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PubMed1979

Coincidence of vitiligo, alopecia areata, onychodystrophy, localized scleroderma and lichen planus.

The unique coincidence of five dermatological disorders, which occurred in a 39-year-old patient, is discussed. The clinical and laboratory examination did not reveal a common underlying cause. It is hoped this report will stimulate the recognition of other cases and thus aid in determining whether the coincidence of these disorders is a true association of diseases with a common underlying factor or a rare abnormality.

Adult↗
PubMed1989

[Parry-Romberg progressive facial hemiatrophy and localized scleroderma. Nosologic and pathogenic problems].

A case of a 65-year-old caucasian woman with progressive hemifacial atrophy (Parry-Romberg syndrome) is reported. The atrophy had begun ten years before. At time of presentation, she was suffering from facial pain and diplopia. She presented with severe enophthalmos associated with eyelid atrophy, loss of cilia and mild blepharoptosis. There was a patch of alopecia and atropic areas of skin, recognised as scleroderma "en coup de sabre". Ophthalmic examination showed miosis. Horner's syndrome and ocular motility disturbance. Biomicroscopic examination of anterior and posterior segments was normal; there was no heterochromia and no disc atrophy. Aesthetic surgical treatment was proposed. The etiology of hemifacial atrophy remains controversial, and its relations with scleroderma and autonomic nervous system disorders are discussed.

Aged↗
PubMed1978

Concurrent localized scleroderma and discoid lupus erythematosus. Cutaneous 'mixed' or 'overlap' syndrome.

Four patients with concurrent, chronic, progessive, localized scleroderma and discoid lupus erythematosus were studied; the condition originated as linear scleroderma in three of them. Three of the four patients were young females at the onset of the first skin disease. Dermatopathologic study confirmed the scleroderma and lupus erythematosus (LE). Direct immunofluorescence showed a positive band test in three cases. Unusual serological results included a positive LE clot test in three cases, a positive extractable nuclear antigen test in one case, and a negative antinuclear antibody test on repeated occasions in all four cases. Rare cutaneous disease similar to systemic, "mixed," or "overlap" connective tissue disease exists and offers an opportunity to study unusual immunologic and pathological events in both scleroderma and LE.

Adolescent↗
PubMed1989

Serum aminoterminal propeptide of type III procollagen in progressive systemic sclerosis and localized scleroderma.

Sera from 31 patients with progressive systemic sclerosis (PSS), 5 patients with widespread localized scleroderma (LS), and 3 patients with lichen sclerosus et atrophicus were analyzed for aminoterminal propeptide of type III procollagen (PIIINP) using a radioimmunoassay based on human propeptide. Thirty-eight per cent of the patients with PSS had levels above normal range, including all of the 3 patients with diffuse scleroderma. The same applies to 4 of 5 patients with widespread localized LS, while PIIINP in all 3 patients with lichen sclerosus et atrophicus were within normal levels. In patients with acrosclerosis, elevated PIIINP seems to be correlated to rapid progression and extension of lesions. A significant increase in PIIINP was found in a patient following discontinuation of prednisone and cyclophosphamide, while the present investigation did not allow judgement of effects of treatment with either penicillamine or cyclosporin A.

Humans↗
PubMed1975

Localized scleroderma.

Familial scleroderma is rare; only seven documented instances of the disease have been reported, to our knowledge. This report adds two more families to the literature. Three children in one family and two in the other had clinically and histiologically established localized scleroderma.

Adolescent↗