Scleredema adultorum of Buschke: an under recognized skin complication of diabetes.

Scleredema of Buschke or scleredema diabetorum is a skin complication of diabetes with deposits of collagen and aminoglycans in the dermis. This disease characterized by thickening and hardening of the skin, is usually localized in nape, back and shoulder areas. Consequences could be a decrease in motility of the shoulders and an impairment of respiratory function. Other possible complications are sleep apnoea syndrome and monoclonal gammapathy. Type 1 or type 2 diabetes may be associated with scleredema of Buschke in more than 50% of cases. Diabetes-related risk factors are long duration of the disease, presence of microangiopathy, overweight and need of insulin. Various specific treatments proposed in the literature are poorly validated. In most severe cases, radiation therapy may be useful.

[Death during migraine crisis]. / Décès au cours d'une crise de migraine.

A 42 year old man presented with a rapidly progressive syndrome, including headache, aphasia, fever, stiff neck and impaired consciousness. Following a transient improvement, the patient died after a six weeks course. The autopsy showed foci of ischaemic necrosis in the white matter of the brain. The patient's clinical history suggests the diagnosis of migraine.

[Intestinal spirochetosis in homosexuals infected with HIV. 3 cases]. / Spirochétose intestinale chez les homosexuels infectés par le virus de l'immunodéficience humaine. Trois observations.

We report 3 cases of intestinal spirochetosis in homosexuals infected with the human immunodeficiency virus (2 group III and 1 group IV C1, according to the Centers for Disease Control classification) presenting with moderate, chronic diarrhea. The diagnosis was made based on the histological examination of colorectal biopsies showing a layer of spirochetes carpeting the epithelium. Electron microscopy evaluation and culturing of the microorganism provided information on the bacterium's morphology. Metronidazole effectively treated the diarrhea. Intestinal spirochetes, whose existence has been recognized for more than a century, constitute a heterogeneous group of bacteria whose pathogenic role in man remains controversial. The systemic search for these organisms in a large series of patients would help to situate their place among the various etiologies of infectious diarrhea in immunodepressed subjects.

[Waldenström's macroglobulinemia with cerebral lymphoproliferative involvement of tumor appearance (Bing and Neel syndrome). An anatomo-clinical case]. / Macroglobulinémie de Waldenström avec atteinte lymphoproliférative cérébrale d'allure tumorale (syndrome de Bing et Neel). Un cas anatomo-clinique.

The authors report the case of a 58 year old woman with a 5 year history of Waldenström's disease, who presented with perivascular confluent and multifocal lymphoproliferative cerebral involvement (Bing and Neel syndrome). The lymphoproliferative process was limited to the brain matter and respected the meninges, posing the problem as to its origin. It resulted in a tumoral syndrome with intracranial hypertension leading to coma and death after 6 months. This case is reviewed in the light of two similar cases in the medical literature.

Eosinophilic pustular folliculitis (Ofuji's disease): efficacy of isotretinoin.

The case of a 30-year-old man with a 6-year history of eosinophilic pustular folliculitis (EPF) is reported. Isotretinoin (1 mg/kg/day) led to a dramatic improvement of all the lesions within 2 weeks. The withdrawal of the drug was followed by a recurrence after 10 days of the papulopustular, follicular and pruritic lesions. Reintroduction of isotretinoin was successful. The benefits of isotretinoin in the treatment of EPF have, to the best of our knowledge, never been reported previously. The mechanisms underlying this efficacy may involve the inhibition of the eosinophilic chemotactic factors thought to be present in sebaceous lipids and in the stratum corneum of patients suffering from EPF.

Glomerulonephritis in dogs with canine leishmaniasis.

Kidney specimens, obtained from 11 dogs of diverse species that had contracted spontaneous leishmaniasis, were submitted to a histopathological investigation using a classical preparation of sections embedded in paraffin, semi-thin and ultra-thin sections embedded in plastic, and sections frozen under immunofluorescence. Eight animals presented moderate to severe renal insufficiency. Two major categories of glomerular lesions were identified in the kidneys examined: acute glomerulonephritis and extra-membranous glomerulonephritis. These two categories were never associated and no transition from from one to the other was found. Immune complex deposits prevailed sharply over proliferative phenomena, which remained infrequent. Study of these glomerulonephrities could represent a model for better understanding of the immunological aspects of leishmaniasis.

Multiple clustered dermatofibromas. Evolution over 20 years.

A 37 year-old man developed, over 20 years, multiple clustered dermatofibromas on the left thigh. Three similar cases have been described previously and the benign course of this lesion has been referred to. Analysis of collagen amino acids revealed a sharp increase of hydroxylysine on the fibrous central zone, suggesting an abnormal accumulation of type IV collagen.

[Cutaneous signs of Noonan's syndrome. Apropos of a case with ulerythema ophyogenes, disseminated pilar and sudoral keratosis and progressive alopecia]. / Les signes cutanés du syndrome de Noonan. A propos d'une observation avec ulérythème ophryogène, kératose pilaire et sudorale disséminée et alopécie progressive.

A case of typical Noonan syndrome (NS) with eye abnormalities, pterygium colli, cryptorchid testes, lymphoedema and asymmetrical cardiac septal hypertrophy is reported in a 8-month old infant. This case was particularly interesting since it included skin manifestations which enabled an early diagnosis to be made. Ulerythema ophryogenes has already been proposed as a cutaneous marker of NS, but the keratinization disorders in our patient also included disseminated keratosis of both hair follicles and sweat glands orifices. Abnormally brittle short curly hair has already been reported, but our patient exhibited progressive alopecia which is very rare in NS. Biochemical hair analysis did not show any abnormalities of aminoacids. All these features were suggestive of keratosis follicularis spinulosa decalvans. It therefore seems very likely that NS is associated with keratinization disorders but ulerythema ophryogenes might only be the limited form of these disorders. The other skin manifestations of NS are reviewed. Since the patient had 4 "café au lait" spots, the relation of NS with Von Recklinghausen syndrome, and neurofibromatosis-Noonan syndrome is discussed. Watson's Leopard and cardio-facial syndromes overlap with, and may represent subsets of NS.

Immune complex vasculitis associated with mediterranean spotted fever.

We report a case of mediterranean spotted fever complicated by leucocytoclastic vasculitis. Rickettsia conorii, IgA, complement and fibrin deposits were found in a skin biopsy. Treatment with tetracycline was successful.

[Melanic proliferations of the conjunctiva. Histopathologic study of a series of 40 cases]. / Les proliférations mélaniques de la conjonctive. Etude histopathologique à propos d'une série de 40 cas.

A retrospective study of 40 melanocytic conjunctival tumors (22 naevi and 18 malignant melanoma) is reported. Cases of non-degenerated conjunctival melanosis were not observed. The particular aspects of diagnosis and the prognostic behaviour of these proliferations are discussed and compared with their cutaneous homologues. Epithelial inclusions within tumor cell proliferation are considered as the best criterion of benignity. Because conjunctival melanomas are rather uncommon and technical difficulties in their study are very frequent (poor quality of tissue samples, small size of specimens) many reports are contradictory. Some diagnostic signs are specific for this kind of tumor in the conjunctiva: nevertheless, the particular conjunctival anatomy makes it impossible to classify according to criteria defined for cutaneous malignant melanoma. No criteria would have a predictive value. The severe prognosis of conjunctival melanoma (44% deaths in the present series over 10 years after diagnosis) has been underestimated. Most often carcinologic therapies have not been used in previously reported cases.

[Pre- and postoperative endocrine function in children with craniopharyngioma ]. / L'exploration endocrinienne pré- et post-opératoire des craniopharyngiomes de l'enfant.

17 children presenting with craniopharyngioma were studied from 1970 to 1980. Presenting symptoms were usually reflecting endocrine dysfunction (13 of 17 cases), such as growth retardation, weight abnormalities, diabetes insipidus and hypothyroidism. Unfortunately, these signs were most often misinterpreted (10 of 13 cases) so that when diagnosis was made dissociated hypopituitarism was present. Further impairment of endocrine function always followed surgical excision. In most children pituitary insufficiency becomes complete and permanent. These results are similar whatever the tumor site and surgical procedure. Growth goes on and puberty occurs without treatment in a few cases but in the majority of them substitutive therapy is necessary.