446
CORRESPONDENCE
Case i. A 21-year-old woman, skin type 2. with long-standing psoriasis, had previously received four courses of oral 8-MOP PUVA and two courses of Tl,-01 phototherapy with good results, and no adverse effects. During her third course of narrow-band UVB (TL-01) treatment she developed painful blistering affecting psoriatic plaques on her shoulders, 24 h after hertwenty-third treatment atan irradiation dose of 1856 mj/cm'. representing 2 4 times her initial MED. The blisters healed in 2 days and treatment was resumed using our routine protocol, with complete clearance of her psoriasis. Case 4. A 44-year-old male psoriatic, skin type 2, receiving his first course of narrow-band UVB phototherapy, developed painful blistering on psoriatic plaques in the interscapular area and the backs of his shoulders. This occurred after his seventeenth treatment session at a dose of 1745 mJ/cm-. which was 4 5 times his initial MED. Routine haematological and biochemical parameters were normal, as were plasma and urine porphyrin analyses. Antinuclear antibody was negative. A skin biopsy showed a large blister containing fibrin and occasional neutrophil polymorphs. The roof of the blister was formed by necrotic keratinocytes. In places, the lack of an underlying epithelium suggested a subepidermal split. Elsewhere, there was underlying epithelium lacking a granular layer, probably indicating re-epithelialization. There was a perivascular lymphocytic inflammatory infiltrate in the superficial dermis. Immunofluorescence was negative. Interestingly, this patient had previously had a course of PUVA and had developed pseudoporphyria on the dorsa of his feet in the later stages of his treatment.
British Journal of Dermatology {1992) 1 2 7 .
two of the four reported cases were asymptomatic incidental findings. Photobiology Unit. NineweUs Hospital. Dundee DDl 9SY. U.K.
.
S.A.GEORGE ).FERGIISON
Reference 1 Green C. Ferguson J. Lakshmipathi T. Joiinson BE. 311 nm UVB phototherapy—an effective treatment for psoriasis. Br / Dermatol 1988: 119:691-6. Discoid lupus erythematosus-like lesions in carriers of X-linked chronic granulomalous disease
Blistering during PUVA therapy following mild trauma (pseudoporphyria) is well described. Our cases do not conform to this pattern because only lesional skin was affected. Similarly, the lack of significant erythema and the unusual distribution of the lesions differentiates them from the acute blistering that may result from a generalized overdose of UV exposure. This blistering phenomenon, which in our experience is not a feature of broad-band UVB (Philips TL-1 2) phototherapy, has no obvious explanation, it is possible that plaques of psoriasis. during a course of treatment with narrow-band phototherapy, have not gained the photoprotection level of surrounding normal skin. The treatment efficacy of this new UVB source may result in a loss of photoprotection by causing a rapid loss of plaque scales at involved sites, thus exposing them to what, for these areas, is a burning dose. The onset of blistering in ail our patients during the second half of their treatment supports this hypothesis. It is probable that a gentler incremental irradiation regimen would reduce the risk of lesional blistering, but this must be balanced against a possible decrease in clearance rates. We are now investigating the therapeutic efficacy of less aggressive irradiation regimens than that which we are currently using.
SiR.J.H.SillevisSmitteifl/.' found discoid lupus erythematosuslike skin lesions with atypical histological findings and negative direct immunofluorescence (DIF) in five of 1 5 carriers of the X-linked cytochrome-b-558-negative variant of chronic granulomatous disease (CGI)). In contrast, we have seen a carrier of CGD with a centrofacial skin eruption resembling perioral dermatitis, thus clinically atypical for discoid lupus erythematosus (DLE). whereas histology and DIF were typical of DLK. Our patient, a 29-year-old woman, has a 5-year-old son with CGD and is a proven carrier of the disease. Recurrent facial rashes after sun exposure began at the age of 13. at first affecting only the cheeks, but later also the forehead and chin. On examination a centrofacial erythema, consisting of confiuent maculo-papules sparing the immediate perioral area. was present, producing a picture suggestive of perioral dermatitis. She was otherwise well. Histology was consistent with lupus erythematosus. showing an atrophic epidermis with focal hydropic degeneration of the basal layer. In the dermis there was a patchy lymphocytic infiltrate around dilated blood vessels and hair follicles. Direct immunofluorescence showed granular IgG. IgM and IgA deposits at the basement membrane zone. Tests for antinuclear antibodies, anti-native DNA. anti-SM antigen and rheumatoid factor were negative. Serum protein electrophoresis and urinalysis were normal. Sensitivity to ultraviolet light (short and long wavelength) was normal. The nitroblue-tetrazolium slide test showed 28% positive ceils (normal range 98-100%),^ and superoxide anion production after granulocyte stimulation by phorbol myrestate acetate was 2 0 0 nmol ()2/million granulocytes/min (normal range ]0-52± 1 8J nmol/million/ min).- Cytochrome b determined by dithionate difference spectroscopy was 1-62 pmol/million granulocytes (normal range 6 4 ± 0 7 9 pmol/million).^ The facial eruption cleared within 3 weeks on treatment with chloroquine 2 50 mg daily, sun protection and a mild topical steroid (c!obetasone-l 7butyrate 0-05%).
This is our first experience of lesional blistering during psoriasis phototherapy and leads us to question whether this may be unique to narrow-band (TL-01) UVB treatment. It is possible that the incidence of this phenomenon is higher, as
Brandrup ft a!? studied skin and oral mucosa in an unselected series of 9 CGD-carriers. They described deep rosacea-like infiltrates of the face, resembling LE profundus. In addition, changes similar to chilblain I.F were present on the
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.
CORRESPONDENCE
Case i. A 21-year-old woman, skin type 2. with long-standing psoriasis, had previously received four courses of oral 8-MOP PUVA and two courses of Tl,-01 phototherapy with good results, and no adverse effects. During her third course of narrow-band UVB (TL-01) treatment she developed painful blistering affecting psoriatic plaques on her shoulders, 24 h after hertwenty-third treatment atan irradiation dose of 1856 mj/cm'. representing 2 4 times her initial MED. The blisters healed in 2 days and treatment was resumed using our routine protocol, with complete clearance of her psoriasis. Case 4. A 44-year-old male psoriatic, skin type 2, receiving his first course of narrow-band UVB phototherapy, developed painful blistering on psoriatic plaques in the interscapular area and the backs of his shoulders. This occurred after his seventeenth treatment session at a dose of 1745 mJ/cm-. which was 4 5 times his initial MED. Routine haematological and biochemical parameters were normal, as were plasma and urine porphyrin analyses. Antinuclear antibody was negative. A skin biopsy showed a large blister containing fibrin and occasional neutrophil polymorphs. The roof of the blister was formed by necrotic keratinocytes. In places, the lack of an underlying epithelium suggested a subepidermal split. Elsewhere, there was underlying epithelium lacking a granular layer, probably indicating re-epithelialization. There was a perivascular lymphocytic inflammatory infiltrate in the superficial dermis. Immunofluorescence was negative. Interestingly, this patient had previously had a course of PUVA and had developed pseudoporphyria on the dorsa of his feet in the later stages of his treatment.
British Journal of Dermatology {1992) 1 2 7 .
two of the four reported cases were asymptomatic incidental findings. Photobiology Unit. NineweUs Hospital. Dundee DDl 9SY. U.K.
.
S.A.GEORGE ).FERGIISON
Reference 1 Green C. Ferguson J. Lakshmipathi T. Joiinson BE. 311 nm UVB phototherapy—an effective treatment for psoriasis. Br / Dermatol 1988: 119:691-6. Discoid lupus erythematosus-like lesions in carriers of X-linked chronic granulomalous disease
Blistering during PUVA therapy following mild trauma (pseudoporphyria) is well described. Our cases do not conform to this pattern because only lesional skin was affected. Similarly, the lack of significant erythema and the unusual distribution of the lesions differentiates them from the acute blistering that may result from a generalized overdose of UV exposure. This blistering phenomenon, which in our experience is not a feature of broad-band UVB (Philips TL-1 2) phototherapy, has no obvious explanation, it is possible that plaques of psoriasis. during a course of treatment with narrow-band phototherapy, have not gained the photoprotection level of surrounding normal skin. The treatment efficacy of this new UVB source may result in a loss of photoprotection by causing a rapid loss of plaque scales at involved sites, thus exposing them to what, for these areas, is a burning dose. The onset of blistering in ail our patients during the second half of their treatment supports this hypothesis. It is probable that a gentler incremental irradiation regimen would reduce the risk of lesional blistering, but this must be balanced against a possible decrease in clearance rates. We are now investigating the therapeutic efficacy of less aggressive irradiation regimens than that which we are currently using.
SiR.J.H.SillevisSmitteifl/.' found discoid lupus erythematosuslike skin lesions with atypical histological findings and negative direct immunofluorescence (DIF) in five of 1 5 carriers of the X-linked cytochrome-b-558-negative variant of chronic granulomatous disease (CGI)). In contrast, we have seen a carrier of CGD with a centrofacial skin eruption resembling perioral dermatitis, thus clinically atypical for discoid lupus erythematosus (DLE). whereas histology and DIF were typical of DLK. Our patient, a 29-year-old woman, has a 5-year-old son with CGD and is a proven carrier of the disease. Recurrent facial rashes after sun exposure began at the age of 13. at first affecting only the cheeks, but later also the forehead and chin. On examination a centrofacial erythema, consisting of confiuent maculo-papules sparing the immediate perioral area. was present, producing a picture suggestive of perioral dermatitis. She was otherwise well. Histology was consistent with lupus erythematosus. showing an atrophic epidermis with focal hydropic degeneration of the basal layer. In the dermis there was a patchy lymphocytic infiltrate around dilated blood vessels and hair follicles. Direct immunofluorescence showed granular IgG. IgM and IgA deposits at the basement membrane zone. Tests for antinuclear antibodies, anti-native DNA. anti-SM antigen and rheumatoid factor were negative. Serum protein electrophoresis and urinalysis were normal. Sensitivity to ultraviolet light (short and long wavelength) was normal. The nitroblue-tetrazolium slide test showed 28% positive ceils (normal range 98-100%),^ and superoxide anion production after granulocyte stimulation by phorbol myrestate acetate was 2 0 0 nmol ()2/million granulocytes/min (normal range ]0-52± 1 8J nmol/million/ min).- Cytochrome b determined by dithionate difference spectroscopy was 1-62 pmol/million granulocytes (normal range 6 4 ± 0 7 9 pmol/million).^ The facial eruption cleared within 3 weeks on treatment with chloroquine 2 50 mg daily, sun protection and a mild topical steroid (c!obetasone-l 7butyrate 0-05%).
This is our first experience of lesional blistering during psoriasis phototherapy and leads us to question whether this may be unique to narrow-band (TL-01) UVB treatment. It is possible that the incidence of this phenomenon is higher, as
Brandrup ft a!? studied skin and oral mucosa in an unselected series of 9 CGD-carriers. They described deep rosacea-like infiltrates of the face, resembling LE profundus. In addition, changes similar to chilblain I.F were present on the
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.
