Sir,
Scleredema adultorum of Buschke is an uncommon condition with unknown pathophysiologic characteristics. It is a misnomer as the condition can occur in childhood. Sudden onset after infection, insidious onset with paraproteinemias and those preceded by diabetes are the three types of scleredema [
Scleredema is characterized by diffuse shiny, symmetrical, non pitting and woody induration of skin. It usually begins on back, sides of neck and spreads to face, shoulders, arms, thorax and becomes generalized. The hands, feet and genetalia are usually spared [
A 6 years old boy presented with diffuse hardening of the skin on face, neck and upper trunk of 1 month duration. It was followed by 10 days after the acute febrile illness. The genitals and legs remained uninvolved. Past and family history was insignificant. Examination revealed shiny, symmetrical woody induration of the skin with no sharp line of demarcation between involved and uninvolved areas. There was lack of expression on face however, the movements of chest and abdomen were not affected. There was no clinically detectable systemic abnormality.
Haematological and urine examination was normal. Erythrocyte sedimentation rate (ESR) was raised (35 mm/h), absolute eosinophil count (AEC): 540 cells/cumm. Anti Nuclear antibody (ANA), lupus erythematosus (LE) phenomenon and anti streptolysin O (ASO) titre were negative. Chest X-ray showed streaky interstial opacities.
Histopathological examination showed no epidermal changes. Increase in the thickness of reticular dermis with separated collagen fibres were observed (Figs.
(a) Epidermis is normal with increased thickness of reticular dermis, (b) Increased collagen fibres in dermis.
(a) Von Geisson stain: Swollen collagen fibres, (b) Alcian Blue: Mucin deposition in dermis.
Various differential diagnosis like scleredema, scleromyxedema and scleroderma were considered in our case. Histopathology of scleredema, epidermis and appendages are unaffected (
In our case acute febrile illness could have triggered scleredema in a 6 years old boy. Scleredema is confirmed by clinical findings, histopathology and use of special stains for collagen and mucin.
Scleredema is a rare connective tissue disorder. It is characterized by the development of skin induration that resolves spontaneously [
The pathogenesis of the condition is obscure, various causes have been proposed. These include immune sensitization phenomenon, hormonal stress reaction, lymphatic stasis, endocrine disturbance, enzyme dysfunction, dysautonomia [
Scleredema is a self limiting condition that resolves within 6 months to 2 years [
Histopathology and special stains are helpful for diagnosis of scleredema. This case is been reported because of the paucity of documented cases in Indian literature and establishes the significance of histopathology and special stains in the diagnosis of scleredema.