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Porokeratotic eccrine ostial and dermal duct nevus
Porokeratotic eccrine ostial and dermal duct nevus Report o f a case o f adult onset T o m J. Stoof, MD, PhD, Theo M. Starink, MD, and Cornelis Nieboer, M D
Amsterdam, The Netherlands We describe the case of a 38-year-old woman with adult-onset porokeratotic eccrine ostial and dermal duct nevus. The patient had a 9-year history of multiple keratotic papules and comedo-like pits on the medial border of the left foot. Light- and electron microscopic studies showed multiple cornoid lamella-like parakeratotic columns, which invariably were associated with hyperplastic eccrine ostia and distal sweat ducts. It is concluded that this entity, the first reported case of adult-onset porokeratotic eccrine ostial and dermal duct nevus, is a variant of the congenital form described previously by Abell and Read and by Aloi and Pippione. (J AM ACAD DER~ATOL 1989;20:924-7.)
Porokeratotic eccrine ostial and dermal duct nevus was first described in 1980 by Abell and Read. t Since then a number of cases have been reported26; another case could be diagnosed in retrospect. 7 Histologically, the condition is characterized by dilation and hyperplasia of the intraepidermal and distal portion of the eccrine sweat duct and the presence of parakeratotic cornoid lamellalike plugs exclusively associated with the sweat gland pores and ducts. Clinically, porokeratotic eccrine ostial and dermal duct nevus occurs typically on a linear or bandlike plantar or palmar area, with multiple punctate pits and comedo-like plugs. In all cases reported thus far the lesions were congenital and nonhereditary. In this report we describe the first case of porokeratotic eccrine ostial and dermal duct nevus of adult onset.
CASE REPORT Our patient was a 38-year-old white woman.
History. For 9 years the patient had an itching dermatosis on the left foot. After an initial gradual extension the eruption had been stable since the last few years. There was no family history of a similar dermatosis or other skin conditions. Examination, The physical examination revealed a
From the Department of Dermatology, Free University Hospital. Reprint requests: Tom J. Stoof, MD, PhD, Department of Dermatology, Academic Hospital, Free University, de Boelelaan 1117, 1081 HV Amsterdam, The Netherlands.
924
Fig. 1. Multiple small keratotic papules and comedolike pits on the medial border of the left foot.
bandlike area on the medial aspect of the left foot, with numerous small keratotic papules and pits, some filled with comedo-like keratotic plugs (Fig. 1). No other cutaneous abnormalities were present. Results of a general physical examination were normal. Light microscopic examination. Routine light microscopic examination of three biopsy specimens revealed similar abnormalities. Serial sections showed involvement of most of the eccrine ostia and ducts. The typical abnormality was a keratotic plug with a characteristic parakeratotic cornoid lamella-like center that filled an invagination formed by a marked dilated and hyperplastic ostium and distal portion of the eccrine sweat duct (Fig. 2). The parakeratotic column extended to the bottom of the invagination and originated from squarnous cells without formation of a granular layer. Adjacent epithelial cells showed vacuolation and scat-
Volume20 Number 5, Part 2 May 1989
Porokeratotic eccrine ostial and dermal duct nevus
925
tered dyskeratotic cells. The proximal portion of the eccrine sweat ducts and the glandular portion were normal. Electron microscopic studies. Electron microscopic examination of two biopsy specimens revealed almost complete loss of microvilli of the acrosyringium at the dip of the lesions. Secretion granules were found not only in the lumen but also in the wall, in the surrounding cells, and between the horny cells (Fig. 3). Cornoid lamella formation was found adjacent to the acrosyringlum. The ceils were irregularly shaped, partly flattened, and partly triangular. Many of these cells showed degeneration, with remnants of nuclei and cell organdies. Only a few granular cells were present in the region beneath the cornoid lamella. The lower portion of the sweat duct was normal and showed numerous microvilli lining the luminal border. DISCUSSION Our patient showed the clinical features (pits and comedo-like plugs) and histologic abnormalities (cornoid lamella-like parakeratotic columns associated exclusively with hyperplastic eccrine ostia and distal sweat ducts) typical of porokeratotic eccrine ostial and dermal duct nevus. Electron microscopic studies demonstrated sweat duct-associated cornoid lamella formation. The abnormalities of the horny cells of the cornoid lameUa were similar to those described in other forms of porokeratosis.8, 9 In addition, the acrosyringium showed alterations that in our experience also can be found in several other keratotic plantar and palmar diseases and may be due to obstruction of the sweat ducts. The first case of porokeratotie eccrine ostial and dermal duct nevus was described in 1980 by Abell and Read. 1Their patient was a 3-year-old girl with a linear keratotic lesion consisting of tiny papules, which had been present since birth, along the inner side of the left foot. In 1986 Aloi and Pippione2 reported the second and the most extensive case of porokeratotic eccrine ostial and dermal duct nevus in a 6-year-old girl. Also in 1986 two brief reports of congenital lesions appeared in the French literature. 3,4 In 1987 Driban and Cavicchia s reported another congenital case. The authors also performed electron microscopic studies that demonstrated the eccrine nature of the lesions. Recently Moreno et al. 6 reported two more cases of congenital porokeratotic eccrine ostial and dermal duct nevus in a 5-year-old girl and a 7-year-old boy.
Fig. 2. High-power view of the lower portion of a lesion showing the base of the cornoid lamella, focal absence of the granular layer, and adjacent normal proximal eccrine duct. Adjacent epithelial cells show vacuolation and scattered dyskeratotic cells. (Hematoxylin-eosin stain; • 132.) Finally, the case reported under a different name in 1979 by Marsden et a12 is apparently another example of porokeratotic eccrine ostial and dermal duct nevus that had been present since birth on the left foot of a 9-year-old boy. Scanning electron microscopic examination of skin surface replicas revealed keratotic plugs occluding the eccrine ostia. The presence of a parakeratotic horn plug, that is, cornoid lamellation, is a constant and characteristic feature of all forms of porokeratosis. ~~ The distinguishing features in porokeratotic eccrine ostial and dermal duct nevus are the presence of (1) cornoid lamellae that are associated invariably with the eccrine ostium and duct and (2) dilation and hyperplasia of the acrosyringium and the deeper eccrine duct. Both microscopic features help to differentiate porokeratotic eccrine ostial and dermal duct nevus from other conditions that share comedo-like keratoses and/or cornoid lamellation.
926
Journal of the American Academy of Dermatology
Stoofet aL
3. Electron micrograph of acrosyringium (,4) surrounded by vacuolated granulosa cells (G) on the border between the stratum corneum (SC) and stratum spinosum (SSp). L, Fig.
Lumen. Note irregular configuration of the horny cells and several nuclear remnants. (X4125.)
In punctate porokeratosis n, 12 and linear porokeratosis, ~-~cornoid lamella formation is not only connected to the eccrine sweat duct but also to the hair follicle infundibulum and the epidermis between the adnexa. This is also the case in porokeratosis plantaris palmaris et disseminata. 14 Porokeratotic eccrine duct and hair follicle nevus recently described by Coskey et al. ~5 resembles porokeratotic eccrine ostial and dermal d u c t nevus in m a n y respects. In addition to sweat duct involvement, however, there is also involvement of the hair follicles. N e v u s comedonicus occasionally shows palmoplantar involvement, b u t the condition is characterized by a m a l f o r m a t i o n of the pilosebaceous unit, which results in cystic dilation of the follicular canal and keratotic plugging. ~6,17 All cases of porokeratotic eccrine ostial and dermal duct nevus reported thus far had congenital lesions; however, in our patient the lesions did not develop until the patient was 29 years old. In our opinion the adult-onset form is a variant of the congenital f o r m of porokeratotic eccrine ostial and
dermal duct nevus, as m a y occur in other epithelial nevi. ~s REFERENCES
1. Abell E, Read SI. Porokeratotic eccrine ostial and dermal duct naevus. Br J Dermatol 1980;103:435-41. 2. Aloi FG, Pippione M. Porokeratotie eccrine ostial and dermal duct nevus. Arch Dermatol 1986;122:892-5. 3. Balato N, Cusano F, Lembo G, Ayala F. Naevus sudoral eccrine porok6ratosique pseudo-com&tonien pahnaire et plantairc. Ann Dermatol Venereol 1986;113:921-2. 4. Civatte J, Jeanmouging M, Denisart M, et al. Naevus sudora[ eecrine palmaire pseudo-com6donien. Ann Dermatol Venereol 1986;113:923-4. 5. Driban NE, Cavicchia JC. Porokeratotic eccrine ostial and dermal duct nevus. J Cutan Pathol 1987;14:118-21. 6. Morena A, Pujol RM, Salvatella N, Alomar A, de Moragas JM. Porokeratotie eecrine ostial and dermal duet nevus. J Cutan PathoI 1988;15:43-8. 7. Marsden RA, Fleming K, Dawber RPR. Comedo naevus of the palm--a sweat duct naevus? Br J Dermatol 1979; 101:717-22. 8. Mann PR, Cort DF, Fairburn EA, Abdel-Aziz A. Ultrastructural studies on two cases of porokeratosis of Mibelli. Br J Dermatol 1974;90:607-17. 9. Sato A, Anton-Lamprecht I, Sehnyder UW, Ultrastrueture of inborn errors of keratinization. VII. Porokeratosis
Volume 20 Number 5, Part 2 May 1989
10. 11. 12.
13.
Mibelli and disseminated superficial actinic porokeratosis. Arch Dermatol Res 1976;255:271-84. Wade TR, Ackerman AB. Cornoid lamellation: a histologic reaction pattern. Am J Dermatopathol 1980; 2:5-15. Rahbari H, Cordero AA, Mehregan AH. Punctate porokeratosis: a clinical variant of porokeratosis of Mibelli. J Cutan Pathol 1977;4:338-41. Sakes EL, Gentry RH. Porokeratosis punctata palmaris et plantaris (punctate porokeratosis): case report and literature review. J AM ACAD DngMATOL 1985;13: 908-12. Rahbari H, Cordero AA, Mehregan AH. Linear porokeratosis: a distinctive clinical variant of porokeratosis of Mibelli. Arch Dermatol 1974;109:526-8.
Porokeratotic eccrine ostial and dermal duct nevus 14, Shaw JC, White CR Jr. Porokeratosis plantaris palmafis et disseminata. J AM ACAD DERMATOL 1984;11:454-60. 15. Coskey R J, Mehregan AH, Hashimoto K. Porokeratotic eccrine duct and hair follicle nevus. J AM ACAD DEI~MATOL 1982;6:940-3. 16. Nabai H, Mehregan AH. Nevus comedonicus: a review of the literature and report of twelve cases. Aeta Derm Venereol (Stockh) 1973;53:71-4. 17. Wood MG, Thew MA. Nevus comedonicus: a case with palmar involvement and review of the literature. Arch Dermatol 1968;98:111-6. 18. Starink ThM, Woerdeman MJ. Unilateral systematized keratosis follieularis: a variant of Darier's disease or an epidermal naevus (acantholytic dyskeratotic epidermal naevus)? Br J Dermatol 1981;105:207-14.
Ofuji papuloerythroderma Report of a case with T cell skin lymphoma and discussion o f the nature of this disease J. J. Grob, MD," A. M. Collet-Villette, M D ? N. Horchowski, MD, b M. Dufaud, MD," L. Prin, MD, c and J. J. Bonerandi, MD" Marseille and Lille, France This report concerns a white woman with characteristic manifestations of Ofuji papuloerythroderma. This erythroderma results from a coalescence of papular sheets that completely spare skin folds. This rare disease is associated with high blood eosinophilia. In this case superficial lymph nodes appeared a few months later and peripheral T ceU nonepidermotropic skin lymphoma occurred, which raises questions as to the nosologic implications of papuloerythroderma. (J AM AeAD DERMATOL1989;20:927-31.)
In 1981 Ofuji et al. 1 published a report concerning four elderly male Japanese patients with a previously unknown dermatologic disorder that had characteristic clinical features. They named this new disorder papuloerythroderma. It appeared as solid papules coalescing into prurific erythroderma, which notably spared all skin folds. These skin lesions were associated with palpable lymph nodes and high blood eosinophllia. On histologic examination, a relatively dense infiltration of lympho-
cytes and histiocytes was observed in the papillary and mid dermis, chiefly around the vessels. The course of the disease was chronic but apparently benign. In 1987 Staughton et al? reported the first case of papuloerythroderma in a white man who had nailfold and buttock infarcts in addition to the usual signs. We describe a case in a white woman that raises the possibility that Ofuji papuloerythroderma is a form of nonepiderrnotropic peripheral T cell lymphoma.
From the Service de Dermatologie, Hfpital Ste. Marguerite, Marseille," the Service d'H6matologie, Centre Paoli-Calmette, Marseille,b and the Centre d'Immunologie et Biologic Parasitaire, Institut Pasteur, LilleY Reprint requests: J. J. Bonerandi, MD, H6pital Ste. Marguerite, 270 Bd. de Sainte Marguerite, B.P. 29, 13277 Marseille Cedex 09, France.
CASE R E P O R T
A 75-year-old white woman consulted us about a 15-month history of dermatosis that was initially consid-
ered to be lichenified eczema. Topical corticosteroids had no effect and psoralens with ultraviolet A therapy seemingly led to exacerbation.
927
Amsterdam, The Netherlands We describe the case of a 38-year-old woman with adult-onset porokeratotic eccrine ostial and dermal duct nevus. The patient had a 9-year history of multiple keratotic papules and comedo-like pits on the medial border of the left foot. Light- and electron microscopic studies showed multiple cornoid lamella-like parakeratotic columns, which invariably were associated with hyperplastic eccrine ostia and distal sweat ducts. It is concluded that this entity, the first reported case of adult-onset porokeratotic eccrine ostial and dermal duct nevus, is a variant of the congenital form described previously by Abell and Read and by Aloi and Pippione. (J AM ACAD DER~ATOL 1989;20:924-7.)
Porokeratotic eccrine ostial and dermal duct nevus was first described in 1980 by Abell and Read. t Since then a number of cases have been reported26; another case could be diagnosed in retrospect. 7 Histologically, the condition is characterized by dilation and hyperplasia of the intraepidermal and distal portion of the eccrine sweat duct and the presence of parakeratotic cornoid lamellalike plugs exclusively associated with the sweat gland pores and ducts. Clinically, porokeratotic eccrine ostial and dermal duct nevus occurs typically on a linear or bandlike plantar or palmar area, with multiple punctate pits and comedo-like plugs. In all cases reported thus far the lesions were congenital and nonhereditary. In this report we describe the first case of porokeratotic eccrine ostial and dermal duct nevus of adult onset.
CASE REPORT Our patient was a 38-year-old white woman.
History. For 9 years the patient had an itching dermatosis on the left foot. After an initial gradual extension the eruption had been stable since the last few years. There was no family history of a similar dermatosis or other skin conditions. Examination, The physical examination revealed a
From the Department of Dermatology, Free University Hospital. Reprint requests: Tom J. Stoof, MD, PhD, Department of Dermatology, Academic Hospital, Free University, de Boelelaan 1117, 1081 HV Amsterdam, The Netherlands.
924
Fig. 1. Multiple small keratotic papules and comedolike pits on the medial border of the left foot.
bandlike area on the medial aspect of the left foot, with numerous small keratotic papules and pits, some filled with comedo-like keratotic plugs (Fig. 1). No other cutaneous abnormalities were present. Results of a general physical examination were normal. Light microscopic examination. Routine light microscopic examination of three biopsy specimens revealed similar abnormalities. Serial sections showed involvement of most of the eccrine ostia and ducts. The typical abnormality was a keratotic plug with a characteristic parakeratotic cornoid lamella-like center that filled an invagination formed by a marked dilated and hyperplastic ostium and distal portion of the eccrine sweat duct (Fig. 2). The parakeratotic column extended to the bottom of the invagination and originated from squarnous cells without formation of a granular layer. Adjacent epithelial cells showed vacuolation and scat-
Volume20 Number 5, Part 2 May 1989
Porokeratotic eccrine ostial and dermal duct nevus
925
tered dyskeratotic cells. The proximal portion of the eccrine sweat ducts and the glandular portion were normal. Electron microscopic studies. Electron microscopic examination of two biopsy specimens revealed almost complete loss of microvilli of the acrosyringium at the dip of the lesions. Secretion granules were found not only in the lumen but also in the wall, in the surrounding cells, and between the horny cells (Fig. 3). Cornoid lamella formation was found adjacent to the acrosyringlum. The ceils were irregularly shaped, partly flattened, and partly triangular. Many of these cells showed degeneration, with remnants of nuclei and cell organdies. Only a few granular cells were present in the region beneath the cornoid lamella. The lower portion of the sweat duct was normal and showed numerous microvilli lining the luminal border. DISCUSSION Our patient showed the clinical features (pits and comedo-like plugs) and histologic abnormalities (cornoid lamella-like parakeratotic columns associated exclusively with hyperplastic eccrine ostia and distal sweat ducts) typical of porokeratotic eccrine ostial and dermal duct nevus. Electron microscopic studies demonstrated sweat duct-associated cornoid lamella formation. The abnormalities of the horny cells of the cornoid lameUa were similar to those described in other forms of porokeratosis.8, 9 In addition, the acrosyringium showed alterations that in our experience also can be found in several other keratotic plantar and palmar diseases and may be due to obstruction of the sweat ducts. The first case of porokeratotie eccrine ostial and dermal duct nevus was described in 1980 by Abell and Read. 1Their patient was a 3-year-old girl with a linear keratotic lesion consisting of tiny papules, which had been present since birth, along the inner side of the left foot. In 1986 Aloi and Pippione2 reported the second and the most extensive case of porokeratotic eccrine ostial and dermal duct nevus in a 6-year-old girl. Also in 1986 two brief reports of congenital lesions appeared in the French literature. 3,4 In 1987 Driban and Cavicchia s reported another congenital case. The authors also performed electron microscopic studies that demonstrated the eccrine nature of the lesions. Recently Moreno et al. 6 reported two more cases of congenital porokeratotic eccrine ostial and dermal duct nevus in a 5-year-old girl and a 7-year-old boy.
Fig. 2. High-power view of the lower portion of a lesion showing the base of the cornoid lamella, focal absence of the granular layer, and adjacent normal proximal eccrine duct. Adjacent epithelial cells show vacuolation and scattered dyskeratotic cells. (Hematoxylin-eosin stain; • 132.) Finally, the case reported under a different name in 1979 by Marsden et a12 is apparently another example of porokeratotic eccrine ostial and dermal duct nevus that had been present since birth on the left foot of a 9-year-old boy. Scanning electron microscopic examination of skin surface replicas revealed keratotic plugs occluding the eccrine ostia. The presence of a parakeratotic horn plug, that is, cornoid lamellation, is a constant and characteristic feature of all forms of porokeratosis. ~~ The distinguishing features in porokeratotic eccrine ostial and dermal duct nevus are the presence of (1) cornoid lamellae that are associated invariably with the eccrine ostium and duct and (2) dilation and hyperplasia of the acrosyringium and the deeper eccrine duct. Both microscopic features help to differentiate porokeratotic eccrine ostial and dermal duct nevus from other conditions that share comedo-like keratoses and/or cornoid lamellation.
926
Journal of the American Academy of Dermatology
Stoofet aL
3. Electron micrograph of acrosyringium (,4) surrounded by vacuolated granulosa cells (G) on the border between the stratum corneum (SC) and stratum spinosum (SSp). L, Fig.
Lumen. Note irregular configuration of the horny cells and several nuclear remnants. (X4125.)
In punctate porokeratosis n, 12 and linear porokeratosis, ~-~cornoid lamella formation is not only connected to the eccrine sweat duct but also to the hair follicle infundibulum and the epidermis between the adnexa. This is also the case in porokeratosis plantaris palmaris et disseminata. 14 Porokeratotic eccrine duct and hair follicle nevus recently described by Coskey et al. ~5 resembles porokeratotic eccrine ostial and dermal d u c t nevus in m a n y respects. In addition to sweat duct involvement, however, there is also involvement of the hair follicles. N e v u s comedonicus occasionally shows palmoplantar involvement, b u t the condition is characterized by a m a l f o r m a t i o n of the pilosebaceous unit, which results in cystic dilation of the follicular canal and keratotic plugging. ~6,17 All cases of porokeratotic eccrine ostial and dermal duct nevus reported thus far had congenital lesions; however, in our patient the lesions did not develop until the patient was 29 years old. In our opinion the adult-onset form is a variant of the congenital f o r m of porokeratotic eccrine ostial and
dermal duct nevus, as m a y occur in other epithelial nevi. ~s REFERENCES
1. Abell E, Read SI. Porokeratotic eccrine ostial and dermal duct naevus. Br J Dermatol 1980;103:435-41. 2. Aloi FG, Pippione M. Porokeratotie eccrine ostial and dermal duct nevus. Arch Dermatol 1986;122:892-5. 3. Balato N, Cusano F, Lembo G, Ayala F. Naevus sudoral eccrine porok6ratosique pseudo-com&tonien pahnaire et plantairc. Ann Dermatol Venereol 1986;113:921-2. 4. Civatte J, Jeanmouging M, Denisart M, et al. Naevus sudora[ eecrine palmaire pseudo-com6donien. Ann Dermatol Venereol 1986;113:923-4. 5. Driban NE, Cavicchia JC. Porokeratotic eccrine ostial and dermal duct nevus. J Cutan Pathol 1987;14:118-21. 6. Morena A, Pujol RM, Salvatella N, Alomar A, de Moragas JM. Porokeratotie eecrine ostial and dermal duet nevus. J Cutan PathoI 1988;15:43-8. 7. Marsden RA, Fleming K, Dawber RPR. Comedo naevus of the palm--a sweat duct naevus? Br J Dermatol 1979; 101:717-22. 8. Mann PR, Cort DF, Fairburn EA, Abdel-Aziz A. Ultrastructural studies on two cases of porokeratosis of Mibelli. Br J Dermatol 1974;90:607-17. 9. Sato A, Anton-Lamprecht I, Sehnyder UW, Ultrastrueture of inborn errors of keratinization. VII. Porokeratosis
Volume 20 Number 5, Part 2 May 1989
10. 11. 12.
13.
Mibelli and disseminated superficial actinic porokeratosis. Arch Dermatol Res 1976;255:271-84. Wade TR, Ackerman AB. Cornoid lamellation: a histologic reaction pattern. Am J Dermatopathol 1980; 2:5-15. Rahbari H, Cordero AA, Mehregan AH. Punctate porokeratosis: a clinical variant of porokeratosis of Mibelli. J Cutan Pathol 1977;4:338-41. Sakes EL, Gentry RH. Porokeratosis punctata palmaris et plantaris (punctate porokeratosis): case report and literature review. J AM ACAD DngMATOL 1985;13: 908-12. Rahbari H, Cordero AA, Mehregan AH. Linear porokeratosis: a distinctive clinical variant of porokeratosis of Mibelli. Arch Dermatol 1974;109:526-8.
Porokeratotic eccrine ostial and dermal duct nevus 14, Shaw JC, White CR Jr. Porokeratosis plantaris palmafis et disseminata. J AM ACAD DERMATOL 1984;11:454-60. 15. Coskey R J, Mehregan AH, Hashimoto K. Porokeratotic eccrine duct and hair follicle nevus. J AM ACAD DEI~MATOL 1982;6:940-3. 16. Nabai H, Mehregan AH. Nevus comedonicus: a review of the literature and report of twelve cases. Aeta Derm Venereol (Stockh) 1973;53:71-4. 17. Wood MG, Thew MA. Nevus comedonicus: a case with palmar involvement and review of the literature. Arch Dermatol 1968;98:111-6. 18. Starink ThM, Woerdeman MJ. Unilateral systematized keratosis follieularis: a variant of Darier's disease or an epidermal naevus (acantholytic dyskeratotic epidermal naevus)? Br J Dermatol 1981;105:207-14.
Ofuji papuloerythroderma Report of a case with T cell skin lymphoma and discussion o f the nature of this disease J. J. Grob, MD," A. M. Collet-Villette, M D ? N. Horchowski, MD, b M. Dufaud, MD," L. Prin, MD, c and J. J. Bonerandi, MD" Marseille and Lille, France This report concerns a white woman with characteristic manifestations of Ofuji papuloerythroderma. This erythroderma results from a coalescence of papular sheets that completely spare skin folds. This rare disease is associated with high blood eosinophilia. In this case superficial lymph nodes appeared a few months later and peripheral T ceU nonepidermotropic skin lymphoma occurred, which raises questions as to the nosologic implications of papuloerythroderma. (J AM AeAD DERMATOL1989;20:927-31.)
In 1981 Ofuji et al. 1 published a report concerning four elderly male Japanese patients with a previously unknown dermatologic disorder that had characteristic clinical features. They named this new disorder papuloerythroderma. It appeared as solid papules coalescing into prurific erythroderma, which notably spared all skin folds. These skin lesions were associated with palpable lymph nodes and high blood eosinophllia. On histologic examination, a relatively dense infiltration of lympho-
cytes and histiocytes was observed in the papillary and mid dermis, chiefly around the vessels. The course of the disease was chronic but apparently benign. In 1987 Staughton et al? reported the first case of papuloerythroderma in a white man who had nailfold and buttock infarcts in addition to the usual signs. We describe a case in a white woman that raises the possibility that Ofuji papuloerythroderma is a form of nonepiderrnotropic peripheral T cell lymphoma.
From the Service de Dermatologie, Hfpital Ste. Marguerite, Marseille," the Service d'H6matologie, Centre Paoli-Calmette, Marseille,b and the Centre d'Immunologie et Biologic Parasitaire, Institut Pasteur, LilleY Reprint requests: J. J. Bonerandi, MD, H6pital Ste. Marguerite, 270 Bd. de Sainte Marguerite, B.P. 29, 13277 Marseille Cedex 09, France.
CASE R E P O R T
A 75-year-old white woman consulted us about a 15-month history of dermatosis that was initially consid-
ered to be lichenified eczema. Topical corticosteroids had no effect and psoralens with ultraviolet A therapy seemingly led to exacerbation.
927