Papuloerythroderma of Ofuji in a HIV-infected patient.
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Aged↗
Biomedical subjects
J Toonstra
Publications and source records attributed to J Toonstra.
Treatment of primary cutaneous B-cell lymphomas of follicle center cell origin: a clinical follow-up study of 55 patients treated with radiotherapy or polychemotherapy.
PURPOSE: Primary cutaneous follicle center cell lymphomas (PCFCCL) are a distinct group of cutaneous B-cell lymphomas with a favorable prognosis after radiotherapy (RT) or polychemotherapy (PCT). In the literature, conflicting data exist regarding the efficacy and the relapse rate of both treatment modalities. In the present study, treatment results and follow-up data of a large group of PCFCCL are evaluated. PATIENTS AND METHODS: Fifty-five patients with a PCFCCL who presented with skin lesions on either the head (n = 12), the trunk (n = 35), or lower legs (n = 8), and who were initially treated with RT (40 cases) or PCT (15 cases) were studied. RESULTS: RT resulted in a complete remission in all 40 cases. Eight cases relapsed and three of these patients died as a result of their lymphoma. The estimated 5-year survival was 89%. Four of eight relapses and all three lymphoma-related deaths occurred in the group of patients presenting with tumor(s) on the lower legs. Treatment with cyclophosphamide, doxorubicin vincristine, and prednisone (CHOP) or cyclophosphomide, vincristine, and prednisone (COP) resulted in a complete remission in 14 of 15 cases. All four cases treated with COP relapsed, whereas only two of 11 patients treated with CHOP had a relapse. The estimated 5-year survival rate of the PCT group was 93%. CONCLUSION: Both RT and CHOP PCT are highly effective modes of treatment for PCFCCL. In localized PCFCCL, RT is the treatment of choice. In patients with multiple tumors involving anatomic nonrelated parts of the skin, CHOP rather than COP PCT is the preferred mode of treatment. PCFCCL on the lower legs, a subgroup that characteristically occur in elderly patients, have a higher relapse rate and a less favorable prognosis than PCFCCL presenting on the head or trunk.
Adult↗
Mammary Paget's disease confined to the areola and associated with multifocal Toker cell hyperplasia.
A hitherto unreported variant of mammary Paget's disease (MPD) limited to the areola, leaving the nipple unaffected, has been analyzed by serial sectioning of the whole areola and nipple. This otherwise characteristic MPD proved to be confined to the epidermis. There was no underlying carcinoma. This MPD was associated with a multifocal presence of monomorphic but otherwise similar cells in small collections surrounding the ostia of areolar mammary glands in the clinically unaffected area. This condition was interpreted as hyperplasia of mammary gland-related cells also found in normal nipples (so-called Toker cells). The observations hint at a possible derivation of some cases of mammary and extramammary Paget's disease from such Toker cells.
Antigens, Neoplasm↗
An unusual form of localized papulonodular cutaneous histiocytosis in a 6-month-old boy.
We report a 6-month-old boy with an unusual form of cutaneous histiocytosis. The lesions were noticed shortly after birth, and there was no evidence of systemic disease. This histiocytic disorder could not be classified according to the Histiocyte Society classification, and was therefore designated an 'unclassified' group II histiocytic disorder. The clinical picture was characterized by dark-red papulonodules with a tendency to coalesce into plaques. Histologically, the infiltrate was characterized by non-epidermotropic histiocytes showing varying degrees of differentiation, eosinophils and lymphocytes, and by the absence of foamy cells and Touton giant cells. As a most conspicuous feature, electron microscopic examination revealed laminated dense bodies, whereas Birbeck granules and comma-shaped bodies were absent. This further distinguished this uncommon variant from the well-known class II histiocytoses. During a 6-month follow-up period all the lesions showed marked regression.
Anti-Inflammatory Agents↗
The diagnostic value of morphometry on blood lymphocytes in erythrodermic actinic reticuloid.
BACKGROUND: As the differential diagnosis of erythrodermic actinic reticuloid vs Sézary syndrome (SS) can be very difficult, we examined the value of the nuclear contour index (NCI) on blood lymphocytes as the criterion for differential diagnoses. The NCI is defined as the nuclear parameter divided by the square root of the nuclear area. Three different parameters were studied: mean NCI, percentage of cells with an NCI of 6.5 or greater, and the highest NCI. These indexes were studied on blood lymphocyte samples obtained from 10 patients with erythrodermic actinic reticuloid and were compared with the findings in 10 patients with other benign forms of erythroderma and in seven patients suffering from SS. RESULTS: The patients with erythrodermic actinic reticuloid differed significantly from the group with SS regarding the percentage of cells with an NCI of 6.5 or greater and the highest NCI, but not when the mean NCI was considered. All three parameters revealed nonsignificant results for erythrodermic actinic reticuloid compared with other benign forms of erythroderma. The group with SS differed significantly from the patients with other benign forms of erythroderma regarding all three parameters. By combining three morphometric criteria (mean NCI, > or = 5.5; > 30% lymphoid cells with an NCI of > or = 6.5; and highest NCI, > or = 11.5), all patients with erythrodermic actinic reticuloid or other benign forms of erythroderma and six of the seven patients with SS were correctly classified. CONCLUSION: Our data indicate that assessment of the NCI on peripheral blood lymphocytes is of value in the differential diagnosis of erythrodermic actinic reticuloid vs SS.
Aged↗
Cutaneous manifestations and histologic findings in the hyperimmunoglobulinemia D syndrome. International Hyper IgD Study Group.
BACKGROUND AND DESIGN: The hyperimmunoglobulinemia D (hyper-IgD) syndrome is characterized by recurrent febrile attacks with abdominal distress, headache, and arthralgias. Physical examination reveals cervical lymphadenopathy in most cases and, sometimes, splenomegaly. Skin lesions have been observed in isolated cases during attacks. We summarize the features of skin lesions and the histopathologic findings in biopsy specimens in the hyper-IgD syndrome. RESULTS: A total of 44 patients with the hyper-IgD syndrome were studied. Thirty-five (79%) of them, 19 males and 16 females, had skin lesions during febrile attacks. Erythematous macules were the most common cutaneous manifestation (15 cases), followed by erythematous papules (12 cases); urticarial lesions (nine cases) and erythematous nodules (seven cases). Skin biopsy specimens of 10 patients with the hyper-IgD syndrome were available for review. The findings varied considerably. Most biopsy specimens showed mild features of vasculitis. Nonspecific findings were noted in five biopsy specimens; Sweet-like features in two, cellulitis-like findings in one, and deep vasculitis characteristics in one. CONCLUSIONS: Skin lesions are common in the hyper-IgD syndrome suggesting that they are a true manifestation of the disease.
Adolescent↗
Immunophenotyping on simultaneously occurring plaques and tumours in mycosis fungoides and Sézary syndrome.
Skin biopsy specimens from 15 patients with mycosis fungoides and Sézary syndrome, with simultaneously occurring plaques and tumours, were examined to assess phenotypic deviation. We focused on immunophenotypic differences between the two types of lesions with respect to the T-cell markers CD2, CD3, CD4, CD5 and CD8. In six patients (40%) loss of one or more T-cell markers occurred in at least one of the lesions. Three of the patients studied (20%) showed a difference in immunophenotype between plaques and tumours, with an additional loss of one of the T-cell markers in the tumours (respectively, CD5, CD2 and CD4). All three of these patients showed a larger number of blast cells in the tumour compared with the plaque. No correlation between this loss of antigenicity and the prognosis was observed. The results of this study show that different immunophenotypes can occur simultaneously in an individual patient. Furthermore, we were able to confirm a relationship between the number of intraepidermal CD1+ cells in plaque lesions and the prognosis.
Antigens, CD↗
Cutaneous immunocytomas: a clinicopathologic study of 26 cases.
The clinical and histological features of 16 patients with a primary cutaneous immunocytoma and 10 patients with a secondary cutaneous immunocytoma are reported. In all cases the diagnosis was based on the presence of monotypic plasma cells or lymphoplasmacytoid cells. Our data show that primary cutaneous immunocytomas are a distinct type of cutaneous lymphoma, characterized by (a) the presence of solitary or localized skin lesions (13 of 16 cases); (b) preferential localization on arms and legs (15 of 16 cases); (c) excellent response to local treatment (15 of 16 cases) and (d) a favourable prognosis. Histologically, these primary cutaneous immunocytomas are characterized by the presence of nodular or diffuse infiltrates with monotypic lymphoplasmacytoid/plasma cells located at the periphery of the infiltrates. Important clinical and histological differences were noted between primary and secondary immunocytomas. In the latter group more widespread skin disease was seen, often in the presence of paraproteins and/or autoimmune diseases. In contrast with the peripheral localization of the monotypic cells in primary cutaneous immunocytomas the monotypic lymphoplasmacytoid/plasma cells in secondary immunocytomas formed diffuse infiltrates or these cells were found dispersed throughout the infiltrate. There were no differences in clinical presentation or course between the different subtypes of cutaneous immunocytomas (lymphoplasmacytic, lymphoplasmacytoid and polymorphic immunocytomas). The differential diagnosis between primary cutaneous immunocytomas and cutaneous plasmacytomas, primary follicular centre cell lymphomas and cutaneous 'pseudolymphomas' is discussed.
Adult↗
Familial elastosis perforans serpiginosa.
BACKGROUND: Elastosis perforans serpiginosa (EPS) is an uncommon skin disease characterized by transepidermal elimination of abnormal elastic fibers. The disease is frequently associated with congenital connective tissue disorders or Down's syndrome. The pathogenesis of EPS is still unclear. There are a few reports in the literature about a familial occurrence of EPS in which different modes of inheritance are suggested. To support the hypothesis of a congenital origin of the disease, we have studied another family with EPS. OBSERVATIONS: In this study, we describe a family in which two sisters and a brother were affected by EPS. The father and three paternal uncles were most probably affected by the same disease. There were no signs of other congenital connective tissue disease in the family members. CONCLUSION: An autosomal dominant mode of inheritance with variable expression of EPS is suggested.
Adult↗
Ectodermal dysplasia, lipoatrophy, diabetes mellitus, and amastia: a second case of the AREDYLD syndrome.
A 19-year-old female with ectodermal dysplasia, lipoatrophy, diabetes mellitus, and amastia is described. This complex of symptoms is very similar to that of a case published by Pinheiro et al [1983] under the acronym of AREDYLD syndrome.
Adult↗
[Acrodermatitis enteropathica; a literature review].
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Acrodermatitis↗
Plasmacytoid monocytes in Jessner's lymphocytic infiltration of the skin. A valuable clue for the diagnosis.
Plasmacytoid monocytes (PCMs) are mononuclear cells of monocyte/macrophage origin with peculiar plasmacytoid features on electron microscopy. This cell type is largely unknown in dermatopathology, probably due to standard fixation procedures. The authors provide a detailed histopathologic, immunohistochemical, and electron-microscopic study of PCMs in Jessner's lymphocytic infiltration of the skin (LIS). PCMs occurred in 58% of all biopsy specimens from 75 patients with LIS in single or multiple clusters within the dermal infiltrates. Recognition of this cell type was important for differential diagnostic reasons because PCMs did not occur in polymorphic light eruption. In discoid lupus erythematosus, PCMs were present in only 7.3% of 110 biopsy specimens. PCMs are probably involved in the activation, proliferation, and differentiation of T-lymphocytes. Their origin and function are discussed and a special role in the homing of lymphocytes into the skin is suggested.
Diagnosis, Differential↗
Cell-bound IgE and increased expression of Fc epsilon-receptors on dendritic cells in cutaneous infiltrates of mycosis fungoides.
Skin biopsies of 31 non-atopic patients, 20 with mycosis fungoides, six with psoriasis and five with contact dermatitis, and of five non-atopic healthy controls were compared for the presence of cell-bound IgE and vacant IgE binding sites. IgE+ cells were demonstrated in the cutaneous infiltrate of nine (45%) patients with mycosis fungoides, two (33%) with psoriasis and one (20%) with contact dermatitis. Following pre-incubation of skin sections with IgE myeloma protein to saturate vacant IgE-binding sites, 14 out of 16 patients (88%) with stage I mycosis fungoides, five (83%) patients with psoriasis and one (20%) with contact dermatitis showed an increase in the number of IgE+ cells. While cell-bound IgE was positively related to serum IgE levels the expression of IgE-binding sites was not. All IgE+ cells were HLA-DR+ dendritic cells identified as either macrophages (CD68+, CD14+) or Langerhans cells (CD1+). Skin biopsies of non-atopic healthy controls or clinically uninvolved skin in mycosis fungoides had neither any IgE+ cells nor any vacant binding sites. Inhibition studies with IgG1, IgG4 and IgE myeloma proteins as well as with several enzymatic fragments of IgE demonstrated that IgE interacted with Fc epsilon-receptors through isotype-specific structures on the Fc epsilon-fragment. Four anti-CD23 monoclonal antibodies, however, were unable to stain vacant Fc epsilon-receptors nor could they block IgE-binding. We hypothesize that locally-secreted lymphokines, like IL-4 or interferon-gamma, induce Fc epsilon-receptors on dendritic cells in the cutaneous infiltrate and that these receptors become occupied in parallel with elevated serum IgE levels.
Antigens, CD↗
[Balloon-cell variant of the Spitz nevus].
We report on a 2-year-old male patient with a Spitz naevus on the left lower leg. In addition to the characteristic histological features of a Spitz naevus, a balloon cell component was found.
Biomarkers, Tumor↗
Actinic reticuloid.
AR can be regarded as the most severe variant of the spectrum of chronic actinic dermatitis. The exact etiology is still unknown, but is probably multifactorial, involving contact allergic, photoallergenic, phototoxic, immunologic, and metabolic factors. A diagnosis of AR should only be made when the following criteria are present: (1) persistent infiltrated papules and plaques on light-exposed skin, often with extension to covered areas or generalized infiltrated erythroderma; (2) photosensitivity to a broad spectrum of wavelengths, including UV-B, UV-A, and part of the visible spectrum; and (3) on histologic examination, a dermal infiltrate with presence of atypical lymphoid cells. When one or two criteria are lacking, the more general term chronic actinic dermatitis should be preferred. The presence of a lymphoid infiltrate dominated by CD8+ cells seems to be useful as an additional marker for a diagnosis of AR. A CD8+ predominance in dermal infiltrates is exceptional and has been reported in clinically distinguished conditions such as graft-versus-host disease, lichen planus, erythema multiforme, and pityriasis lichenoides acuta, as well as chronica, and, finally, in mycosis fungoides. In erythrodermic patients, the presence of a CD8+ -dominated dermal infiltrate and, especially, a reversed CD4+ to CD8+ ratio in the peripheral blood are highly characteristic for a definite diagnosis of AR. It is not known why preferentially CD8+ lymphocytes occur in AR. Exposure to UV radiation can induce the presence of suppressor cells. In late lesions of polymorphic light eruption, there is also an increase of CD8+ cells as compared with early lesions, perhaps contributing to local suppression of the inflammatory response.(ABSTRACT TRUNCATED AT 250 WORDS)
Humans↗
Carbon dioxide laser treatment of multiple tricho-epitheliomas.
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Adult↗
UVA-induced tumours in pigmented hairless mice and the carcinogenic risks of tanning with UVA.
An animal experiment is presented in which two groups of pigmented hairless mice were exposed daily to suberythemal doses of UVA to study tumourigenesis. The aim of the study was to estimate the carcinogenic risks of tanning by UVA. The pigmented hairless mice, Skh-hr2, were separated by selective breeding into two groups, the "browns" and the "blacks". Both groups were exposed daily to UVA from fluorescent UVA lamps (Philips TL40W/09) purified by rigorously filtering out the shorter wavelengths. No acute actinic damage was observed after any exposure. However, in most UVA exposed animals, especially in the blacks, a marked scratching preceded the development of tumours. Hyperkeratosis was also observed. All animals developed tumours. Histopathologically at least 60% of the tumours were squamous cell carcinomas. Depositions of melanophages were observed, but no melanomas. It is beyond any doubt that UVA is carcinogenic in laboratory animals. The present state of knowledge justifies no preference for tanning with UVA over tanning with UVB.
Animals↗