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Linear psoriasis with porokeratotic eccrine ostial and dermal duct nevus
Linear psoriasis with porokeratotic eccrine ostial and dermal duct nevus Hee-Joon Yu, MD, Joo-Youn Ko, MD, Hyeok-Man Kwon, MD, and Jeong-Soo Kim, MD Seoul, Korea Linear psoriasis is an uncommon form of psoriasis characterized by the linear distribution of the psoriatic lesions. It usually follows the lines of Blaschko with unilateral involvement. Poro keratotic eccrine ostial and dermal duct (PEODD) nevus is another rare dermatosis that follows Blaschko’s line. The pathogenesis of linear psoriasis and PEODD nevus is unclear, but both could be best explained by a specific somatic mutation. Hence, it has been suggested that the mutation responsible for PEODD nevus would constitute a rare but critical psoriasis gene. In the literature, 1 case of linear psoriasis with PEODD nevus was reported, which may support this suggestion. This article describes another case of linear psoriasis and PEODD nevus. A 7-year-old boy had a 4-month history of multiple psoriasiform plaques, arranged in linear distribution, and had congenital linear hyperkeratotic papules and pits on the right side of his trunk and right arm. (J Am Acad Dermatol 2004;50:S81-3.)
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inear psoriasis was first described in 1951 by Leslie1 and Sobel.2 It is a rare form of psoriasis and is characterized by a linear distribution of the psoriatic lesions.3-7 Usually, it follows the lines of Blaschko with unilateral involvement. The pathogenesis is unclear, but it could be explained as a result of the migration of cells harboring a somatic mutation following the lines of Blaschko during early embryogenesis.4,7-9 Porokeratotic eccrine ostial and dermal duct (PEODD) nevus, described by Abell and Read10 in 1980, is another rare dermatosis that typically occurs on a linear or bandlike plantar or palmar area, with multiple punctate pits and comedo-like plugs.11-13 It has been suggested that the mutation responsible for PEODD nevus would constitute a rare but critical psoriasis gene within a spectrum of polygenic predisposition for psoriasis. In the literature, one case of linear psoriasis with PEODD nevus has been reported.9 Now, we describe another case of linear psoriasis with PEODD nevus, which may support the above explanation.
CASE REPORT A 7-year-old Korean boy had a 4-month history of eruption comprising multiple psoriasiform plaques that were arranged in a linear distribution along the lines of Blaschko and confined to the right side of the trunk and arm (Fig 1, A). In addition, he had This supplement is made possible through an unrestricted educational grant from Stiefel Laboratories to the American Academy of Dermatology. From the Department of Dermatology, Hanyang University College of Medicine. Funding sources: None. Conflicts of interest: None identified. Presented as a poster at the 60th Annual Meeting of the American Academy of Dermatology, New Orleans, La, February 22-27, 2002. Reprint requests: Hee-Joon Yu, MD, Department of Dermatology, Hanyang University Guri Hospital, College of Medicine, University of Hanyang, Guri City, Gyunggi-do, Seoul, 471-701 Korea. E-mail: [email protected]. 0190-9622/$30.00 © 2004 by the American Academy of Dermatology, Inc. doi:10.1016/S0190-9622(03)02797-X
discrete hyperkeratotic papules and pits with a linear distribution on the right palm (Fig 2) that had been present since birth. Laboratory investigations, including complete blood cell count, blood biochemistry tests, and urinalysis, produced normal findings. A serologic test for syphilis showed a negative result. Skin biopsy specimens of the plaques on the right shoulder and flank revealed typical features of psoriasis, including parakeratosis, Munro’s microabscesses, thinning of the suprapapillary epidermis with spongiform pustules, and acanthosis with regular elongation of the rete ridges (Fig 3). The eccrine glands did not show any abnormality. In addition, immunohistochemical staining with the monoclonal antibody Ks8.12 (antikeratin 16) showed homogeneous distribution pattern in the suprabasal area. However, histologic findings of the hyperkeratotic lesions on the right palm were quite different; there were parakeratotic columns within an epidermal invagination with a loss of the granular layer (Fig 4). The parakeratotic columns arose over eccrine ducts with a dilated acrosyringium. Clinically and histologically, the lesions on the right palm represented PEODD nevus. Subsequently, the boy was given the diagnosis of linear psoriasis with PEODD nevus. After 1 month of treatment with topical steroids, the psoriasis was much improved (Fig 1, B), but PEODD nevus did not show improvement. During the 13-month follow-up period, psoriatic lesions recurred intermittently, but PEODD nevus had not changed.
DISCUSSION The existence and nosologic position of a linear psoriasis has frequently been a subject of debate. Many authors have disputed the existence of true linear psoriasis and contend that many reports of linear psoriasis represent either inflammatory linear verrucous epidermal nevus (ILVEN) in a patient with psoriasis, a nevus,14,15 or even an invasion of a linear epidermal nevus by psoriasis as a manifestation of the isomorphic reaction. In contrast, some authors believe that linear psoriasis is a separate entity. Therefore, before diagnosis of linear psoriasis, it should be differentiated clinically and histopathologically from ILVEN.3,4,14-16 The criteria for the diagnosis of ILVEN established by Altman and Mehregan17 include early age of onset, 4 times more common among women than men, frequent involvement of the left leg, pruritus, psoriasiform appearance, persistence,
S81
S82 Yu et al
J AM ACAD DERMATOL MAY 2004
Fig 1. A, Multiple psoriasiform plaques arranged in linear distribution along lines of Blaschko and confined to right side of trunk and arm. B, Improvement of linear psoriasiform lesions after 1 month of treatment with topical steroids.
Fig 3. Histopathologic findings of psoriatic plaques showing parakeratosis, Munro’s microabscesses, thinning of suprapapillary epidermis with spongiform pustules, and acanthosis with regular elongation of rete ridges. (Hematoxylin-eosin stain; original magnification ⫻100.)
Fig 2. Linear hyperkeratotic papules and pits on right palm, which were coalescing with poriasiform lesion.
and resistance to treatment. Among these criteria, a differential point is that the lesions of ILVEN take the form of intensely pruritic linear groups of excoriated eczematous papules, which proves extremely refractory to therapy. The histopathologic aspect of ILVEN is very similar to psoriasis, but is characterized by columns of hypergranulosis with orthokeratosis alternating with columns of agranulosis with parakeratosis. From an immunohis-
tochemical point of view, ILVEN and psoriasis show certain differences.14 A focal staining pattern of anti-keratin 16 and a homogeneous distribution of anti-keratin 10 were observed in ILVEN. In psoriasis, a homogeneous staining of anti-keratin 16 was observed and anti-keratin 10 staining showed foci of decreased staining. In this case, the psoriasiform lesions observed in the 7-year-old boy were not representative of ILVEN because, above all else, the lesions were improved with topical steroids treatment alone. Furthermore, the antikeratin staining showed a homogenous distribution pattern in the suprabasal area. PEODD nevus was initially reported in 1979 by Marsden et al.18 It was then further described by Abell and Read10 in 1980. It is usually classified among the adnexal tumors with eccrine differentiation.9,11-13 PEODD nevus is another rare dermatosis that follows the lines of Blaschko. The lesions are typically asymptomatic, congenital, keratotic papules or plaques and multiple punctate pits that often display comedo-like keratotic plugs. They are usually localized on the palms and soles, but may occur
J AM ACAD DERMATOL VOLUME 50, NUMBER 5
Fig 4. Histopathologic findings of papules on right palm showing cornoid lamella-like parakeratotic columns extending from acrosyringium. (Hematoxylin-eosin stain; original magnification ⫻100.)
at other distal areas of the extremities and even on multiple locations. Histopathologically, PEODD nevus is characterized by the following: a comedo-like dilatation and hyperplasia of the acrosyringium; prominent parakeratotic cornoid lamella-like plugs within an epidermal invagination; absence of the granular layer; and hamartomatous changes of the eccrine dermal ducts.19 The precise pathogenesis of linear psoriasis and PEODD nevus remains unclear. But linear psoriasis could be explained as a result of the migration of cells harboring a somatic mutation following the lines of Blaschko during early embryogenesis. By contrast, PEODD nevus could be caused by a specific somatic mutation that induces formation of abnormal clone of epidermal cells, producing a cornoid lamella-like column.9,11,19 According to these hypotheses, the co-occurrence of 2 diseases could be best explained in the following way: the mutation responsible for PEODD nevus could constitute a rare but critical psoriasis gene within a spectrum of polygenic predisposition for psoriasis. Actually, in the literature, the reported case of a patient having linear psoriasis with PEODD nevus9 was explained in this way. This case is another example of linear psoriasis with PEODD nevus. This may support the above explanation and may help in the future to elucidate the unknown cause of psoriasis. REFERENCES 1. Leslie G. Linear psoriasis. Br J Dermatol 1951;63:262-3. 2. Sobel M. Linear psoriasis. Arch Dermatol Syph 1951;63:267.
Yu et al S83 3. Ginarte M, Fernandez-Redondo V, Toriboi J. Unilateral psoriasis: a case individualized by means of involucrin. Cutis 2000;65:167-70. 4. Park BS, Youn JI, Cho HK, Lee KH. A case of linear psoriasis. Korean J Dermatol 1996;34:998-1002. 5. Baselga E, Puig L, Llobet J, Musulen E, de Moragas JM. Linear psoriasis associated with systemic lupus erythematosus. J Am Acad Dermatol 1994;30:130-3. 6. Al-Fouzan AS, Hassab-el-Naby HM, Nanda A. Congenital linear psoriasis: a case report. Pediatr Dermatol 1990;7:303-6. 7. Atherton DJ, Kahana M, Russel-Jones R. Naevoid psoriasis. Br J Dermatol 1989;120:837-41. 8. Happle R. Somatic recombination may explain linear psoriasis. J Med Genet 1991;28:337. 9. van de Kerkhof P, Steijlen P, Happle R. Co-occurrence of linear psoriasis and porokeratotic eccrine ostial and dermal duct naevus. Acta Derm Venereol 1993;73:311-2. 10. Abell E, Read SI. Porokeratotic eccrine ostial and dermal duct naevus. Br J Dermatol 1980;103:435-41. 11. Sassmannshausen J, Bogomilsky J, Chaffins M. Porokeratotic eccrine ostial and dermal duct nevus: a case report and review of the literature. J Am Acad Dermatol 2000;43:364-7. 12. Solis J, Sau P, James WD. Puzzling palmar and plantar papules: porokeratotic eccrine ostial and dermal duct nevus. Arch Dermatol 1991; 127:1220-4. 13. Stoof TJ, Starink TM, Nieboer C. Porokeratotic eccrine ostial and dermal duct nevus: report of a case of adult onset. J Am Acad Dermatol 1989; 20:924-7. 14. de Jong E, Rulo H, van de Kerkhof PC. Inflammatory linear verrucous epidermal nevus (ILVEN) versus linear psoriasis: a clinical, histological and immunohistochemical study. Acta Derm Venereol 1991;71: 343-6. 15. Thivolet J, Goujon C. Linear psoriasis and systematized epidermolytic nevus. Arch Dermatol 1982;118:285-6. 16. Menni S, Restano L, Gianotti R, Boccardi D. Inflammatory linear verrucous epidermal nevus (ILVEN) and psoriasis in a child? Int J Dermatol 2000;39:30-2. 17. Altman J, Mehregan AH. Inflammatory linear verrucous epidermal nevus. Arch Dermatol 1971;104:385-9. 18. Marsden R, Fleming K, Dawber R. Comedo naevus of the palm–a sweat duct naevus? Br J Dermatol 1979;101:717-22. 19. Bergman R, Lichtig C, Cohen A, Friedman-Birnbaum R. Porokeratotic eccrine ostial nevus and dermal duct nevus. Am J Dermatopathol 1992; 14:319-22.
L
inear psoriasis was first described in 1951 by Leslie1 and Sobel.2 It is a rare form of psoriasis and is characterized by a linear distribution of the psoriatic lesions.3-7 Usually, it follows the lines of Blaschko with unilateral involvement. The pathogenesis is unclear, but it could be explained as a result of the migration of cells harboring a somatic mutation following the lines of Blaschko during early embryogenesis.4,7-9 Porokeratotic eccrine ostial and dermal duct (PEODD) nevus, described by Abell and Read10 in 1980, is another rare dermatosis that typically occurs on a linear or bandlike plantar or palmar area, with multiple punctate pits and comedo-like plugs.11-13 It has been suggested that the mutation responsible for PEODD nevus would constitute a rare but critical psoriasis gene within a spectrum of polygenic predisposition for psoriasis. In the literature, one case of linear psoriasis with PEODD nevus has been reported.9 Now, we describe another case of linear psoriasis with PEODD nevus, which may support the above explanation.
CASE REPORT A 7-year-old Korean boy had a 4-month history of eruption comprising multiple psoriasiform plaques that were arranged in a linear distribution along the lines of Blaschko and confined to the right side of the trunk and arm (Fig 1, A). In addition, he had This supplement is made possible through an unrestricted educational grant from Stiefel Laboratories to the American Academy of Dermatology. From the Department of Dermatology, Hanyang University College of Medicine. Funding sources: None. Conflicts of interest: None identified. Presented as a poster at the 60th Annual Meeting of the American Academy of Dermatology, New Orleans, La, February 22-27, 2002. Reprint requests: Hee-Joon Yu, MD, Department of Dermatology, Hanyang University Guri Hospital, College of Medicine, University of Hanyang, Guri City, Gyunggi-do, Seoul, 471-701 Korea. E-mail: [email protected]. 0190-9622/$30.00 © 2004 by the American Academy of Dermatology, Inc. doi:10.1016/S0190-9622(03)02797-X
discrete hyperkeratotic papules and pits with a linear distribution on the right palm (Fig 2) that had been present since birth. Laboratory investigations, including complete blood cell count, blood biochemistry tests, and urinalysis, produced normal findings. A serologic test for syphilis showed a negative result. Skin biopsy specimens of the plaques on the right shoulder and flank revealed typical features of psoriasis, including parakeratosis, Munro’s microabscesses, thinning of the suprapapillary epidermis with spongiform pustules, and acanthosis with regular elongation of the rete ridges (Fig 3). The eccrine glands did not show any abnormality. In addition, immunohistochemical staining with the monoclonal antibody Ks8.12 (antikeratin 16) showed homogeneous distribution pattern in the suprabasal area. However, histologic findings of the hyperkeratotic lesions on the right palm were quite different; there were parakeratotic columns within an epidermal invagination with a loss of the granular layer (Fig 4). The parakeratotic columns arose over eccrine ducts with a dilated acrosyringium. Clinically and histologically, the lesions on the right palm represented PEODD nevus. Subsequently, the boy was given the diagnosis of linear psoriasis with PEODD nevus. After 1 month of treatment with topical steroids, the psoriasis was much improved (Fig 1, B), but PEODD nevus did not show improvement. During the 13-month follow-up period, psoriatic lesions recurred intermittently, but PEODD nevus had not changed.
DISCUSSION The existence and nosologic position of a linear psoriasis has frequently been a subject of debate. Many authors have disputed the existence of true linear psoriasis and contend that many reports of linear psoriasis represent either inflammatory linear verrucous epidermal nevus (ILVEN) in a patient with psoriasis, a nevus,14,15 or even an invasion of a linear epidermal nevus by psoriasis as a manifestation of the isomorphic reaction. In contrast, some authors believe that linear psoriasis is a separate entity. Therefore, before diagnosis of linear psoriasis, it should be differentiated clinically and histopathologically from ILVEN.3,4,14-16 The criteria for the diagnosis of ILVEN established by Altman and Mehregan17 include early age of onset, 4 times more common among women than men, frequent involvement of the left leg, pruritus, psoriasiform appearance, persistence,
S81
S82 Yu et al
J AM ACAD DERMATOL MAY 2004
Fig 1. A, Multiple psoriasiform plaques arranged in linear distribution along lines of Blaschko and confined to right side of trunk and arm. B, Improvement of linear psoriasiform lesions after 1 month of treatment with topical steroids.
Fig 3. Histopathologic findings of psoriatic plaques showing parakeratosis, Munro’s microabscesses, thinning of suprapapillary epidermis with spongiform pustules, and acanthosis with regular elongation of rete ridges. (Hematoxylin-eosin stain; original magnification ⫻100.)
Fig 2. Linear hyperkeratotic papules and pits on right palm, which were coalescing with poriasiform lesion.
and resistance to treatment. Among these criteria, a differential point is that the lesions of ILVEN take the form of intensely pruritic linear groups of excoriated eczematous papules, which proves extremely refractory to therapy. The histopathologic aspect of ILVEN is very similar to psoriasis, but is characterized by columns of hypergranulosis with orthokeratosis alternating with columns of agranulosis with parakeratosis. From an immunohis-
tochemical point of view, ILVEN and psoriasis show certain differences.14 A focal staining pattern of anti-keratin 16 and a homogeneous distribution of anti-keratin 10 were observed in ILVEN. In psoriasis, a homogeneous staining of anti-keratin 16 was observed and anti-keratin 10 staining showed foci of decreased staining. In this case, the psoriasiform lesions observed in the 7-year-old boy were not representative of ILVEN because, above all else, the lesions were improved with topical steroids treatment alone. Furthermore, the antikeratin staining showed a homogenous distribution pattern in the suprabasal area. PEODD nevus was initially reported in 1979 by Marsden et al.18 It was then further described by Abell and Read10 in 1980. It is usually classified among the adnexal tumors with eccrine differentiation.9,11-13 PEODD nevus is another rare dermatosis that follows the lines of Blaschko. The lesions are typically asymptomatic, congenital, keratotic papules or plaques and multiple punctate pits that often display comedo-like keratotic plugs. They are usually localized on the palms and soles, but may occur
J AM ACAD DERMATOL VOLUME 50, NUMBER 5
Fig 4. Histopathologic findings of papules on right palm showing cornoid lamella-like parakeratotic columns extending from acrosyringium. (Hematoxylin-eosin stain; original magnification ⫻100.)
at other distal areas of the extremities and even on multiple locations. Histopathologically, PEODD nevus is characterized by the following: a comedo-like dilatation and hyperplasia of the acrosyringium; prominent parakeratotic cornoid lamella-like plugs within an epidermal invagination; absence of the granular layer; and hamartomatous changes of the eccrine dermal ducts.19 The precise pathogenesis of linear psoriasis and PEODD nevus remains unclear. But linear psoriasis could be explained as a result of the migration of cells harboring a somatic mutation following the lines of Blaschko during early embryogenesis. By contrast, PEODD nevus could be caused by a specific somatic mutation that induces formation of abnormal clone of epidermal cells, producing a cornoid lamella-like column.9,11,19 According to these hypotheses, the co-occurrence of 2 diseases could be best explained in the following way: the mutation responsible for PEODD nevus could constitute a rare but critical psoriasis gene within a spectrum of polygenic predisposition for psoriasis. Actually, in the literature, the reported case of a patient having linear psoriasis with PEODD nevus9 was explained in this way. This case is another example of linear psoriasis with PEODD nevus. This may support the above explanation and may help in the future to elucidate the unknown cause of psoriasis. REFERENCES 1. Leslie G. Linear psoriasis. Br J Dermatol 1951;63:262-3. 2. Sobel M. Linear psoriasis. Arch Dermatol Syph 1951;63:267.
Yu et al S83 3. Ginarte M, Fernandez-Redondo V, Toriboi J. Unilateral psoriasis: a case individualized by means of involucrin. Cutis 2000;65:167-70. 4. Park BS, Youn JI, Cho HK, Lee KH. A case of linear psoriasis. Korean J Dermatol 1996;34:998-1002. 5. Baselga E, Puig L, Llobet J, Musulen E, de Moragas JM. Linear psoriasis associated with systemic lupus erythematosus. J Am Acad Dermatol 1994;30:130-3. 6. Al-Fouzan AS, Hassab-el-Naby HM, Nanda A. Congenital linear psoriasis: a case report. Pediatr Dermatol 1990;7:303-6. 7. Atherton DJ, Kahana M, Russel-Jones R. Naevoid psoriasis. Br J Dermatol 1989;120:837-41. 8. Happle R. Somatic recombination may explain linear psoriasis. J Med Genet 1991;28:337. 9. van de Kerkhof P, Steijlen P, Happle R. Co-occurrence of linear psoriasis and porokeratotic eccrine ostial and dermal duct naevus. Acta Derm Venereol 1993;73:311-2. 10. Abell E, Read SI. Porokeratotic eccrine ostial and dermal duct naevus. Br J Dermatol 1980;103:435-41. 11. Sassmannshausen J, Bogomilsky J, Chaffins M. Porokeratotic eccrine ostial and dermal duct nevus: a case report and review of the literature. J Am Acad Dermatol 2000;43:364-7. 12. Solis J, Sau P, James WD. Puzzling palmar and plantar papules: porokeratotic eccrine ostial and dermal duct nevus. Arch Dermatol 1991; 127:1220-4. 13. Stoof TJ, Starink TM, Nieboer C. Porokeratotic eccrine ostial and dermal duct nevus: report of a case of adult onset. J Am Acad Dermatol 1989; 20:924-7. 14. de Jong E, Rulo H, van de Kerkhof PC. Inflammatory linear verrucous epidermal nevus (ILVEN) versus linear psoriasis: a clinical, histological and immunohistochemical study. Acta Derm Venereol 1991;71: 343-6. 15. Thivolet J, Goujon C. Linear psoriasis and systematized epidermolytic nevus. Arch Dermatol 1982;118:285-6. 16. Menni S, Restano L, Gianotti R, Boccardi D. Inflammatory linear verrucous epidermal nevus (ILVEN) and psoriasis in a child? Int J Dermatol 2000;39:30-2. 17. Altman J, Mehregan AH. Inflammatory linear verrucous epidermal nevus. Arch Dermatol 1971;104:385-9. 18. Marsden R, Fleming K, Dawber R. Comedo naevus of the palm–a sweat duct naevus? Br J Dermatol 1979;101:717-22. 19. Bergman R, Lichtig C, Cohen A, Friedman-Birnbaum R. Porokeratotic eccrine ostial nevus and dermal duct nevus. Am J Dermatopathol 1992; 14:319-22.