Clinical and Experimental Dermatology 1992; 17: 279-28t.
ILVEN responding to occlusive potent topical steroid therapy R.CERIO, E.WILSON JONES AND R.A.J.EADY St. Thomas' Hospital. London
St. John's Dermatology Centre,
Accepted for publication 1 October 1991
Summary We report two adult patients with inflamtnatory linear verrucous epidermal naevi (ILVEN) who failed to respond to treatment hy surgical excision or with dithranol hut benefited from topical fluorinated steroids with occlusion.
Case reports Patient 1 A 37-year-old Ghanaian woman presented with a 14-year hi.story of a pruritic linear lesion affecting the right forearm. The lesion had not responded to numerous topical reagents including fluorinated steroids. Previous attempted surgical excision of an area over her wrist resulted in recurrence. There was no personal or family history of psoriasis. On examination she had a linear psoriasiform lesion corresponding to the tines of Blaschko. It consisted of discrete hyperkeratotic coalescing plaques (Fig. 1). Residual scarring from previous surgery was evident. No classical signs of psoriasis were seen. Mycological examination was negative. Histology showed prominent psoriasiform acanthosis, patchy spongiosis and columns of alternating orthokeratotic and parakeratotic hyperkeratosis. There was an associated moderately chronic inflammatory infiltrate in the underlying upper dermis but Munro microabscesses were absent (Fig. 2). Using toluidene blue and the chloracetate esterase reaction an increa.sed number of mast cells were identified in the underlying dermis. After 4 months treatment with 0-05% clobetasol propionate with 5"o salicylic acid oitment under occlusion there was a marked improvement: the psoriasiform lesions had largely disappeared leaving residual patchy hypopigmentation. The scar resulting from previous surgery was clearly visible (Fig. 3a). The patient remains well 3 years later on intermittent 0-05"o betamethasone ointment with S^,, salicylic acid. The lesion recurs if therapy is stopped.
Figure 1. Case t: Inflammatory tinear verrucous naevus (ILVEN) affecting the outer right forearms. Lesions consist of descrete psoriasiform plaques corresponding to the linear of Blaschtto,
F i g u r e 2 . HlJ.toioy,_\ \\u\\\
l...as(.- i O t i l i y i i s l i j i m u iiLa]kt:u p s o n i i ^ i l o i i i i
acanthosis, patchy spongiosis and atternating orthokeratosis and paralceratosis. There is a moderate upper dermat perivascutar lympho histiocytic infittrate (Originat x 65 H & E),
Patient 2
A 49-year-old Caucasian male presented with a linear psorasiform lesion corresponding to lines of Blaschko on the posterior left leg since he was a teenager. His main Correspondence: Dr Rino Cerio, Department of Dermatotogy, The symptom was intense pruritus. Despite the prolonged use Royal London Hospital, Whitechapel, London El IBB, UK, of numerous topical therapies there was no therapeutic 279
280
R.CKRIO, E.WILSON JONES AND R.A.J.EAHY
Figure 3. (a) (iase 1: Dramatic therapeutic response to 4 nionihs therapy with potent topical steroids under occlusion, Rcsiduat hypopigmentation is seen proximatt_\ with scarring following previous surgery distally, (b)(;ase 1: After 4 months treatment, showing loss of psorisiform acanthosis, parat^eratosis and reduced dermal inflammation with some telangiectasia. The biopsy was taken adjacent to that shown in i'"igure 1 {Original x 250 H & E).
benefit from steroids, salicylic acid and Ingram's regime. His cousin had mild psoriasis. The naevus extended the whole length of the limb involving the buttock, posterior thigh, calf, and medial and dorsal aspects of the foot (Fig. 4). Similarly, he demonstrated no classical signs of psoriasis. Histology was very similar to the preceding case with the same degree of dermal inflammation. Similarly, he received 005*',) clobetasol propionate ointment under occlusion for 3 months and although clinical response was not as dramatic {Fig. 5) the patient remained asymptomatic on intermittent topical treatment 2 years later. On therapy repeat skin biopsies on both cases revealed reversal of epidermal thickening and absence of dermal infiammation {Fig. 3b). In addition there appeared to be a subjective reduction in mast cell numbers using special stains.
Figure 4. Case 2: ILVP^N affecting most of the buttock and tower timb with smiitar clinical features to Patient t.
Discussion Inflammatory linear verrucous epidermal naevus {ILVEN) can be distinguished from lichen striatus because of its refractory clinical course, intense pruritus, resistance to therapy and histology.' It is a relatively rare disorder first described by Unna in 18%.' The condition was reported as 'dermatitic naevus' by Calnan in 1956' but the term ILVEN^ has been adopted by most clinicians. Most patients are children but adults can be affected/ It is four times more common in females than males and curiously the left lower limb is the commonest
OCCLUSIVE POTENT TOPICAL STEROID THERAPY
281
including surgery as illustrated by Patient 1. Only generous excision to include underlying dermis may prevent recurrence. In smaller lesions cryotherapy has been usCAil.' The unsuccessful response of both patients to treatment with tar, dithranol and UVB supports the concept that ILVEN is not just linear or naevoid psoriasis. Both our cases illustrate the therapeutic henefit of potent topical steroid therapy under occlusion. Fox and Lapins** described a patient with ILVEN who responded to intralesional injections of cortico-steroids. However, there was only a temporary decrease in hyperkeratosis and erythema. Recent studies had demonstrated that topical (luorinated steroids applied under occlusion will cause a reduction in numbers or even total depletion of identifiable dermal mast cells." In both our patients there appeared to he a reduction in mast-cell numbers following topical steroid therapy. Moreover, mast-cell degranulation has been found to be a feature of developing lesions in psoriasis.'^ It is interesting to speculate that these cells may also have a role in the psoriasiform hyperplasia and intense pruritis which is characteristic of this uncommon disorder. References
Figure S. Case 2: Marked improvement both symptomaticalty and ctinicalty fottowing occtiisive therapy with potent topical steroids.
site. Characteristically, as demonstrated by our two patients, the lesion is psoriasiform both clinically and histopathologically. Some instances of purported linear or naevoid psoriasis could probably be reclassified as ILVEN, particularly as most of these patients did not have psoriasis elsewhere,'' Although not diagnostic, the typical alternating bands of ortho- and parakeratosis with lack of neutrophils have been used to differentiate ILVEN from psoriasis.'** Most treatment for this condition is unsatisfactory^"*
t. Kd. Rook A, Wilkinson DS, Ebling FJG, Champion RM, Burton JL, Test Book of Derrnatology Vot. t, 4th F.dn, Oxford: Btackwett Scientific Pubtications, 171-172, 2, Unna PG, l'he Histopathology of Diseases of the Shin. New York: MacMillan, 1896; 1148, 3. Calnan CD, Fxzematous tinear naevus. Creut Ormond Street Journal 1956; 10: 66 71, 4, Attman J, Mehregan AU, Inflammatory tinear verruco.se epidermal naevus. Archives of Dermatology 1971; 104: 383 389, 5, Hodge SJ, Barr JM, Owen Ig. Inflammatory linear verrucose epidermal naevus, .irchives oJ Dermatology t97X; 114: 436-438. 6, Bennett RG, Burns L, Graywood M, Systematized epidermat naevi. Arehives of Dermatotogy t973; 108: 705-707. 7. Toribio J, Quinoncs B, Inflammatory linear verrucous epidermat naevus. Archives of Dermatotogy 1975; 150: 65-69, 8. Dupre A, C^hristot B, Inflammatory tinear verrucous epidermal naevus a pathotogic study. Archives oJ Dermatotogy t977; 113: 767-769. 9. Fox BJ, Lapins NA. Comparison of treatment modalities of epidermat naevus. A case report and vcvievi. Joumat of Dermatologie Surgery and Oncology t983; 9: 879 885," 10, Rulo HFC, Van de Kerkhof PCM. Treatment of Inflammatory tinea verrucous epidermal naevus, Dermatologica 1991; 182: 112114, 11 Barton J, l.avker RM, Schechter NM, Lazarus S, Treatment of urticaria pigmentosa with corticosteroids. Archives of Dermatotogy t9X.S; 121: 15t6-t523, t2, Brody I, Mast cetl degranulation in the evotution of Acute eruptive guttate psoriasis vutgaris, 7"'"'""/';/ Investigative Dermatotogy 1984; 82: 460-464,
ILVEN responding to occlusive potent topical steroid therapy R.CERIO, E.WILSON JONES AND R.A.J.EADY St. Thomas' Hospital. London
St. John's Dermatology Centre,
Accepted for publication 1 October 1991
Summary We report two adult patients with inflamtnatory linear verrucous epidermal naevi (ILVEN) who failed to respond to treatment hy surgical excision or with dithranol hut benefited from topical fluorinated steroids with occlusion.
Case reports Patient 1 A 37-year-old Ghanaian woman presented with a 14-year hi.story of a pruritic linear lesion affecting the right forearm. The lesion had not responded to numerous topical reagents including fluorinated steroids. Previous attempted surgical excision of an area over her wrist resulted in recurrence. There was no personal or family history of psoriasis. On examination she had a linear psoriasiform lesion corresponding to the tines of Blaschko. It consisted of discrete hyperkeratotic coalescing plaques (Fig. 1). Residual scarring from previous surgery was evident. No classical signs of psoriasis were seen. Mycological examination was negative. Histology showed prominent psoriasiform acanthosis, patchy spongiosis and columns of alternating orthokeratotic and parakeratotic hyperkeratosis. There was an associated moderately chronic inflammatory infiltrate in the underlying upper dermis but Munro microabscesses were absent (Fig. 2). Using toluidene blue and the chloracetate esterase reaction an increa.sed number of mast cells were identified in the underlying dermis. After 4 months treatment with 0-05% clobetasol propionate with 5"o salicylic acid oitment under occlusion there was a marked improvement: the psoriasiform lesions had largely disappeared leaving residual patchy hypopigmentation. The scar resulting from previous surgery was clearly visible (Fig. 3a). The patient remains well 3 years later on intermittent 0-05"o betamethasone ointment with S^,, salicylic acid. The lesion recurs if therapy is stopped.
Figure 1. Case t: Inflammatory tinear verrucous naevus (ILVEN) affecting the outer right forearms. Lesions consist of descrete psoriasiform plaques corresponding to the linear of Blaschtto,
F i g u r e 2 . HlJ.toioy,_\ \\u\\\
l...as(.- i O t i l i y i i s l i j i m u iiLa]kt:u p s o n i i ^ i l o i i i i
acanthosis, patchy spongiosis and atternating orthokeratosis and paralceratosis. There is a moderate upper dermat perivascutar lympho histiocytic infittrate (Originat x 65 H & E),
Patient 2
A 49-year-old Caucasian male presented with a linear psorasiform lesion corresponding to lines of Blaschko on the posterior left leg since he was a teenager. His main Correspondence: Dr Rino Cerio, Department of Dermatotogy, The symptom was intense pruritus. Despite the prolonged use Royal London Hospital, Whitechapel, London El IBB, UK, of numerous topical therapies there was no therapeutic 279
280
R.CKRIO, E.WILSON JONES AND R.A.J.EAHY
Figure 3. (a) (iase 1: Dramatic therapeutic response to 4 nionihs therapy with potent topical steroids under occlusion, Rcsiduat hypopigmentation is seen proximatt_\ with scarring following previous surgery distally, (b)(;ase 1: After 4 months treatment, showing loss of psorisiform acanthosis, parat^eratosis and reduced dermal inflammation with some telangiectasia. The biopsy was taken adjacent to that shown in i'"igure 1 {Original x 250 H & E).
benefit from steroids, salicylic acid and Ingram's regime. His cousin had mild psoriasis. The naevus extended the whole length of the limb involving the buttock, posterior thigh, calf, and medial and dorsal aspects of the foot (Fig. 4). Similarly, he demonstrated no classical signs of psoriasis. Histology was very similar to the preceding case with the same degree of dermal inflammation. Similarly, he received 005*',) clobetasol propionate ointment under occlusion for 3 months and although clinical response was not as dramatic {Fig. 5) the patient remained asymptomatic on intermittent topical treatment 2 years later. On therapy repeat skin biopsies on both cases revealed reversal of epidermal thickening and absence of dermal infiammation {Fig. 3b). In addition there appeared to be a subjective reduction in mast cell numbers using special stains.
Figure 4. Case 2: ILVP^N affecting most of the buttock and tower timb with smiitar clinical features to Patient t.
Discussion Inflammatory linear verrucous epidermal naevus {ILVEN) can be distinguished from lichen striatus because of its refractory clinical course, intense pruritus, resistance to therapy and histology.' It is a relatively rare disorder first described by Unna in 18%.' The condition was reported as 'dermatitic naevus' by Calnan in 1956' but the term ILVEN^ has been adopted by most clinicians. Most patients are children but adults can be affected/ It is four times more common in females than males and curiously the left lower limb is the commonest
OCCLUSIVE POTENT TOPICAL STEROID THERAPY
281
including surgery as illustrated by Patient 1. Only generous excision to include underlying dermis may prevent recurrence. In smaller lesions cryotherapy has been usCAil.' The unsuccessful response of both patients to treatment with tar, dithranol and UVB supports the concept that ILVEN is not just linear or naevoid psoriasis. Both our cases illustrate the therapeutic henefit of potent topical steroid therapy under occlusion. Fox and Lapins** described a patient with ILVEN who responded to intralesional injections of cortico-steroids. However, there was only a temporary decrease in hyperkeratosis and erythema. Recent studies had demonstrated that topical (luorinated steroids applied under occlusion will cause a reduction in numbers or even total depletion of identifiable dermal mast cells." In both our patients there appeared to he a reduction in mast-cell numbers following topical steroid therapy. Moreover, mast-cell degranulation has been found to be a feature of developing lesions in psoriasis.'^ It is interesting to speculate that these cells may also have a role in the psoriasiform hyperplasia and intense pruritis which is characteristic of this uncommon disorder. References
Figure S. Case 2: Marked improvement both symptomaticalty and ctinicalty fottowing occtiisive therapy with potent topical steroids.
site. Characteristically, as demonstrated by our two patients, the lesion is psoriasiform both clinically and histopathologically. Some instances of purported linear or naevoid psoriasis could probably be reclassified as ILVEN, particularly as most of these patients did not have psoriasis elsewhere,'' Although not diagnostic, the typical alternating bands of ortho- and parakeratosis with lack of neutrophils have been used to differentiate ILVEN from psoriasis.'** Most treatment for this condition is unsatisfactory^"*
t. Kd. Rook A, Wilkinson DS, Ebling FJG, Champion RM, Burton JL, Test Book of Derrnatology Vot. t, 4th F.dn, Oxford: Btackwett Scientific Pubtications, 171-172, 2, Unna PG, l'he Histopathology of Diseases of the Shin. New York: MacMillan, 1896; 1148, 3. Calnan CD, Fxzematous tinear naevus. Creut Ormond Street Journal 1956; 10: 66 71, 4, Attman J, Mehregan AU, Inflammatory tinear verruco.se epidermal naevus. Archives of Dermatology 1971; 104: 383 389, 5, Hodge SJ, Barr JM, Owen Ig. Inflammatory linear verrucose epidermal naevus, .irchives oJ Dermatology t97X; 114: 436-438. 6, Bennett RG, Burns L, Graywood M, Systematized epidermat naevi. Arehives of Dermatotogy t973; 108: 705-707. 7. Toribio J, Quinoncs B, Inflammatory linear verrucous epidermat naevus. Archives of Dermatotogy 1975; 150: 65-69, 8. Dupre A, C^hristot B, Inflammatory tinear verrucous epidermal naevus a pathotogic study. Archives oJ Dermatotogy t977; 113: 767-769. 9. Fox BJ, Lapins NA. Comparison of treatment modalities of epidermat naevus. A case report and vcvievi. Joumat of Dermatologie Surgery and Oncology t983; 9: 879 885," 10, Rulo HFC, Van de Kerkhof PCM. Treatment of Inflammatory tinea verrucous epidermal naevus, Dermatologica 1991; 182: 112114, 11 Barton J, l.avker RM, Schechter NM, Lazarus S, Treatment of urticaria pigmentosa with corticosteroids. Archives of Dermatotogy t9X.S; 121: 15t6-t523, t2, Brody I, Mast cetl degranulation in the evotution of Acute eruptive guttate psoriasis vutgaris, 7"'"'""/';/ Investigative Dermatotogy 1984; 82: 460-464,
