Background: Symmetric lipomatoses are characterized by marked symmetric deposition of diffusely distributed fatty tissue. Though relatively common disorders, they are rather rarely reported in the literature, possibly being misdiagnosed as general obesity. While the differential diagnosis of symmetric lipomatosis versus general obesity may not appear difficult in males, it is obviously problematic in females. Observations: We describe the findings in 6 representative female patients with symmetric lipomatoses: 3 with benign (multiple) symmetric lipomatosis and 3 with female zonal obesity. The former disorder was characterized by massive, firm, symmetric fat deposition predominantly around the neck and shoulder girdle and was clearly associated with alcohol abuse and/or liver disease. There were no malignant tumors of the upper airways. In the latter case, fatty tissue had accumulated mainly at the buttocks and thighs but characteristically spared the feet and hands. Tenderness was a common symptom. This disorder showed familial predisposition. Histology in both cases revealed normal fatty tissue which was neither encapsulated nor septally divided. Conclusions: We suggest that the term ‘symmetric lipomatoses’ refers to two separate disorders, benign (multiple) symmetric lipomatosis and female zonal obesity.
In a multi-centre study 291 patients with psoriasis were treated with (a) oral doses of the recently developed retinoid Ro 10-9359, (b) classical local dithranol application, and (c) both. In a preliminary evaluation of 203 patients treated orally excellent or good results were obtained in 120 (61%), no response in 31 (15.8%). The initial dose was 1.0 mg/kg body-weight daily, i.e. 50-75 mg, which was then reduced to 25-50 mg daily. A clinical response was noted after 2-3 weeks. Particularly, severe erythrodermic and pustular forms of the disease responded surprisingly well to the drug so that cytostatic agents were avoided. Under long-term administration, however, relapses were still seen. Most side-effects were reasonably well tolerated. But in 14% of patients the drug had to be discontinued because of hair loss, paronychia or slight elevation of transaminases (up to 40 U/I). This new drug is thus a potent antipsoriatic agent: it is effective, easily controlled and causes only moderate side-effects.
In a multicentre, cooperative study into the treatment of extensive psoriasis with a new aromatic retinoid (Ro 10--9359) trichogram, liver function tests and the light erythema threshold were investigated. In some of the patients hair loss occurred, usually in the fifth to eighth week after a total dose of 1.9 g retinoid. In all cases this improved an average of six weeks after dose reduction or cessation of treatment. The trichogram in 27 patients showed a diffuse toxic hair loss. In 70% the effluvium was telogenic, in 22% telogen-dystrophic. GPT, GOT, alkaline phosphatase and prothrombin index showed no significant alterations during retinoid treatment. However, in individual cases there was a rise in GOT and GPT up to 80 U/l. Furthermore there was a statistical tendency in rising bilirubin levels. Finally there was no evidence for an increase in light sensitivity after three weeks of retinoid treatment. Measurement of the erythema threshold showed rather more a reduction in light sensitivity under treatment.
The so-called amyopathic dermatomyositis is a rare variant of dermatomyositis which has attracted increasing interest during the last years. One finds the classical signs of dermatomyositis such as periorbital edema and erythema, erythematous macular and papular lesions localized at bony prominences (so-called Gottron's papules), generalized pruritus, photosensitivity, and a cutaneous histopathologic picture compatible with skin lesions of dermatomyositis. Crucial for the diagnosis is the exclusion of myositis by clinical examination, EMG and histology. Furthermore, longterm supervision of patients is advisable in order not to miss the appearance of early signs of myositis. The longest reported follow-up of amyopathic dermatomyositis patient is 4 years; however, it cannot be excluded that these cases will eventually culminate in classical dermatomyositis. In this paper we describe two cases and discuss the differential diagnosis and therapy; also, the term "Premyopathic dermatomyositis" is proposed, to indicate that the full picture is to be expected in most cases.
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