Clinicat and Experimental Dermatology 1997; 22: 157-158.

Scottish Dermatological Society Abstracts219th Meeting: Aberdeen, 13 February 1997

Acute urticaria as a cutaneous manifestation of the antiphospholipidsyndrome - a first report. Isabelle C. Hay and A.D. Ormerod. Ward29, Aberdeen Royal Infirmary, Foresterhill, Aberdeen AB2S 2ZN, UK

We report the case of a 31 year old female referred urgently by her GPwith a severe episode of urticaria. Due to the extensive nature of theurticaria her GP had commenced her on oral Prednisolone. Onexamination she had widespread urticaria and extensive livedo reti-cularis which pointed to the diagnosis.

She has an interesting past medical history. She was diagnosed ashaving Raynaud's phenomenon in 1987 with investigations for "inter-mittent claudication" proving unfruitful. She was admitted to medicalwards on several occasions in 1992/93 with chest pain and palpita-tions. At that time she was found to have evidence of mixed aorticvalve disease. SLE was suspected but ANF was negative. In 1995 shewas admitted to a psychiatric hospital with "hypomania/depression"which resolved spontaneously. She was para 2 -|- 0.

This patient's protracted and varied medical history is explained bythe most recent investigation revealing a markedly raised anticardio-lipin antibody. She has Ijeen diagnosed as having the antiphospholipidsyndrome (APS).

APS is similar to SLE with a plethora of systemic manifestations andis a condition which can be fatal if not recognised and treatedappropriately. The syndrome has numerous cutaneous manifestationswhich include livedo reticularis, thrombophlebitis, skin ulceration andsplinter haemorrhages. Whilst APS is now recognised more fre-quently, we propose that acute urticaria should also be included inthe list of cutaneous manifestations.

References

1. Fitzgerald D, Heagerty A. English J. Cold urticaria as occupationaldermatitis in APS. Contact Dermatitis 1995; 32: 328.

2. Krivda S, Beard S, Kovach J. Cutaneous ulceration and a newcardiac murmur in a young woman. Archives of Dermatology 1995;131: 897-899.

An unusual hypersensitivity reaction to denture base resin constitu-ents. S.C. Barclay and A. Forsyth*. Department of Periodontology,Newcastle Dental Hospital, Netpcastle upon Tyne and *Contact DermatitisInvestigation Unit, Glasgow Royal Infirmary, Glasgow, UK

Allergy to the commonly used denture base resins is infrequentlyreported. When they have been reported, most acrylic allergies havebeen described as local contact allergic reactions, with few reportsidentifying any significant systemic symptoms^.

This paper reports a case where the patient suffered extensivesystemic symptoms which were strongly linked to denture wear.

A variety of alternative dentures of different resin content wereconstructed over time with varying reactions. The patient was patch-tested and responded with positive reactions to pure dye samplessupplied by manufacturers, of the resins. She has also failed to react todentures made in a clear acrylic with no dye components. Thesefactors strongly support the hypothesis that the reactions experiencedby this patient to some denture resins is the result of the incorporatedcolouring agents. Only one other report in the literature appears, inconsidering allergic reactions to acrylic resins in dentures, to haverecognised the possibility of dyestuffs being involved in the patientsreaction to the prostheses'.

It is therefore suggested that in cases where an allergy withsystemic manifestations to a denture base resin is suspected, question-ing with regard to other reactions to colourants and patch testing fordyestuffs should be considered in addition to the use of a resin with nocolouring agents in construction of replacement prostheses.

1. Weaver RE, Goebel WM. Reactions in acrylic resin dental^rosthtsts. j Prosthet Dent 1980, 43, 138-142.

2. Devlin H and Watts DC. Acrylic "Allergy".? Brit. Dent J 1984,157, 272-75.

3. Sim J. Allergic reaction to Denture Base Material. J Can DentAssoc 1958, 24, 292-94.

Porphyria cutanea tarda and sideroblastic anaemia: treatment withdesferrioxamine. A. Butler, R. Dawe* and D. Bilsland. Department ofHaematology, Southern General Hospital, Glasgow and *Department ofPhotobiology, Ninemells Hospital, Dundee, UK

Sideroblastic anaemia associated with porphyria has been reportedpreviously in 6 patients and we report a further case successfullymanaged by the use of the iron chelating agent, desferrioxamine.

A 70 year old female was noted to have a normochromic,normocytic anaemia (91 g/1) in Jan 1993. Bone marrow examina-tion demonstrated ring sideroblasts, consistent with idiopathicsideroblastic anaemia.

In April 1995 she developed a blistering eruption associated withskin fragility on the dorsum of both hands which healed leaving miliaand hyperpigmentation. Biochemistry confirmed porphyria cutaneatarda.

The presence of sideroblastic anaemia presented difficulty inmanagement as the treatment of choice, venesection, would haveexacerbated the anaemia without reducing iron load (serum ferritin770ug/l,NR 20-300).

The patient was commenced on desferrioxamine 1.5 g sc 5 days/week over a six month period. Clinical improvement with a reductionin fragility and blistering was noted within one month of commencingtreatment. Clinical remission was observed at 6 months andwas paralleled by sequential falls in serum ferritin and urinaryporphyrins.

Patients with porphyria cutanea tarda commonly have abnormal-ities in iron metabolism. Increased iron absorption has been demon-strated and 80% have hepatic siderosis. In addition iron ingestion can

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158 ABSTRACTS

provoke disease activity, while depletion of body iron stores caninduce remission. In this case, desferrioxamine was used successfullyas an alternative to phlebotomy which was contraindicted. The casehighlights the potential for this iron chelator in the management ofporphyra cutanea tarda in the context of other medical conditionswhich preclude the use of phlebotomy.

References

1. Photosensitivity, abnormal porphyrin profile and sideroblasticanaemia.Lim HW. Cooper D, Sassa S et al. J Am Acad Dermatol 1992: 27,287-292.

2. Iron removal therapy in porphyria cutanea tarda: phlebotomyversus slow subcutaneous desferrioxamine infusion.Rocchi E, Gibertini P, Cassanelli M et al BrJ Dermatol 1986: 114:621-629.

Erythrokeratoderma variabiiis in a North Eastern Scottish kindredmaps to the same region on Chromosome lp as other European andNorth American kindreds. S.M. Morley, W.H.I. McLean, E.B.Lane*, N. Haitesf, M. White and J. HewittJ. *CRC Cell StructureGroup, University of Dundee, and Departments of ^Genetics andXDermatology, Aberdeen Royal Infirmary, Foresterhill, Aberdeen AB252ZN, UK

Erythrokeratoderma variabiiis (EKV), first described by Mendes daCosta in 1925 is a rare autosomal dominant inherited disorder. It ismanifested by discrete erythematous scaly patches, often symmetricaland commonly affecting face, buttocks and limbs. The striking featureof the conditions is the variability of the size and position of thelesions. Keratoderma of palms and soles may occur but teeth and nailsare generally normal. Linkage analysis first reported in 1988' sug-gested EKV was close to the Rh locus on Chromosome lp, and thishas been confirmed and further pinpointed to an approximately 5cMregion on Ip34 by Richards et al, in 1996 .

We have performed linkage analysis on a large pedigree fromNE Scotland (10 affected members) with similar probes, and con-firmed linkage in this pedigree also to the same locus on chromosomelp. This extends the evidence for genetic homogeneity in EKV infamilies from French-Canadian, Swiss, Dutch and now Scottishorigin. Richards et al suggest that two members of the connexingene family which map to the same region, encoding gap junctionproteins, are potential candidate genes. It is proposed that the familiesare combined for further analysis in collaboration with Richards et al.

'van der Schroeff e< al, 1988. Hum Genet 80: 97-98.^Richard et al. 1996. J Invest Dermatol 106: 586.

Nitric oxide synthase correlates with the severity of the psoriaticlesion. A.D. Ormerod and P. Copeland. Ward 29, Aberdeen RoyalInfirmary, Foresterhill, Aberdeen AB25 2ZN, UK

We have previously reported significant increases in inducible nitricoxide synthase (iNOS) in psoriasis by quantitative APAAP immuno-histochemical detection with a mouse monoclonal aiNOS (Transduc-tion labs)'. We and others have since shown the RNA message foriNOS to be upregulated by RT-PCR. As nitric oxide has both pro-infiammatory and immunosuppressive and cytostatic actions, it couldbe having a suppressive function rather than contributing to thepathogenesis.

With the same archive material in which positive staining wasdetermined in paired lesional and non-lesional psoriatic biopsiesfrom 8 subjects and a strept avidin biotin complex immunoperoxidasemethod, we stained for CD3 (pan T cell marker) and Ki67 (prolif-erating cell nuclear antigen) to determine the immunological andproliferative activity in each biopsy. Computerised image analysis wasused to quantify staining and Spearman's two sided correlationcoefficient tested using SPSS statistical software. iNOS stainingcorrelated with CD3 (rank coefficient 0.93 p< 0.0005) and Ki67(rank coefficient 0.88 p< 0.0005). Ki67 and CD3 also correlate(0.78, p < 0.0005). Similar correlations were found when only lesionalbiopsies were included.

With plastic devices to trap air above psoriatic lesions and chemi-luminescent detection ^ of NO in 48 measurements from 12 psoriaticpatients, NO production was also shown to correlate with scores forerythema (rank coefficient 0.37, /)<0.013).

As iNOS and NO correlate with active disease, it is implied that NOparticipates in promoting pathogenic events in psoriasis.

1. Ormerod AD, Benjamin H, Herriot R, Ralston SH. Induciblenitric oxide synthase is upregulated in psoriasis. Clin ExperDermatol 1995; 20: 277.

2. Weller R, Ormerod AD. Increased expression of inducible nitricoxide synthase. BrJ Dermatol 1997; 136:136-137.

© 1997 Blackwell Science Ltd, Climcal and Experimental Dermatology, 11, 1S7-158