Case Of Granulomatous Slack Skin Research Articles

A rare case of granulomatous slack skin with scarce multinucleated giant cells

A rare case of granulomatous slack skin with scarce multinucleated giant cells

Epi-O-07 - Granulomatous slack skin: clinical retrospective study of 8 cases of the Cutaneous Lymphoma French Study Group

Granulomatous slack skin (GSS) is a rare variant of mycosis fungoides (MF) presenting clinically as pendulous and lax plaques or tumours and histologically as a dermal infiltrate of neoplastic T-cells with multinucleated giant cells, loss of dermal elastin fibres and sometimes elastophagocytosis and lymphophagocytosis. Because of its scarcity, GSS is poorly known. The aim of this study was to describe the clinical and evolutive features and the response to treatment of a GSS cohort. In this retrospective multicenter study, we have identified the cases of GSS in the Cutaneous Lymphoma French Study Group database and member centers.

Outcome of a case of granulomatous slack skin, recalcitrant leg ulcers and pruritus, managed with Miculicz ointment and homeopathy

Outcome of a case of granulomatous slack skin, recalcitrant leg ulcers and pruritus, managed with Miculicz ointment and homeopathy

Granulomatous slack skin: case report with electron microscopic features

Granulomatous slack skin (GSS) is a rare subtype of mycosis fungoides. It usually presents as slowly evolving, erythematous, slack plaques that usually involve folds of lax skin. Herein, we report a case of GSS and we show electron microscopy examination. Atypical T cells with convoluted and cerebriform nuclei, lymphophagocytosis, and elastophagocytosis are key features of GSS under electron microscopy.

Mycosis fongoïde chalazodermique associé à un séminome testiculaire métastatique

Granulomatous slack skin (GSS) is an extremely rare subtype of T-cell lymphoma, a variant of mycosis fungoides (MF). Herein, we describe the first reported case of GSS associated with metastatic testicular seminoma. A 28-year-old male patient presented with circumscribed erythematous loose skin masses, especially in the body folds and which had been relapsing for 4years. Skin biopsy showed a loss of elastic fibers and an atypical granulomatous T-cell infiltrate with epidermotropism, enabling a diagnosis of GSS to be made. A biopsy of a retroperitoneal lymphadenopathy showed testicular seminoma metastasis. Patients suffering from GSS have a statistically higher risk of developing a second primary cancer, especially Hodgkin's lymphoma. The association found between GSS and a lymphoproliferative malignancy requires long-term follow-up and determines the patient's prognosis. It is not possible to prove a formal link between GSS and testicular seminoma. However, this case illustrates the value of screening for a second cancer, particularly where extra-cutaneous lesions appear during GSS treatment. Lymph node biopsy should be performed routinely in the event of GSS with possible lymph node involvement.

Clinicopathological analysis of six patients with granulomatous slack skin

Objective To investigate clinicopathological features of granulomatous slack skin (GSS) . Methods Clinical and pathological data were obtained from 6 cases of GSS, and analyzed retrospectively. Results All the 6 patients were males, and presented with slack plaques and even pendulous skin folds. These skin lesions mainly affected flexural areas in 5 patients, and only occurred in the right chest in 1 patient. One patient was diagnosed with GSS complicated by mycosis fungoides (MF) . Histopathological examination showed non-caseating granulomas with infiltration of medium-sized lymphoid cells and multinucleated giant cells in the dermis in all the patients, atypical lymphoid cells in 1 patient, and epidermotropism in another patient. Immunohistochemical study revealed monoclonal CD4+ lympho-cytosis. Elastic fiber staining was performed in 4 patients, and showed crimp, fracture and disappearance of elastic fibers, and elastic fiber phagocytized by multinuclear giant cells in 2 patients. There were monoclonal rearrangements of T-cell receptor (TCR) γ gene in 2 patients. After radiotherapy with electron beam or superficial X-ray, lesions of 2 patients became smaller, and then were excised completely without recurrence. The other 4 patients received intramuscular injection of interferon α-2b and topical application of 0.02% mechlore-thamine solution, and their condition was improved. Conclusions GSS has a slowly progressive course, and overtreatment is not recommended. Surgical excision may be the first choice for GSS localized in flexural areas. Key words: Lymphoma, T-cell, cutaneous; Cutis laxa; Mycosis fungoides; Skin manifestations; Pathology; Treatment outcome

Granulomatous Slack Skin Presenting as Acquired Ichthyosis and Muscle Masses

We describe a case of granulomatous slack skin in a 31-year-old woman with an unusual presentation of acquired ichthyosis and muscular masses involving four limbs over 3 years. Vesicles and ulcerative skin nodules first appeared only 3 months prior to diagnosis. The diagnosis was confirmed after sequential biopsies of muscle, skin lesions, and lymph nodes, together with molecular genetic studies. The patient responded poorly to various therapies, including thalidomide, and died of doxorubicin-related cardiomyopathy.

Topical Nitrogen Mustard for the Treatment of Granulomatous Slack Skin

Granulomatous slack skin (GSS) is a rare variant of mycosis fungoides in which inflammation leads to elastolysis manifested by pendulous skin folds with a predilection for flexural areas. Histologic findings include many multinucleated giant cells with large numbers of nuclei and loss of dermal elastic tissue. Definitive therapy has yet to be established, but recently interferon-alpha and radiation, interferon-gamma, and pentostatin have shown some success in the treatment of GSS. We present two cases of GSS treated with topical nitrogen mustard (chlormethine; mechlorethamine; mustine), one of whom has long-term remission and the other partial remission. Topical nitrogen mustard appears to be an effective skin-directed therapy for this rare condition.

Granulomatous slack skin without evidence of a clonal T-cell proliferation

Granulomatous slack skin is a rare cutaneous disorder with less than 50 cases presented in the English-language literature. The disease is characterized by circumscribed erythematous lax skin accentuated most commonly in the axillary and inguinal areas. A strong association with a preceding or evolving diagnosis of mycosis fungoides or Hodgkin's disease has been reported. Previous reports describe the entity as a lymphoproliferative disease in the same spectrum as mycosis fungoides and Hodgkin's disease with a monoclonal T-cell population. Our case, without an evident β-T-cell receptor rearrangement, suggests that not all cases of granulomatous slack skin are a result of an indolent lymphoma. Granulomatous slack skin probably represents a spectrum of diseases that can eventuate into a lymphoproliferative process.

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We reported 2 cases of granulomatous slack skin, one is a 39-year-old woman and the other is a 75-year-old man.In both cases, the biopsy specimens showed massive infiltration of small to medium sized lymphocytes and the loss of elastic fibers. Monoclonal rearrangement of the TCRβ gene was detected in both cases by southern blot analysis.In case 1, 33Gy X-ray irradiation for the skin lesion and subcutaneous nodules on the right upper arm and the lymphonodes in the axilla and PUVA therapy for the whole body induced almost completely regression of the lesions. In case 2, we performed 36Gy-electron beam therapy to irradiate the tumorous lesions of the right upper arm, axilla, and chest. Both patients responded to the treatment and have been almost free of disease up to the time of writing, August 2003. [Skin Cancer (Japan) 2004; 19: 238-242]

Polymerase chain reaction-denaturing gradient gel electrophoresis (PCR/DGGE)-based detection of clonal T-cell receptor gamma gene rearrangements in paraffin-embedded cutaneous biopsies in cutaneous T-cell lymphoproliferative diseases.

Polymerase chain reaction (PCR)-based amplification of T-cell receptor (TCR)-gamma genes is a novel technique that can detect a clone of T cells comprising less than 1% of the total T cells in a lymphoid infiltrate. Besides greater sensitivity than Southern blotting, this technique can be performed with smaller quantities of lower molecular weight genomic DNA as template. We retrospectively analyzed 12 paraffin-embedded biopsies of cutaneous T-cell lymphoma (CTCL), 1 case suspicious for CTCL, 1 case of granulomatous slack skin, and 8 cases of inflammatory skin diseases to determine if PCR-denaturing gradient gel electrophoresis (PCR-DGGE) analysis can detect TCR-gamma gene rearrangements on paraffin-embedded specimens. We were able to amplify Vgamma1-8 TCR sequences in each case and detected a dominant clone in 9 of 12 cases of CTCL and in granulomatous slack skin. We analyzed Vgamma9 sequences in 9 cases of CTCL and detected a dominant clone in 4 cases. This study demonstrates that PCR-DGGE can easily be applied retrospectively to cutaneous biopsies of lymphoproliferative diseases when fresh tissue is not available.

Granulomatous slack skin: Cytogenetic and molecular analyses

Granulomatous slack skin (GSS) is a rare disorder which is considered a slowly evolving T-cell lymphoma associated with granulomatous inflammation that mediates clastolysis. A combined cytogenetic, molecular, and cellular analysis was conducted on a clinically and histologically defined case of GSS. Cell cultures obtained from the skin biopsy showed trisomy of chromosome 8, and the DNA sample extracted from the skin biopsy showed a T-cell receptor β-chain rearrangement.

Granulomatous Slack Skin: Clonal Rearrangement of the T-Cell Receptor β Gene Is Evidence for the Lymphoproliferative Nature of a Cutaneous Elastolytic Disorder

Granulomatous slack skin (GSS) is characterized by the slow evolution of bulky, erythematous skin folds that have a granulomatous histology, and show destruction of dermal elastic tissue. Several cases have been putatively associated with Hodgkin's disease, and histologic similarities to mycosis fungoides have also been noted. We examined tissue from 3 cases of GSS to determine whether the condition was inflammatory or lymphoproliferative in nature. We found an abnormal, monomorphous T-helper cell immunophenotype, and in all 3 cases, clonal rearrangement of the T-cell receptor beta gene. We conclude that GSS is an indolent cutaneous T-cell lymphoma associated with granulomatous inflammation that mediates elastolysis, producing a distinctive clinical appearance.