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We report 3 cases of lipodermatosclerosis (LDS) and discuss the nosology of similar disorders caused by venous insufficiency of the legs. These cases are characterized by (1) occurrence in middle-aged or aged woman, (2) painful, indurated erythema with hyperpigmented scleroderma-like hardening on the lower leg, (3) lobular panniculitis with membranocystic fat necrosis and various degrees of septal fibrosis. Although the designation LDS has been used particularly in the UK and in the USA, this entity is not familiar in other countries including Japan.

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Pseudo-scleroderma should not be confused with true scleroderma, the prognosis of which is unpredictable and often serious. Progressive acrosclerosis must be differentiated from Raynaud's disease, congenital or hereditary disorders of unknown aetiology: Werner's syndrome, acrogeria and progeria; Rothmund-Thomson's syndrome, Steinert's disease, phenylketonuria, disorders of glycogen metabolism; metabolic disorders: mutilating acropathies, scleromyxoedema, porphyria cutanea tarda; occupational and iatrogenic disorders: acroosteolysis, toxic epidermic syndrome (Spain), scleroderma-like change induced by bleomycin, chronic graft-versus-host disease; and leprosy. Acute diffuse scleroderma should not be confused with Buschke's scleroedema, sclerema neonatorum, systemic amyloidosis and scleroderma-like changes in hypothyroidism.

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The histologic finding of variably acid-fast coccoid forms in all the available biopsy material (skin, lymph nodes, and lung) from a case of coexisting scleroderma-like cutaneous disease (hypodermitis sclerodermiformis) and systemic sarcoidosis is reported. The morphologic size, shape, and staining characteristics of these microbes, along with the presence of the lung of 'large bodies', suggest that these microbes are cell wall deficient L forms of mycobacteria. Culture of the skin of the scleroderma-like lesion yielded Staphylococcus epidermidis, and the relationship of this isolate to the histologic findings of bacteria is discussed, as well as the possible pathogenic role played by L forms of mycobacteria in collagen disease and systemic sarcoidosis.

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We described two patients with localized, chronic, painful, scleroderma-like lesions of the lower part of the leg associated with venous stasis. This type of lesion has been termed "hypodermitis sclerodermiformis," and we review the literature pertinent to this entity. In addition, skin biopsy material was studied for the presence of acid-fast microbes.

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