Scleredema of Buschke (SB) is a rare condition characterised by a diffuse, symmetrical, non-pitting induration of the skin usually affecting the neck, shoulders, trunk, face and arms. Following the classification of Graff (1968), SB can be classified in: 1) self-limiting SB following acute infection; 2) chronic and non-progressive SB, associated with tumors (myeloma, paraproteinaemia, insulinoma) or endocrine disease (hyperparathyroidism); 3) SB with chronic progression, associated with diabetes. Differential diagnose as to be done with sclerodermia, localized and generalized morphea, cutaneous mucinosis, pseudoscleroderma, localized scleromixedema, trichinosis and subcutaneous fat necrosis. We describe a 68 years old patient, non diabetic, who developed induration of the skin involved thorax, neck, arms and face three weeks after a pharyngitis due to Staphylococcus β-haemoliticus. The infection was treated with amoxicilline 2g/day for 12 days. Laboraory data were negative as instrumental examination. Histopathology and direct immunofluorescence were performed on a skin specimen taken from the neck; direct immunofluorescence was negative while the histology disclosed a typical pattern of SB with sclerosis of derma and deposition of mucin between the collagen bundles. The patient was treated with prednisone 25 mg on every other day for 45 days with resolution of the scleredema. Two weeks after the therapy the patient presented again progressive induration of the skin at the same areas. Laboratory data and instrumental examination were negative and in about three weeks he presented a spontaneous and progressive regression of the skin involvement. Considering the absence of diabetes or other diseases described in association with SB, and the history of a previous acute infection, we concluded with a diagnose of SB type 1 (Graff, 1968). Many hypothesis were proposed about the pathogenesis of SB considering the relationship whit the associated disease; considering the literature, an hypothesis about the pathogenetic role of immunoglobulines in acute infection is proposed.
Scleredema of Buschke after upper respiratory tract infection. A case report
Brazzelli V.;Borroni G.
2002-01-01
Abstract
Scleredema of Buschke (SB) is a rare condition characterised by a diffuse, symmetrical, non-pitting induration of the skin usually affecting the neck, shoulders, trunk, face and arms. Following the classification of Graff (1968), SB can be classified in: 1) self-limiting SB following acute infection; 2) chronic and non-progressive SB, associated with tumors (myeloma, paraproteinaemia, insulinoma) or endocrine disease (hyperparathyroidism); 3) SB with chronic progression, associated with diabetes. Differential diagnose as to be done with sclerodermia, localized and generalized morphea, cutaneous mucinosis, pseudoscleroderma, localized scleromixedema, trichinosis and subcutaneous fat necrosis. We describe a 68 years old patient, non diabetic, who developed induration of the skin involved thorax, neck, arms and face three weeks after a pharyngitis due to Staphylococcus β-haemoliticus. The infection was treated with amoxicilline 2g/day for 12 days. Laboraory data were negative as instrumental examination. Histopathology and direct immunofluorescence were performed on a skin specimen taken from the neck; direct immunofluorescence was negative while the histology disclosed a typical pattern of SB with sclerosis of derma and deposition of mucin between the collagen bundles. The patient was treated with prednisone 25 mg on every other day for 45 days with resolution of the scleredema. Two weeks after the therapy the patient presented again progressive induration of the skin at the same areas. Laboratory data and instrumental examination were negative and in about three weeks he presented a spontaneous and progressive regression of the skin involvement. Considering the absence of diabetes or other diseases described in association with SB, and the history of a previous acute infection, we concluded with a diagnose of SB type 1 (Graff, 1968). Many hypothesis were proposed about the pathogenesis of SB considering the relationship whit the associated disease; considering the literature, an hypothesis about the pathogenetic role of immunoglobulines in acute infection is proposed.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
