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Akilov and colleagues present a case of very unusual cutaneous T-cell lymphoma with granulomatous features that disseminated rapidly all over the skin. The pontine lesion manifested by dysarthria. While multiple chemotherapeutic regimens failed to provide the improvement, whole-body skin irradiation resolved in long-lasting complete response.

The presence of a granulomatous reaction in cutaneous lymphomas has been described in the past, especially in mycosis fungoides (MF), where a "granulomatous" variant of the disease is well known. We describe a patient with granulomatous MF (GMF) who has been followed for 13 years presenting with erythematosquamous plaques on his fingers and toes, ankles, heels, and abdomen, which on microscopic examination showed a lichenoid granulomatous reaction admixed with a neoplastic proliferation of small-sized, atypical CD4 lymphocytes. GMF is characterized by a granulomatous reaction intermingled with the dermal infiltrate of MF which may even reach the subcutaneous tissue. Only 7 cases of GMF in which the granulomas were located within the papillary or superficial dermis have been described to date. We report for the first time a unique case of lichenoid GMF where the granulomatous reaction obscures the interface between the epidermis and dermis. Sequential biopsies and complete phenotypic studies were necessary to get an accurate diagnosis.

Introduction Granulomatous mycosis fungoides (MF) is a rare variant in which granulomas are associated with other typical signs of MF. Its prognosis is worse than that of classical MF. Plasmacytoid dendritic cells (PDCs) are a subset of interferon-producing dendritic cells that link the innate and the adaptative immune responses. They have also been related to tolerance to certain tumors such as melanoma. Materials and methods In this article, we examined for the presence of CD123+ PDC in six cases of granulomatous MF from our archives. Results We found clusters of 10 or more positive cells in three of six cases of granulomatous MF (two women and a man, in their sixth and seventh decade). Although in two of these three cases the granulomatous response was extensive, in the other, it only represented 10% of the infiltrate of the biopsy. In all three cases, the granulomas were epithelioid, sarcoidal type. Conclusions CD123+ PDC can be identified in granulomatous MF. The pathogenic and prognostic role of this finding requires further clarification.

Granulomatous mycosis fungoides (GMF) is an unusual histologic subtype of cutaneous T-cell lymphoma.1 The diagnosis of GMF is usually established after observation of a granulomatous inflammatory reaction associated with a malignant lymphoid infiltrate. Epidermotropism, a clue to diagnosis in classical mycosis fungoides (MF) may be absent in about 47% of cases of GMF.2 In some instances, the granulomatous component may be intense and obscures the lymphomatous component of the infiltrate.1 There are no distinctive clinical patterns associated with GMF.1, 3

Granulomatous cutaneous T-cell lymphomas (CTCL) and lymphomatoid granulomatosis are considered granulomatous lymphoproliferative disorders. The most common types of granulomatous CTCL are granulomatous mycosis fungoides and granulomatous slack skin. Lymphomatoid granulomatosis is a rare Epstein-Barr virus driven lymphoproliferative disorder. This article reviews the etiopathogenesis, clinical presentation, systemic associations, and management of both granulomatous slack skin syndrome and lymphomatoid granulomatosis.

Granulomatous mycosis fungoides is a rare subtype of T-cell cutaneous lymphoma. Due to its clinical heterogenicity the diagnosis is delayed and based on histopathological and immuno-histochemical findings, sometimes requiring gene rearrangement studies for confirmation. We report the case of a patient who was submitted to several biopsies before diagnostic conclusion.

Granulomatous mycosis fungoides (GMF) is a rare type of cutaneous T cell lymphoma. A 38-year-old married male presented with decreased sweating all over the body for last 8 years, progressive redness and scaling over body for 2 years and multiple noduloulcerative lesions over the body for 1 year. Cutaneous examination revealed generalized erythema and scaling with poikilodermatous changes over chest and upper back along with multiple noduloulcerative lesions. Skin biopsy from a nodular lesion revealed dense granulomatous infiltrate of atypical lymphocytes with epidermotropism and sparing of appendages. Diagnosis of GMF was made. Computed tomographic scan of thorax, abdomen and pelvis revealed axillary and inguinal lymphadenopathy. Immunohistochemistry revealed leukocyte common antigen and CD3 positivity suggestive of T cell origin. Patient was started on CHOP (Cyclophosphamide, Hydroxydaunorubicin, Oncovin and Prednisolone) regimen of chemotherapy with marked improvement after three cycles of chemotherapy. This case had some clinical resemblance to lepromatous leprosy.

Background Granulomatous mycosis fungoides is an extremely rare type of cutaneous T-cell lymphoma. Two cases are described and checked for clinical, histological and therapeutic differences to other variants of mycosis fungoides. Case reports Case 1: a 52-year-old patient with livid plaques covering the whole integument. Histological findings showed granulomas with multinuclear giant cells in addition to a malignant lymphohistiocytic infiltrate (monoclonal T-cell receptor (TCR) -γ rearrangement). Despite various chemotherapeutic regimens, progression to tumour stage was observed. Case 2: an 88-year-old man with plaques and ulcerating tumours on the trunk and head. Histological findings showed malignant T-lymphocyte infiltrate (monoclonal TCR-γ rearrangement) and granulomas with multinuclear foreign-body giant cells. Complete regression of all lesions was achieved using both local psoralen-ultraviolet A and electron radiotherapy. Conclusions The diagnosis of a granulomatous mycosis fungoides depends exclusively on the histological demonstration of granulomas. Distinct clinical characteristics are not present. Apart from granuloma formation, no other noticeable histological features are evident. The presence of granulomas in mycosis fungoides does not have prognostic implications, as cases with aggressive, but also with a prolonged course have been described.

We report a patient with granulomatous mycosis fungoides whose disease transformed into a high grade blast lymphoma with angiocentric features within 12 months of the initial diagnosis. This repudiates previous claims that granulomatous inflammation is protective in cutaneous T-cell lymphoma.

Granulomatous mycosis fungoides is a rare form of mycosis fungoides with controversial histogenesis. Early reports seemed to indicate a favorable prognosis for these patients. We report two cases of granulomatous mycosis fungoides, both of which had other unusual clinical features. The cases were studied with routine light microscopy, immunohistochemistry, electron microscopy, and gene probe studies. Despite some clinical and histopathologic similarities, the results of the immunohistochemical and molecular biologic studies were diverse. These results suggest that granulomatous mycosis fungoides does not define a single subset of cases, immunophenotypically or biologically.

We describe a 68-year-old man with plaque stage mycosis fungoides (MF) for 8 years. He developed tumorous lesions of granulomatous MF (GrMF) and generalized granuloma annulare (GA) after a previously indolent clinical course. Since then, the clinical course was aggressive with involvement of the bone marrow and lymph nodes, and leukemic change occurred. Systemic chemotherapy was given, but the patient died 9 months later due to neutropenic fever and septic shock. GA in malignant lymphoma has been reported most frequently in association with Hodgkin’s disease. To the best of our knowledge, GA associated with GrMF has never been reported in the English language literature. The prognostic significance of the association of granulomatous inflammation and malignancy is reviewed.

The purpose of this report is to describe a further distinctive cause of crazy-paving pattern on thin-section computed tomography in a patient affected by pulmonary granulomatous mycosis fungoides. It was possible to reconcile the crazy-paving appearance on thin-section computed tomography scan with the histopathologic pattern termed acute fibrinous organizing pneumonia pattern.

We report a case of granulomatous mycosis fungoides that progressed into fatal gastrointestinal involvement 4 years after the onset of skin lesions, despite improvement of the skin lesions in response to a combination of PUVA and systemic interferon-gamma therapy. Histological examination showed Pautrier's microabscesses with granuloma annulare-like features and sarcoidal granuloma formation in the plaque stage, proliferation of blast-transformed atypical lymphocytes with persistent granuloma formation in the tumour stage, and metastatic lesions. A literature review of granulomatous mycosis fungoides revealed that 11 of the 24 reported cases died of the disease, and like our case, seven died within 5 years. We suggest that mycosis fungoides with granulomatous reactions does not indicate a favourable prognosis.

Granulomatous slack skin (GSS) is a rare lymphoproliferative disorder that is an unusual variant of cutaneous T-cell lymphoma (CTCL).l It is characterized by the progressive laxity of affected skin, with predilection for flexural areas.‘, 3 The disease has a marked male predominance, with age at onset typically ranging from adolescence to middle age! Although histologically similar to granulomatous mycosis fungoides (MF), GSS can be distinguished from MF by both clinical and histologic criteria? Approximately one third of the reported patients with GSS have subsequently had Hodgkin’s disease.4 We describe a patient with GSS in whom Hodgkin’s disease developed approximately 11 years after the initial appearance of his skin lesions.

Cutaneous T cell lymphoma first presenting at the tumor stage is described in three patients, including one with the rarely reported granulomatous mycosis fungoides. The rapid progression and resistance to the currently available treatment modalities seem to characterize this unique form of lymphoma.

This is a 14-year follow-up of a patient with granulomatous mycosis fungoides. The patient has had nodular lesions of mycosis fungoides for more than 30 years, an unusually long survival for the nodular form of the disease that is often quickly fatal. The role of the granulomatous inflammatory response in his survival is speculated upon.

Granulomatous mycosis fungoides is an unusual histologic variant of mycosis fungoides, a condition that is ordinarily indolent. Granulomatous slack skin, like granulomatous mycosis fungoides, shows epidermotropism, granulomatous inflammation, a clonal T-helper cell population, and progression to systemic lymphoma in some cases. Unlike granulomatous mycosis fungoides, it is characterized clinically by bulky, pendulous skin folds. The similarities between the two conditions prompted us to compare the histologic features. We reviewed 24 biopsies from 10 patients with granulomatous mycosis fungoides. These showed several distinct histologic patterns, including three cases that mimicked granuloma annulare. We also reviewed biopsy specimens from four patients with granulomatous slack skin. These specimens had a more stereotypic appearance, with permeation of the entire dermis and subcutis by lymphocytes, marked epidermotropism, and a more even distribution of granulomas and giant cells within the infiltrate. Biopsies of fully developed lesions of granulomatous slack skin showed elastolysis involving the full thickness of the dermis--a feature not seen in any of our granulomatous mycosis fungoides cases. Biopsy specimens from granulomatous mycosis fungoides and granulomatous slack skin may be mistaken for nonneoplastic granulomatous dermatitides, but they can usually be distinguished from these by the presence of epidermotropism or atypical lymphocytes. Because several of our patients with granulomatous mycosis fungoides died after courses of unremarkable length, it seems unlikely that the presence of granulomas is invariably correlated with a more benign course than nongranulomatous mycosis fungoides.

Summary— An unusual form of mycosis fungoides was characterized clinically by the spontaneous resolution of ulcerated nodular lesions into poikiloderma and histologically by a granulomatous malignant lymphoma.

A 47-year-old black man had typical papulonodular lesions of lymphomatoid papulosis (LyP) with concurrent plaque-stage mycosis fungoides (MF). Both diagnoses were confirmed histologically. This supports the concept that LyP is part of the spectrum of cutaneous T-cell lymphoproliferative disorders. The patient also had a large nodule and a deeply infiltrated plaque, each of which exhibited a deep granulomatous reaction. These were interpreted as representing granulomatous MF. Clinically, there was an evolution from a predominance of LyP lesions to a predominance of MF plaques. Topical carmustine therapy resulted in a substantial decrease in the number and size of both LyP and MF lesions. Both lesion types involuted with hypopigmentation.