M.L. Geerts

Intravascular lymphoma: clinical presentation, natural history, management and prognostic factors in a series of 38 cases, with special emphasis on the 'cutaneous variant'.

Despite its recognition as a distinct, extremely rare entity, no large studies of intravascular lymphoma (IVL) have been reported. The clinico‐pathological characteristics of 38 human immunodeficiency virus‐negative patients with IVL diagnosed in Western countries were reviewed to better delineate clinical presentation, clinical variants, natural history and optimal therapy. The IVL is an aggressive and usually disseminated disease (Ann Arbor stage IV in 68% of cases) that predominantly affects elderly patients (median age 70 years, range: 34–90; male:female ratio 0·9), resulting in poor Eastern Cooperative Oncology Group Performance Status (ECOG‐PS >1 in 61%), B symptoms (55%), anaemia (63%) and high serum lactate dehydrogenase level (86%). The brain and skin are the most common sites of disease. In contrast to previous reports, hepatosplenic involvement (26%) and bone marrow infiltration (32%) were found to be common features in IVL, while nodal disease was confirmed as rare (11% of cases). Patients with disease limited to the skin (‘cutaneous variant’; 26% of cases) were invariably females with a normal platelet count, and exhibited a significantly better outcome than the remaining patients, which deserves further investigation. Overall survival was usually poor; however, the early use of intensive therapies could improve outcome in young patients with unfavourable features. ECOG‐PS >1, ‘cutaneous variant’, stage I and chemotherapy use were independently associated with improved survival.

Evidence for a polyclonal nature of the cell infiltrate in sinus histiocytosis with massive lymphadenopathy (Rosai‐Dorfman disease)

Summary. Sinus histiocytosis with massive lymphadenopathy (SHML), or Rosai‐Dorfman disease, is rare histiocytic disorder of known origin which shares several cell markers with Langerhans’cell histiocytosis (LCH). Although Rosai‐Dorfman cells exhibit an aberrant immunophenotype, the indolent clinical course of SHML suggests a reactive disorder rather than a neoplastic process. Until recently this was prevailing opinion concerning LCH also, but recent studies have detected clonal histiocytes in all forms of this latter condition, which is therefore considered a clonal neoplastic disorder with highly variable biologial behaviour. To determine whether the histiocytic proliferation in SHML is polyclonal or clonal we used X‐linked polymorphic loci to assess clonality in lesional tissues in two women. Polymorpic regions of the human androgen receptor (HUMARA) locus were amplified by polymerase chain reaction (PCR) analysis. The HUMARA locus was informative in both cases and, following digestion with methylation‐sensitive enzymes, typical polyclonal X‐inactivation patterns were observed. Since abnormal cells accounted for >90% lesional tissue cells, we conclude that Rosai‐Dorfman histiocytic proliferation was polyclonal in the women studied.

The onychomatricoma: An electron microscopic study.

The electron microscopic findings in the onychomatricoma are described. In the proximal zone of the onychomatricoma, basal cells have various aspects, some being lacunar while others have only a limited cytoplasmic rim containing mitochondria and tonofilaments. In the parakeratotic cell columns the cells elongate and homogenized tonofilaments appear. Around the lacunae the cells are poorly differentiated and their cytoplasm is granular. It can be concluded that in an onychomatricoma the basal cells have a decreased amount of tonofilaments and desmosomes and that their evolution is not uniform. The tumour can be considered as being the result of a disturbed differentiation of nail matrix cells.

Acitretin as cancer chemoprophylaxis in a renal transplant recipient

The use of acitretin in a renal transplant recipient who had been treated for several premalignant and malignant skin lesions is the subject of this case report. During the treatment period no new dysplastic lesions developed.

Lichen sclerosus et atrophicus: an electron microscopic study.

The ultrastructural changes in seven cases of lichen selerosus et atrophicus are described. An interesting epidermal alteration was the presence of collagen fibrils in the intercellular space; furthermore, the basal cells were altered by condensation and homogenization of the tonofibrils. The basement membrane was not uniform and often multilayered. The anchoring fibrils were numerous. In the cutis intertwining strands of fine filaments, bundles of immature collagen and normal collagen could be found. In lichen sclerosus et atrophicus the dermo‐epidermal interrelationship is disturbed.

Le syndrome de Lyell

In Lyell’s disease different clinical patterns can be observed: (a) an acute scarlatiniform erythema, on which more or less extended bullae rapidly appear; (b) a morbilliform erythema, with bullae; (c

Xanthomas due to generalized oedema

A unique case of normolipaemic eruptive xanthomas due to generalized oedema is described. We propose that in this patient eruptive xanthomas were caused by the capillary leak syndrome. The increased vascular permeability could be responsible for leakage of lipoproteins into t he dermis with subsequent phagocytosis by histiocytic cells.

Erythromelalgia: a clue to the diagnosis of polycythemia vera.

We report the case of a 74-year-old woman with recurrent episodes of symmetrical congestion and erythema in the distal lower legs causing a burning distress. Laboratory and clinical investigations revealed an underlying myeloproliferative disorder. The cutaneous symptoms were atypical of erythromelalgia. Salicylates and treatment of the underlying polycythemia were able to eliminate the skin lesions but not entirely suppress the subjective discomfort.

Comparative electron microscopic study between Mendes da Costa's disease and recessive epidermolysis bullosa dystrophica

An electron microscopic study of Mendes da Costas disease (MCD) has been undertaken to determine whether this disease is a variant of epidermolysis bullosa dystrophica, or should be classified as a separate disease entity. It was shown that in MCD lacunae occur in the lower layers of the epidermis, in the presence of normal anchoring fibrils. In the recessive form of epidermolysis bullosa dystrophica (EBD), however, no lacunae were found in the epidermis, and anchoring fibrils were absent. These findings support the concept that MCD should not be considered as a special form of epidermolysis bullosa dystrophica.

Alkaline phosphatase positive lymphoma.

Alkaline phosphatase (AP) activity on cryostat sections of the skin of 38 cases of non-Hodgkins lymphomas of the B cell type and of 10 cases of lymphadenosis benigna cutis have been studied. Membrane-bound AP activity has been found in 8 out of 28 cases of low-grade malignant lymphomas of the B cell type and in 2 out of 10 cases of lymphadenosis benigna cutis. This is a known phenomenon in the latter. In the literature of non-Hodgkins lymphoma AP activity is related to the intermediate type of lymphocytic lymphoma. Further studies are needed to demonstrate that the AP activity correlates with a specific cell type in the B lymphoid cell differentiation.