CORRESPONDENCE
British Journal of Dermatology (1992) 127.
fitigers and toes. The histological changes ranged from almost pure lymphocytic infiltration of the Jessner-Kanof type to features consistent with IJi, and changes similar to chronic dermatitis. This variahitity in histoiogical changes is demonstrated by the finding of an appearance consistent with LE in our case, whereas in the cases of Sillevis Smitt et «/.' histological findings generally did not show all the elements typical of I,R. The pathogenesis of IJi-like manifestations in carriers of CCiD is unknown. By immunophenotyping of dermal infiltrates in CGI) carriers. Sillevis Sniitt t'l «/.' showed that these infiltrates did not differ from those in common DM',. This suggests that the pathogenesis is the same. In CGD, ineffective phagocytosis might lead to chronic antigenaemia. eventually provoking autoantibody formation. On the other hand, negative autoimmunological findings in most cases are an important argument against this theory/^ Other common mucocutaneous disorders in carriers of CC^U are recurrent aphthous stomatitis (RAS)' '^ and hidradenitis suppurativa.'^ In contrast with common RAS, ulcers occur predominantly on the gingivae. show a more extensive erythematous halo, and have negative DIF. Prenatal diagnosis is available for pregnancies at risk of C(iD. In order to establish an effective procedure to detect and counsel carriers, women with DEJ^ in combination with recurrent aphthous stomatitis, hidradenitis suppurativa, or a family history of early childhood deaths should he screened.'* Departiiifut of DeriniHohgy. fJniversitii Hospital of Zurich, 'Aurich. Switzerland
Median nail dystrophy associated with isotretinoin therapy SIR, Aromatic retinoids are known to cause various dystrophic nail changes. These include splitting, softening, shedding of the nail and chronic paronychia.' Whilst chronic paronychia is seen during therapy with isotretinoin the other changes are encountered more frequently in association with etretinate therapy. We report the case of a 38-year-old woman who developed a median canaliform dystrophy of a thumb nail, a change not previously reported following treatment with either etretinate or isotretinoin. The patient had severe nodulocystic acne which required three separate 4-montb courses of isotretinoin (i mg/kg/day). The time intervals between each course varied from 9 to 12 months. Six weeks after commencing each of the courses of isotretinoin she developed an obvious central ridge and proximal splitting. On both sides of the central defect there were horizontal ridges, although these were more prominent on one aspect and at the proximal end. where a fir-^tree configuration could be seen (Fig. 1). These dystrophic changes subsequently resolved approximately 4 weeks after the isotretinoin was discontinued. The patient had no previous personal or family history of nail abnormalities. Whilst we accept that median canaliform dystrophy might occur by chance in a patient taking isotretinoin, the temporal relationship to the multiple courses strongly implicates isotretinoin as the causative agent. The aetiology of median canaliform dystrophy is unknown but it is typically seen in one or more nails, the thumb-nail being most commonly
A.ENDERLIN
R.A.SEGtR" B.WiiTHRlCH L.BRUCKNER-TL DERM ANN*
*Childri'n'fi Hospital. Division of Immunologij and Haematology, University of Zurich. Steinwiesslr 7 5 . HOU 'Zurich. Switzerland.
P.PANEZZONI G.BlJRG
References 1 Sillevis Sniitl |H, Weening RS. Kricg SR. Bos |D. Discoid lupus erythematosus-iike lesions in carriers of X-Iinked chronic granulomatous disease. Hr //>miflW/ 1990: 122: 643-50. 2 Miihlebach T). Hrny C. Suter R et al Analysis of various types of chronic granulomaluus disease with the monoclonal antibody 7D5 directed against the small subunit of surface cytochrome b^^s. I'nlkar .AUcrgii Immunol 1991: 2: 124-30. J Brandrup I'. Koch C. Petri M cI al. Discoid iupus erytheraatosus-like iesions and stomatitis in female carriers of X-iinked chronic granuiomatous disease. Br j Dermatal 1981: 104: 495-505. 4 Barton LL. Johnson CR. Discoid lupus erythematosus and X-linked chronic granulomatous disease. Pediatr Dermatol l9Sb; i: J76-9. 5 ("iarioch JJ. Sampson JR. Sey wright M. Thomson J. Dermatoses in five related female carriers of X-linked chronic granulomatous disease. BrlDermatol 1989; 121: 391-6. (i Schaller ]. Illness resembling lupus erythemalosus in mothers of boys with chronic granulomatous disease. Ann/rilern Med 1972: 76: 747-50.
447
Figure 1. Median naii dystrophy, VMIIJ [in and ridging.
448
CORRESPONDENCE
British Journal of Dermatology (1992) 127.
involved. It has a tendency to spontaneous remission and can occasionally be familial. In this case the cause of the dystrophy may be a dyskeratotic reaction of the keratinocytes in a localized area of the nail matrix induced by isotretinoin in a predisposed individual. We are not aware of any recorded cases of a drug-induced median canaliform dystrophy. Department of Dermatology. The General Infirmary at Leeds. Great George Street. Ueds. U.K.
W. W.BOTTOMLEY W.J.CUNLIFFE
References 1 Baran R. Retlnoids and the nails. / Dermatohgical Treatment 1990; 1: 151-4. Extracorporeal photochemolherapy in the treatment of severe psoriatic arthropathy SrR. The efficacy of extracorporeal photochemotherapy (EP) in psoriasis and psoriatic arthropathy was first evaluated by Wilfert ('( a/.' At present, the value of this treatment is questionable. We would like to add our experience of the use of EP in the management of one patient with long-standing psoriatic arthropathy. resistant to conventional treatment. A 29-year-oid man had a 1 3-year history of psoriasis and psoriatic polyarthritis. His skin had been treated with topical steroids, short-contact anthralin therapy. PUVA. etretinate. methotrexate IMTX) and cyclosporin A, following established therapeutic schedules. Arthritic symptoms improved only with oral phenylbutazone IPBZ). Although there was a marked decrease in PASI score (from bS-i) to 14-7). his arthropathy remained active, with pain, morning stiffness, disabling weakness and significant radiographic changes. In February 1991. while he was receiving treatment with weekly MTX and daily PBZ. the patient was included in an KP programme (treatment on 2 consecutive days, with a treatment-free period of 4 weeks, using a Therakos UVAR System). In August 1991. he showed a decrease in PASI score (from 14-7 to 1 0 ) and marked reduction in pain and morning stiffness, resulting in adequate mobility. Based on animal models.' the efficacy of EP on inflammatory and autoimmune diseases is at present being evaluated, with encouraging results. '--^ After 6 months treatment with FP, our patient showed a moderate improvement of both skin lesions and arthropathy. During this period, the oral MTX dose was gradually reduced from 1 5 mg weekly to 10 mg every 4 weeks, and at the end of the treatment period, the initial PBZ dose of 200 mg daily was reduced to 2(H) mg on alternate days. No adverse effects were observed. Our data agree with those of Wilfert et o/.' and Vonderheid et air Thus, EP could be a safe and effective alternative for the treatment of refractory cases of psoriatic arthropathy. However, further objective studies are necessary to establish the real efficacy of such an expensive and time-consuming procedure.
Department of Dermatology.
Universidad de Alcald de Renares, Hospital Ramon y Cajal. Apartado 37.
R.F.DE M I S A
J.M.AZANA A.HARTO A.LKIX)
28034-Madrid. Spain Correspondence: Dr A.Ledo. Paseo de la Castellana 167. 28n46-Madrid. Spain
References 1 Wilfert H, Honigsmann H, Steiner G et al. Treatment of psoriatic arthritis by extracorporeal photocheraotherapy. Brf Dermatol 1990; 122:225-32. 2 Vonderheid EC. Bigler RD. Rogers Tj et al. Effect of extracorporeal photopheresis on selected immunologic parameters in psoriasis vulgaris. Yale } Biol Med 19S9: 62: 65 3-64. 5 Ben-Nun A. Wekerle H.Cohen IR. Vaccination against autoimmune cnLTphalomyclitis with T-lymphocyle line cells reactive against myelin basic protein, feature 1981: 292: 6 0 - 1 . 4 Rook AH. Heald PW. Nahass C.J ei ul. Treatment of autoimmune disease with extracorporeal photochemotherapy: pemphigus vulgaris—preliminary report. Yale Biol Med 1989: 62: 647-52.
Malignant melanoma occurring as a second cancer following primary germ cell tumour in males: report of three
SIR, We report three male patients with malignant melanoma which developed following a primary germ cell tumour. None of the patients had the familial cancer syndrome or the familial atypical mole/melanoma syndrome. The occurrence of a second malignancy in cancer patients is well known, and it is important for those involved in their management to be alert to this possibility. The occurrence of a malignant tumour is distressing for any affected individual. Despite the reassurance of effective therapy now available for many primary malignancies, resulting in long-term survival, it is now well known that such an individual faces an increased risk of developing a second malignancy. The following three cases illustrate the development of malignant melanoma after the occurrence of a pritnary germ cell tumour (seminoma or teratoma) in young adult males. Case 1. A 26-year-old man presented with right testicular swelling in March 1986. He was found to have stage 1 seminoma, for which a right orchidectoniy was performed. In February 1987. he noticed gradual enlargement of a pigmented lesion over his left scapula. Fxcision biopsy of the 1-6 X 1 6 cm lesion showed a malignant melanoma 0-8 mm thick, with invasion of the papillary dermis (Clark level 2). Following this, wide excision with primary closure was undertaken. There was no family history of dyspiastic naevi or
British Journal of Dermatology (1992) 127.
fitigers and toes. The histological changes ranged from almost pure lymphocytic infiltration of the Jessner-Kanof type to features consistent with IJi, and changes similar to chronic dermatitis. This variahitity in histoiogical changes is demonstrated by the finding of an appearance consistent with LE in our case, whereas in the cases of Sillevis Smitt et «/.' histological findings generally did not show all the elements typical of I,R. The pathogenesis of IJi-like manifestations in carriers of CCiD is unknown. By immunophenotyping of dermal infiltrates in CGI) carriers. Sillevis Sniitt t'l «/.' showed that these infiltrates did not differ from those in common DM',. This suggests that the pathogenesis is the same. In CGD, ineffective phagocytosis might lead to chronic antigenaemia. eventually provoking autoantibody formation. On the other hand, negative autoimmunological findings in most cases are an important argument against this theory/^ Other common mucocutaneous disorders in carriers of CC^U are recurrent aphthous stomatitis (RAS)' '^ and hidradenitis suppurativa.'^ In contrast with common RAS, ulcers occur predominantly on the gingivae. show a more extensive erythematous halo, and have negative DIF. Prenatal diagnosis is available for pregnancies at risk of C(iD. In order to establish an effective procedure to detect and counsel carriers, women with DEJ^ in combination with recurrent aphthous stomatitis, hidradenitis suppurativa, or a family history of early childhood deaths should he screened.'* Departiiifut of DeriniHohgy. fJniversitii Hospital of Zurich, 'Aurich. Switzerland
Median nail dystrophy associated with isotretinoin therapy SIR, Aromatic retinoids are known to cause various dystrophic nail changes. These include splitting, softening, shedding of the nail and chronic paronychia.' Whilst chronic paronychia is seen during therapy with isotretinoin the other changes are encountered more frequently in association with etretinate therapy. We report the case of a 38-year-old woman who developed a median canaliform dystrophy of a thumb nail, a change not previously reported following treatment with either etretinate or isotretinoin. The patient had severe nodulocystic acne which required three separate 4-montb courses of isotretinoin (i mg/kg/day). The time intervals between each course varied from 9 to 12 months. Six weeks after commencing each of the courses of isotretinoin she developed an obvious central ridge and proximal splitting. On both sides of the central defect there were horizontal ridges, although these were more prominent on one aspect and at the proximal end. where a fir-^tree configuration could be seen (Fig. 1). These dystrophic changes subsequently resolved approximately 4 weeks after the isotretinoin was discontinued. The patient had no previous personal or family history of nail abnormalities. Whilst we accept that median canaliform dystrophy might occur by chance in a patient taking isotretinoin, the temporal relationship to the multiple courses strongly implicates isotretinoin as the causative agent. The aetiology of median canaliform dystrophy is unknown but it is typically seen in one or more nails, the thumb-nail being most commonly
A.ENDERLIN
R.A.SEGtR" B.WiiTHRlCH L.BRUCKNER-TL DERM ANN*
*Childri'n'fi Hospital. Division of Immunologij and Haematology, University of Zurich. Steinwiesslr 7 5 . HOU 'Zurich. Switzerland.
P.PANEZZONI G.BlJRG
References 1 Sillevis Sniitl |H, Weening RS. Kricg SR. Bos |D. Discoid lupus erythematosus-iike lesions in carriers of X-Iinked chronic granulomatous disease. Hr //>miflW/ 1990: 122: 643-50. 2 Miihlebach T). Hrny C. Suter R et al Analysis of various types of chronic granulomaluus disease with the monoclonal antibody 7D5 directed against the small subunit of surface cytochrome b^^s. I'nlkar .AUcrgii Immunol 1991: 2: 124-30. J Brandrup I'. Koch C. Petri M cI al. Discoid iupus erytheraatosus-like iesions and stomatitis in female carriers of X-iinked chronic granuiomatous disease. Br j Dermatal 1981: 104: 495-505. 4 Barton LL. Johnson CR. Discoid lupus erythematosus and X-linked chronic granulomatous disease. Pediatr Dermatol l9Sb; i: J76-9. 5 ("iarioch JJ. Sampson JR. Sey wright M. Thomson J. Dermatoses in five related female carriers of X-linked chronic granulomatous disease. BrlDermatol 1989; 121: 391-6. (i Schaller ]. Illness resembling lupus erythemalosus in mothers of boys with chronic granulomatous disease. Ann/rilern Med 1972: 76: 747-50.
447
Figure 1. Median naii dystrophy, VMIIJ [in and ridging.
448
CORRESPONDENCE
British Journal of Dermatology (1992) 127.
involved. It has a tendency to spontaneous remission and can occasionally be familial. In this case the cause of the dystrophy may be a dyskeratotic reaction of the keratinocytes in a localized area of the nail matrix induced by isotretinoin in a predisposed individual. We are not aware of any recorded cases of a drug-induced median canaliform dystrophy. Department of Dermatology. The General Infirmary at Leeds. Great George Street. Ueds. U.K.
W. W.BOTTOMLEY W.J.CUNLIFFE
References 1 Baran R. Retlnoids and the nails. / Dermatohgical Treatment 1990; 1: 151-4. Extracorporeal photochemolherapy in the treatment of severe psoriatic arthropathy SrR. The efficacy of extracorporeal photochemotherapy (EP) in psoriasis and psoriatic arthropathy was first evaluated by Wilfert ('( a/.' At present, the value of this treatment is questionable. We would like to add our experience of the use of EP in the management of one patient with long-standing psoriatic arthropathy. resistant to conventional treatment. A 29-year-oid man had a 1 3-year history of psoriasis and psoriatic polyarthritis. His skin had been treated with topical steroids, short-contact anthralin therapy. PUVA. etretinate. methotrexate IMTX) and cyclosporin A, following established therapeutic schedules. Arthritic symptoms improved only with oral phenylbutazone IPBZ). Although there was a marked decrease in PASI score (from bS-i) to 14-7). his arthropathy remained active, with pain, morning stiffness, disabling weakness and significant radiographic changes. In February 1991. while he was receiving treatment with weekly MTX and daily PBZ. the patient was included in an KP programme (treatment on 2 consecutive days, with a treatment-free period of 4 weeks, using a Therakos UVAR System). In August 1991. he showed a decrease in PASI score (from 14-7 to 1 0 ) and marked reduction in pain and morning stiffness, resulting in adequate mobility. Based on animal models.' the efficacy of EP on inflammatory and autoimmune diseases is at present being evaluated, with encouraging results. '--^ After 6 months treatment with FP, our patient showed a moderate improvement of both skin lesions and arthropathy. During this period, the oral MTX dose was gradually reduced from 1 5 mg weekly to 10 mg every 4 weeks, and at the end of the treatment period, the initial PBZ dose of 200 mg daily was reduced to 2(H) mg on alternate days. No adverse effects were observed. Our data agree with those of Wilfert et o/.' and Vonderheid et air Thus, EP could be a safe and effective alternative for the treatment of refractory cases of psoriatic arthropathy. However, further objective studies are necessary to establish the real efficacy of such an expensive and time-consuming procedure.
Department of Dermatology.
Universidad de Alcald de Renares, Hospital Ramon y Cajal. Apartado 37.
R.F.DE M I S A
J.M.AZANA A.HARTO A.LKIX)
28034-Madrid. Spain Correspondence: Dr A.Ledo. Paseo de la Castellana 167. 28n46-Madrid. Spain
References 1 Wilfert H, Honigsmann H, Steiner G et al. Treatment of psoriatic arthritis by extracorporeal photocheraotherapy. Brf Dermatol 1990; 122:225-32. 2 Vonderheid EC. Bigler RD. Rogers Tj et al. Effect of extracorporeal photopheresis on selected immunologic parameters in psoriasis vulgaris. Yale } Biol Med 19S9: 62: 65 3-64. 5 Ben-Nun A. Wekerle H.Cohen IR. Vaccination against autoimmune cnLTphalomyclitis with T-lymphocyle line cells reactive against myelin basic protein, feature 1981: 292: 6 0 - 1 . 4 Rook AH. Heald PW. Nahass C.J ei ul. Treatment of autoimmune disease with extracorporeal photochemotherapy: pemphigus vulgaris—preliminary report. Yale Biol Med 1989: 62: 647-52.
Malignant melanoma occurring as a second cancer following primary germ cell tumour in males: report of three
SIR, We report three male patients with malignant melanoma which developed following a primary germ cell tumour. None of the patients had the familial cancer syndrome or the familial atypical mole/melanoma syndrome. The occurrence of a second malignancy in cancer patients is well known, and it is important for those involved in their management to be alert to this possibility. The occurrence of a malignant tumour is distressing for any affected individual. Despite the reassurance of effective therapy now available for many primary malignancies, resulting in long-term survival, it is now well known that such an individual faces an increased risk of developing a second malignancy. The following three cases illustrate the development of malignant melanoma after the occurrence of a pritnary germ cell tumour (seminoma or teratoma) in young adult males. Case 1. A 26-year-old man presented with right testicular swelling in March 1986. He was found to have stage 1 seminoma, for which a right orchidectoniy was performed. In February 1987. he noticed gradual enlargement of a pigmented lesion over his left scapula. Fxcision biopsy of the 1-6 X 1 6 cm lesion showed a malignant melanoma 0-8 mm thick, with invasion of the papillary dermis (Clark level 2). Following this, wide excision with primary closure was undertaken. There was no family history of dyspiastic naevi or
