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Biomedical subjects

S Aractingi

Publications and source records attributed to S Aractingi.

At least 91 records · Page 5Linked to original sources

Immunohistochemical and virological study of skin in the papular-purpuric gloves and socks syndrome.

The pathogenesis of viral exanthems remains unclear. We have undertaken an immunohistochemical study of lesional skin biopsies in patients with the papular-purpuric gloves and socks syndrome (PPGSS) secondary to parvovirus B19 infection. Intracytoplasmic staining of the dermal endothelial cells, keratinocytes, and sweat glands was shown with an antibody to parvovirus B19. There were perivascular dermal infiltrates with T cells, sometimes with exocytosis. By polymerase chain reaction, virus DNA was detected in all skin biopsies and in one serum sample. The cutaneous manifestations of parvovirus infection seem secondary to infection of the endothelium and epidermis.

Adolescent↗

Raynaud's phenomenon and digital necrosis induced by interferon-alpha.

We report two patients treated with interferon-alpha who developed Raynaud's phenomenon followed by multiple digital necrosis. Arteriography of the legs revealed diffuse distal narrowing. Histological examination of the resected tissue showed ischaemic necrosis within the dermis and subcutaneous tissue without vasculitis. Resolution of the symptoms was observed when interferon-alpha was withdrawn. Since other causes of Raynaud's phenomenon and digital necrosis were ruled out, a drug cause is likely. Raynaud's phenomenon should be looked for in patients treated with interferon-alpha. If present, immediate withdrawal of the drug is required.

Antineoplastic Agents↗

[Pyogenic granuloma of the tongue in chronic graft versus host disease].

INTRODUCTION: The development of pyogenic granuloma of the tongue is rare. We report such a case in a patient with chronic graft-versus-host disease. CASE REPORT: A 5-year-old girl was treated with allogenic bone marrow transplantation for aplastic anemia. Complications consisted in chronic graft-versus-host disease with cutaneous, mucosal and hepatic involvement. Seven months later, a voluminous pyogenic granuloma of the tongue developed on previous erosive lichenoid lesions of chronic graft-versus-host disease while the patient was under cyclosporin. Surgical resection was done. Relapse occurred and treatment was identical. DISCUSSION: Six cases of pyogenic granuloma of the oral cavity in chronic graft-versus-host disease have been reported in the literature. Pyogenic granuloma must be considered in tumoral lesion of the oral cavity of patients with chronic graft-versus-host disease. All patients had erosive lichenoid lesions and were under cyclosporin; its role in the genesis of these lesions is debated. Treatment relies on surgical resection confirming the diagnosis.

Bone Marrow Transplantation↗

[Cutaneous graft versus host disease].

Graft versus host disease is frequently encountered in patients with an allogenic bone marrow transplantation. The disease apparently results from the activity of the donor T lymphocytes which react against the recipient's cells. There are two categories of graft versus host disease: the acute form which occurs within 3 months of the graft and the chronic form which occurs thereafter. Skin is the predominant site of manifestations, although the liver, the gut and the eye may be involved. In acute graft versus host disease, there is a characteristic maculo-papular rash raising a difficult differential diagnosis which pathology examination of biopsy cannot always resolve. The chronic disease is easier to recognize on the basis of local or generalized lichenoid or sclerodermal skin lesions. Several risk factors have been identified including the degree of donor-recipient HLA mismatch, recipient age and the number of T cells grafted. Graft versus host disease leads to immune deficiency with lymphoid depletion and increased risk of infection. Treatment depends on the site of organ involvement and extension. Corticosteroids and immunosuppressors are used.

Acute Disease↗

Unusual specific cutaneous lesions in myelodysplastic syndromes.

BACKGROUND: Early diagnosis of leukemia cutis in myelodysplastic syndrome (MDS) is important because these lesions can precede acute peripheral blood or bone marrow transformation. Leukemia cutis is usually easy to recognize, but atypical lesions are not well described. OBJECTIVE: Our purpose was to describe unusual specific lesions in MDS. METHODS: Data from patients with myeloid malignancies and leukemia cutis were reviewed. Only patients with MDS and cutaneous lesions different from typical tumors were included. Clinical features were analyzed. RESULTS: Four patients with MDS and unusual specific cutaneous lesions were found. They had ecchymoses, necrotic plaques or ulcers, and prurigo-like lesions. In three of four patients appearance of these skin lesions heralded or was concomitant with acute transformation. CONCLUSION: Specific cutaneous lesions can display unusual patterns. Early biopsy of cutaneous lesions in MDS is indicated.

Aged↗

Hyperkeratotic spicules and monoclonal gammopathy.

Hyperkeratotic spicules are a rare disorder that has been associated with different types of malignancies. An association with multiple myeloma raises the question of a relation between the monoclonal component and skin manifestations. We describe hyperkeratotic spicules in a 61-year-old man with a monoclonal gammopathy of undetermined significance. Immunofluorescence and immunoelectron microscopic findings demonstrated the deposition of the monoclonal immunoglobulin at the dermoepidermal junction. Our findings support previous reports that the association between hyperkeratotic spicules and monoclonal gammopathy is not fortuitous and may be related to particular properties of the monoclonal immunoglobulin.

Humans↗

Hydroxyurea-induced dermatomyositis-like eruption.

Hydroxyurea is frequently used to treat myeloproliferative syndromes. Cutaneous lesions resembling those seen in dermatomyositis have rarely been reported in the course of treatment with hydroxyurea. We report six additional patients with this unusual adverse effect. All of the patients had a very typical and similar cutaneous eruption, with scaly, linear erythema on the dorsa of the hands. Leg ulceration occurred in two cases. Muscle involvement was never observed. One patient had unexplained lung disease. In all the others the disorder pursued a benign course, even when hydroxyurea was not withdrawn. Dermatomyositis-like lesions seem to be a not infrequent and characteristic adverse reaction to hydroxyurea. Investigations are not required, and the course is usually benign.

Aged↗

Neutrophilic dermatoses during granulocytopenia.

BACKGROUND AND DESIGN: Noninfectious cutaneous neutrophilic lesions can occur during granulocytopenia, but their mechanism remains unknown. We undertook a retrospective study of the neutrophilic dermatoses that developed during granulocytopenia induced by chemotherapy for acute myelogenous leukemia. RESULTS: Seven men and one woman were included (2.6% of treated cases of acute myelogenous leukemia); half had acute myelogenous leukemia subtypes 4 and 5. The male-to-female ratio was 7:1. Neutrophilic eccrine hidradenitis was diagnosed in five cases, Sweet's syndrome in two cases, and difficult-to-classify neutrophilic dermatoses in one case. Cutaneous lesions appeared 12.5 days after the start of chemotherapy, and the mean leukocyte count was 0.426 x 10(9)/L. Three patients needed corticosteroids systemically. CONCLUSION: Neutrophilic dermatoses during chemotherapy-induced granulocytopenia seem to occur more frequently in men with acute myelogenous leukemia subtypes 4 and 5.

Adult↗

Fasciitis in chronic graft-versus-host disease. A clinicopathologic study of 14 cases.

OBJECTIVE: To describe the clinicopathologic features of fasciitis in patients with chronic graft-versus-host disease (GVHD) after allogeneic bone marrow transplantation from human leukocyte antigen (HLA)-identical donors. DESIGN: A retrospective cohort study. SETTING: Tertiary care center. PATIENTS: Patients who had allogeneic bone marrow transplantation and developed chronic GVHD with clinical and pathologic signs of fasciitis. MAIN OUTCOME MEASURE: Analysis of clinical presentations and of deep cutaneo-muscular biopsy specimens. RESULTS: Between January 1974 and January 1991, 14 of 475 patients who had allogeneic bone marrow transplantation developed chronic GVHD that began with the sicca syndrome and liver or digestive tract involvement, or both, 60 to 170 days after the graft was received. Sudden and painful skin swelling was reported 350 to 3745 days after the graft was received. Follow-up over 2 to 7 years showed failure of the fasciitis to respond to steroid therapy or to any conventional treatment of chronic GVHD. Although 7 patients showed moderate improvement, the others remained functionally disabled because of skin tightness, joint stiffness, contractures, and sores. Patients with fasciitis in chronic GVHD had no specific immunogenetic profile and no history of L-tryptophan intake or phytonadione injections. CONCLUSION: Among alloimmune syndromes, fasciitis is a distinct entity that leads to functional disability. This rare form of chronic GVHD may provide clues to understanding the mechanisms involved in fasciitis from other causes.

Adolescent↗

Simultaneous occurrence of two rare cutaneous markers of poor prognosis in myelodysplastic syndrome: erythema elevatum diutinum and specific lesions.

We report the concomitant occurrence of erythema elevatum diutinum and specific skin lesions in a patient with a myelodysplastic syndrome (MDS). This patient's course, and review of other reported cases, support the opinion that neutrophilic dermatoses are associated with a poor prognosis of MDS. The simultaneous appearance of these manifestations could be the consequence of a particular chemotactism of myeloid cells, expressed after acute transformation.

Aged↗

[Abnormal fibrinolysis in Degos disease. A study of 3 cases].

INTRODUCTION: Degos' disease is a rare dermatosis characterized by papular lesions with a porcelain-white central atrophy and histopathological aspect of wedge-shaped infarction necrosis and an endovasculitis in the dermis. Its pathogenesis is unknown but many abnormalities of haemostasis have been reported. PATIENTS AND METHODS: Platelets functions, coagulation and fibrinolysis were estimated in three patients with Degos' disease. For one patient, direct immunoelectron microscopy using an antibody to von Willebrand factor was performed on lesional skin. RESULTS: In all the patients, prolonged euglobulin lysis time, increased plasminogen activator (PA) and plasminogen activator inhibitor (PAI) activities before and after a venous occlusion test were detected and indicated an inhibition of fibrinolysis. Electron microscopy demonstrated in one case an increased number of Weibel-Palade bodies and a raised staining of von Willebrand factor in endothelial cells. Tests for coagulation and circulating anticoagulant were normal. Results of platelets adhesion showed decrease of adhesion in one case and increased adhesion in another. Platelets aggregation studies were normal in two cases and showed hyperactive spontaneous and induced aggregation in one case. CONCLUSION: We showed an inhibition of fibrinolysis in three patients with Degos' disease. These abnormalities could induce a prethrombotic state. The release of PA and PAI from the endothelial cells into the blood stream and the modifications observed with electron microscopy may signify a primary lesion of endothelial cell of still unknown origin.

Atrophy↗

Umbilical Paget's disease and prostatic carcinoma.

We report a case of umbilical Paget's disease occurring in a patient with a prostatic carcinoma. No other malignancy was found, and the patient was treated by surgical excision of the lesion. To our knowledge, the occurrence of extramammary Paget's disease of the umbilicus has not been reported previously. The clinical presentation, its association with prostatic carcinoma, and the possible pathogenesis are discussed.

Aged↗

The 'euthyroid sick syndrome': incidence, risk factors and prognostic value soon after allogeneic bone marrow transplantation.

We studied the incidence of thyroid function abnormalities observed soon after allogeneic bone marrow transplantations (BMT) and their predictive value on the overall prognosis. Free serum thyroxine, free serum triiodothyronine, total serum reverse triiodothyronine and serum thyrotropin levels were systematically measured in 78 patients before and 3 months after BMT. 41 (52%) had normal hormone levels and 37 (48%) had abnormal ones, among whom four (5%) had peripheral compensated hypothyroidism and 33 (43%) were described as having 'euthyroid sick syndrome' (low thyroxine state, or low T3 syndrome). Two factors strongly influenced the appearance of thyroid abnormalities: steroid dose at the time of thyroid function testing, and age (< or = 16 years/ > 16 years). Among the younger patients, 21 had no thyroid abnormalities, while five did. Among the older patients, 20 had no thyroid abnormalities, while 32 did (P < 0.001). The occurrence of thyroid abnormalities seemed to influence survival strongly, since the 30-month projected survival time was 83% for patients without abnormalities whereas it was 49% for patients with an abnormal profile (P < 0.001). In conclusion, evidence obtained among our population reveals that euthyroid sick syndrome indicates a poor prognosis and that it is very important to monitor thyroid hormone levels (particularly free hormones) soon after allogeneic BMT and regularly thereafter.

Adolescent↗

Sarcoidosis associated with leucocytoclastic vasculitis. A case report and review of the literature.

We report on a patient presenting with cutaneous leucocytoclastic vasculitis in association with sarcoidosis. Detailed investigations did not show evidence of infectious or neoplastic diseases. Vasculitis was therefore considered to be secondary to sarcoidosis. A review of 4 other previously reported cases reveals a common presentation with the association of vasculitic skin lesions with deep cutaneous nodules, mediastinal lymph nodes and arthritis. The role of antibody response in leucocytoclastic vasculitis is discussed.

Female↗

Fusarium solani cutaneous infection in a neutropenic patient.

A 47-year-old woman with acute myeloblastic leukemia developed disseminated papules and pustules after chemotherapy. A skin biopsy specimen revealed fungal vasculitis and grew Fusarium solani. The patient died despite treatment with amphotericin B. The isolate was resistant to amphotericin B, imidazole and 5-fluocytosine. Fusarium should be recognized as a potential cause of deep fungal infection in immunocompromised patients.

Dermatomycoses↗

Persistent acantholytic dermatosis: sex-related differences in clinical presentation?

We report the case of a 58-year-old man with a chronic papular eruption of 10 years' duration. Histopathology revealed focal acantholytic dyskeratosis. This condition is thought to represent a distinct entity which has been reported under several names and frequently referred to as persistent acantholytic dermatosis. The relationship between this condition and transient acantholytic dermatosis (Grover's disease), emphasizing sex-related presentations, is discussed.

Acantholysis↗