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PubMed2002

Follicular mucinosis: a critical reappraisal of clinicopathologic features and association with mycosis fungoides and Sézary syndrome.

CONTEXT: Beginning in 1957, patients have been described with localized alopecia characterized histopathologically by mucin deposition within hair follicles (follicular mucinosis [FM]). At least 2 distinct diagnostic entities have been proposed: one occurring in children and young adults without association with other diseases ("idiopathic" FM), the other occurring in elderly patients and associated with mycosis fungoides or Sézary syndrome ("lymphoma-associated" FM). OBJECTIVE: To determine whether idiopathic and lymphoma-associated FM are distinct or related entities. DESIGN: Case series. SETTING: Department of Dermatology, University of Graz, Graz, Austria. PATIENTS: Forty-four patients with FM were divided into 2 groups. Group 1 comprised 16 patients (mean age, 37.5 years) with no associated mycosis fungoides or Sézary syndrome; group 2 was made up of the other 28 (mean age, 52.2 years), who had clinicopathologic evidence of cutaneous T-cell lymphoma. RESULTS: Mean age was lower in patients with idiopathic FM, but a considerable overlapping among the 2 groups was present. Location on the head and neck region was common in both groups, but most patients with lymphoma-associated FM had lesions also on other body sites. In fact, solitary lesions at presentation were common in patients with idiopathic FM (11 [68.8%] of 16 patients), but uncommon in those with lymphoma-associated FM (2 [7.1%] of 28 patients). Histopathologic findings did not allow clear-cut differentiation of the 2 groups. Finally, a monoclonal rearrangement of the T-cell receptor gamma gene was demonstrated by polymerase chain reaction analysis in about 50% of tested cases from each group. CONCLUSIONS: Criteria previously reported to differentiate idiopathic from lymphoma-associated FM proved ineffective. In analogy to localized pagetoid reticulosis (Woringer-Kolopp disease), small-plaque parapsoriasis, and so-called solitary mycosis fungoides, idiopathic FM may represent a form of localized cutaneous T-cell lymphoma.

Adolescent↗
PubMed2006

Mycosis fungoides in a hemodialysis patient with intractable pruritus.

A 69-year-old Japanese man developed pruritus 3 years after beginning hemodialysis. Although eczema was not apparent at first, erythematous patches and plaques developed gradually on the affected skin. Secondary hyperparathyroidism was considered to be a main cause of this patient's pruritus, but skin lesions worsened even after parathyroidectomy had markedly decreased parathyroid hormone concentrations. Two months later, he developed an antibiotic-refractory fever of unknown origin and cervical, axillary, and inguinal lymphadenopathy. Elevations of soluble interleukin-2 receptor with 7410 U/mL and IgE with 24 600 U/mL in serum were noted, as was eosinophilia. The skin showed multiple slightly scaly patches and infiltrative plaques, which were reddish brown and distributed widely over the body surface except for the scalp and face. Mycosis fungoides, a cutaneous T-cell lymphoma, was diagnosed from biopsy specimens findings in skin and lymph node. Mycosis fungoides has not been documented as a cause of pruritus in hemodialysis patients. However, if skin lesions steadily worsen in hemodialysis patients, malignant diseases such as mycosis fungoides should be considered.

Aged↗
PubMed1978

Treatment of mycosis fungoides lymphoma: effectiveness of infusions of methotrexate followed by oral citrovorum factor.

Eleven patients with stage II or III mycosis fungoides lymphoma were treated with a regimen consisting of iv infusions of methotrexate (MTX) (60-240 mg/m2) administered sequentially and oral citrovorum factor. All 11 patients experienced a good to excellent response. Complete remissions were induced in seven of the 11 patients. In most cases remissions were sustained with weekly administration of low-dose (25--50 mg) MTX. Adverse drug reactions, such as leukopenia, anemia, and mucositis, were minimal in degree. Some unique adverse reactions included skin ulcerations (three patients), a leukocytoclastic angiitis (one patient), and painful hands and feet during MTX infusions (two patients). Our experience with this regimen has led us to conclude that it is safe and very effective in the treatment of stages II and III of mycosis fungoides.

Administration, Oral↗
PubMed1981

Megavolt electron irradiation for localized mycosis fungoides.

A retrospective analysis was carried out to evaluate the effectiveness of small field megavolt electron irradiation for localized mycosis fungoides. Only local field electron beam therapy was employed for limited disease reserving total skin electron irradiation for multiple lesions or diffuse disease covering at least 25 per cent of the entire body surface. Of the 14 patients with limited disease treated between 1964 and 1973 with the local field technique, 10 patients (71 per cent) are alive without evidence of disease at a minimum of 5 years. In contrast, of 200 patients with extensive cutaneous disease who received total skin electron irradiation, only 16 (8 per cent) were considered cured. It is concluded that early localized mycosis fungoides is potentially curable, and that limited field electron beam therapy with a relatively low total dose is adequate to obtain excellent response.

Humans↗
PubMed1990

[Comparison of the results obtained with ultraviolet radiation and photochemotherapy in the early stages of mycosis fungoides].

The Authors have performed a retrospective study, comparing the results obtained in two groups of patients affected by Mycosis Fungoides at early stages. Thirty-four patients were treated with ultraviolet rays, 32 with photochemotherapy. The patients, in the first stages of the disease, classified on the basis of TNM system, were treated with UVR or PUVA according to standard schedules. The results obtained in the two groups were the following: 1. UVR treated group: complete remission 32%, partial remission 65%, no results 3%; 2. PUVA treated group: complete remission 84%, partial remission 16%. These data show the evidence that photochemotherapy is undoubtedly more advantageous than simple phototherapy in inducing complete remission of early Mycosis Fungoides. UVR therapy still presents, however, as a worthwhile non-aggressive therapeutic choice when the disease has not a progressive character or when photochemotherapy has serious contraindications.

Follow-Up Studies↗
PubMedDate not supplied

[The ultrastructural characteristics of the early diagnosis of mycosis fungoides].

As a result of studies made (biopsy specimens of damaged skin from seven patients presenting with the erythrodermic stage of mycosis fungoides) a conclusion was reached that at the given stage the following two processes take place: (1) a rise in the proliferative activity of keratinocytes and (2) disturbances their differentiation. These ultrastructural manifestations can serve as criteria for a well-timed diagnosis of mycosis fungoides to be made.

Biopsy↗
PubMed1977

Total skin electron irradiation in mycosis fungoides.

Total skin irradiation with fast electrons (4 MeV, 3,500 rad) was applied in 10 cases of histologically proven mycosis fungoides without any signs of internal dissemination. The technique is described. Complete disappearance of both lesions and symptoms occurred in all patients after treatment. However, half of the patients relapsed after the initial irradiation and were subsequently treated with a booster dose of 1,000 rad 4 MeV electrons or with topical nitrogen mustard. Irradiation with fast electrons seems to be of great importance in the treatment of mycosis fungoides.

Female↗
PubMed1986

Mycosis fungoides involving the central nervous system.

The clinical records of nine patients, followed at the University of Chicago, with mycosis fungoides (MF) and neurologic deficits due to direct CNS involvement were reviewed. Lymph node and visceral involvement was present in all patients. Clinical findings included cranial nerve deficits in four patients, altered mental status in eight, gait disturbances in three, and leg weakness in one. Helpful diagnostic studies included lumbar puncture with CSF cytology, computerized tomography (CT) brain scans, brain biopsy, and myelogram. Two patients, who were treated with radiation therapy (RT), survived 14 and 40 weeks, respectively. Survival of less than 4 weeks limited treatment in the other patients. Autopsies were performed on six patients and revealed meningeal infiltration and perivascular infiltration of the brain. In addition, the cerebral parenchyma was infiltrated in one patient and a lymphomatous tumor was found in the brainstem of another. We conclude that the varied clinical and pathologic features of CNS involvement with mycosis fungoides resemble those seen in leukemias and other lymphoproliferative disorders. Better awareness of the clinical features may permit earlier diagnosis and initiation of therapy.

Adult↗
PubMed1982

Hypopigmented mycosis fungoides: report of five cases with ultrastructural observations.

Five dark-skinned individuals presented with widespread well-demarcated hypopigmented lesions, biopsy of which revealed the histopathological features of mycosis fungoides. Ultrastructural studies showed focal invasion of the epidermis by mycosis cells with degenerative changes in adjacent melanocytes and keratinocytes. The majority of melanocytes exhibited swelling of cytoplasmic organelles and disordered melanogenesis with production of spherical incompletely melanized melanosomes. In addition disintegrating melanocytes were occasionally seen. Perifollicular repigmentation within hypopigmented areas occurred in two patients following clearing of the epidermal infiltrate with PUVA therapy. Mycosis fungoides may present with areas of cutaneous hypopigmentation.

Adolescent↗
PubMed1987

A case-control study of possible causative factors in mycosis fungoides.

A detailed case control study was carried out on 53 patients (33 males and 20 females) with histologically proven mycosis fungoides and on an age- and sex-matched control population. Possible causative factors investigated included occupation, recreation, and exposure to petrochemicals, pesticides, insecticides, and potential carcinogens. Exposure to plants of the Compositae family, tanning history, and chronic sun exposure were also investigated, as were smoking history, drug ingestion history, and other skin disease. Personal and family histories of other malignancies were also investigated. The only statistically significant difference to emerge was that the patients with mycosis fungoides had significantly more family history of atopic dermatitis. In view of the absence of any significant difference between patients and controls with regard to personal history of atopic dermatitis, this difference may be the result of multiple statistical testing rather than a phenomenon of true biological significance.

Adolescent↗
PubMed1984

The treatment of ulcers in patients with mycosis fungoides with split-thickness skin grafts.

Five patients have recently been treated at this institution for skin ulcerations caused by mycosis fungoides and its treatment with total body electron beam radiation therapy. Both the disease and its treatment can injure epidermal appendages as well as the blood supply to the skin. As a result of this potential damage, there was initial concern that skin grafts might not take and donor sites might not heal in these patients. Though skin grafting has not been universally successful, we have been able to skin graft the majority of the ulcerated lesions in these patients with good results. Donor sites have healed, though more slowly than usual. Skin grafting can be effective for treating ulcers in patients who have undergone total body electron beam radiation therapy for mycosis fungoides.

Adult↗