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[5- year old boy with Weber-Christian Syndrome or histiocytic cytophagic panniculitis? Diagnostic difficulties. Case presentation].

The aim of the paper is to present a case of a 5-year-old boy hospitalised because of recurrent high temperature and reddish blue infiltration of skin and hypodermis lasting 3 months. Because of pancytopenia and hepatomegaly, we suspected a proliferative disease of the haematopoietic or lymphatic system. Several bone marrow biopsies, did not show neoplastic changes. Serological tests excluded virus and animal borne infections. In the histopathologic examination of skin and hypodermis biopsy there was an inflammatory infiltration of the adipose tissue without features of vessel inflammation (the Weber-Christian syndrome). Al though steroid therapy was applied, the infiltration of the adipose tissue did not regress, while liver dysfunction symptoms intensified and pancytopenia and fever continued. The patient died with symptoms or respiratory and circulatory failure. This case of a 5-year-old boy is presented because of extreme diagnostic difficulties, which occurred at each phase of investigations. The repeated tests, as well as several verifications of the sampled histopathological material did not bring any unequivocal proof of the postulated diagnosis.

Adipose Tissue↗

Prevalence of obesity, overweight and underweight in a Hong Kong community: the United Christian Nethersole Community Health Service (UCNCHS) primary health care program 1996-1997.

The aim of this cross-sectional observation study was to assess thoroughly the body mass index (BMI) profile in Hong Kong Chinese and report all categories of BMI cutoff points as suggested by the World Health Organization (WHO). A cohort of 17,242 subjects (4822 men and 12420 women) aged 15 or above from the community of Hong Kong presenting themselves voluntarily from April 1996 to August 1997 for primary health assessment at the Three Health Centers of the United Christian Nethersole Community Health Service (UCNCHS). The mean age (+/- SD) was 51.0 +/- 16.2 years (range 15-96 years, median 49.6 years). The mean BMI of the 17,242 subjects was 23.5 +/- 3.3 kg/m2 in men and 23.0 +/- 3.7 kg/m2 in women. The BMI peaked at age 30 to 50 years in men and 50 to 70 years in women. The age-standardized prevalence of BMI > or = 30 kg/m2 or > or = 25 kg/m2 in Hong Kong Chinese was 3.0% in men and 3.2% in women, and 29.1% in men and 21.3% in women, respectively. For underweight, 35.9% and 27.8% of women and 27.5% and 10.3% of men aged 15-20 and 20-30 years, respectively, had BMI < 18.5 kg/m2. In conclusion, a significant proportion of Hong Kong Chinese had a BMI > or = 25 kg/m2. Among Hong Kong Chinese aged < 30 years, the prevalence of underweight was also high.

Adolescent↗

[Christian Andreas Cothenius (1708-1789). A pro-memoria on the occasion of the 200th anniversary of his death].

The 200th anniversary of the death of Christian Andreas Cothenius gave occasion to appreciate life and work of this personage of a physician. Cothenius maintained manifold connections to Halle, of which the golden doctorate and the heritage of the pharmaceutic enterprises of his teacher Friedrich Hoffmann were treated in this place. The picture of the local relations was supplemented by the history of the Cothenius medal which is today awarded by the Leopoldina of Halle.

Germany↗

[Long-term observation of a case of Hand-Schüller-Christian disease].

A case is presented of a 28-year-old man with Hand-Schüller-Christian's disease whose initial manifestations were skull bones lesions and a diffuse interstitial fibrosis. Three years later the patient developed diabetes insipidus without changes in the hypothalamic-hypophysial region on computed tomography. Some features of the clinical picture are discussed.

Adult↗

Weber-Christian disease associated with multiple perforations of the ileum and colon.

Panperitonitis developed in a 57-yr-old Japanese man during the course of Weber-Christian disease (WCD). Emergent operation disclosed three perforations of the ascending colon and two of the distal ileum. Histological study of the surgical specimen showed that all perforations were located at the centers of acute subserosal panniculitic foci. No vasculitis was discerned. In the previously reported three cases of WCD with intestinal perforation, perforation was solitary and seen in the small intestine. The cause of perforation was considered to be vasculitis in two cases. In the remaining one, panniculitis around the perforation was in chronic granulomatous stage. This was the first case of WCD in which acute subserosal panniculitis caused multiple ileocolonic perforations.

Acute Disease↗

[Weber Christian disease with immunoblastic lymphoma. Apropos of a case].

The clinical case of a female patient suffering Weber Christian disease of long evolution with cutaneous and systemic manifestations, involvement of the mesenteric and epicardic fat and that later involved to immunoblastic lymphoma is presented. The different etiopathogenic factors are discussed.

Female↗

[Weber-Christian syndrome and pancreatic involvement. Apropos of a case with review of the literature].

The Weber-Christian syndrome associates generalized nodular inflammatory panniculitis with osteoarticular manifestations. Pathogenesis of this rare affection is still obscure but a pancreatic origin has been reported in the literature. A case is presented having the particular features of being revealed by a subacute episode of chronic pancreatitis, and by the intensity of radiologic bony manifestations.

Adult↗

[Pfeifer-Weber-Christian panniculitis (PWCP). Description of a case and review of the literature].

We report on a case of Pfeifer-Weber-Christian panniculitis (PWCP) in a 40 year old woman. PWCP is a rare inflammatory disorder of the subcutaneous fatty tissue. It is characterized by painful relapsing, subcutaneous nodules occurring preferentially at the upper arm, thigh and trunk regions. The disease is often accompanied by recurrent temperatures and constitutional symptoms. The clinical course of our patient had already lasted for four years when we saw her for the first time. Besides local panniculitis of the arms and the trunk she suffered from recurrent temperatures. Antibiotics and/or antiinflammatory therapy failed to control the disease. Laboratory tests and chest X-ray did not reveal noteworthy pathological results. PWCP was proven histologically and distinguished from other soft tissue disorders. The findings are discussed in the context of previous reports. The etiopathology of PWCP remains unclear and a specific therapy still awaits introduction.

Adipose Tissue↗

[Histiocytosis X. 3 different cases: eosinophilic granuloma, Hand-Schueller-Christian disease, Abt-Letterer-Siwe disease].

The authors present three different cases of histiocytosis X: the eosinophilic granuloma, the Hand-Schuller-Christian syndrome and the Letterer-Siwe syndrome. As it appears in the main articles concerning this argument, the Aa. emphasize the common features belonging to the three different forms of histiocytosis: the involvement of the bones and of other tissues. Moreover the prognosis is defined as good in the first case, uncertain in the second case and severe in the third.

Child, Preschool↗

Weber-Christian disease. Analysis of 15 cases and review of the literature.

We report 15 patients encountered over 13 years who presented with inflammation of subcutaneous fat and were given clinical and pathologic diagnoses of Weber--Christian disease (WCD). Prominent clinical features included female predominance, lower extremity nodules, fevers, arthritis/arthralgias, and myalgias. Notable laboratory features were elevated erythrocyte sedimentation rate, anemia, leukopenia, and hypocomplementemia, frequently with circulating 7S IgM or immune complexes at times of active symptoms. Histologic findings were lobular--together with frequent septal--panniculitis, fat-laden macrophages, variable cellular infiltrates, necrosis, and occasional vasculitis. Follow-up revealed the death of 2 patients and disease stabilization or improvement in 13 patients. Six patients developed features of other diseases (factitial disease, erythema nodosum, acute myelogenous leukemia, rheumatoid arthritis, systemic lupus erythematosus, and sarcoid) and a seventh may have had erythema induratum. We suggest that classic WCD, as originally described, reflects an increasingly recognized spectrum of panniculitides. These are syndromes of diverse etiology that share many clinical, inflammatory, and immunologic features.

Adult↗

Clinical features of yellow fever cases at Vom Christian Hospital during the 1969 epidemic on the Jos Plateau, Nigeria.

Between 27 September and 27 December 1969, 103 patients diagnosed as having yellow fever were admitted to Vom Christian Hospital, near Jos, Nigeria. Headache and vomiting were the commonest presenting complaints, and 95% of the patients showed scleral icterus or bile pigments in the urine. Haemorrhage, signs of renal failure, and CNS involvement (agitation, seizures) were associated with a grave prognosis. The overall case-fatality ratio was 45.6%. The average duration of illness for fatal cases was 6.4 days and for non-fatal cases 17.8 days. Six illustrative case histories are presented.

Adolescent↗

[Growth hormone deficiency during the course of histiocytoses. A report on a case of Hand-Schuller-Christian disease (author's transl)].

Hand-Schuller-Christian disease frequently products histiocytic localization in the part of the posterior hypothalamus which is responsible for diabetes insipidus. Retarded growth is also relatively frequent and has multiple etiologies. An isolated deficiency in growth hormone (GH) which may be due to localization of the disease in the anterior hypothalamus is much rarer. In the case reported the possibility of this localization was only suspected because of the presence of a hyperprolactinemia. Retarded growth was obviously present but it was difficult to relate this to a GH defian 7 ng/ml. Follow-up examinations did however provide definite proof of a hormonal deficiency. This confirms the notion of partial growth hormone deficiency and the difficulty of providing proof during the usual stimulation tests in some cases.

Adolescent↗

[Case of nodular non-suppurative paniculitis (Weber-Christian syndrome)].

It is reported on a 42-year-old female patient with panniculitis nodularis non suppurativa (Weber-Christian's syndrome). Anamnesis and histological findings are discussed and the therapy is explained. In the discussion the finally still unknown etiology (allergic, autoimmunological) as well as the differential diagnosis (focal panniculitis, lipomatosis subcutanea, erythema nodosum and others) are explained.

Adult↗

Experience of prolonged treatment of hand-Schüller-Christian disease with prednisolone.

An account is presented of the experience acquired in the prolonged prednisolone treatment, at 2-day intervals, of 6 patients with Hand-Schüller-Christian disease. A substantial improvement was observed in the basic process, with scarcely any side-effects. On the basis of the literature data and the present findings, glucocorticoid administration every second day is recommended for the treatment of these patients.

Adolescent↗