W.A. van Vloten

Dermatopathic lymphadenopathy and lymph node involvement in mycosis fungoides

Lymph node involvement in mycosis fungoides (MF) is associated with a poor prognosis. Histologically, in most cases of clinical lymphadenopathy the excised lymph node shows dermatopathic lymphadenopathy (DL). The diagnosis of MF involvement can readily be made when the lymph node tissue has partly or wholly been replaced by atypical lymphoreticular tissue. Early involvement of a dermatopathic lymph node by MF may be difficult to diagnose. A histologic study was performed on 30 lymph nodes from 24 patients with MF. Most of these lymph nodes had been excised as part of the staging procedure. The maximal follow‐up period was five years. A classification of lymph node involvement into four categories is suggested and correlations with clinical courses and results of DNA cytophotometry of lymph node imprints are shown. Lymph nodes showing the histologic picture of DL can be divided into two groups: a group without MF involvement (category I) and a group with MF involvement (category II). The latter group is considered to represent early involvement of lymph nodes by MF. Partial or complete replacement of lymph node tissue by atypical lymphoreticular tissue corresponds with categories III and IV, respectively.

Early diagnosis of mycosis fungoides and Sézary's syndrome by morphometric analysis of lymphoid cells in the skin

Mycosis fungoides (MF) and Sézarys syndrome (SS) are cutaneous T‐cell lymphomas characterized ultrastructurally by the presence of lymphoid cells with deep and narrow nuclear indentations (cerebri‐form mononuclear cells or CMC). Early diagnosis of MF and SS is difficult because in their early stages they often resemble various forms of chronic, benign skin lesions. By measuring the frequency distribution of a nuclear shape parameter (nuclear contour index or NCI) of lymphoid cells in skin infiltrates using computer assisted planimetry, we tried to classify suspect cases into the malignant and benign groups. From 12 patients with MF or SS (malignant group) and 11 patients with chronic, benign skin lesions (benign group) the frequency distribution of the NCI of the lymphoid cell population was measured. Nonlinear discriminant analysis selected the 70th and 25th percentile of the NCI distribution of the lymphoid cells in the skin infiltrates as parameters by which these patients could be classified correctly into the malignant or benign groups with a probability of over 95%. The predictive value of these parameters was tested on ten patients suspected of having cutaneous T‐cell lymphomas. Three cases were classified as benign and 7 as malignant. In a three‐year follow up cutaneous T‐cell lymphomas did not develop in any of the 3 cases classified as benign, MF developed in 5 of 7 cases classified as malignant, 1 patient has lymphomatoid papulosis and 1 patient is still suspect for MF. These results are compared with those of DNA cytophotometry performed on skin imprint preparations. It is concluded that morphometry of lymphoid cells in skin lesions of patients suspect for MF and SS can make an important contribution to an early diagnosis of MF or SS.

Photodynamic therapy with violet light and topical δ‐aminolaevulinic acid in the treatment of actinic keratosis, Bowen’s disease and basal cell carcinoma

Background Most clinical studies using photodynamic therapy (PDT) with topical application of δ‐aminolaevulinic acid (δ‐ALA) use red light because it allows greater depth of penetration. However, given the porphyrin‐like spectrum of δ‐ALA‐induced photosensitivity, violet light provides a maximal overlap with the excitation spectrum of protoporphyrin IX, meaning that PDT with violet light uses less light energy to induce the phototoxic reaction.

Psychosocial impact of acne vulgaris: Evaluation of the relation between a change in clinical acne severity and psychosocial state

Background: Although knowledge concerning the impact of acne vulgaris on quality of life has increased in recent years, relatively few studies have assessed the effect of a change in clinical severity on psychosocial state. Objective: Assessment of the effect of a change in clinical acne severity on psychosocial state. Methods: This was investigated by means of questionnaires and clinical assessements by acne patients and dermatologists. Fifty females with mild to moderate facial acne were seen before and after a 9-month treatment with oral contraceptives. Results: The results showed a great variability in psychosocial impairment between individuals. After 9 months, a significant reduction in clinical severity was seen overall which did not relate to the significant improvements in self-esteem, stability of self-esteem and acceptance of appearance. Conclusion: Perceived psychosocial impairment is individually based, is greater in women who subjectively overrate their acne and does not relate to clinical improvement.

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

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 β 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.

Melanocytic atypia in dysplastic nevi. Immunohistochemical and cytophotometrical analysis

In a double‐blind study a correlation was found between the histologically assessed degree of nevomelanocytic atypia in 58 dysplastic nevi (DN) and the presence of two markers associated with malignant transformation. The markers included a marked expression of histocompatibility locus Class I antigens on nevomelanocytes (P < 0.01) and abnormalities in the nuclear DNA content as measured by DNA cytophotometry (P = 0.01). Both markers were present in most of the markedly atypical DN, in about half of the moderately atypical DN, and in less than 30% of the mildly atypical DN. These findings suggest that a DN with marked or moderate melanocytic atypia indicates a premalignant condition and identifies a patient at risk for melanoma.

The prognostic value of membrane markers and morphometric characteristics of lymphoid cells in blood and lymph nodes from patients with mycosis fungoides

For 26 Patients With Mycosis Fungoides (Mf), The Type And Extent Of The Skin Lesions, The Percentage Of Cerebriform Mononuclear Cells (Cmc), And T And B Lymphocytes In The Peripheral Blood And Lymph Nodes Were Correlated With Mf Involvement Of Regional Lymph Nodes, The Clinical Course, And Response To Therapy. Skin Tumors And An Involvement Of More Than 25% Of The Skin Correlated Well With Lymph Node Involvement. Normal Percentages (2–18%) Of Cmc In The Peripheral Blood Were Found For Mf Patients Without Lymph Node Involvement When Compared With Those Found For Patients With Benign Erythroderma And Healthy Donors. Elevated Circulating Cmc Percentages (>20%) Were Observed In 9 Of 11 Mf Patients With Lymph Node Involvement. In The Lymph Node Cell Suspensions From Nine Of Ten Mf Patients With Lymph Node Involvement, Increased Cmc Values Were Found (>15%), Whereas Two Of Three Mf Patients Without Lymph Node Involvement Showed Percentages Comparable (4% And 7%, Respectively) With Those Of The Control Lymph Nodes. In The Peripheral Blood Of Patients With Mf, Decreased Percentages Of T Cells (≦55%) Were Found Predominantly For Patients With Lymph Node Involvement, Whereas Normal Percentages Were Noted For Most Of The Patients Without Lymph Node Involvement. No Consistent Differences In The Percentage Of T And B Cells In The Lymph Node Cell Suspensions Were Found Between Mf Patients With And Without Lymph Node Involvement. Patients With Lymph Node Involvement Showed In General A Partial Response To Therapy With An Unfavorable Clinical Course In Contrast To Patients Without Lymph Node Involvement. Increased Percentages Of Cmc (>20%) And Decreased Percentages Of T Cells (≦55%) In The Peripheral Blood, The Presence Of Skin Tumors, And Involvement Of More Than 25% Of The Skin Are Prognostically Unfavorable Signs For Patients With Mycosis Fungoides.

A histologic study of lymph nodes from patients with the sézary syndrome

Lymph node sections from 12 patients with Sézary syndrome (SS) were studied histologically. The histopathologic alterations were compared with those in lymph nodes from four patients with (erythrodermatic) mycosis fungoides (MF) and two patients with a benign form of erythroderma. Most SS lymph nodes showed a rather monotonous and diffuse infiltration of cerebriform mononuclear cells (CMC), which tended to efface the normal lymph node architecture. By contrast, in lymph nodes involved by MF there was not only an increase in the number of CMC, but also a marked increase in the number of interdigitating reticulum cells that often showed a considerable degree of nuclear polymorphia. In the advanced stages of lymph node involvement by MF, blastic transformation was much more pronounced than in the SS lymph nodes. These histologic differences between MF and SS lymph nodes suggest that different pathogenetic mechanisms may be operative in the development of either of these conditions. Cancer 57:2375–2380, 1986.

Management of Nonstaphylococcal Toxic Epidermal Necrolysis: Follow-Up Study of 16 Case Histories

In this study the clinical and laboratory data of 14 patients who experienced 16 attacks of nonstaphylococcal toxic epidermal necrolysis (TEN) are presented. All patients were treated in the Department of Dermatology of the University Hospital of Utrecht. Only 1 patient died. Attention is focused on a successful management consisting of reversed barrier nursing, painstakingly executed skin care, timely use of antibiotics and a therapy consisting of high dosages of corticosteroids. Thorough management in centers experienced in the treatment of TEN is essential for good treatment results. Further investigations concerning the use of corticosteroids in treating TEN are needed.

Idiopathic erythroderma : A follow-up study of 28 patients

BACKGROUND Erythroderma may result from different causes, but a proportion remains undetermined (idiopathic erythroderma). Patients with idiopathic erythroderma have often been regarded to have a pre-Sézary syndrome because some of these patients have developed a cutaneous T-cell lymphoma during follow-up. OBJECTIVE The aim of this study was to investigate if this was true for our group and also if it is possible to identify further which patients are at high risk of developing cutaneous T-cell lymphoma. METHODS We analyzed clinical and follow-up data and reviewed the skin histopathology of all patients who were diagnosed with idiopathic erythroderma in our clinic between 1977 and 1994. RESULTS Twenty-eight patients, 16 males and 12 females, were diagnosed with idiopathic erythroderma. This is 27% of the patients who were diagnosed with erythroderma in our clinic, during this period. During the median follow-up of 33 months, 35% of the patients went into complete remission and 52% showed partial remission. Three patients (13%), all females, had persistent chronic erythroderma. Two of the latter group progressed to cutaneous T-cell lymphoma, i.e. 1 to Sézary syndrome and 1 to mycosis fungoides. CONCLUSION Based on our results we conclude that only patients with persistent chronic idiopathic erythroderma, which is a minority, have an increased risk of developing cutaneous T-cell lymphoma and therefore need a close and long-term follow-up.