Rapidly progressive diffuse systemic sclerosis after local vitamins A, D and E complex injections: literature review and report of two cases.

The term autoimmune/autoinflammatory syndrome induced by adjuvants (ASIA) or Shoenfeld's syndrome refers to a wide group of immune-mediated diseases triggered by external agents. Several substances, such as vaccine adjuvants, squalene and silicone implants, are implied in the pathogenesis of ASIA syndrome. Treatment and prognosis of this complex condition are not completely known due to lack of good quality evidence. After a brief introductory literature review on ASIA, we report here two cases of patients that developed rapidly progressive systemic sclerosis clinical features after multiple intramuscular local injections of a substance recommended by a non-medical professional called ADE. ADE is an oily vitamin complex for veterinary use, and it was used in these cases for cosmetic muscular definition and enhancement purpose. To our knowledge, this is the first paper to describe the relation between injections of ADE and the development of ASIA with severe systemic sclerosis phenotype. Further investigation is needed to better understand the pathophysiology and to provide the basis for the treatment of this condition.

[Pansclerotic porphyria cutanea tarda after chronic exposure to organic solvents]. / Pansklerotische Skleroporphyrie nach langjähriger Exposition gegenüber organischen Lösungsmitteln.

A 63 year old man developed generalized scleroderma with massive sclerotic areas, particularly in the abdominal region, four years after being diagnosed with porphyria cutanea tarda (PCT). He had almost daily exposure to organic solvents (benzene, trichlorethylene) for many years. The cutaneous fibrosis progressed dramatically leading to a pansclerosis, even though the uroporphyrin levels were borderline and the liver enzyme values were normal. Organic solvents are among those substances which can cause a cutaneous fibrosis. The unusually complicated clinical development in our patient was a combination of the two initial factors, the PCT and the long term exposure to organic solvents. The pansclerotic PCT was differentiated from a systemic sclerosis, a disabling pansclerotic morphea and a generalized morphea by means of histological examinations, the absence of a Raynaud phenomenon and the non-involvement of additional organs. Auto-antibodies typical for systemic sclerosis were negative. Using a medium dosage of UVA1 phototherapy and intensive physiotherapy, the progression of the skin disease was stopped and the sclerosis improved.

[Scleroderma-like skin changes following administration of paclitaxel for the treatment of ovarian carcinoma]. / Sklerodermiforme Hautveränderungen bei Therapie eines Ovarialcarcinoms mit Paclitaxel.

A 61-year-old women with ovarian carcinoma was treated with surgery followed by 6 cycles of paclitaxel (Taxol) and carboplatin, producing a complete remission. During the course of therapy, she developed diffuse cutaneous sclerosis and thickening along with edema, weight gain and malaise. While antinuclear antibodies were elevated, specific antibodies against Scl 70 were not found. Other routine laboratory investigations were normal or negative. 20 MHz sonography showed a thickened, echo-rich dermis, while the Cutometer documented decreased cutaneous elasticity. Esophageal manometry identified abnormal motility. Additional screening and imaging studies showed no further evidence of systemic involvement. A skin biopsy showed dermal sclerosis. Treatment with immunosuppressive agents - prednisolone, azathioprine, cyclophosphamide- and phototherapy, including both UVA-1 and PUVA combined with penicillin, produced no definite improvement. One year after the administration of paclitaxel, the sclerosis and malaise are only minimal improved. This case is the second documented association of scleroderma-like cutaneous changes with paclitaxel therapy. Similar cases have been more often described following treatment with the analog substance docetaxel (Taxotere).