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A 49-year-old woman with a history of end-stage renal disease, uncontrolled type 2 diabetes, and congestive heart failure visited the hospital for an acute heart failure exacerbation secondary to missed dialysis appointments. On admission, her provider noted that she had tender, pruritic lesions on the extensor surface of her arms. She said they had appeared 2 to 3 months after she started dialysis. She had attempted to control the pain and pruritus with over-the-counter topical hydrocortisone and oral diphenhydramine but nothing provided relief. She was recommended for follow-up at the hospital for further examination and biopsy of one of her lesions.
At this follow-up visit, the patient noted that the lesions had spread to her left knee. Multiple firm discrete papules and nodules, with central hyperkeratotic plugs, were noted along the extensor surfaces of her forearms, left extensor knee, and around her ankles (FIGURES 1A and 1B). Some of the lesions were tender. Examination of the rest of her skin was normal. A punch biopsy was obtained.
WHAT IS YOUR DIAGNOSIS?
HOW WOULD YOU TREAT THIS PATIENT?
Diagnosis: Kyrle disease
The patient’s end-stage renal disease and type 2 diabetes—along with findings from the physical examination—led us to suspect Kyrle disease. The punch biopsy, as well as the characteristic keratotic plugs (FIGURE 2) within epidermal invagination that was bordered by hyperkeratotic epidermis, confirmed the diagnosis.
Kyrle disease (also known as hyperkeratosis follicularis et follicularis in cutem penetrans) is a rare skin condition. It is 1 of 4 skin conditions that are classified as perforating skin disorders; the other 3 are elastosis perforans serpiginosa, reactive perforating collagenosis, and perforating folliculitis (TABLE1,2).3 Perforating skin disorders share the common characteristic of transepidermal elimination of material from the upper dermis.4 These disorders are typically classified based on the nature of the eliminated material and the type of epidermal disruption.5
There are 2 forms of Kyrle disease: an inherited form often seen in childhood that is not associated with systemic disease and an acquired form that occurs in adulthood, most commonly among women ages 35 to 70 years who have systemic disease.3,4,6 The acquired form of Kyrle disease is associated with diabetes and renal failure, but there is a lack of data on its pathogenesis.7,8
Characteristic findings include discrete pruritic, dry papules and nodules with central keratotic plugs that are occasionally tender. These can manifest over the extensor surface of the extremities, trunk, face, and scalp.4,7,9 Lesions most commonly manifest on the extensor surfaces of the lower extremities.
Other conditions that feature pruritic lesions
In addition to the other perforating skin disorders described in the TABLE,1,2 the differential for Kyrle disease includes the following:
Prurigo nodularis (PN) is a skin disorder in which the manifestation of extremely pruritic nodules leads to vigorous scratching and secondary infections. These lesions typically have a grouped and symmetrically distributed appearance. They often appear on extensor surfaces of upper and lower extremities.10 PN has no known etiology, but like Kyrle disease, is associated with renal failure. Biopsy can help to distinguish PN from Kyrle disease.
Continue to: Hypertrophic lichen planus
Hypertrophic lichen planus is a pruritic skin disorder characterized by the “6 Ps”: planar, purple, polygonal, pruritic, papules, and plaques. These lesions can mimic the early stages of Kyrle disease.11 However, in the later stages of Kyrle disease, discrete papules with hyperkeratotic plugs develop, whereas large plaques will be seen with lichen planus.
Keratosis pilaris (KP) is an extremely common, yet benign, disorder in which hair follicles become keratinized.12 KP can feature rough papules that are often described as “goosebumps” or having a sandpaper–like appearance. These papules often affect the upper arms. KP usually manifests in adolescents or young adults and tends to improve with age.12 The lesions are typically smaller than those seen in Kyrle disease and are asymptomatic. In addition, KP is not associated with systemic disease.
Target symptoms and any underlying conditions
In patients who have an acquired form of the disease, symptoms may improve by
For patients whose Kyrle disease is inherited or whose underlying condition is not easily treated, there are a number of treatment options to consider. First-line treatment includes topical keratolytics (salicylic acid and urea), topical retinoids, and ultraviolet light therapy.5,7 Systemic retinoids, topical steroids, cryotherapy, electrosurgery, CO2 laser surgery, and surgical excision have also been used with some success.7,14 Oral histamines and emollients also may help to relieve the pruritus. Lesions often recur upon discontinuation of therapy.
Our patient was referred to Dermatology for ultraviolet light therapy. She was also treated with topical 12% ammonium lactate twice daily. Within a few months, she reported improvement of her symptoms.
1. Rapini R. Perforating disorders. Plastic Surgery Key. Published April 22, 2017. Accessed February 18, 2021. https://plasticsurgerykey.com/perforating-disorders/
2. Patterson JW. The perforating disorders. J Am Acad Dermatol. 1984;10:561-581
3. Azad K, Hajirnis K, Sawant S, et al. Kyrle’s disease. Indian Dermatol Online J. 2013;4:378-379.
4. Arora K, Hajirnis KA, Sawant S, et al. Perforating disorders of the skin. Indian J Pathol Microbiol. 2013;56:355-358.
5. Ataseven A, Ozturk P, Kucukosmanoglu I, et al. Kyrle’s disease. BMJ Case Rep. 2014;2014: bcr2013009905.
6. Cunningham SR, Walsh M, Matthews R. Kyrle’s disease. J Am Acad Dermatol. 1987;16(pt 1):117-123.
7. Nair PA, Jivani NB, Diwan NG. Kyrle’s disease in a patient of diabetes mellitus and chronic renal failure on dialysis. J Family Med Prim Care. 2015;4:284-286.
8. Hurwitz RM, Melton ME, Creech FT 3rd, et al. Perforating folliculitis in association with hemodialysis. Am J Dermatopathol. 1982;4:101-108.
9. Kolla PK, Desai M, Pathapati RM, et al. Cutaneous manifestations in patients with chronic kidney disease on maintenance hemodialysis. ISRN Dermatol. 2012;2012:679619.
10. Lee MR, Shumack S. Prurigo nodularis: a review. Australas J Dermatol. 2005;46:211-220.
11. Usatine RP, Tinitigan M. Diagnosis and treatment of lichen planus. Am Fam Physician. 2011;84:53-60.
12. Thomas M, Khopkar US. Keratosis pilaris revisited: is it more than just a follicular keratosis? Int J Trichology. 2012;4:255-258.
13. Chang P, Fernández V. Acquired perforating disease: report of nine cases. Int J Dermatol. 1993;32:874-876.
14. Wagner G, Sachse MM. Acquired reactive perforating dermatosis. J Dtsch Dermatol Ges. 2013;11:723-729.
A 49-year-old woman with a history of end-stage renal disease, uncontrolled type 2 diabetes, and congestive heart failure visited the hospital for an acute heart failure exacerbation secondary to missed dialysis appointments. On admission, her provider noted that she had tender, pruritic lesions on the extensor surface of her arms. She said they had appeared 2 to 3 months after she started dialysis. She had attempted to control the pain and pruritus with over-the-counter topical hydrocortisone and oral diphenhydramine but nothing provided relief. She was recommended for follow-up at the hospital for further examination and biopsy of one of her lesions.
At this follow-up visit, the patient noted that the lesions had spread to her left knee. Multiple firm discrete papules and nodules, with central hyperkeratotic plugs, were noted along the extensor surfaces of her forearms, left extensor knee, and around her ankles (FIGURES 1A and 1B). Some of the lesions were tender. Examination of the rest of her skin was normal. A punch biopsy was obtained.
WHAT IS YOUR DIAGNOSIS?
HOW WOULD YOU TREAT THIS PATIENT?
Diagnosis: Kyrle disease
The patient’s end-stage renal disease and type 2 diabetes—along with findings from the physical examination—led us to suspect Kyrle disease. The punch biopsy, as well as the characteristic keratotic plugs (FIGURE 2) within epidermal invagination that was bordered by hyperkeratotic epidermis, confirmed the diagnosis.
Kyrle disease (also known as hyperkeratosis follicularis et follicularis in cutem penetrans) is a rare skin condition. It is 1 of 4 skin conditions that are classified as perforating skin disorders; the other 3 are elastosis perforans serpiginosa, reactive perforating collagenosis, and perforating folliculitis (TABLE1,2).3 Perforating skin disorders share the common characteristic of transepidermal elimination of material from the upper dermis.4 These disorders are typically classified based on the nature of the eliminated material and the type of epidermal disruption.5
There are 2 forms of Kyrle disease: an inherited form often seen in childhood that is not associated with systemic disease and an acquired form that occurs in adulthood, most commonly among women ages 35 to 70 years who have systemic disease.3,4,6 The acquired form of Kyrle disease is associated with diabetes and renal failure, but there is a lack of data on its pathogenesis.7,8
Characteristic findings include discrete pruritic, dry papules and nodules with central keratotic plugs that are occasionally tender. These can manifest over the extensor surface of the extremities, trunk, face, and scalp.4,7,9 Lesions most commonly manifest on the extensor surfaces of the lower extremities.
Other conditions that feature pruritic lesions
In addition to the other perforating skin disorders described in the TABLE,1,2 the differential for Kyrle disease includes the following:
Prurigo nodularis (PN) is a skin disorder in which the manifestation of extremely pruritic nodules leads to vigorous scratching and secondary infections. These lesions typically have a grouped and symmetrically distributed appearance. They often appear on extensor surfaces of upper and lower extremities.10 PN has no known etiology, but like Kyrle disease, is associated with renal failure. Biopsy can help to distinguish PN from Kyrle disease.
Continue to: Hypertrophic lichen planus
Hypertrophic lichen planus is a pruritic skin disorder characterized by the “6 Ps”: planar, purple, polygonal, pruritic, papules, and plaques. These lesions can mimic the early stages of Kyrle disease.11 However, in the later stages of Kyrle disease, discrete papules with hyperkeratotic plugs develop, whereas large plaques will be seen with lichen planus.
Keratosis pilaris (KP) is an extremely common, yet benign, disorder in which hair follicles become keratinized.12 KP can feature rough papules that are often described as “goosebumps” or having a sandpaper–like appearance. These papules often affect the upper arms. KP usually manifests in adolescents or young adults and tends to improve with age.12 The lesions are typically smaller than those seen in Kyrle disease and are asymptomatic. In addition, KP is not associated with systemic disease.
Target symptoms and any underlying conditions
In patients who have an acquired form of the disease, symptoms may improve by
For patients whose Kyrle disease is inherited or whose underlying condition is not easily treated, there are a number of treatment options to consider. First-line treatment includes topical keratolytics (salicylic acid and urea), topical retinoids, and ultraviolet light therapy.5,7 Systemic retinoids, topical steroids, cryotherapy, electrosurgery, CO2 laser surgery, and surgical excision have also been used with some success.7,14 Oral histamines and emollients also may help to relieve the pruritus. Lesions often recur upon discontinuation of therapy.
Our patient was referred to Dermatology for ultraviolet light therapy. She was also treated with topical 12% ammonium lactate twice daily. Within a few months, she reported improvement of her symptoms.
A 49-year-old woman with a history of end-stage renal disease, uncontrolled type 2 diabetes, and congestive heart failure visited the hospital for an acute heart failure exacerbation secondary to missed dialysis appointments. On admission, her provider noted that she had tender, pruritic lesions on the extensor surface of her arms. She said they had appeared 2 to 3 months after she started dialysis. She had attempted to control the pain and pruritus with over-the-counter topical hydrocortisone and oral diphenhydramine but nothing provided relief. She was recommended for follow-up at the hospital for further examination and biopsy of one of her lesions.
At this follow-up visit, the patient noted that the lesions had spread to her left knee. Multiple firm discrete papules and nodules, with central hyperkeratotic plugs, were noted along the extensor surfaces of her forearms, left extensor knee, and around her ankles (FIGURES 1A and 1B). Some of the lesions were tender. Examination of the rest of her skin was normal. A punch biopsy was obtained.
WHAT IS YOUR DIAGNOSIS?
HOW WOULD YOU TREAT THIS PATIENT?
Diagnosis: Kyrle disease
The patient’s end-stage renal disease and type 2 diabetes—along with findings from the physical examination—led us to suspect Kyrle disease. The punch biopsy, as well as the characteristic keratotic plugs (FIGURE 2) within epidermal invagination that was bordered by hyperkeratotic epidermis, confirmed the diagnosis.
Kyrle disease (also known as hyperkeratosis follicularis et follicularis in cutem penetrans) is a rare skin condition. It is 1 of 4 skin conditions that are classified as perforating skin disorders; the other 3 are elastosis perforans serpiginosa, reactive perforating collagenosis, and perforating folliculitis (TABLE1,2).3 Perforating skin disorders share the common characteristic of transepidermal elimination of material from the upper dermis.4 These disorders are typically classified based on the nature of the eliminated material and the type of epidermal disruption.5
There are 2 forms of Kyrle disease: an inherited form often seen in childhood that is not associated with systemic disease and an acquired form that occurs in adulthood, most commonly among women ages 35 to 70 years who have systemic disease.3,4,6 The acquired form of Kyrle disease is associated with diabetes and renal failure, but there is a lack of data on its pathogenesis.7,8
Characteristic findings include discrete pruritic, dry papules and nodules with central keratotic plugs that are occasionally tender. These can manifest over the extensor surface of the extremities, trunk, face, and scalp.4,7,9 Lesions most commonly manifest on the extensor surfaces of the lower extremities.
Other conditions that feature pruritic lesions
In addition to the other perforating skin disorders described in the TABLE,1,2 the differential for Kyrle disease includes the following:
Prurigo nodularis (PN) is a skin disorder in which the manifestation of extremely pruritic nodules leads to vigorous scratching and secondary infections. These lesions typically have a grouped and symmetrically distributed appearance. They often appear on extensor surfaces of upper and lower extremities.10 PN has no known etiology, but like Kyrle disease, is associated with renal failure. Biopsy can help to distinguish PN from Kyrle disease.
Continue to: Hypertrophic lichen planus
Hypertrophic lichen planus is a pruritic skin disorder characterized by the “6 Ps”: planar, purple, polygonal, pruritic, papules, and plaques. These lesions can mimic the early stages of Kyrle disease.11 However, in the later stages of Kyrle disease, discrete papules with hyperkeratotic plugs develop, whereas large plaques will be seen with lichen planus.
Keratosis pilaris (KP) is an extremely common, yet benign, disorder in which hair follicles become keratinized.12 KP can feature rough papules that are often described as “goosebumps” or having a sandpaper–like appearance. These papules often affect the upper arms. KP usually manifests in adolescents or young adults and tends to improve with age.12 The lesions are typically smaller than those seen in Kyrle disease and are asymptomatic. In addition, KP is not associated with systemic disease.
Target symptoms and any underlying conditions
In patients who have an acquired form of the disease, symptoms may improve by
For patients whose Kyrle disease is inherited or whose underlying condition is not easily treated, there are a number of treatment options to consider. First-line treatment includes topical keratolytics (salicylic acid and urea), topical retinoids, and ultraviolet light therapy.5,7 Systemic retinoids, topical steroids, cryotherapy, electrosurgery, CO2 laser surgery, and surgical excision have also been used with some success.7,14 Oral histamines and emollients also may help to relieve the pruritus. Lesions often recur upon discontinuation of therapy.
Our patient was referred to Dermatology for ultraviolet light therapy. She was also treated with topical 12% ammonium lactate twice daily. Within a few months, she reported improvement of her symptoms.
1. Rapini R. Perforating disorders. Plastic Surgery Key. Published April 22, 2017. Accessed February 18, 2021. https://plasticsurgerykey.com/perforating-disorders/
2. Patterson JW. The perforating disorders. J Am Acad Dermatol. 1984;10:561-581
3. Azad K, Hajirnis K, Sawant S, et al. Kyrle’s disease. Indian Dermatol Online J. 2013;4:378-379.
4. Arora K, Hajirnis KA, Sawant S, et al. Perforating disorders of the skin. Indian J Pathol Microbiol. 2013;56:355-358.
5. Ataseven A, Ozturk P, Kucukosmanoglu I, et al. Kyrle’s disease. BMJ Case Rep. 2014;2014: bcr2013009905.
6. Cunningham SR, Walsh M, Matthews R. Kyrle’s disease. J Am Acad Dermatol. 1987;16(pt 1):117-123.
7. Nair PA, Jivani NB, Diwan NG. Kyrle’s disease in a patient of diabetes mellitus and chronic renal failure on dialysis. J Family Med Prim Care. 2015;4:284-286.
8. Hurwitz RM, Melton ME, Creech FT 3rd, et al. Perforating folliculitis in association with hemodialysis. Am J Dermatopathol. 1982;4:101-108.
9. Kolla PK, Desai M, Pathapati RM, et al. Cutaneous manifestations in patients with chronic kidney disease on maintenance hemodialysis. ISRN Dermatol. 2012;2012:679619.
10. Lee MR, Shumack S. Prurigo nodularis: a review. Australas J Dermatol. 2005;46:211-220.
11. Usatine RP, Tinitigan M. Diagnosis and treatment of lichen planus. Am Fam Physician. 2011;84:53-60.
12. Thomas M, Khopkar US. Keratosis pilaris revisited: is it more than just a follicular keratosis? Int J Trichology. 2012;4:255-258.
13. Chang P, Fernández V. Acquired perforating disease: report of nine cases. Int J Dermatol. 1993;32:874-876.
14. Wagner G, Sachse MM. Acquired reactive perforating dermatosis. J Dtsch Dermatol Ges. 2013;11:723-729.
1. Rapini R. Perforating disorders. Plastic Surgery Key. Published April 22, 2017. Accessed February 18, 2021. https://plasticsurgerykey.com/perforating-disorders/
2. Patterson JW. The perforating disorders. J Am Acad Dermatol. 1984;10:561-581
3. Azad K, Hajirnis K, Sawant S, et al. Kyrle’s disease. Indian Dermatol Online J. 2013;4:378-379.
4. Arora K, Hajirnis KA, Sawant S, et al. Perforating disorders of the skin. Indian J Pathol Microbiol. 2013;56:355-358.
5. Ataseven A, Ozturk P, Kucukosmanoglu I, et al. Kyrle’s disease. BMJ Case Rep. 2014;2014: bcr2013009905.
6. Cunningham SR, Walsh M, Matthews R. Kyrle’s disease. J Am Acad Dermatol. 1987;16(pt 1):117-123.
7. Nair PA, Jivani NB, Diwan NG. Kyrle’s disease in a patient of diabetes mellitus and chronic renal failure on dialysis. J Family Med Prim Care. 2015;4:284-286.
8. Hurwitz RM, Melton ME, Creech FT 3rd, et al. Perforating folliculitis in association with hemodialysis. Am J Dermatopathol. 1982;4:101-108.
9. Kolla PK, Desai M, Pathapati RM, et al. Cutaneous manifestations in patients with chronic kidney disease on maintenance hemodialysis. ISRN Dermatol. 2012;2012:679619.
10. Lee MR, Shumack S. Prurigo nodularis: a review. Australas J Dermatol. 2005;46:211-220.
11. Usatine RP, Tinitigan M. Diagnosis and treatment of lichen planus. Am Fam Physician. 2011;84:53-60.
12. Thomas M, Khopkar US. Keratosis pilaris revisited: is it more than just a follicular keratosis? Int J Trichology. 2012;4:255-258.
13. Chang P, Fernández V. Acquired perforating disease: report of nine cases. Int J Dermatol. 1993;32:874-876.
14. Wagner G, Sachse MM. Acquired reactive perforating dermatosis. J Dtsch Dermatol Ges. 2013;11:723-729.
