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Biomedical subjects

R Degos

Publications and source records attributed to R Degos.

At least 19 recordsLinked to original sources
PubMed1981

[Silvery hair in children: a symptom of leucogranulocytic and melanocytic diseases (author's transl)].

Nine children with silvery hair have been dermatologically studied: eight out nine presented a context of abnormal sensitivity to infections. The study of pigmentation, granulocytes functions and immunity system allows a separation in several types: 1) 4 patients had a characteristic Chediak-Higashi syndrome, this diagnosis being based on pigmentary and immunohematological grounds. 2) Two siblings exhibited functional disturbance of the Chediak-Higashi type, but without its cytological abnormalities. A third patient resembled closely to this picture, but was insufficiently investigated. 3) The eighth patient has been studied by Drs. Griscelli and Pruniéras who had previously described a new disease on immunological, hematological and pigmentary grounds. All these case with defective polymorphonuclear functions have febrile peaks with accelerated aggravation close to Chediak-Higashi syndrome. 4) The ninth patient with silvery hair does not have any immunological abnormalities, nor any accelerated phase at the age of eight but suffers from diffuse epilepsy, cerebellar and chorioretinal atrophy.

Chediak-Higashi Syndrome↗
PubMed1979

Malignant atrophic papulosis.

The lethal intestino-cutaneous syndrome which we described in 1942 as malignant atrophic papulosis (MAP) has gained various other visceral sites. However, the cutaneous eruption remains the constant and pathognomonic symptom, which, despite its benign appearance harbours a serious prognosis because of the frequently very severe lesions in the small intestine, and sometimes of the nervous system. The very special histological structure shows zones of necrosis (dermal in the skin) due to vasculitis with a tendency to thrombosis, affecting the small vessels below the lesion, and with little or no inflammatory reaction, which differentiates it from other angiitis. The aetiology remains uncertain (?viral) and the treatment is disappointing although heparin appears to have been helpful occasionally.

Adolescent↗
PubMed1978

[Idiopathic eruptive macular pigmentation (author's transl)].

The feeling of the authors is that their seven reported cases of a pigmented dermatosis are different from the ashy dermatosis and from the erythema dyschromicum perstans. This disease, which affects children and teenagers, males as well as females, is characterized by pigmented macules 5-25 mm in diameter, affecting the neck, the trunk and the limbs. The first symptom is whether a pigmented spot, or an erythematous, papular or achromic lesion; in the latter instance the pigmentation occurs only secundarily. In most of the cases this dermatose is slowly and spontaneously regressive. The histological picture is not really specific. In one case there was a marked intraepidermal dyskeratosis of the sweat duct openings. The etiology remains unknown.

Adolescent↗
PubMed1977

[The difference forms of "parapsoriasis en plaques". A report of 90 cases (author's transl)].

The term parapsoriasis was used by Brocq (1902) to group a number of conditions previously described under different names. This group has since then been modified, the same conditions being described under separate names and these have led to a great confusion especially between countries. In this study of 90 cases, three types of parapsoriasis en plaques are distinguished. The "parapsoriasis digitiforme" (40 cases) or benign type, or xanthoerythrodermia perstans, or chronic superficial dermatitis is characterized by small, oval or finger-like, yellow or pink patches. The histology is frequently not characteristic, but in a few cases, there is an exocytosis localized "en flammèches" in the epidermis. The condition is usually permanent but none of these cases has progressed to mycosis fongoides. The parapsoriasis "en grandes plaques simples" (25 cases) is characterized by few pink patches (3 to 5), larger than in benign type. In our cases the transformation to poikiloderma atrophicans vasculare is not observed. One of these cases progressed to mycosis fongoides. The "parapsoriasis en grandes plaques poïkilodermiques" (25 cas), or poikiloderma atrophicans vasculare, prereticulotic poikiloderma, atrophic parapsoriasis, parapsoriasis lichenoides, is characterized by large patches, in limited number, showing a reticulated pigmentation and slight atrophy with telangiectasia. Five cases changed into mycosis fongoides and 4 cases showed some symptoms of malignancy; The histologic features are the same in the two last types: sometimes they are non-specific, in numerous cases the picture is characteristic with micro-abscesses or "flammèches"; in some cases there is a dense infiltrate with a clearly defined lower limit: this histologic appearance can be seen in cases without transformation into mycosis fongoides.

Adult↗
PubMed1976

Churg and Strauss granulomatous angeitis.

The authors report a new case of Churg and Strauss allergic angeitis, observed during the patients lifetime. Severe asthma preceded the cutaneous lesions by 16 months. There was an eosinophilia of 66 p. 100 of 21,000 white cells. The histological picture was characteristic. A general review of the literature is made in the light of this case.

Adult↗
PubMed1976

[Mucocutaneous Bowen's disease. 243 clinical cases].

The authors studied 243 cases of patients with 269 muco-cutaneous localisations of Bowen's disease. After considering the aetiological factors, the various clinical signs and the pathological associations, they draw histological conclusions. Poikilocarynosis seems to be the most reliable histological sign. They conclude their study with the prognosis and treatment.

Adult↗