Treatment of pyoderma gangrenosum with intravenous immunoglobulin.

BACKGROUND: Intravenous immunoglobulin (IVIG) is increasingly being used to treat inflammatory and autoimmune disease. OBJECTIVES: To elucidate the efficacy of IVIG as an adjunct treatment for pyoderma gangrenosum (PG). PATIENTS/METHODS: Ten patients with PG were treated with IVIG at Johns Hopkins Department of Dermatology. All patients had severe mutilating and/or refractory disease requiring multi-agent therapy. The charts were reviewed retrospectively. RESULTS: Seven of the ten patients had clearance of PG lesions in the setting of IVIG and six of these patients maintained efficacy with repeated IVIG treatment. Five patients complained of nausea with treatment, and in one case nausea was severe and intractable. One patient developed an immune reaction requiring diphenhydramine and methylprednisolone and another experienced aseptic meningitis. CONCLUSIONS: IVIG may be an effective adjuvant in the treatment of PG and has an acceptable side-effect profile. Randomized, placebo-controlled, double-blinded trials are needed to confirm this hypothesis.

Photoprotection by thalidomide in patients with chronic cutaneous and systemic lupus erythematosus: discordant effects on minimal erythema dose and sunburn cell formation.

BACKGROUND: Thalidomide is an anti-inflammatory and immunomodulatory agent with proven efficacy in several refractory inflammatory skin conditions including photoexacerbated skin diseases. The effects of thalidomide on ultraviolet (UV)-induced cutaneous damage in humans have not been extensively studied. We describe the results of minimal erythema dose (MED) testing in nonlesional skin of three patients with chronic cutaneous lupus erythematosus (CCLE) before and after treatment with thalidomide. OBJECTIVES: To determine whether thalidomide treatment provides clinical and histological evidence of photoprotection from acute UV injury. METHODS: MED testing was performed in nonlesional skin of three patients with CCLE before and after treatment with thalidomide. Skin biopsy specimens were taken from MED sites for in situ immunochemistry. RESULTS: In each patient, the MED to UVB irradiation was significantly higher while the patient was receiving thalidomide treatment than in the absence of thalidomide, suggesting a systemic photoprotective effect. Thalidomide treatment had no significant effect on markers of apoptosis including sunburn cell formation and terminal deoxynucleotidyl transferase-mediated biotinylated deoxyuridine triphosphate nick end labelling, which identifies single-strand breaks in DNA. CONCLUSIONS: Thalidomide inhibits acute UVB erythema at 24 h after exposure, as a 100-mg daily dose of this drug for 4 weeks conveyed a sun protection factor of 1.56 to > 4.0. We conclude that inhibition of UVB-induced inflammation may, in part, explain the therapeutic benefits of this agent on photosensitive diseases.

Henoch-Schönlein purpura in pregnancy.

Henoch-Schönlein purpura (HSP) is an IgA-mediated small vessel vasculitis which commonly involves the skin, gastrointestinal system and kidneys. Numerous HSP triggers have been identified, and pregnancy has been reported as an exacerbating factor. After a pregnant woman had been diagnosed as having new-onset HSP, we reviewed all cases of immunofluorescence-proven HSP evaluated by the Department of Dermatology at the Johns Hopkins Hospital between 1990 and 2002, and report three cases of HSP occurring during pregnancy. Two patients developed new-onset HSP, one at 16 weeks gestation and one at 22 weeks, while the third developed a recurrence of HSP at 12 weeks gestation after 19 years of remission. We conclude that pregnancy may be a trigger for HSP onset or recurrence in susceptible individuals.