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Biomedical subjects

M C Koeppel

Publications and source records attributed to M C Koeppel.

At least 19 recordsLinked to original sources
PubMed1999

[KID syndrome (keratitis, ichthyosis and deafness)].

Keratitis, ichthyosis and deafness are the dominant signs of KID syndrome. The lesions involving cornea, epidermis and internal ear are probably the result of a congenital ectodermal abnormality. Associated signs such as increased sensitivity to infections, and dermoskeleton dystrophies are also useful for the diagnosis. There are no specific biological signs. Most cases are sporadic but familial cases have been described with unclear mode of inheritance. Treatment is disappointing. Thus management mainly relies upon early detection of complications.

Diagnosis, Differential↗
PubMed1998

Radiosurgery without whole brain radiotherapy in melanoma brain metastases. Club de Cancérologie Cutanée.

To evaluate the effectiveness of radiosurgery without whole brain radiotherapy in the palliative treatment of melanoma brain metastases, we retrospectively assessed the results in 35 patients: 4 with a solitary brain metastasis, 13 with a single brain metastasis and metastases elsewhere and 18 with multiple brain metastases. The local control rate was 98.2% (55/56 metastases) at 3 months. Median survival was 22 months in patients with a solitary brain metastasis, 7.5 months in patients with a single brain metastasis and metastases elsewhere, and 4 months in patients with multiple brain metastases. Complications were unusual and surgery was required in 2 of 35 patients. These results show for the first time that melanoma patients with a unique brain metastasis with or without metastases elsewhere clearly benefit from tumour control easily obtained by radiosurgery. Although the comparison of radiosurgery with surgery and/or whole brain radiotherapy cannot be adequately addressed, radiosurgery alone seems to provide similar results with lower morbidity and impact on quality of life.

Adult↗
PubMed1998

Cutaneous and gastrointestinal mastocytosis associated with cerebral toxoplasmosis.

A patient with systemic cutaneous and gastrointestinal mastocytosis and associated meningoencephalitic toxoplasmosis is reported. The simultaneous occurrence of the two conditions and their regression after treatment of the toxoplasmosis suggest a possible relationship between the mast cell proliferation and the parasitic infection. Mast cell activation and stimulation may be transitory in response to a stimulus, causing reactional mastocytosis.

Adult↗
PubMed1997

[Vulvar dermatoses].

Vulvar dermatoses are the localizations to the vulva of a great variety of dermatoses. In genodermatoses, the expression of diseases on the vulva is a part of a complex syndrome. It is non indispensable for the diagnosis. In the immuno-allergic dermatoses or in dermatoses where the pathogenesis is not clearly understood, the vulvar disease is very important because the vulva is often the exclusive localization. The vulvar disease can be the first manifestation before the other expressions of the mucocutaneous disease. Sometimes, there is a risk of scars or transformation in epithelioma. So the diagnosis must be soon established. At least, hidradenitis suppurativa is disease where the choice of the must appropriate treatment is difficult.

Female↗
PubMed1997

[Edema caused by retroperitoneal and tricuspid fibrosis with sclerodermatous cutaneous involvement disclosing carcinoid tumor. Apropos of a case and review of the literature].

A 65-year-old female was admitted with leg edema by retroperitoneal fibrosis and tricuspid valve incompetence by fibrosis, cutaneous fibrosis, moderate flushing over the upper body without diarrhea. It revealed an ileal carcinoid tumor with hepatic metastases. Octreotide (Sandostatine), tumor excision and interferon alpha 2b treatment led to a regression of flushing and edema, a reduction of fibrosis and a stabilization of the metastatic carcinoid, with normal serotonin levels.

Aged↗
PubMed1996

[Urticaria caused by cold. 104 cases].

INTRODUCTION: Superficial and deep cold urticaria is a heterogeneous group of manifestations induced by exposure to cold (0 to 4 degrees C). Frequency is generally estimated at 2 to 3 p. 100. We studied retrospectively 104 patients meeting clinical, biological and therapeutic criteria of cold urticaria. PATIENTS AND METHODS: From 1981 to 1995, 1802 patients with urticaria were included in a standardized allergy survey. Among these patients, 104 met the criteria for the diagnosis of cold urticaria: positive test with 3 ice cubes placed on the skin for 5, 10 and 15 minutes or positive immersion test (immersion in 4 degrees C water for 15 minutes). Two groups of patients were excluded, those with systemic cold urticaria and those with minimal thermo-differential urticaria. RESULTS: Cold urticaria was found in 5.7 p. 100 of our urticaria patients, predominantly in young women. The triggering effect of cold was found at history taking in 71 p. 100 of the cases. The urticaria was usually superficial, more rarely associated with deep and/or mucosal urticaria. General signs were uncommon. The three ice cube test demonstrated significative biological and immunological abnormalities. In 5 cases, discrete cryoprecipitate was found (4 cryoglobulins, 1 cryofibrinogen). Although the search for an infectious agent was not conducted in absolutely all cases, there was no apparently remarkable association with infection the exception of VIH infection. Anti-H1 agents were given in 88.3 p. 100 of the cases leading to short-term improvement. Follow-up is insufficient to evaluate long-term outcome. DISCUSSION: This retrospective survey of 104 cases of cold urticaria was compared with data in the literature, particularly with the small number of studies including a large number of patients. We conclude that the diagnosis of cold urticaria can be based on history taking alone and the three ice cube tests (prognosis value, indication of reaction threshold). An exhaustive search for the cause is not indicated. A search for cryopathy should however always be done and followed by a complete work-up in case of positivity. Clinical signs other than cold urticaria suggesting a pathological response to cold require complete investigations.

Adolescent↗
PubMed1995

[Cutaneovisceral leishmaniasis following severe nervous depression].

Leishmania is considered an opportunistic agent during cellular immunodeficiency. The authors report the case of a patient living in the south of France presenting visceral leishmaniasis with cutaneous manifestations in the course of a severe nervous depression. This case report illustrates the possible relationships between stress and immunity.

Adult↗
PubMed1994

Electron-beam therapy in scleredema adultorum with associated monoclonal hypergammaglobulinaemia.

We report a case of scleredema adultorum (Buschke's disease) associated with an IgA kappa monoclonal hypergammaglobulinaemia. A significant improvement in the skin was obtained with electron-beam therapy. Scleredema would appear to be linked to monoclonal hypergammaglobulinaemia, but the relationship between the skin disorder and the immunoglobulin abnormality remains to be elucidated.

Adolescent↗
PubMed1994

Lymphomatoid papulosis and Hodgkin's disease: report of a case.

The authors report the case of a 67-year-old-man who presented with stage IIIAa Hodgkin's disease (HD) almost 17 years after developing CD30+ type A lymphomatoid papulosis (LP). Combination chemotherapy resulted in a complete remission of the HD for 2 years, although the LP continued relentlessly as before. A possible link between HD and LP is discussed in the light of similar cases reported in the literature.

Aged↗
PubMed1994

Secondary pancreatic involvement of mycosis fungoides detected by a clinically palpable mass.

A patient with a 4-year history of mycosis fungoides, who presented a pancreatic spreading of the disease, detected by a clinically palpable mass, is reported and discussed with a review of the literature. Mycosis fungoides (MF) is a T cell lymphoma primarily localized to the skin. Laparoscopic staging and autopsy studies, however, have shown that lymph node involvement or visceral spreading is common in the course of the disease [1-3]. Among visceral manifestations of MF, pancreas infiltration is found in 20-40% of autopsied cases [1, 2, 4]. Occurrence of a palpable pancreatic mass has never been described. We report a patient with a long-standing MF, and a secondary pancreatic extension detected by a palpable mass.

Aged↗
PubMed1994

[Acquired zinc deficiency in a breast-fed premature infant].

Acquired zinc deficiency in exclusively breast-fed premature babies is a recently described entity which surprisingly, has not been reported more frequently. Its pathogenesis would appear to be the result of various factors, prematurity, exclusive breast-feeding and a suspected maternal defect, acquired or inherited, for the transfer of zinc from the blood to the breast milk. We report the case of a girl who was born at 31 weeks, exclusively breast-fed for 28 weeks and who presented a characteristic clinical feature of zinc deficiency. The mother's zinc level in the breast-milk was abnormally low. After zinc therapy and progressive weaning, the lesions dramatically improved in few days. There was no recurrence of the lesions one year after the treatment was completely stopped. With regard to this characteristic case, we outline the many pathophysiological mechanisms involved in acquired zinc deficiency. Recognition of this clinical feature by dermatologists seems essential because the required treatment is spectacularly effective and definitive. Finally, we also examine possible forms with few symptoms and we wonder if serum zinc levels should be checked in at-risk babies.

Acrodermatitis↗
PubMed1993

Paroxysmal pruritus and multiple sclerosis.

We report a patient who presented with paroxysmal pruritus in association with multiple sclerosis. Magnetic resonance imaging of the cervical spinal cord showed a lesion at a level corresponding to the dermatomal location of the pruritus.

Acute Disease↗
PubMed1993

Electron-beam therapy in Arndt-Gottron's scleromyxoedema.

We report a patient with Arndt-Gottron's scleromyxoedema whose skin lesions responded to electron-beam therapy. This treatment appears to be effective in improving the cutaneous signs and functional impairment of this disease.

Combined Modality Therapy↗