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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

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

No evidence for a spirochaetal origin of localized scleroderma.

We looked for evidence of a Borrelia infection in 15 patients with morphoea. We were not able to detect antibodies to Borrelia burgdorferi in any of these 15 patients. None of the 14 skin biopsies examined by immunohistochemistry showed evidence of spirochaetes. Skin biopsies were cultured in 10 patients. All were negative. These results do not support a spirochaetal origin of localized scleroderma.

Adolescent

[Histological study on localized scleroderma].

To define stage-specific and type-specific histological findings in morphea, 21 (14 plaque, 2 linear, and 5 en coup de sabre type) patients were examined. A) Fibrotic changes; 1) Every case had the fibrotic change of various degrees. 2) Nodular sclerotic fibrosis was found in 7 cases with morphea, namely in 5 of 11 cases with a plaque type and 2 of 4 en coup de sabre type morphea. And the above mentioned 7 cases were within two years since the onset, and no nodular fibrosis was found in the morphea with the longer duration than two years in history. Nodular fibrosis was located in the middle or lower dermis adjacent to the typical sclerotic dermis. It was strongly suggested that this nodular fibrosis is as an initial change of localized scleroderma. 3) Nodular fibrosis expanded to the neighboring area and made a typical histological feature of morphea which is completely different from that of diffuse scleroderma. B) Inflammatory findings; 1) Inflammatory changes were divided into, a perivascular, a diffuse or non-perivascular, and a mixed type. 2) The pure perivascular type was found in 10, the pure diffuse type only in one, the mixed type in 6 cases, and the other 4 cases had no inflammation. 3) Marked or moderate inflammation was found in cases with short history of the disease except for one case. 4) Inflammatory cells in the morphea were mainly composed of lymphocytes and histiocytes, but occasionally of plasma cells in 11 of 17 cases. C) Pigmentary changes; Incontinentia pigmenti was found in 18 of 21 cases.

Adolescent

[Localized scleroderma (morphea) and septic arthritis. Clinical manifestations of Lyme borreliosis seen in El Ferrol].

Two cases of Lyme's disease seen at El Ferrol (Spain) were described. One of them developed a recurrent knee arthritis and the other had a localized sclerodermia (morphea) syndrome. Diagnosis was established by means of clinical picture and serologic tests (enzyme-linked analysis and/or indirect immunofluorescence tests). Joint involvement has often been described in patients diagnosed of having Lyme's disease in Spain, however, the relationship between morphea and borreliosis is still a matter of controversy. We believe that patients with localized sclerodermia and high significant titers of specific antibodies against B. burgdorferi should be treated with antimicrobial agents.

Adult