Papular-purpuric gloves and socks syndrome with hepatitis B infection.
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Adult↗
Biomedical subjects
M Rybojad
Publications and source records attributed to M Rybojad.
Long-term follow-up of non-HIV Kaposi's sarcoma treated with low-dose recombinant interferon alfa-2b.
BACKGROUND AND DESIGN: We reviewed the follow-up of 16 patients with Kaposi's sarcoma not related to human immunodeficiency virus (13 with classic Kaposi's sarcoma and three with endemic Kaposi's sarcoma; median age, 58 years) treated by low-dose recombinant interferon alfa-2b (5 million U three times weekly for at least 6 months). RESULTS: One patient had a complete response, nine had a major response, three had stable disease, and one had a minor response. Visceral disease stabilized and symptoms improved in three patients. Limited relapse was noted in four patients after withdrawal of interferon. CONCLUSION: Our results confirm the efficacy and safety of low-dose recombinant interferon alfa-2b in the long-term treatment of both cutaneous and visceral lesions of Kaposi's sarcoma not related to human immunodeficiency virus.
Adolescent↗
Melanoderma revealing primary antiphospholipid syndrome.
Acute adrenal insufficiency is a rare complication of the primary antiphospholipid syndrome. We report a case of melanoderma of rapid onset revealing an addisonian crisis associated with primary antiphospholipid syndrome, which was associated with life-threatening hypercalcemia. Melanoderma does not belong to the clinical presentation of acute adrenal insufficiency. Its very rapid onset in our patient is probably due to her very high level of serum ACTH, in relation with her recent abortion.
Abortion, Spontaneous↗
[Blau syndrome or familial form of sarcoidosis with onset during infancy].
INTRODUCTION: Blau syndrome is a granulomatous disease with dominant autosomal transmission. Skin, joint and ocular manifestations usually appear in childhood. CASE REPORTS: A father and his son had granulomatous disease with skin and joint manifestations beginning in childhood. Both patients had inflammatory polyarticular deformations of the small and medium sized joints with formation of synovial cysts. Skin manifestations were seen only in the son who presented diffuse micropapulous eruptions. Histology examination of the superficial and deep derma revealed an epithelioid granuloma without necrosis. DISCUSSION: Our case are similar to the syndrome described by Blau who recognized the familial nature of early onset sarcoidosis and probable autosomal dominant transmission together with joint deformation and development of synovial cysts without pulmonary involvement.
Age Factors↗
[Bone marrow autograft in the treatment of cutaneous lymphoma].
INTRODUCTION: The prognosis of advanced stage or high grade cutaneous lymphomas is very poor in case of recurrence after conventional polychemotherapy. Recent studies have confirmed the importance of intensified treatment with autologous bone marrow transplantation in case of recurrence. We used this method in patients with a cutaneous lymphoma with poor prognosis. PATIENTS AND METHODS: Seven patients with a high-grade or disseminated cutaneous lymphoma were given an autologous bone marrow graft in case of recurrence after one or more polychemotherapy protocols. In 4 patients, treatment included total body irradiation and high-dose chemotherapy (cyclophosphamide/etoposide, or aracytine/melphalan) and in the 3 others chemotherapy alone (BEAM or BEAC) was used prior to transplantation. RESULTS: Two complete remissions of 46 and 34 months duration after graft were achieved without complementary treatment. One patient had partial remission. Recurrence was observed in 2 patients 5 months after the graft and in 1 other 30 months later. Prolonged complete remission was observed in patients given total body irradiation and the early recurrences in those given chemotherapy alone. DISCUSSION: This pilot study demonstrates that patients with a poor prognosis cutaneous lymphoma can achieve prolonged complete remission by therapy intensification using autologous bone marrow transplantation after total body irradiation.
Adolescent↗
[A case for diagnosis: Osler subacute endocarditis].
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Adult↗
Omenn's reticulosis associated with the nephrotic syndrome.
Omenn's reticulosis is an inherited severe combined immunodeficiency characterized by neonatal exfoliative erythroderma. A newborn baby who had minimal change nephrotic syndrome and Omenn's reticulosis is reported. Abnormalities in lymphocyte function could explain both the nephropathy and the cutaneous changes.
Dermatitis, Exfoliative↗
[Perianal papulonodular dermatitis in Hirschsprung disease].
INTRODUCTION: Macerations resulting from anal and/or urinary incontinence can lead to perineal or peristomal pseudo-verrucosis. The papulo-nodular perianal lesions observed in children after surgery for Hirschsprung syndrome is an example. CASE REPORT: A 28-month-old boy with rectosigmoid Hirschsprung disease developed chronic diarrhea after surgery with colo-anal anastomosis followed rapidly by perineal rash. The rash did not respond to local treatments and pseudo-condylomatous papulonodules developed. Infection, deficiency and tumoral causes were eliminated. Skin biopsy showed benign epidermal hyperplasia. The lesions regressed spontaneously within a few weeks after the diarrhea had been controlled and diapers were no longer used, confirming the diagnosis of perianal papulo-nodular dermatosis following surgical treatment for Hirschprung disease. DISCUSSION: The presentation of the perianal rash was similar to Sevestre and Jacquet dermatitis and infantile gluteal granuloma. The pathogenesis is similar to perianal or peristomal pseudoverrucosis seen in incontinent or stomy patients. Diagnosis is based on the clinical presentation, the periorificial localization and the pseudo-tumoral aspect of the lesions. Removal of the cause of maceration, when possible, is the basis of treatment.
Anus Diseases↗
[Aggressive cutaneous T-cell lymphoma associated with the presence of Epstein-Barr virus. 2 cases].
INTRODUCTION: The factors of prognosis of the cutaneous T-cell lymphomas are less well known as those of the B-cell lymphomas and the role of the Epstein-Barr virus (EBV) is not yet definitively evaluated. CASE REPORTS: Two male patients aged 62 and 82 years had a mycosis fungoides with a lethal outcome. The first patient had mutilating facial tumors; the RNA m of EBV and the genome of EBV were demonstrated in the diseased skin. The second patient had an erythrodermic course with enlarged peripheral lymph nodes and circulating Sézary's cells; the genome of EBV was demonstrated by PCR in the diseased skin. DISCUSSION: The role of the EBV has already been demonstrated in peripheral aggressive T-cell lymphomas. In the mycosis fungoides, the EBV is associated with the lesions in 0 to 32 p. cent according to the published series. EBV associated T-cell lymphomas have a poor survival rate and the EBV infection may be associated with the expression of the multidrug resistant gene-1 (MDR-1) and the risk of a terminal hemophagocytosis. In our both patients the presence of the EBV in the lymphocytes of the skin lesions is also an argument in favour of the pathogenic role of the virus.
Aged↗
[Scleroderma-like patch on the thigh in infants after vitamin K injection at birth: six observations].
INTRODUCTION: Texier's disease or pseudosclerodermatous reaction after intramuscular injection of vitamin K1 is well known in adults although only 1 report of a case in a newborn was found in the literature. We report 6 cases. CASE REPORTS: Six infants (4 boys, 2 girls) developed "peau d'orange" skin lesions after the age of 6 months which was localized in the lower third of the medial aspect of the thigh. Initial rapid locoregional extension was followed by stabilization and then regression. In all 6 cases, histology showed lesions of the fascia and/or the deep hypoderma associated with variable mononuclear inflammatory infiltration and hyalin fibrosis. When performed, immunological studies (complement fixation, search for autoantibodies) were always negative or normal. No visceral involvement was found. DISCUSSION: A pseudosclerodermatous lesion of the lower third of the thigh occurred in 6 infants at the site of an intramuscular injection of vitamin K1 administered at birth. The history, clinical manifestations, histology and outcome of these cases are compatible with the diagnosis of Texier's disease. We discuss the role of the solvent in the Roche vitamin K1 injection. The pathogenesis of this side effect remains unknown. CONCLUSION: Texier's disease in infants after injection of vitamin K1 at birth is a stereotypic dermatosis. Diagnosis is based on history and clinical presentation. The causal effect of injectable vitamin K1 should be entertained whenever pseudosclerodermatous lesions are observed in a young child.
Antifibrinolytic Agents↗
[Rosacea with ocular involvement in a child].
INTRODUCTION: Rosacea ia an uncommon facial eruption in children. We report a case of rosacea associated with a specific episcleritis. CASE REPORT: A 10-year-old girl consulted for an erythematous papular and pustular eruption of the mid face of 1 month duration. The child complained that she had had a red painful right eye for 6 months. There was no argument for acne, periorificial granulomatous dermititis or for sarcoidosis. The ophthalmological examination gave the diagnosis of ocular and cutaneous rosacea. Oral antibiotics followed by erythromycin gave favorable results for both skin and ocular lesions. DISCUSSION: Ocular involvement is a frequent complication of rosacea in adults. It is exceptional in children in whom rosacea is in itself rare. The presence of blepharitis, keratoconjunctivitis or episcleritis may suggest the diagnosis in a child with a mid facial eruption. As in the adult, skin and ocular rosacea responds well to prolonged oral antibiotics. The choice of the antibiotic depends on the age of the child. Cyclins are not authorized in children under the age of 8 years.
Anti-Bacterial Agents↗
Kaposi's sarcoma and new herpesvirus.
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Acquired Immunodeficiency Syndrome↗
Late, painful, subungual hyperkeratosis in incontinentia pigmenti.
The postpubertal appearance of subungual, painful keratotic tumors is a rare feature of incontinentia pigmenti. A patient affected by incontinentia pigmenti developed subungual, painful, nontumoral, hyperkeratotic lesions of the hands at 10 years of age. The mildness of the subungual lesions may be explained by the early stage of the disorder, but it is difficult to correlate the severity of the fingertip pain with the absence of true tumoral swelling. To our knowledge this is the youngest patient reported so far and the only one with a prepubertal expression of this puzzling disorder.
Abnormalities, Multiple↗
High-dose recombinant interleukin-2 in advanced cutaneous T-cell lymphoma.
BACKGROUND AND DESIGN: Treatment of cutaneous T-cell lymphoma is still a difficult challenge, once the usual therapies (topical chemotherapy, phototherapy, radiation therapy, and chemotherapy) have proved to be unsuccessful. New therapies, mostly immunotherapies, are currently under investigation. The use of recombinant interleukin-2 has already been evaluated in hematopoietic malignancies. We decided to treat patients with advanced cutaneous T-cell lymphoma relapsing or progressing in spite of the usual treatments with high-dose recombinant interleukin-2. Seven patients (three with mycosis fungoides, three with Sézary syndrome, and one with nonepidermotropic large-cell cutaneous lymphoma) were included in this open study. They were scheduled to receive recombinant interleukin-2 at a dose of 20 x 10(6) IU/m2 per day, administered by continuous infusion during three fortnightly induction cycles and five monthly consolidation cycles. RESULTS: Three complete responses (two responses to mycosis fungoides; one response to large-cell lymphoma) and two partial responses were obtained. The clinical response appeared after the first cycle of treatment in the good responders. The complete responses are still ongoing 33, 28, and 6 months after completion of recombinant interleukin-2 therapy and without any further treatment. Sequential immunophenotypic studies showed an increase of the CD1+ cells in the dermal infiltrates. No significant modification of natural killer or cytotoxic T cells could be seen. CONCLUSIONS: Despite our low number of cases, our results clearly show that some advanced cutaneous T-cell lymphomas can benefit from high-dose recombinant interleukin-2 therapy. Further studies are necessary to determine the exact place of recombinant interleukin-2 in the therapeutic arsenal of cutaneous T-cell lymphoma.
Adult↗
[Dermatoses of the nipple and the areola].
Dermatoses of the nipple and areola are rare. The commonest dermatosis is Paget's disease, which presents in the form of a well demarcated erythematous area, sometimes erosive, oozing or hyperkeratotic. Histological examination reveals an intraepidermal proliferation of large clear cells, either isolated or grouped in clumps, predominantly in the suprabasal layers. Immunohistochemistry shows that these cells express low molecular weight cytokeratins and the epithelial membrane antigen, fairly frequently carcinoembryonic antigen. In 96% of cases, Paget's disease is associated with underlying breast carcinoma, either in situ or invasive. Erosive adenomatosis presents in the form of an erosion of the nipple, which is sometimes increased in size. Histologically, it consists of a benign tumour which may ulcerate the epidermis, composed of tubes and papillae lined by a double layer of epithelial and myoepithelial cells. The syringomatous tumour is exceptional. In places, it forms rudimentary sweat ducts and is considered to have an intermediate malignancy; its resection must be complete. Other tumours may also be observed in this site: leiomyoma, leiomyosarcoma, benign cutaneous lymphocytoma, basal cell carcinoma, naevoid areolar hyperkeratosis. They are exceptional except areolar neurofibromas in case of neurofibromatosis. Infectious dermatoses (viral warts, molluscum contagiosum, scabies) are accompanied by lesions in other sites. They same applied to the majority of inflammatory dermatoses such as eczema or Fox-Fordyce disease. Supernumerary nipples are situated on a line extending from the anterior part of the axillary crease to the medial part of the inguinal crease.
Adenomatoid Tumor↗
[Intra-epidermal pustulosis in a child. Demonstration of a target antigen similar to foliaceus pemphigus antigen].
INTRODUCTION: Intraepidermal IgA pustulosis is a vesiculopustular dermatosis defined by pemphigus type intercellular deposit exclusively of IgA. It is a member of the pemphigus group and may be related to neurtrophilic dermatoses. CASE REPORT: A child had vesiculopustular lesions of the limbs since the age of 11 years. Biopsy showed the subcorneal intraepidermal nature of the pustules and exclusive IgA deposit throughout the epiderma. Indirect immunofluorescence and protein immunoelectrophoreses were negative. Immunotransfer to beef tongue extract evidenced a 150-160 kDa band corresponding to IgA and IgG desmoglein. Treatment with general corticosteroids followed by pristinamycin was successful. DISCUSSION: This is the first case report showing evidence of antiepidermal antibodies directed against the superficial pemphigus antigen.
Age Factors↗
[Mycosis fungoides in the child. Three cases].
INTRODUCTION: Cutaneous lymphoma is unusual in children but according to data in the literature, approximated 5 p. 100 of the cases observed would begin in childhood. CASE REPORT: We retrospectively studied 3 cases of mycosis fungoides where the first manifestations occurred before 10 years of age. In one of the patients, the diagnosis was not definitively confirmed until adulthood. DISCUSSION: Diagnosis in these forms which begin in childhood is usually achieved after a long delay. Clinically, these lymphomas form a homogeneous group. In approximately 15 p. 100, guttate parapsoriasis occurs before mycosis fungoides. Biopsy is indicated if the lesions change in aspect or become atypical. The most frequent presentation in children or young adults is vitiligoid hypo-pigmented macules. Histologically, childhood forms do not differ from the adult forms and also respond to local treatment as in adults. Prospective studies conducted conjointly by paediatricians and dermatologists would be needed to describe the natural history of these cutaneous lymphomas in light of progression to aggressive lymphoma of Hodgkin's disease described in certain cases.
Antigens, CD↗
Schnitzler's syndrome associated with sensorimotor neuropathy.
We describe a patient with chronic urticaria in association with monoclonal IgM gammopathy (Schnitzler's syndrome). Seven years after the onset of the cutaneous lesions sensorimotor neuropathy developed. Myelin-associated glycoprotein was detected in the patient's serum. High doses of corticosteroids improved the skin condition but failed to prevent the neuropathy. Six months of treatment with immunoglobulins was without benefit.
Aged↗