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Skin thicking simulating scleroderma is not rare in diabetes mellitus (about 2% among patients with diabetes mellitus), however it is not well known to rheumatologists and endocrinologists. The basis of the syndrome are the abnormalities of collagen and glycosaminoglycans metabolism accompanying by the acid mucopolysaccharides accumulation in the deep part of the reticular dermis. Three cases of pseudosclerodrma in patients with long-standing complicated insulin-dependent diabetes mellitus are presented. The main clinical manifestations of pseudoscleroderma were marked cutaneous induration of the neck, body and proximal parts of extremities liked that of scleredema of Buschke ( 2 patients), and primary paraarticular induration leaded to the flexion contractures liked that of paraneoplastic scleroderma ( I patient). Possible mechanisms of development, clinical and morphological peculiarities and treatment for this syndrome are discussed.

In the period 1994-2002, we saw 71 patients with sclerotic skin changes; 66 were diagnosed with systemic sclerosis (SSc) while five (7%) were diagnosed with pseudoscleroderma associated with various malignancies. The mean duration of disease in these five patients was significantly shorter than that of patients with SSc. The incidence of positive antinuclear antibodies, Raynaud's phenomenon or oesophageal involvement in patients with pseudoscleroderma was significantly lower than that in patients with SSc. The distributions of skin sclerosis varied in each case. Serum basic fibroblast growth factor (bFGF) levels in the five patients with pseudoscleroderma were very elevated compared with levels in controls. Elevated expression of bFGF was detected on fibroblasts of affected skin and in one lung-cancer tissue sample obtained by excision.

The Moore Federman syndrome (MFS) is characterized by short stature, stiffness of the joints, characteristic facies and ocular abnormalities. Herein, we report the case of a 45 year-old lady with short stature, thickening of the skin, stiffness of the joints, typical facies, iridodonesis and cataract since the age of 12 years. She had short digits and no family history of similar illness. To the best of our knowledge, this could be the fourth report of MFS.

Contact reactions may present as non-eczematous lesions. A variety of non-eczematous occupational contact reactions has been described. The exact patho-mechanism of these reactions is unknown. The following non-eczematous contact reactions have been described: 1. Erythema multiforme-like eruption [urticarial papules and plaque eruptions (UPPE)] 2. Purpuric eruption 3. Lichen planus-like eruption 4. Papular and nodular eruption 5. Granulomatous reaction 6. Pustular eruption 7. Erythema and exfoliation 8. Pseudoscleroderma

A 65-year-old female began experiencing arthralgia, morning stiffness and psychroesthesia in April 2000. She visited a rheumatologist and was suspected of having early-stage rheumatoid arthritis. She was referred to our hospital, and was admitted in December 2000. At that time, sclerosis of the skin was advanced, and Raynaud's phenomenon was confirmed. The patient also exhibited nailfold bleeding, and was diagnosed as having systemic scleroderma. She visited a gynecologist for screening and cervical uteri carcinoma was confirmed. She underwent surgery in March 2001, and subsequently, sclerosis in her skin was almost stopped. We believe that these clinical symptoms were related to paraneoplastic syndrome. We therefore immunochemically investigated the pathogenesis of paraneoplastic syndrome and found that connective tissue growth factor (CTGF) might be involved and transforming growth factor-beta (TGF-beta) might not be involved in this case.

We report a case of a 30-year-old Caucasian patient with progressive sclerosis of the skin mainly on the upper limbs which was diagnosed as eosinophilic fasciitis (Shulman syndrome). Circulating antibodies against Borrelia burgdorferi were detected. The association of B. burgdorferi infection with eosinophilic fasciitis is discussed.

Sir, Lipodermatosclerosis (LDS), also reported as liposclerosis, hypodermatitis sclerodermaformis, sclerosing panniculitis, pseudoscleroderma, indurated cellulitis, stasis panniculitis and chronic cellulitis, is characterized by a scleroderma-like induration of the skin and a typical ‘inverted champagne bottle’ sign. Although considerable progress has been made in understanding the pathophysiology, no unifying therapeutic concept exists and the efficiency of the various treatment options has been reported very controversially. In the present case we describe successful treatment of LDS with the weak androgen danazol.

A case of bulbous swelling of the tips of fingers and toes, central acro-osteolysis of terminal phalanges and bound-down skin of the extremities and face in a 14 year old girl, is reported.

"Collagens: Their Structure and Metabolism, Cornelia Mauch and Thomas Krieg Proteoglycans: Structure, Biosynthesis, and Degradation, Hiroshi Shinkai Elastic Fibers, Jouni Uitto, Michael Fazio, and Veli-Matti KAhAri Cutaneous Basement Membranes, David T. Woodley and Yves Sarret Fibronectin, Edward J. O'Keefe Collagenase in Disease, Rosalind A. Grymes, Annemarie Kronberger, and Eugene A. Bauer Connective Tissue Protein Receptors, Monika Borchert Mechanical Properties of the Dermis: An Engineer's Nightmare, Christopher Edwards and Ronald Marks The Family of Stromal Cells, Giulio Gabbiani and Marie-Luce Bochaton-Piallat Chemotaxis and Invasiveness, Adriana Albini, Antonella Melchiori, Silvio Parodi, and Hynda K. Kleinman Cell-Cell and Cell-Matrix Interactions, Betty V. Nusgens Effect of Cytokines and Growth Factors on Fibroblast Activity, Konrad Herrmann and Hans-Joachim Boehme Aging and Premature Aging Syndromes, Charles M. Lapiere Ehlers-Danlos Syndrome, Charles M. Lapiere Cutis Laxa, F. Michael Pope Marfan's Syndrome, F. Michael Pope Osteogenesis Imperfecta, F. Michael Pope Pseudoxanthoma Elasticum, F. Michael Pope Epidermolysis Bullosa, Leena Bruckner-Tuderman Acquired Bullous Diseases, Fenella Wojnarowska Localized and Systemic Scleroderma, Raul Fleischmajer Pseudoscleroderma: Clinical, Morphological, and Biochemical Aspects, Stefania Jablonska Healing, Ulcers, and Keloids, Charles M. Lapiere Collagen in Dermatological Applications, Frank DeLustro and Samuel J. Stegman In Vitro Reconstructured Skin for Grafting in Humans, Bernard Coulomb and Louis Dubertret Drugs Acting on Connective Tissue, Aarne Oikarinen "

An infant girl with partial phenylketonuria developed pseudoscleroderma. After six years of follow up, both the neurologic and cutaneous conditions improved under a phenylalanine restricted diet. The probable roles of phenylalanine, tryptophan, tyrosine, and their metabolites may cause both conditions through possible transient hypersensitivity of cutaneous and muscular tissues.

A case of IgG-χ multiple myeloma is reported with coexistent scleroderma-like skin lesions. Histological, histochemical, electron microscopic and immunohistochemical studies failed to reveal amyloid in the skin, muscles, intestine and kidney. No macroglossia or other features characteristic of systemic amyloi dosis have been revealed. Attention is called to disturbances of tryptophan metabolism (increased level of indoles) which may play some role in the pathomechanism of the indurations. The possible transformation of paraprotein to histochemically-detectable amyloid is discussed.

Summary We report localized pseudoscleroderma occurring around the sites of intramuscular injection of phytomenadione (Konakion®), in a patient with drug-induced liver disease. This is the first report of pseudoscleroderma following phytomenadione injection (Texier's syndrome), to appear in the British literature.

Pseudoscleroderma occurs occasionally in patients with phenylketonuria, but as far as we know scleroderma with visceral involvement has never been reported in phenylketonuria. Here we present a case of progressive systemic scleroderma with mild phenylketonuria

Fourteen years ago, I had the pleasure of reviewing Prof Stefania Jablonska's monograph, Sclerodermaand Pseudoscleroderma, for this journal. The publication of the current volume in 1988 permits us to understand the major advances in diagnosis, understanding of the disease, and treatment that the interval provided. And yet, if we accept the five outcome measures of disease championed by Fries and carefully defined in our book Prognosis, namely, death, disease activity, disability, treatment, and costs (listed, for the convenience of having five D's, as drugs and dollars), there are grounds for optimism. While mortality figures after diagnosis may not be particularly favorable, the introduction of angiotensinconverting enzyme inhibitors and other contemporary treatments may well influence the equation favorably.Systemic sclerosis is not rare in specialized practices and university centers, which concentrate such cases. A recently concluded cooperative study in the United States contributed hundreds of cases, and more than 5000

We describe 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. In both cases, Fite-Faraco-stained tissue sections contained many acid-fast coccoid and giant microbial forms suggestive of transitional L forms. Culture of the lesion in both cases was positive forStaphylococcus epidermidis. Until the cause is fully clarified, the search for acid-fast bacteria appears warranted in forms of pseudoscleroderma such as hypodermitis sclerodermiformis. (Arch Dermatol115:449-452, 1979)

This book appeared in Poland in 1963 and was translated for the National Library of Medicine in 1965. It is unlike most American textbooks, particularly as it takes an editorial stance and evaluates data from this perspective. The author favors the neurogenic theory, which is at variance with most American opinions. She is aware of the other etiologic hypotheses but dismisses them in favor of her own candidate. There is as much evidence against as there is for most hypotheses, and this holds true for the author's concept as well, though one would never guess it from this volume. The clinical manifestations of progressive systemic sclerosis (a term the author does not like), as well as histopathology and roentgenography, are well covered. By pseudoscleroderma , the author means syndromes that may be confused with scleroderma. This designation includes various skin diseases (including morphea), as well as rheumatoid arthritis, dermatomyositis, endocrine disorders