Proc. roy. Soc. Med Volume 69 May 1976
20
patient had developed a linear band of ulcers round the leg, and gave a dissociated answer when questioned about an elastic band. She continued to produce other artifact lesions until she retired from work she resented. Should not child abuse be considered in the p-esent case ?
Discussion Hewitt et al. (1975) described 9 cases of pemphigus from D-penicillamine, commonly of the seborrhceic type (presumably Senear-Usher-like). In some the eruption cleared when D-penicillamine was stopped, but in others new lesions appeared. This patient is still developing lesions four months after discontinuance of the drug. The mechanism of pemphigus production by penicillamine is uncertain. Penicillamine is a sulphur amino acid with a close stereochemical relationship to the naturally occurring amino acid cysteine. Several distinct pharmacological actions of penicillamine are recognized. Because it acts as a chelating agent it is used for heavy metal poisoning, Wilson's disease, cystinosis and cystinuria. It also acts as a decomplexing agent and is used for macroglobulincmia and, as it splits aldamine cross-links in the collagen molecule, in rheumatoid arthritis. Sinfilarly, it can break disulphide linkages and perhaps this is relevant for pemphigus production. A possibility is that the mechanism is immunological, comparable with the drug-induced SLE syndrome.
384
Dr F A Ive: A similar type of phenomenon of chronic constriction of bodily appendages by threads and human hair has been described by Quinn (1971, Pediatrics 48, 145). Dr Knight: The capacity of rubber bands to erode through skin and deeper tissues is well known by some farmers who use them to castrate sheep. Dr A A Ross: I know of a case where a woman's wedding ring was too tight for her finger, worked through the skin and became covered by that tissue. This over the years produced a groove in the underlying bone.
Penicillamine-induced Pemphigus R J Cairns FRCP (St Bartholomew's Hospital, Rochester, Kent)
REFERENCE Hewitt J, Benveniste M & Lessana-Leibowitch M (1975) British Journal of Dermatology Supplement No. 2, p 12
J F, man aged 51. Storekeeper History: In November 1973 commenced Dpenicillamine 500 mg daily for rheumatoid arthritis. The dose was slowly increased and by March 1974 he was taking 1500 mg daily. In February 1975 he noted a scaly area I cm across on the right deltoid region and found the surface abraded easily. When seen in the skin clinic in The following cases were also shown: June 1975 he had many Senear-Usher-like lesions, 1-2 cm across, on the trunk, upper arms and Xeroderma Pigmentosum thighs. Some appeared on sites of trauma. Dr R J Cairns Nikolsky's sign positive. Despite the discontinu- Familial Palmo-plantar Keratoderma ance of D-penicillamine new lesions continue to with Dermatophytosis Dr H M Barnes (for Dr I Sarkany) appear. Previous history: No atopy. No penicillin COliac Disease, Dermatitis Herpetiformis and Hypothyroidism sensitivity. On examination: On the affected sites are round Dr D Thompson and oval discs, 1-2 cm across. Some of the areas (for Dr A G White and Dr C M Ridley) show small vesicles and flaccid bulle. The surface Severe Motor Involvement in Morphcea abrades easily. The seborrhoeic areas are not Dr D A Burns (for Dr C D Calnan) Intestinal Involvement in Acrosclerosis particularly affected. Investigations: Latex test positive 1/160. Histology Dr D A Burns (for Dr I Sarkany) (Professor Wilson Jones):, 'The section shows Bathing Trunk Ichthyosis eroded epidermis above an intact basal layer. Dr C D Calnan Elsewhere there is evidence of acantholysis.' Juvenile Xanthogranuloma Immunofluorescent studies are typical of pem- Dr T W E Robinson phigus. No circulating anti-intercellular anti- Kokarden Nevus bodies demonstrated. Full blood count, ESR, Dr A P Warin (for Dr G C Wells) antinuclear factor and blood urea normal; no LE (meeting to be continued) cells. Printed in England by Staples Printers Limited, at The Stanhope Press, Rochester, Kent.
20
patient had developed a linear band of ulcers round the leg, and gave a dissociated answer when questioned about an elastic band. She continued to produce other artifact lesions until she retired from work she resented. Should not child abuse be considered in the p-esent case ?
Discussion Hewitt et al. (1975) described 9 cases of pemphigus from D-penicillamine, commonly of the seborrhceic type (presumably Senear-Usher-like). In some the eruption cleared when D-penicillamine was stopped, but in others new lesions appeared. This patient is still developing lesions four months after discontinuance of the drug. The mechanism of pemphigus production by penicillamine is uncertain. Penicillamine is a sulphur amino acid with a close stereochemical relationship to the naturally occurring amino acid cysteine. Several distinct pharmacological actions of penicillamine are recognized. Because it acts as a chelating agent it is used for heavy metal poisoning, Wilson's disease, cystinosis and cystinuria. It also acts as a decomplexing agent and is used for macroglobulincmia and, as it splits aldamine cross-links in the collagen molecule, in rheumatoid arthritis. Sinfilarly, it can break disulphide linkages and perhaps this is relevant for pemphigus production. A possibility is that the mechanism is immunological, comparable with the drug-induced SLE syndrome.
384
Dr F A Ive: A similar type of phenomenon of chronic constriction of bodily appendages by threads and human hair has been described by Quinn (1971, Pediatrics 48, 145). Dr Knight: The capacity of rubber bands to erode through skin and deeper tissues is well known by some farmers who use them to castrate sheep. Dr A A Ross: I know of a case where a woman's wedding ring was too tight for her finger, worked through the skin and became covered by that tissue. This over the years produced a groove in the underlying bone.
Penicillamine-induced Pemphigus R J Cairns FRCP (St Bartholomew's Hospital, Rochester, Kent)
REFERENCE Hewitt J, Benveniste M & Lessana-Leibowitch M (1975) British Journal of Dermatology Supplement No. 2, p 12
J F, man aged 51. Storekeeper History: In November 1973 commenced Dpenicillamine 500 mg daily for rheumatoid arthritis. The dose was slowly increased and by March 1974 he was taking 1500 mg daily. In February 1975 he noted a scaly area I cm across on the right deltoid region and found the surface abraded easily. When seen in the skin clinic in The following cases were also shown: June 1975 he had many Senear-Usher-like lesions, 1-2 cm across, on the trunk, upper arms and Xeroderma Pigmentosum thighs. Some appeared on sites of trauma. Dr R J Cairns Nikolsky's sign positive. Despite the discontinu- Familial Palmo-plantar Keratoderma ance of D-penicillamine new lesions continue to with Dermatophytosis Dr H M Barnes (for Dr I Sarkany) appear. Previous history: No atopy. No penicillin COliac Disease, Dermatitis Herpetiformis and Hypothyroidism sensitivity. On examination: On the affected sites are round Dr D Thompson and oval discs, 1-2 cm across. Some of the areas (for Dr A G White and Dr C M Ridley) show small vesicles and flaccid bulle. The surface Severe Motor Involvement in Morphcea abrades easily. The seborrhoeic areas are not Dr D A Burns (for Dr C D Calnan) Intestinal Involvement in Acrosclerosis particularly affected. Investigations: Latex test positive 1/160. Histology Dr D A Burns (for Dr I Sarkany) (Professor Wilson Jones):, 'The section shows Bathing Trunk Ichthyosis eroded epidermis above an intact basal layer. Dr C D Calnan Elsewhere there is evidence of acantholysis.' Juvenile Xanthogranuloma Immunofluorescent studies are typical of pem- Dr T W E Robinson phigus. No circulating anti-intercellular anti- Kokarden Nevus bodies demonstrated. Full blood count, ESR, Dr A P Warin (for Dr G C Wells) antinuclear factor and blood urea normal; no LE (meeting to be continued) cells. Printed in England by Staples Printers Limited, at The Stanhope Press, Rochester, Kent.
