Acrokeratosis paraneoplastica (Bazex's syndrome): association with liposarcoma.

Acrokeratosis paraneoplastica (Bazex's syndrome) is a rare obligate paraneoplastic dermatosis characterized by erythematosquamous lesions localized symmetrically at the acral sites. The condition almost exclusively affects Caucasian men older than 40 years. It is usually associated with primary malignant neoplasms of the upper aerodigestive tract. In most cases, the skin changes precede the clinical manifestation of the underlying neoplasm. The dermatosis can be cured only by removal of the underlying carcinoma. We describe a case of acrokeratosis paraneoplastica associated with a retroperitoneal liposarcoma in a 71-year-old Caucasian man. The liposarcoma was surgically removed but recurred several times, with acrokeratosis paraneoplastica showing a parallel development. We, therefore, add liposarcoma to the growing list of malignant neoplasms associated with acrokeratosis paraneoplastica.

Prospective, randomized, multicenter, double-blind placebo-controlled trial comparing adjuvant interferon alfa and isotretinoin with interferon alfa alone in stage IIA and IIB melanoma: European Cooperative Adjuvant Melanoma Treatment Study Group.

PURPOSE: The combination of interferon alfa (IFNalpha) and isotretinoin has shown a direct antiproliferative effect on human melanoma cell lines, but it remained unclear whether this combination is more effective than IFNalpha alone in patients with metastatic melanoma. We evaluated safety and efficacy of IFNalpha and isotretinoin compared with IFNalpha alone as adjuvant treatment in patients with primary malignant melanoma stage IIA and IIB. PATIENTS AND METHODS: In a prospective, randomized, double-blind, placebo-controlled trial, 407 melanoma patients in stage IIA (301 patients) and IIB (106 patients) were randomly assigned to either IFNalpha and isotretinoin (isotretinoin group; 206 patients) or IFNalpha and placebo (placebo group; 201 patients) after excision of the primary tumor. IFNalpha was administered three times a week at a dose of 3 million units subcutaneously for 24 months. Isotretinoin at a dose of 20 mg for patients < or = 73 kg, 30 mg for patients greater than 73 kg, or placebo daily for 24 months. RESULTS: A scheduled interim analysis revealed no significant differences in survival rates, with the isotretinoin group and the placebo group showing 5-year disease-free survival rates of 55% (95% CI, 46% to 65%) and 67% (95% CI, 59% to 75%), respectively, and overall 5-year survival rates of 76% (95% CI, 67% to 84%) and 81% (95% CI, 74% to 88%), respectively. The trial was stopped for futility. CONCLUSION: The addition of isotretinoin to an adjuvant treatment of low-dose IFNalpha in patients with stage IIA and IIB melanoma had no significant effect on disease-free or overall survival and is therefore not recommended.

Papular xanthoma: a clinicopathological study of 10 cases.

BACKGROUND: Papular xanthoma (PX) is one of several clinicopathologic variants of normolipemic cutaneous non-Langerhans cell histiocytoses (n-LCH). PX represents a monomorphous reaction pattern of n-LCH characterized by the presence of predominantly xanthomatized macrophages. OBJECTIVE: The purpose of this study was to identify the clinical, histological and immunohistochemical characteristics of PX. METHODS: A series of 10 cases of PX was identified and the results compared with the other histologic subtypes, namely the polymorphous and the remaining other monomorphous reaction patterns in n-LCH. RESULTS: In this clinicopathologic study, papular xanthoma presented clinically mainly as solitary papule, with a male to female ratio of4 : 1, in an age range from 13 to 57 years and a biphasic occurrence: in the young adolescence and middle ages. It was predominantly located on the trunk, the extremities, and rarely on the head. Clinically, PX was described as xanthoma, 'cutaneous tumor', but also as atheroma, keloid, histiocytoma, Spitz's nevus or clear cell acanthoma. Histology showed moderately well circumscribed exoendophytic papules with a regular epidermis and a dense infiltration of xanthomatized macrophages interspersed by numerous Touton type giant cells. Immunohistochemically mono- and multinucleated macrophages were consistently positive with KiM1p; while only giant cells were labeled with KP1 (CD68), the reactivity with HAM 56 was much more variable. Up to 50% of the xanthomatized cells labeled positive for the lectin peanut agglutinin. In one case the xanthomatized cells stained positive for CD34. Staining for factor XIIIa and CD1a were negative. CONCLUSIONS: This series confirms PX as a rare, but distinguished clinicopathologic entity in the spectrum of n-LCH of the skin.

Schönlein-Henoch purpura during pregnancy with successful outcome for mother and newborn.

BACKGROUND: Schönlein-Henoch purpura is a systemic vasculitis that affects vessels of a small caliber and rarely reported in the literature. CASE PRESENTATION: We report on a 35-year-old woman who developed palpable purpura with necrotizing cutaneous lesions on the lower limbs at 27 weeks of gestation. She also complained of epigastric pain and arthralgias. Histologic examination of a skin biopsy showed leukocytoclastic vasculitis with intravascular fibrin thrombi. The direct immunofluorescence analysis evidenced vascular deposits of IgA and C3 in the upper and mid-dermis. These findings were consistent with Schönlein-Henoch purpura. There was no evidence of renal involvement or placental dysfunction. The patient was treated with low-dose oral corticosteroids and a healthy infant was delivered by cesarean section. Examination of the placenta and the navel string disclosed no signs of vasculitis or infarction. CONCLUSION: Schönlein-Henoch purpura is rarely reported in pregnancy. Treatment with orally administered corticosteroids may lead to a beneficial outcome for mother and newborn.