Successful Treatment of Poststreptococcal Scleredema Adultorum Buschke With Intravenous Immunoglobulins

Scleredema. A multicentre study of characteristics, comorbidities, course and therapy in 44 patients

“…Our study confirms that there is no single universally effective therapy for scleredema [8][9][10][11][12][13] . Physical therapies such as UVA1, bath PUVA, cream PUVA, low-dose broad-band UVA and narrow-band UVB [14][15][16][17][18] , localized electron beam therapy and photopheresis 19,20 , intravenous immunoglobulin (IVIg) [21][22][23] have been reported to improve the disease in anecdotal reports or small case series. Among the 19 patients of ours who received one systemic therapy, only two had a complete resolution, one with UVA1 and one with systemic steroids.…”

Successful treatment with IVIg therapy of diabetes-associated scleredema severe progressive case and review of the literature

“…Response was typically noted early in the course of treatment, but often improvement continued for the duration of therapy (Adam et al, 2015;Aichelburg et al, 2012;Barde et al, 2009;Eastham et al, 2014). All four patients reported decreased induration and increased range of motion, allowing better functioning in daily activities.…”

“…They are most likely with the first treatment (Orange et al, 2006) and increasing doses and infusion rate (Struff, Klasser, Eckert, & Dietrich, 2005). IVIg was tolerated well with only one patient reporting a slight headache (Adam et al, 2015;Aichelburg et al, 2012;Barde et al, 2009;Eastham et al, 2014). Severe adverse events are rare but may include anaphylaxis, hypotension, aseptic meningitis, thromboembolic events, or acute renal failure (Cherin et al, 2016;Orange et al, 2006).…”

“…All four previous cases were associated with one of the three major associated diseases. There were two males and two females, 34-60 (mean 49) years at the time of initiating IVIg (Adam et al, 2015;Aichelburg et al, 2012;Barde, Masouy e, Saurat, & Le Gal, 2009;Eastham, Femia, Velez, Smith, & Vleugels, 2014). IVIg was administered monthly at 2 g/kg/cycle over 2 days in three patients.…”

Scleredema adultorum of Buschke over an unusual site associated with pregnancy

“…Antibiotics are recommended in those cases associated with streptococcal infections, though they do not appear to shorten or cure skin conditions in scleredema. [4] Other reported therapies include oral or intralesional steroids, cyclosporine, colchicine, low-dose methotrexate, high-dose penicillin, electron beam therapy, extracorporeal photopheresis, psoralen followed by ultraviolet A Psoralen followed by ultra violet A (PUVA) and intravenous immunoglobulins[5] Prognosis depends on the etiology. Though in most cases it is self-limited, death due to cardiac and pulmonary involvement has been documented.…”