Colchicine for epidermolysis bullosa acquisita.

BACKGROUND: Epidermolysis bullosa acquisita (EBA) is a chronic subepidermal blistering disease that is difficult to treat. Recently one patient with severe EBA was described who responded dramatically to colchicine. OBJECTIVE: Our purpose was to determine the efficacy of colchicine in the treatment of EBA. METHODS: Four patients with severe EBA refractory to conventional therapy were treated with colchicine 0.6 to 1.5 mg a day for up to 4 years. RESULTS: In all four patients the lessening of skin fragility and the decrease in spontaneous blister formation were dramatic; few side effects were noted. CONCLUSION: Colchicine should be considered in the treatment of EBA.

Use of CD34 in assessing the relationship between stroma and tumor in desmoplastic keratinocytic neoplasms.

It has been hypothesized that the ability for neoplastic growth of epithelial-derived neoplasms depends upon the stroma. There are currently some studies which show that the stroma surrounding basal cell carcinomas (BCC) is derived from the tumor. In contrast, other studies provide evidence that the stroma is a host-derived response to the tumor. In order to further examine the nature of stroma enveloping cutaneous epithelial neoplasms, we examined a series of tumors which contain abundant stroma, including morpheic type BCC (MBCC), desmoplastic trichoepitheliomas (DTE), and microcystic adnexal carcinomas (MAC). The spindle-shaped cells surrounding the epithelial islands of the two malignant tumors, MBCCs and MACs, were negative in 70% and 100% of cases, respectively, for CD34. In contrast, the spindle-shaped cells surrounding the islands of the benign DTEs were positive for CD34 in 80% of cases. The results suggest that whereas stromal cells surrounding DTEs resemble the CD34-positive perifollicular cells, the spindle-shaped stromal cells surrounding MBCC and MAC are CD34 negative, and may be derived from sources other than the normal mesenchymal tissue surrounding cutaneous appendages.

Cutaneous angiosarcoma as a second malignancy in a lymphedematous leg in a Hodgkin's disease survivor.

Angiosarcoma is a rare and highly malignant neoplasm. Cutaneous angiosarcoma not associated with postmastectomy lymphedema is rare. There are only eight reported cases of development of cutaneous angiosarcoma in a lymphedematous extremity in a patient with preexisting malignancy other than breast cancer. This is the first reported case of development of cutaneous angiosarcoma in a lymphedematous extremity in a Hodgkin's disease survivor. A review of cutaneous angiosarcoma associated with lymphedema and a discussion of the pathogenesis of angiosarcoma are included.

CD34 staining pattern distinguishes basal cell carcinoma from trichoepithelioma.

BACKGROUND AND DESIGN: Trichoepithelioma is a benign skin tumor with follicular differentiation, which sometimes is difficult to distinguish clinically and histologically from basal cell carcinoma. One of the most helpful differences is the histologic appearance of the stroma. CD34 is an antigen known to stain the spindle-shaped cells located around the middle portion of normal hair follicles. We have stained formalin-fixed, paraffin-embedded sections of 16 trichoepitheliomas and 19 basal cell carcinomas for CD34 (anti-HPCA-1, Becton Dickinson, San Jose, Calif) to detect differences in the staining pattern and to facilitate discrimination of these two types of tumors. RESULTS: The spindle-shaped cells surrounding the islands of trichoepithelioma cells were focally strongly positive for CD34. In all basal cell carcinomas, the spindle-shaped cells surrounding the nests of tumor cells were negative; in these areas only the blood vessels were positive with this antibody. CONCLUSIONS: CD34 staining pattern differentiates between trichoepithelioma and basal cell carcinoma. CD34 stain may be helpful in distinguishing between these two tumors on small punch biopsies or in difficult diagnostic cases.

Ethanol inhibits electrically-induced calcium transients in isolated rat cardiac myocytes.

The fluorescent Ca2+ indicator fura-2 was used to follow cytosolic Ca2+ transients during excitation-contraction coupling in suspensions of isolated rat heart cells induced to beat synchronously by electrical field stimulation. The Ca2+ transient reached a maximum at about 30 ms after application of the electrical stimulus and then relaxed to the basal level over the following 200 ms. Treatment of the myocytes with 0.25 to 2.0% ethanol (40 to 340 mM) caused a decrease in the peak of the Ca2+ transient, with no apparent change in the time to peak. This effect of ethanol occurred progressively over a period of about 1 min before a new stable state was achieved. At 1% ethanol the peak Ca2+ level was reduced by 50%. Ethanol reversed the stimulatory effect of isoproterenol on peak Ca2+ and at high levels of ethanol the beta-adrenergic agonist no longer caused any enhancement of the Ca2+ transient. Ethanol did not cause any marked change in the basal Ca2+ level between beats. The effects of ethanol were readily reversible. These results suggest that the negative inotropic effect of ethanol observed in intact cardiac muscle preparations may result in part from interference with the Ca2+ fluxes responsible for excitation-contraction coupling in ventricular myocytes.