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 © 2001-2009, DermAtlas | Image Name: | Arteriovenous_fistula_1_071031 | File Type: | jpg | |
| Diagnosis: | ARTERIOVENOUS FISTULA / FISTULA, ARTERIOVENOUS | Category: | vascular malformation / treatment / renal disease / cutaneous sign of systemic disease | ||
| Body Site: | arm / forearm / forearm | Age: | 77 years | ||
| Contributor: | Jerzy Pawlak, MSc,PhD | ||||
| Description: | tortuous compressible linear mass | ||||
| Comments: | This 77-year-old man with a history of renal failure complained of a dry itchy scaly patch overlying the arteriovenous fistula configured for dialysis therapy. | ||||
 © 2001-2009, DermAtlas | Image Name: | acquired_perforating_dermatosis_5_070804 | File Type: | jpg | |
| Diagnosis: | KYRLE DISEASE / ACQUIRED PERFORATING DERMATOSIS | Category: | papulosquamous eruptions / cutaneous sign of systemic disease / renal disease / immunodeficiency related | ||
| Body Site: | leg | Age: | 43 years | ||
| Contributor: | Laura McGirt | ||||
| Description: | Histopathology was read initially as a perforating collagenosis, but review showed a keratinaceous plug without diagnostic features of a perforating disorder. | ||||
| Comments: | This 43-year-old man withg a history of Human Immunodeficiency Virus infection, chronic renal failure requiring dialysis, and diabetes developed an eruptive pruritic eruption on his extremities, back, and head 2 years ago. The lesions usually begin as small dark bumps which gow in size and develop small “crystals” in the center which eventually fall out leaving a small hole in the skin. On examination most of the lesions were located on the extensor surfaces of the arms and legs but some were scattered on the trunk, scalp, and forehead. Histopathology was read initially as a perforating collagenosis, but review showed a keratinaceous plug without diagnostic features of a perforating disorder.Perforating disorders comprise a collection of skin disease that are papulonodular in nature and have the hallmark feature of a central hyperkeratotic plug through which dermal components are eliminated. Specific perforating disorders include elastosis perforans serpiginosa, reactive perforating collagenosis, and acquired perforating dermatosis (APD), which has been used as a synonym for or a category which encompasses those cases designated as Kyrle’s disease. Kyrle’s disease (hyperkeratosis follicularis et parafollicularis in cutem penetrans) was originally described in 1916 in a diabetic woman with generalized hyperkeratotic nodules1. There is some controversy in the literature in regards to classification of the perforating disorders, as there is a great deal of overlap, clinically and histologically. We use the diagnosis of APD to refer to a perforating skin disease that presents in adulthood in association with diabetes mellitus or renal failure. APD is relatively common, with a reported incidence of 4.5 to 11% of those on chronic hemodialysis2,3. There is no sex or racial predilection. The pathogenesis is not well understood but it is theorized that pruritus, often found in uremic patients, leads to excessive scratching of the skin. In the setting of diabetic vasculopathy this could lead to dermal necrosis, which is then eliminated through the epidermis (can include both elastin and collagen)2. Another hypothesis is that there is an “uncoupling of epidermal proliferation and differentiation,” with keratinization occurring faster that new cell production. Keratinization of the basal layer incites a dermal inflammatory reaction, leading to the classic papulonodule with a hyperkeratotic plug4. Clinically the lesions start as small pin-head size papules and can grow to close to one centimeter. There is a predilection for the extensor surfaces of the legs, and the arms, trunk, head and neck can be involved. There are reports of koebnerization. The duration of these lesions ranges from 4 months to 43 years4. APD has been reported to develop in patients with lymphoma, sclerosing cholangitis, congestive heart failure, and HIV, but many of these patients also had concurrent renal failure or diabetes2-5. Treatment includes oral retinoids, phototherapy (PUVA and UVB), and anecdotal evidence has shown improvement with topical, intralesional, oral steroids, topical retinoids and salicylic acid, among others. References: 1. Kyrle J. Hyperkeratosis follicularis et parafollicularis in cutem penetrans. Arch Dermatol Syphilol 1916;123:466-93. 2. Morton CA, Henderson IS, Jones MC, Lowe JG. Acquired perforating dermatosis in a British dialysis population. Br J Dermatol. 1996;135:671-77. 3. Hood A, Hardegen GL, Zarate AR et al. Kyrle’s disease in patients with chronic renal failure. Arch Dermatol. 1982;118:85-8. 4. Patterson JW. The perforating disorders. J Am Acad Dermatol. 1984;10:561-81. 5. Skiba G, Milkiewicz P, Mutimer D, et al. Successful treatment of acquired perforating dermatosis with rifampicin in an Asian patient with sclerosing cholangitis. Liver. 1999;19(2):160-3. | ||||
| Related Images: | All related Images acquired_perforating_dermatosis_1_070804 acquired_perforating_dermatosis_2_070804 acquired_perforating_dermatosis_3_070804 acquired_perforating_dermatosis_4_070804 | ||||
 © 2001-2009, DermAtlas | Image Name: | acquired_perforating_dermatosis_4_070804 | File Type: | jpg | |
| Diagnosis: | KYRLE DISEASE / ACQUIRED PERFORATING DERMATOSIS | Category: | papulosquamous eruptions / cutaneous sign of systemic disease / renal disease / immunodeficiency related | ||
| Body Site: | leg | Age: | 43 years | ||
| Contributor: | Laura McGirt | ||||
| Description: | Histopathology was read initially as a perforating collagenosis, but review showed a keratinaceous plug without diagnostic features of a perforating disorder. | ||||
| Comments: | This 43-year-old man withg a history of Human Immunodeficiency Virus infection, chronic renal failure requiring dialysis, and diabetes developed an eruptive pruritic eruption on his extremities, back, and head 2 years ago. The lesions usually begin as small dark bumps which gow in size and develop small “crystals” in the center which eventually fall out leaving a small hole in the skin. On examination most of the lesions were located on the extensor surfaces of the arms and legs but some were scattered on the trunk, scalp, and forehead. Histopathology was read initially as a perforating collagenosis, but review showed a keratinaceous plug without diagnostic features of a perforating disorder.Perforating disorders comprise a collection of skin disease that are papulonodular in nature and have the hallmark feature of a central hyperkeratotic plug through which dermal components are eliminated. Specific perforating disorders include elastosis perforans serpiginosa, reactive perforating collagenosis, and acquired perforating dermatosis (APD), which has been used as a synonym for or a category which encompasses those cases designated as Kyrle’s disease. Kyrle’s disease (hyperkeratosis follicularis et parafollicularis in cutem penetrans) was originally described in 1916 in a diabetic woman with generalized hyperkeratotic nodules1. There is some controversy in the literature in regards to classification of the perforating disorders, as there is a great deal of overlap, clinically and histologically. We use the diagnosis of APD to refer to a perforating skin disease that presents in adulthood in association with diabetes mellitus or renal failure. APD is relatively common, with a reported incidence of 4.5 to 11% of those on chronic hemodialysis2,3. There is no sex or racial predilection. The pathogenesis is not well understood but it is theorized that pruritus, often found in uremic patients, leads to excessive scratching of the skin. In the setting of diabetic vasculopathy this could lead to dermal necrosis, which is then eliminated through the epidermis (can include both elastin and collagen)2. Another hypothesis is that there is an “uncoupling of epidermal proliferation and differentiation,” with keratinization occurring faster that new cell production. Keratinization of the basal layer incites a dermal inflammatory reaction, leading to the classic papulonodule with a hyperkeratotic plug4. Clinically the lesions start as small pin-head size papules and can grow to close to one centimeter. There is a predilection for the extensor surfaces of the legs, and the arms, trunk, head and neck can be involved. There are reports of koebnerization. The duration of these lesions ranges from 4 months to 43 years4. APD has been reported to develop in patients with lymphoma, sclerosing cholangitis, congestive heart failure, and HIV, but many of these patients also had concurrent renal failure or diabetes2-5. Treatment includes oral retinoids, phototherapy (PUVA and UVB), and anecdotal evidence has shown improvement with topical, intralesional, oral steroids, topical retinoids and salicylic acid, among others. References: 1. Kyrle J. Hyperkeratosis follicularis et parafollicularis in cutem penetrans. Arch Dermatol Syphilol 1916;123:466-93. 2. Morton CA, Henderson IS, Jones MC, Lowe JG. Acquired perforating dermatosis in a British dialysis population. Br J Dermatol. 1996;135:671-77. 3. Hood A, Hardegen GL, Zarate AR et al. Kyrle’s disease in patients with chronic renal failure. Arch Dermatol. 1982;118:85-8. 4. Patterson JW. The perforating disorders. J Am Acad Dermatol. 1984;10:561-81. 5. Skiba G, Milkiewicz P, Mutimer D, et al. Successful treatment of acquired perforating dermatosis with rifampicin in an Asian patient with sclerosing cholangitis. Liver. 1999;19(2):160-3. | ||||
| Related Images: | All related Images acquired_perforating_dermatosis_1_070804 acquired_perforating_dermatosis_2_070804 acquired_perforating_dermatosis_3_070804 acquired_perforating_dermatosis_5_070804 | ||||
 © 2001-2009, DermAtlas | Image Name: | acquired_perforating_dermatosis_3_070804 | File Type: | jpg | |
| Diagnosis: | KYRLE DISEASE / ACQUIRED PERFORATING DERMATOSIS | Category: | papulosquamous eruptions / cutaneous sign of systemic disease / renal disease / immunodeficiency related | ||
| Body Site: | leg | Age: | 43 years | ||
| Contributor: | Laura McGirt | ||||
| Description: | multiple 0.5 cm to 1 cm hyperpigmented papules with a central hyperkeratotic plug | ||||
| Comments: | This 43-year-old man withg a history of Human Immunodeficiency Virus infection, chronic renal failure requiring dialysis, and diabetes developed an eruptive pruritic eruption on his extremities, back, and head 2 years ago. The lesions usually begin as small dark bumps which gow in size and develop small “crystals” in the center which eventually fall out leaving a small hole in the skin. On examination most of the lesions were located on the extensor surfaces of the arms and legs but some were scattered on the trunk, scalp, and forehead. Histopathology was read initially as a perforating collagenosis, but review showed a keratinaceous plug without diagnostic features of a perforating disorder.Perforating disorders comprise a collection of skin disease that are papulonodular in nature and have the hallmark feature of a central hyperkeratotic plug through which dermal components are eliminated. Specific perforating disorders include elastosis perforans serpiginosa, reactive perforating collagenosis, and acquired perforating dermatosis (APD), which has been used as a synonym for or a category which encompasses those cases designated as Kyrle’s disease. Kyrle’s disease (hyperkeratosis follicularis et parafollicularis in cutem penetrans) was originally described in 1916 in a diabetic woman with generalized hyperkeratotic nodules1. There is some controversy in the literature in regards to classification of the perforating disorders, as there is a great deal of overlap, clinically and histologically. We use the diagnosis of APD to refer to a perforating skin disease that presents in adulthood in association with diabetes mellitus or renal failure. APD is relatively common, with a reported incidence of 4.5 to 11% of those on chronic hemodialysis2,3. There is no sex or racial predilection. The pathogenesis is not well understood but it is theorized that pruritus, often found in uremic patients, leads to excessive scratching of the skin. In the setting of diabetic vasculopathy this could lead to dermal necrosis, which is then eliminated through the epidermis (can include both elastin and collagen)2. Another hypothesis is that there is an “uncoupling of epidermal proliferation and differentiation,” with keratinization occurring faster that new cell production. Keratinization of the basal layer incites a dermal inflammatory reaction, leading to the classic papulonodule with a hyperkeratotic plug4. Clinically the lesions start as small pin-head size papules and can grow to close to one centimeter. There is a predilection for the extensor surfaces of the legs, and the arms, trunk, head and neck can be involved. There are reports of koebnerization. The duration of these lesions ranges from 4 months to 43 years4. APD has been reported to develop in patients with lymphoma, sclerosing cholangitis, congestive heart failure, and HIV, but many of these patients also had concurrent renal failure or diabetes2-5. Treatment includes oral retinoids, phototherapy (PUVA and UVB), and anecdotal evidence has shown improvement with topical, intralesional, oral steroids, topical retinoids and salicylic acid, among others. References: 1. Kyrle J. Hyperkeratosis follicularis et parafollicularis in cutem penetrans. Arch Dermatol Syphilol 1916;123:466-93. 2. Morton CA, Henderson IS, Jones MC, Lowe JG. Acquired perforating dermatosis in a British dialysis population. Br J Dermatol. 1996;135:671-77. 3. Hood A, Hardegen GL, Zarate AR et al. Kyrle’s disease in patients with chronic renal failure. Arch Dermatol. 1982;118:85-8. 4. Patterson JW. The perforating disorders. J Am Acad Dermatol. 1984;10:561-81. 5. Skiba G, Milkiewicz P, Mutimer D, et al. Successful treatment of acquired perforating dermatosis with rifampicin in an Asian patient with sclerosing cholangitis. Liver. 1999;19(2):160-3. | ||||
| Related Images: | All related Images acquired_perforating_dermatosis_1_070804 acquired_perforating_dermatosis_2_070804 acquired_perforating_dermatosis_4_070804 acquired_perforating_dermatosis_5_070804 | ||||
 © 2001-2009, DermAtlas | Image Name: | acquired_perforating_dermatosis_2_070804 | File Type: | jpg | |
| Diagnosis: | KYRLE DISEASE / ACQUIRED PERFORATING DERMATOSIS | Category: | papulosquamous eruptions / cutaneous sign of systemic disease / renal disease / immunodeficiency related | ||
| Body Site: | leg | Age: | 43 years | ||
| Contributor: | Laura McGirt | ||||
| Description: | multiple 0.5 cm to 1 cm hyperpigmented papules with a central hyperkeratotic plug | ||||
| Comments: | This 43-year-old man withg a history of Human Immunodeficiency Virus infection, chronic renal failure requiring dialysis, and diabetes developed an eruptive pruritic eruption on his extremities, back, and head 2 years ago. The lesions usually begin as small dark bumps which gow in size and develop small “crystals” in the center which eventually fall out leaving a small hole in the skin. On examination most of the lesions were located on the extensor surfaces of the arms and legs but some were scattered on the trunk, scalp, and forehead. Histopathology was read initially as a perforating collagenosis, but review showed a keratinaceous plug without diagnostic features of a perforating disorder.Perforating disorders comprise a collection of skin disease that are papulonodular in nature and have the hallmark feature of a central hyperkeratotic plug through which dermal components are eliminated. Specific perforating disorders include elastosis perforans serpiginosa, reactive perforating collagenosis, and acquired perforating dermatosis (APD), which has been used as a synonym for or a category which encompasses those cases designated as Kyrle’s disease. Kyrle’s disease (hyperkeratosis follicularis et parafollicularis in cutem penetrans) was originally described in 1916 in a diabetic woman with generalized hyperkeratotic nodules1. There is some controversy in the literature in regards to classification of the perforating disorders, as there is a great deal of overlap, clinically and histologically. We use the diagnosis of APD to refer to a perforating skin disease that presents in adulthood in association with diabetes mellitus or renal failure. APD is relatively common, with a reported incidence of 4.5 to 11% of those on chronic hemodialysis2,3. There is no sex or racial predilection. The pathogenesis is not well understood but it is theorized that pruritus, often found in uremic patients, leads to excessive scratching of the skin. In the setting of diabetic vasculopathy this could lead to dermal necrosis, which is then eliminated through the epidermis (can include both elastin and collagen)2. Another hypothesis is that there is an “uncoupling of epidermal proliferation and differentiation,” with keratinization occurring faster that new cell production. Keratinization of the basal layer incites a dermal inflammatory reaction, leading to the classic papulonodule with a hyperkeratotic plug4. Clinically the lesions start as small pin-head size papules and can grow to close to one centimeter. There is a predilection for the extensor surfaces of the legs, and the arms, trunk, head and neck can be involved. There are reports of koebnerization. The duration of these lesions ranges from 4 months to 43 years4. APD has been reported to develop in patients with lymphoma, sclerosing cholangitis, congestive heart failure, and HIV, but many of these patients also had concurrent renal failure or diabetes2-5. Treatment includes oral retinoids, phototherapy (PUVA and UVB), and anecdotal evidence has shown improvement with topical, intralesional, oral steroids, topical retinoids and salicylic acid, among others. References: 1. Kyrle J. Hyperkeratosis follicularis et parafollicularis in cutem penetrans. Arch Dermatol Syphilol 1916;123:466-93. 2. Morton CA, Henderson IS, Jones MC, Lowe JG. Acquired perforating dermatosis in a British dialysis population. Br J Dermatol. 1996;135:671-77. 3. Hood A, Hardegen GL, Zarate AR et al. Kyrle’s disease in patients with chronic renal failure. Arch Dermatol. 1982;118:85-8. 4. Patterson JW. The perforating disorders. J Am Acad Dermatol. 1984;10:561-81. 5. Skiba G, Milkiewicz P, Mutimer D, et al. Successful treatment of acquired perforating dermatosis with rifampicin in an Asian patient with sclerosing cholangitis. Liver. 1999;19(2):160-3. | ||||
| Related Images: | All related Images acquired_perforating_dermatosis_1_070804 acquired_perforating_dermatosis_3_070804 acquired_perforating_dermatosis_4_070804 acquired_perforating_dermatosis_5_070804 | ||||
 © 2001-2009, DermAtlas | Image Name: | acquired_perforating_dermatosis_1_070804 | File Type: | jpg | |
| Diagnosis: | KYRLE DISEASE / ACQUIRED PERFORATING DERMATOSIS | Category: | papulosquamous eruptions / cutaneous sign of systemic disease / renal disease / immunodeficiency related | ||
| Body Site: | leg | Age: | 43 years | ||
| Contributor: | Laura McGirt | ||||
| Description: | multiple 0.5 cm to 1 cm hyperpigmented papules with a central hyperkeratotic plug | ||||
| Comments: | This 43-year-old man withg a history of Human Immunodeficiency Virus infection, chronic renal failure requiring dialysis, and diabetes developed an eruptive pruritic eruption on his extremities, back, and head 2 years ago. The lesions usually begin as small dark bumps which gow in size and develop small “crystals” in the center which eventually fall out leaving a small hole in the skin. On examination most of the lesions were located on the extensor surfaces of the arms and legs but some were scattered on the trunk, scalp, and forehead. Histopathology was read initially as a perforating collagenosis, but review showed a keratinaceous plug without diagnostic features of a perforating disorder.Perforating disorders comprise a collection of skin disease that are papulonodular in nature and have the hallmark feature of a central hyperkeratotic plug through which dermal components are eliminated. Specific perforating disorders include elastosis perforans serpiginosa, reactive perforating collagenosis, and acquired perforating dermatosis (APD), which has been used as a synonym for or a category which encompasses those cases designated as Kyrle’s disease. Kyrle’s disease (hyperkeratosis follicularis et parafollicularis in cutem penetrans) was originally described in 1916 in a diabetic woman with generalized hyperkeratotic nodules1. There is some controversy in the literature in regards to classification of the perforating disorders, as there is a great deal of overlap, clinically and histologically. We use the diagnosis of APD to refer to a perforating skin disease that presents in adulthood in association with diabetes mellitus or renal failure. APD is relatively common, with a reported incidence of 4.5 to 11% of those on chronic hemodialysis2,3. There is no sex or racial predilection. The pathogenesis is not well understood but it is theorized that pruritus, often found in uremic patients, leads to excessive scratching of the skin. In the setting of diabetic vasculopathy this could lead to dermal necrosis, which is then eliminated through the epidermis (can include both elastin and collagen)2. Another hypothesis is that there is an “uncoupling of epidermal proliferation and differentiation,” with keratinization occurring faster that new cell production. Keratinization of the basal layer incites a dermal inflammatory reaction, leading to the classic papulonodule with a hyperkeratotic plug4. Clinically the lesions start as small pin-head size papules and can grow to close to one centimeter. There is a predilection for the extensor surfaces of the legs, and the arms, trunk, head and neck can be involved. There are reports of koebnerization. The duration of these lesions ranges from 4 months to 43 years4. APD has been reported to develop in patients with lymphoma, sclerosing cholangitis, congestive heart failure, and HIV, but many of these patients also had concurrent renal failure or diabetes2-5. Treatment includes oral retinoids, phototherapy (PUVA and UVB), and anecdotal evidence has shown improvement with topical, intralesional, oral steroids, topical retinoids and salicylic acid, among others. References: 1. Kyrle J. Hyperkeratosis follicularis et parafollicularis in cutem penetrans. Arch Dermatol Syphilol 1916;123:466-93. 2. Morton CA, Henderson IS, Jones MC, Lowe JG. Acquired perforating dermatosis in a British dialysis population. Br J Dermatol. 1996;135:671-77. 3. Hood A, Hardegen GL, Zarate AR et al. Kyrle’s disease in patients with chronic renal failure. Arch Dermatol. 1982;118:85-8. 4. Patterson JW. The perforating disorders. J Am Acad Dermatol. 1984;10:561-81. 5. Skiba G, Milkiewicz P, Mutimer D, et al. Successful treatment of acquired perforating dermatosis with rifampicin in an Asian patient with sclerosing cholangitis. Liver. 1999;19(2):160-3. | ||||
| Related Images: | All related Images acquired_perforating_dermatosis_2_070804 acquired_perforating_dermatosis_3_070804 acquired_perforating_dermatosis_4_070804 acquired_perforating_dermatosis_5_070804 | ||||
 © 2001-2009, DermAtlas | Image Name: | reactive_perforating_collagenosis_3_060220 | File Type: | jpg | |
| Diagnosis: | REACTIVE PERFORATING COLLAGENOSIS | Category: | perforating disorders / cutaneous sign of systemic disease / renal disease | ||
| Body Site: | leg / trunk | Age: | 57 years | ||
| Contributor: | Greg Hosler, MD, PhD | ||||
| Description: | A Masson trichrome stain helps confirm the presence of collagen fibers within the elimination channel. Collagen stains an intense blue with this special stain. | ||||
| Comments: | This 57-year-old man had chronic diabetes and renal failure. After being on dialysis for three years, he developed pruritic papules over his legs and trunk. He unfortunately died soon after diagnosis due to his multiple medical conditions. Reactive perforating collagenosis is one of the acquired perforating disorders. Some patients get this following trauma, usually during childhood. The other population with this disorder has a presentation similar to this patient, i.e. chronic renal failure and diabetes. This presentation overlaps with the other acquired perforating disorders such as Kyrle disease. Although distinction is largely academic, reactive perforating collagenosis is characterized by transelimination of collagen through the epidermis, detected by H&E or special stains, as in our case. | ||||
| Related Images: | All related Images reactive_perforating_collagenosis_1_060220 reactive_perforating_collagenosis_2_060220 | ||||
 © 2001-2009, DermAtlas | Image Name: | reactive_perforating_collagenosis_2_060220 | File Type: | jpg | |
| Diagnosis: | REACTIVE PERFORATING COLLAGENOSIS | Category: | perforating disorders / cutaneous sign of systemic disease / renal disease | ||
| Body Site: | leg / trunk | Age: | 57 years | ||
| Contributor: | Greg Hosler, MD, PhD | ||||
| Description: | Higher power at the base of the epidermal disruption allows close inspection of the granular basophilic debris, consistent with necrosis. Eosinophilic fibers resembling collagen extend up into the channel. Collagen degeneration is present in the subjacent dermis. | ||||
| Comments: | This 57-year-old man had chronic diabetes and renal failure. After being on dialysis for three years, he developed pruritic papules over his legs and trunk. He unfortunately died soon after diagnosis due to his multiple medical conditions. Reactive perforating collagenosis is one of the acquired perforating disorders. Some patients get this following trauma, usually during childhood. The other population with this disorder has a presentation similar to this patient, i.e. chronic renal failure and diabetes. This presentation overlaps with the other acquired perforating disorders such as Kyrle disease. Although distinction is largely academic, reactive perforating collagenosis is characterized by transelimination of collagen through the epidermis, detected by H&E or special stains, as in our case. | ||||
| Related Images: | All related Images reactive_perforating_collagenosis_1_060220 reactive_perforating_collagenosis_3_060220 | ||||
 © 2001-2009, DermAtlas | Image Name: | reactive_perforating_collagenosis_1_060220 | File Type: | jpg | |
| Diagnosis: | REACTIVE PERFORATING COLLAGENOSIS | Category: | perforating disorders / cutaneous sign of systemic disease / renal disease | ||
| Body Site: | leg / trunk | Age: | 57 years | ||
| Contributor: | Greg Hosler, MD, PhD | ||||
| Description: | Histologic sections of skin show a flask-shaped central disruption of the epidermis, containing abundant basophilic granular material. The surrounding epidermis is acanthotic with overlying scale crust. | ||||
| Comments: | This 57-year-old man had chronic diabetes and renal failure. After being on dialysis for three years, he developed pruritic papules over his legs and trunk. He unfortunately died soon after diagnosis due to his multiple medical conditions. Reactive perforating collagenosis is one of the acquired perforating disorders. Some patients get this following trauma, usually during childhood. The other population with this disorder has a presentation similar to this patient, i.e. chronic renal failure and diabetes. This presentation overlaps with the other acquired perforating disorders such as Kyrle disease. Although distinction is largely academic, reactive perforating collagenosis is characterized by transelimination of collagen through the epidermis, detected by H&E or special stains, as in our case. | ||||
| Related Images: | All related Images reactive_perforating_collagenosis_2_060220 reactive_perforating_collagenosis_3_060220 | ||||
 © 2001-2009, DermAtlas | Image Name: | kyrle_1_060206 | File Type: | jpg | |
| Diagnosis: | HYPERKERATOSIS FOLLICULARIS ET PARAFOLLICULARIS IN CUTEM PENETRANS / KYRLE DISEASE | Category: | papulosquamous eruptions / Metabolic disorders / renal disease / perforating disorders | ||
| Body Site: | leg | Age: | 42 years | ||
| Contributor: | Greg Hosler, MD, PhD | ||||
| Description: | Histologic sections of skin show a large keratotic plug, traversing the epidermis in a parafollicular location, with a slight epidermal invagination. The channel is filled with keratin, dense smudgy basophilic material, and inflammatory cells. At the base of the channel, there is a granulomatous inflammatory reaction. Irregular epidermal acanthosis is also noted. | ||||
| Comments: | This 42-year-old male developed numerous erythematous nodules and scaly papules over his lower extremities. He has chronic renal failure secondary to diabetes mellitus. This is a typical presentation for Kyrle disease (hyperkeratosis follicularis et parafollicularis in cutem penetrans), that is young to middle-age with diabetes and renal failure. Kylre disease is thought to represent an end-pathway for abnormal keratinization, possibly causing a "foreign-body-like" reaction to the patients own stratum corneum, but the etiology is largely unknown. This histology is also typical, in this case showing a parafollicular keratotic plug. A granulomatous reaction resides at the base of the keratin-filled channel. The differential diagnosis includes other perforating disorders, such as the perforating disorder of renal failure and/or diabetes, reactive perforating collagenosis, and perforating folliculitis. These entities show extreme overlap and may represent similar or the same processes, but can be distinguished by the location and the content of the channel. | ||||
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