[Waldenstrom's macroglobulinemia with antibasement membrane activity of monoclonal immunoglobulin]. / Macroglobulinémie de Waldenström avec activité anti-membrane basale de l'immunoglobuline monoclonale.

INTRODUCTION: Waldenström macroglobulinemia is a rare hematologic disorder with characteristic malignant plasma cell proliferation associated with the secretion of a IgM monoclonal immunoglobulin which is the cause of most of the clinical manifestations. Skin involvement is exceptional. We report a new case of Waldenström macroglobulinemia discovered in a patient who developed specific skin lesions with monoclonal immunoglobulin deposits. CASE REPORT: A 50-year-old woman developed spontaneously painful infiltrated erythematopapular plaques over the extension aspects of the limbs. There were no other clinical manifestations. The patient had a kappa IgM monoclonal gammapathy. The diagnosis of Waldenström macroglobulinemia was proposed after the discovery of voluminous intra-abdominal adenopathies and was confirmed by immunohistochemistry of the biopsies, indirect immunofluorescence evidenced reactivity against the epidermal basal membrane. Chemotherapy led to successful regression of the skin lesions, the tumoral mass and circulating IgM. DISCUSSION: This is the second reported case of Waldenström macroglobulinemia with anti-basal membrane monoclonal immunoglobulin. This IgM would recognize an 82 kD antigen on the dermal side of the dermo-epidermal junction situated in the anchoring fibres of the lamina densa, a zone classically involved in acquired bullous epidermolysis.

[Acquired regressive cutaneous non-Langerhans-cell histiocytosis in an infant]. / Histiocytose cutanée acquise régressive non langerhansienne de l'enfant.

INTRODUCTION: Cutaneous histiocytosis of childhood often regresses spontaneously without treatment. In some cases however, it is difficult to differentiate aggressive forms and electron microscopy and immunohistochemistry can be a valuable help. We report a case of cutaneous histiocytosis in a child which illustrates the difficulties encountered in the classification of histiocytosis. CASE REPORT: An 18-month old girl was brought to consultation with a cutaneous nodule which had developed at the age of 15 months on the labia majora. A second nodule on the chin had regressed spontaneously. Histology showed evidence of dermal histiocyte proliferation. Immunohistochemistry demonstrated is non Langerhans nature which was confirmed by electron microscopy. The clinical course was benign after surgical exeresis of the lesion on the labia majora. DISCUSSION: Different forms of histiocytosis can be classed on the basis of 4 criteria: Langerhans origin or not, acquired or congenital forms, cutaneous or visceral involvement, benign or malignant course. Four diagnosis were possible in our case: histiocytosis X, self-healing congenital histiocytosis, benign cephalic histiocytosis, juvenile xanthogranuloma. We preferred to use the descriptive term of acquired regressive cutaneous non-X histiocytosis of childhood.

[Cutaneous hypersensitivity at the site of injection of vitamin K1]. / Hypersensibilité cutanée au point d'injection de vitamine K1.

INTRODUCTION: Skin reactions after vitamin K injections are uncommon and only seen with vitamin K1 (phytomenadione). Possible association with liver disease is debated. The pathophysiological mechanism would be related to hypersensitivity to phytomenadione. CASE REPORT: Two new cases of hypersensitivity reactions at the point of vitamin K1 injection are reported. Neither of the patients had liver disease. DISCUSSION: Injectable vitamin K1 can cause skin reactions whatever the dose and mode of injection. Two clinical presentations have been described: an acute eczematous aspect and a late onset sclerous and atrophic form. The first cases of hypersensitivity to vitamin K were reported in patients with liver disease. Several recent publications did not find such an association. Our two observations would confirm this hypothesis. The pathophysiological mechanism of the acute form would involve type IV allergy to phytomenadione as suggested by the delay between sensitization and reactivation, the histology, the patch tests which are positive with phytomenadione and negative with the carrier and the presence of reaction at rechallenge. However, the lack of the necessary sensitization phase and abnormally slow regression of eczematous lesions are unusual and might be explained by a particularly active antigenic effect of the phytomenadione molecule possibly related to the phytyl moiety.

[Lymphomatoid papulosis and anaplastic giant-cell lymphoma]. / Papulose lymphomatoïde et lymphome anaplasique à grandes cellules.

INTRODUCTION: The association between lymphomatoid papulosis and malignant Hodgkin or non-Hodgkin lymphoma is well known but still raises the problem of nosology between these two pathologies. Is lymphomatoid papulosis a pseudolymphoma, a prelymphomatous state or a true skin lymphoma? CASE REPORT: We observed a patient who had lymphomatoid papulosis and anaplastic large-cell lymphoma within an interval of 8 years between. This case was particularly interesting because identical immunophenotypes were observed in the atypical large-cells of the skin and the lymphomatous cells of the lymph nodes (positive for CD43, CD45, CD25, CD30, CD15, EMA). DISCUSSION: This case points out that atypical large-cells of lymphomatoid papulosis express the CD15 antigen which is only expressed by atypical large-cells in half of the cases of lymphomatoid papulosis. In addition, EMA is classically expressed in primary lymph node lymphomas rather than in primary cutaneous anaplastic large cell lymphomas which could predict extracutaneous dissemination of lymphomatoid papulosis. Furthermore, the demonstration that the skin lesions and the lymph nodes responded differently to the same treatment would suggest that there are other unrecognized biological differences. Lymphomatoid papulosis appears to be a range of disorders of the lymphoproliferation of activated T-cells and could include varioliform parapsoriasis and cutaneous lymphoma.

[Follicular atrophoderma, basal cell proliferation and hypotrichosis (Bazex-Dupré-Christol syndrome). A study in 2 families]. / Atrophodermie folliculaire, proliférations baso-cellulaires et hypotrichose (syndrome de Bazex, Dupré, Christol). Etude de deux families.

We describe a new case of Bazex, Dupré and Christol (BDC) syndrome in a family (family B...) where the disease affects four generations. Moreover, we report the clinical and genealogical evolution of the first family (family A...), described in 1966 by Bazex. A review of the literature has enabled us to collect the various symptoms of BDC syndrome, to discuss the relationship of this syndrome with basal cell naevomatosis and X-linked dominant chondrodysplasia punctata, and to discuss the kind of heredity. In family B..., two patients were examined. A one-month-old girl, with hypotrichosis and pseudo pili torti, and her 29-year-old father, with hypotrichosis and dorsal follicular atrophoderma of the hands. In this family, the paternal grand mother, one of her sisters, and the great grand father were also affected, but no basal cell carcinomas were found. In family A..., five patients were followed up. Four brothers, adult men aged 43 to 55 years initially presented only dorsal follicular atrophoderma the hands and face, hypotrichosis and numerous basal cell carcinomas of the face, especially in palpebral, temporal, nasal and forehead sites. Surgery was required and gave better results than radiotherapy, which left sites of sclerosis. Their mother was also affected and had been treated for numerous basal cell carcinomas of the face. The other members of the same generation (two women, two men) were unaffected. But the uncle and the grand father had the disease and at the last generation, a girl, child of one of the brothers affected, presented follicular atrophoderma and hypotrichosis.(ABSTRACT TRUNCATED AT 250 WORDS)

Cutaneous metastasis from follicular carcinoma of the thyroid gland.

A 65-year-old man with a history of malignant melanoma presented a reddish nodule on the left temporal region of the scalp. Histologic features and immunoperoxidase staining on the skin biopsy specimen confirmed the diagnosis of metastatic thyroid follicular carcinoma. The patient presented recurrences of metastatic skin nodules, lung and bone metastases, and died after a 3-year follow-up.