Derek J. Cripps

Porphyria turcica due to hexachlorobenzene: a 20 to 30 year follow-up study on 204 patients

During 1955–1961 in south‐east Turkey, over 3000 patients developed porphyria due to ingestion of hexachlorobenzene, a fungicide added to wheat seedlings. Subsequently they developed pigmentation, hirsutism, weakness, porphyrinuria and bullae. The condition was called kara yara or ‘black sore’. Many of the breast‐fed children under the age of I year died from a disease known as pembe yara or ‘pink sore’.

PUVA and skin cancer: A historical cohort study on 492 patients

BACKGROUND The safety of psoralen plus ultraviolet A (PUVA) light therapy has been an issue of debate. A few multiple-center cooperative studies have reported an increase of basal cell and squamous cell carcinomas among PUVA-treated patients. In our institute, more than 1000 patients have been treated with PUVA since 1975. OBJECTIVE We investigated the incidence of skin cancer among patients who received high doses of PUVA to see whether such incidence increased. METHODS This is a historical cohort study of two comparison groups of patients. Subjects under study were 492 psoriasis patients who received PUVA treatments between 1975 and 1989. One group of 103 patients, defined as the high-dose group, received an accumulated PUVA dose of 1000 joules/cm2 or more; another group of 389 patients, as the low-dose group, received 200 joules/cm2 or less. The occurrence of skin cancer in the two comparison groups is analyzed. RESULTS In the high-dose group we observed an increased number of patients with squamous cell carcinoma, keratoacanthoma, and actinic keratosis. We did not see any patients with genital cancer, melanoma, or an increased number of patients with basal cell carcinoma. CONCLUSION The risk of squamous cell carcinoma developing in patients who received a high dose of PUVA is confirmed. We speculate a combination of factors, including PUVA, may contribute to this risk.

Action spectra of topical psoralens: a re-evaluation

The action spectra for producing minimal phototoxic erythema with topical 0 1% trimethyl psoralen (TMP) and 8‐mcthoxypsoralen (8‐MOP) were determined with a double monochromator in the range of 295–380 nm. Both psoralens induced photosensitivity in the range of 313–365 nm; TMP was 54% more effective than 8‐MOP. There was no difference in the dose needed to produce minimal UV erythema or phototoxic erythema with 8‐MOP and TMP at 295 and 305 nm, but at 313 nm with 8‐MOP, photosensitivity was enhanced 3.5 times, and with TMP, sensitivity was enhanced 55 times. The peak sensitivity with 8‐MOP was at 330 nm and for TMP it was 335 nm. No photosensitivity occurred above 380 nm. Results suggest that TMP and 8‐MOP are significantly photoreactive at 320–335 nm. Commonly used UV‐A light sources show peak emission around 360 nm. If there is a relationship between development of erythema and therapeutic effectiveness then this raises the possibility of alternative UV light sources for phototherapy with psoralens.

Rothmund-Thomson syndrome: A case report, phototesting, and literature review

Rothmund-Thomson syndrome is a rare hereditary syndrome with developmental defects. Characteristics of this syndrome, based on a review of 107 reported cases in the literature, are (in descending order) as follows: early onset of poikiloderma, short stature, absence or sparseness of eyebrow and/or eyelash hair, familial juvenile cataracts, small hands and bone defects, sunlight sensitivity, hypogonadism, defective dentition, nail abnormality, hyperkeratosis, and mental retardation. Recently we encountered a 25-year-old white woman who had developed this syndrome but without juvenile cataracts, hypogonadism, or mental retardation. She had developed a basal cell epithelioma, which has not previously been described in this syndrome. Phototesting with monochromatic radiation and with a solar simulator showed photosensitivity in the ultraviolet A range but not in the ultraviolet B range. This case may represent an example of the Thomson type. The case is described and the literature reviewed.

Selective T cell killing of human lymphocytes by ultraviolet radiation

Abstract The effects of ultraviolet radiation (uv) on human B and T lymphocytes were studied. In vitro studies showed that T lymphocytes were more sensitive to uv than B lymphocytes as assessed by eosin-dye exclusion. Following uv exposure, the viable lymphocytes responded to mitogens (PHA, PWM), and functional B lymphocytes were present at a time when no viable T cells were detected. Varying doses of uv were required to abrogate different in vitro responses (proliferative response to antigen or allogeneic cells, MIF production, and cell-mediated lympholysis). In vivo , uv was able to diminish an established cutaneous delayed hypersensitivity response. In vitro uv treatment of parental mouse spleen cells eliminated a graft-versus-host reaction in F 1 recipients as determined by the spleen index. The basis for the differential effect of uv on B and T lymphocyte viability and functional responses is unknown.

Comparative efficacy of oral erythromycin versus oral tetracycline in the treatment of acne vulgaris. A double-blind study.

The efficacy of erythromycin base (E-Mycin tablets, 333 mg) and the efficacy of tetracycline hydrochloride (Panmycin tablets) were compared in this double-blind, randomized study. Two hundred patients with moderate to moderately severe acne vulgaris were randomly assigned to the study. One hundred patients received 1 gm of erythromycin base by mouth per day for 4 weeks, followed by 333 mg/day for 8 weeks, plus placebo for tetracycline. The second group of patients received 1 gm of tetracycline by mouth per day for 4 weeks, followed by 500 mg/day for 8 weeks, plus placebo for erythromycin. Both drugs reduced acne severity to the same extent. Pustules, papules, and open comedo counts decreased significantly over the 12-week period. Seventy-seven percent of the erythromycin-treated patients and 89% of the tetracycline-treated patients stated that their acne was markedly improved or improved by week 12. Most of the side effects in patients treated with erythromycin were gastrointestinal symptoms. Among the side effects in patients treated with tetracycline were Candida vaginitis in one patient and pseudotumor cerebri in one patient.

ULTRAVIOLET ACTION SPECTRUM WITH A PRISM‐GRATING MONOCHROMATOR

SUMMARY.— A prism‐grating monochromator has been assembled for use with various xenon or xenon–mercury radiation sources. The main advantage of the double monochromator is the low level of stray radiation. The disadvantage of the double instrument is the lowered intensity of radiation at the final exit; comparisons of various arc sources showed that a 2·5 kW xenon–mercury lamp gave adequate output in the ultraviolet (UV) range for action spectra studies. The UV action spectra were determined in normal subjects at 8 and 24 hr. and the results have been compared with those of other investigators.

Multiple glomus tumors.

A man had widespread, slowly evolving vascular lesions since infancy suggestive of the Blue Rubber Bleb Nevus Syndrome. His son had two painless lesions typical of Multiple Glomus Tumors. Many of the mans nodular lesions were painful. Post‐excision recurrences were noted. Histologic studies of asymptomatic tumors from both cases showed irregular, dilated, vascular channels surrounded by narrow mantles of glomus cells, whereas a painful tumor had large foci of glomus cells with wider mantles around the flattened channels. Electron microscopy showed the glomus cells to be modified smooth muscle cells. The anatomy and pathology of glomus tumors are reviewed. Differentiation from other syndromes of multiple hemangiomata, particularly the Blue Rubber Bleb Nevus Syndrome is stressed. It is suggested that Multiple Glomus Tumors may be derived from simple cutaneous vessels and not the Sucquet‐Hoyer canal of the normal cutaneous glomus body described by Masson in 1924.

Porphyria and lipid proteinosis. A clinical, histological and biochemical comparison of 19 South African cases.

AT an International Congress on Porphyria held in Cape Town in 1963, it was suggested that a skin condition resembling lipid protoinosis could he produced hy erythropoietic protoporphyria (Findlay, lllfilJ). Cripps (1964) [irovided the first pnhlished evidence that tliis was in fact the case. The relationships hetween li|)id proteinosis and porphyria of various types have now heen specially studied in li> white South African patients. They comprise -•) patients with erythropoietic protopor}>hvria with changes like lipid proteino.̂ is, 7 with pure lipid proteinosis, 6 with variegate porphyria with changes like lipid proteinosis and one who appeared to have both lipid proteinosis and orythropoietic protoporphyria. A general description of tlie case findings ia given here, individual details heing summarized in the appendix.

Erythropoietic protoporphyria: hepatic cirrhosis.

Observations on liver pathology and function were made on 12 patients with erythropoietic protoporphyria (EPP). Needle liver biopsies in 3 of 11 patients showed mild portal or periportal fibrosis without evidence of abnormal liver function. One patient (Case 12) died from hepatic cirrhosis and failure at the age of 11 years, the youngest yet reported. He was not suspected of pending liver disease until 3 months before death. The quantity of protoporphyrin present in his liver was approximately 575% of total liver weight. Fatal liver disease in EPP has occurred in 13 patients, 7 male and 6 female, in which the mean age of death was 38 years. The pathology of the liver in EPP is characteristic and is described.