoma. The axillae, abdomen and groins were the most frequently affected areas. No definitive management for GSS has been established. Surgery has been performed in localized forms, and systemic treatments have included corticosteroids, dapsone, chlorambucil, nitrogen mustard, and radiotherapy.
Our patient was treated with chemotherapy for his Hodgkin's disease, and this resulted in complete remission of the lymphoma. Subsequent maintenance therapy with interferon-alpha produced good control of the cutaneous lesions.
PATHOGENESIS CHARACTERIZATION T-cell lymphoma J Invest Dermatol 1987;89:183
Elastolysis and lymphophagocytosis may be mediated by phagocytosis and digestion within multinucleated giant cells provoked by the underlying T-cell lymphoma
CLINICAL VARIANTS/GROSS DISEASE CHARACTERIZATION Granulomatous slack skin. Report of three patients with an updated review of the literature.
van Haselen CW, Toonstra J, van der Putte SJ, van Dongen JJ, van Hees CL, van Vloten WA.
Department of Dermatology, University Hospital of Utrecht, The Netherlands.
Dermatology 1998;196(4):382-91 Abstract quote
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 beta 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.
HISTOLOGICAL TYPES CHARACTERIZATION General Non-necrotizing granulomas with numerous multi-nucleated giant cells, mononuclear histiocytes, and atypical lymphocytes
Characteristic loss of elastic fibers, confirmed by elastic staining
VARIANTS
SPECIAL STAINS/IMMUNOPEROXIDASE/OTHER CHARACTERIZATION Electron microscopy (EM) J Cutan Pathol 2001;28:44-48
Autophagolysosomes
Heterophagolysosomes morphologically similar to Birbeck granules
Morphologically abnormal elastic and collagen fibers
PROGNOSIS AND TREATMENT CHARACTERIZATION TREATMENT INTERFERON Granulomatous slack skin: successful treatment with recombinant interferon-gamma.
Kono T, Nagayasu TS, Nakanishi T, Tsuruta D, Ishii M, Taniguchi S, Takeda T, Hino M.
Department of Dermatology, Osaka City University Medical School, Japan.
Br J Dermatol 2000 Feb;142(2):353-7 Abstract quote
Granulomatous slack skin is a rare variant of indolent cutaneous lymphoma, characterized by a cutis laxa-like clinical appearance and widespread granulomatous infiltration.
A 25-year-old man had suffered from slowly progressive infiltrative, scaly and atrophic plaques and flaccid nodules with deep induration in the axillae, trunk and thighs.
Histopathologically, dense lymphoid cell infiltration with numerous multinucleated giant cells (MGC) throughout the dermis to the subcutaneous tissue was observed. T-cell receptor gene rearrangement was detected in the skin lesions. Granulomatous infiltration with MGC was found in enlarged lymph nodes and the liver.
Acute exacerbation was successfully treated with systemic recombinant interferon-gamma.
Arch Dermatol 1984;120:1085
Arch Dermatol 1985;121:250
Dermatol Clin 1994;12:375
Am J Dermatopathol 1996;18:199
Histopathology 1994;25:49
Mycosis Fungoides
Last Updated 2/4/2002
Send mail to The Doctor's Doctor with questions or comments about this web site.
Copyright © 2004 The Doctor's Doctor