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Biomedical subjects

D Lipsker

Publications and source records attributed to D Lipsker.

At least 55 records · Page 3Linked to original sources
PubMed1997

[Lymph node and cutaneous syndrome associated with bone plasmacytoma].

INTRODUCTION: We report two patients in whom a slowly growing erythematous thoracic lesion led to the diagnosis of an underlying plasmocytoma. After the treatment of the latter, the cutaneous lesions disappeared, strongly suggesting a link between the two manifestations. CASE REPORTS: The two male patients, aged respectively 66 and 73 years old, had erythematous thoracic plaques. In both cases, extensive laboratory work-up and a histological examination of a cutaneous biopsy did not allow a precise diagnosis. The two patients had a solitary bony plasmocytoma located beneath the cutaneous plaques. The plasmocytomas were discovered respectively 2 and 4 years after the first cutaneous manifestations and were associated to histological non-specific lymph node hyperplasia. Treatment of the plasmocytoma led to the disappearance of skin lesions in both patients. DISCUSSION: In both cases, an erythematous scleroderma-like plaque associated to palpable peripheral and mediastinal lymph nodes, was located over a solitary bony plasmocytoma. Treatment of the plasmocytoma led to the disappearance of the cutaneous lesions, strongly suggesting a link between the two manifestations. Such an association has not been previously reported. We think this is a new entity, characterised by a scleroderma-like cutaneous plaque overlying a solitary bony plasmocytoma and associated to superficial and deep lymph node hyperplasia. We suggest to call it "plasmocytoma associated cutaneous lymph node syndrome". Its relationship to the POEMS syndrome and scleromyxedema are discussed. The pathophysiology remains completely unknown; the syndrome regresses after the treatment of the plasmocytoma.

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PubMed1997

[Primary cutaneous monomelic B-cell lymphoma].

BACKGROUND: Cutaneous B cell lymphomas, especially when appearing as a monomelic papulonodular eruption, are rare. PATIENT: Ms H. 87-year-old, consulted for a papulonodular eruption of the left lower limb which developed during the past 5 months. This limb had been the site of a lymphedema since a traumatism 8 years ago. Histopathological analysis and immunostaining of a nodule showed that it was a large cell lymphoma of follicular stem cells. There was no extracutaneous involvement and the patient was successfully treated with radiotherapy. Two months after the completed radiotherapy a cutaneous relapse on the trunk and the upper limbs was treated with cyclophosphamide-vincristine-prednisone chemotherapy. DISCUSSION: Lymphedema probably played a role in the genesis of this lymphoma presumably by reducing the local immune response. It may have harmed endothelial cells and maintained an antigenic stimulation leading first to lymphocyte hyperplasia and eventually to a true lymphoma, in the same way this has been proved for some MALT lymphomas.

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PubMed1997

[Macrophage activation syndrome disclosing leukemic transformation of mycosis fungoides].

BACKGROUND: Mycosis fungoides can mimic pigmented purpuric dermatitis. We report such a case which progressed to peripheral T-cell lymphoma; progression was revealed by reactive hemophagocytic syndrome (RHS). CASE REPORT: A 65-year old male patient was hospitalized for a pigmented and purpuric eruption. The skin lesions appeared 2 years earlier and at that time biopsy had shown pigmented and purpuric dermatitis. One month before hospitalization, general signs appeared. On admission, he had papular and purpuric rash, mainly on the trunk, hepatosplenomegaly, enlarged axillar and inguinal lymph nodes, and fever at 38.2 degrees. A skin biopsy showed histologic changes typical of mycosis fungoides. He also had bicytopenia, hepatitis, and increased triglyceride and ferritin levels suggesting RHS which was proved by means of bone marrow biopsy. These tests also evidenced peripheral T-cell lymphoma. The patient was treated with two courses of chemotherapy (CHOP) but the disease progressed and he deceased. DISCUSSION: Mycosis fungoides can occasionally begin with an eruption very closely resembling pigmented purpuric dermatitis. Therefore, repeated biopsies should be done in case of widespread permanent pigmented purpuric dermatitis of no apparent origin. RHS is a life-threatening disease. The diagnosis should be suspected in any cytopenic patient with fever, increased triglyceride levels and abnormal liver tests. A search for an etiology must then be undertaken a prompt treatment is needed.

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PubMed1996

[Blaschkolinear eruption with biological signs of lupus].

INTRODUCTION: Acquired inflammatory dermatoses of the adult with a blaschkolinear arrangement are rare. Their cause is unknown. CASE REPORT: We report the case of a 35-year-old man who presented an unilateral skin eruption covering the forearm and with secondary blaschkolinear extension to the arm, the axillary region and the chest. In this particular case, the patient was positive for RNP type anti-nuclear antibodies and direct immunofluorescence was positive in both biopsies of the lesions and of healthy skin. The eruption lasted 6 months and regressed spontaneously. DISCUSSION: Such cases are exceptional and the nosology is difficult to establish. A particular form of lupus erythematosus? Fortuitous coexpression of lupus antibodies and blaschkitis in an adult?

Adult↗
PubMed1996

Examination of cutaneous macroglobulinosis by immunoelectron microscopy.

Cutaneous macroglobulinosis is a rare cutaneous manifestation of Waldenström's disease. Lesions are though to result from accumulation of macroglobulin in the dermis and are therefore called IgM storage papules. Ultrastructural findings in the previously reported cases were contradictory and the nature of the deposits was not established by electron microscopy. The purpose of this study was to analyse such deposits by the use of immunoelectron microscopy. A 60-year-old woman had multiple erythematous papules for 1 year. The histopathological changes consisted of plasmocytic infiltration of the dermis and eosinophilic deposits. The skin changes and other investigations led to a diagnosis of Waldenström's disease. Samples from normal and diseased skin were analysed by electron microscopy and by immunogold labelling with anti-IgM antibodies, after Lowicryl K4M embedding. An extracellular electron dense granular and filamentous material was observed in the mid- and upper dermis, between and within the collagen bundles. No periodicity was noted and no deposits were seen at the dermoepidermal junction. Immunoelectron microscopy showed a positive labelling located only on these deposits, in both normal-appearing skin and in lesions. In this patient, immunoelectron microscopy clearly demonstrated the presence of large amounts of IgM in the dermis, which were found in the lesions of cutaneous macroglobulinosis and in normal skin. These results suggest that the IgM storage papules result from a greater density of deposits rather than a site-specific accumulation.

Female↗
PubMed1993

[Squamous syringometaplasia: an original manifestation of pathomimesis].

INTRODUCTION: Epidermoid syringometaplasia consists in rare changes of the eccrine sweat glands, which show keratinization of their ductal part. Many conditions are associated with epidermoid syringometaplasia: chronic ulcers, scars, keratoacanthoma and various drugs or toxic agents, suggesting a toxic effect of drugs or an inflammatory mechanism. OBSERVATION: A 22-year-old woman presented with large painless ulcero-vegetating lesions of the forearm; the X-ray examination showed an major emphysema. She also had multiple scars on her trunk and limbs. The curious features of the lesions and the psychiatric troubles suggested the diagnosis of dermatitis artefacta. Histopathological examination showed a pseudocarcinomatous epidermal hyperplasia; in the deep dermis the eccrine ducts were connected to the epidermal cell masses or seemed to be included into the proliferation. Keratinization was noted within the keratinocytes surrounding the duct lumen. A dense inflammatory cell infiltrate was observed, containing many macrophages with a foamy cytoplasm, probably resulting from the injection of a foreign substance. All lesions healed within a few days using an occlusive dressing. COMMENTS: In this patient, the voluntary injection of an unknown substance probably induced these metaplastic changes of the sweat ducts, which are probably related to a toxic mechanism. Epidermoid syringometaplasia has never been described after cutaneous injections.

Adult↗
PubMed1993

[Cutis verticis gyrata: reduction surgical treatment].

Cutis verticis gyrata (CVG) is a rare cause of acquired scalp thickening; in its primary form, the disease does not show any bone involvement. A 19-year old man had noticed a moderate thickening of the scalp at the age of 15. He progressively developed ridges and furrows in the occipital and parietal areas: after a four-year period, there were ten sagittal and irregularly parallel folds. There was neither pain nor pruritus, and the hair had a normal appearance. Complete neurological examination was unremarkable and the patient was of normal intelligence. All biological investigations, including STH and TSH levels, were normal. X-ray examination and tomodensitometry did not show any bone involvement. Our patient was treated by surgical reduction of the scalp areas involved under general anesthesia, with a very good result. This is a typical case of primary CVG. Differential diagnosis of primary cutis verticis gyrata comprises pachydermoperiostosis, cerebriform intradermal nevus and many secondary conditions associated with hypertrophy and folding of the scalp. A relatively simple surgical treatment results in a major reduction of the scalp thickening, and avoids maceration.

Adult↗
PubMedDate not supplied

[Oral ulcers induced by nicorandil: prevalence and clinicopathological aspects].

INTRODUCTION: The first observations of "giant buccal aphthosis" induced by nicorandil were published in 1996. Nicorandil is a potassium channel activator used in the treatment of angina pectoris, which seems to induce specific buccal ulcerations. The purpose of this study was to analyze the clinicopathologic data of patients with aphthosis induced by nicorandil and to study the prevalence of this side effect. PATIENTS AND METHODS: We have seen 3 patients who spontaneously consulted, and 5 patients who were addressed to us after a telephone survey. We have then examined 100 consecutive patients treated by nicorandil for at least 1 month, who were hospitalized in 3 departments of cardiology in Strasbourg, and 100 age- and sex-matched controls who were treated by other antianginal drugs. RESULTS: Our 8 patients suffered from large, chronic and painful ulcerations of a 4-week duration, located on the tongue, the gingiva and the cheeks despite various symptomatic treatments. In one case, histopathologic data were consistent with an eosinophilic ulcer. Prospective study: among 100 patients treated by nicorandil, 5 had unusual chronic buccal ulcerations, whereas none of the 100 controls had aphthosis (p = 0.03). The confidence interval (99 p. 100) of this side effect prevalence was therefore 1 p. 100 to 14 p. 100. DISCUSSION: Nicorandil can induce large and painful buccal ulcerations with severe dysphagia, weight loss, and depression. Dermatologists should be aware of this particular side-effect, since our study showed a high prevalence, and because lesions heal rapidly after withdrawal of nicorandil. Why nicorandil may be associated with mouth ulcers remains unanswered. A past history of aphthae could be a cofactor of this side-effect.

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PubMedDate not supplied

[Painful edema of the feet revealing ankylosing spondylitis].

INTRODUCTION: Painful peripheral oligoarthritis can reveal ankylosing spondylitis. In some instances, an acral pitting edema can be the sign of this affection. CASE REPORT: A 39-year-old man, with no significant previous medical history, consulted in a dermatology department for acral pain and edema of both feet, which were exacerbated during the second part of the night. On examination, he had a pitting edema and a livedo of the distal part of the feet. Biological investigations revealed an inflammatory syndrome and the presence of the histocompatibility HLA-B27 antigen. Bone scintigraphy revealed distal hyperfixation on both feet. Diagnosis of ankylosing spondylitis was established. DISCUSSION: Late-onset ankylosing spondylitis, which appears in subjects older than 35 years, can manifest as peripheral arthritis with systemic signs, often in the absence of involvement of the axial skeleton. Peripheral pitting edema of lower limbs can be the presenting sign. Since cutaneous involvement can be the presenting sign, dermatologists should be aware of this entity.

Adult↗