A Report of Kyrle's Disease (Hyperkeratosis Penetrans) in a 43
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A Report of Kyrle’s Disease (Hyperkeratosis Penetrans) in a 43-Year-Old Male with End-Stage Renal Disease Ryan Skinner, DO,* Nina Sabzevari, BS,** Daniel Hurd, DO*** *Chief resident, Dermatology Department, LewisGale Hospital Montgomery, Blacksburg, VA **4th-year medical student, Edward Via College of Osteopathic Medicine, Blacksburg, VA ***Program Director, Dermatology Residency Program, LewisGale Hospital Montgomery, Blacksburg, VA Disclosures: None Correspondence: Ryan Skinner, DO; [email protected] Abstract Kyrle’s disease, also known as hyperkeratosis penetrans or hyperkeratosis follicularis et parafollicularis in cutem penetrans, is a rare condition, classified as one of the perforating dermatoses. Clinical presentation is typically numerous red-brown nodules with a scaly crust and central hyperkeratotic plug. Although an identifiable cause has yet to be established, there appears to be a strong relationship with end-stage renal disease and diabetes mellitus. In this report, we present a case of Kyrle’s disease in a 43-year-old male with multiple comorbid medical conditions and provide a review of efficacious treatments. Introduction was first described in 1916 and usually presents The etiology of Kyrle’s disease is unknown, Perforating dermatoses, including Kyrle’s disease as an extensive, painless papular eruption with a and although in some cases it appears to be (or hyperkeratosis follicularis et parafollicularis hyperkeratotic central plug. It most commonly a primary perforating skin disorder, in others involves the lower extremities but can also it occurs secondary to chronic kidney disease, in cutem penetrans), perforating folliculitis, 3 elastosis perforans serpiginosa, and reactive involve the upper extremities and trunk. There liver disease, congestive heart failure or diabetes is no involvement of the acral surfaces or mucous mellitus.4 Treatment is focused on managing perforating collagenosis, are disorders of 3 transepithelial destruction of dermal structures, membranes. Histologically, there is epidermal underlying medical conditions, if present, as well atrophy with extension into the papillary dermis as keratolytic agents, although no one treatment commonly occurring secondary to chronic renal 4 disease or diabetes mellitus.1,2 Kyrle’s disease and the presence of a hyperkeratotic plug. option has proven to be efficacious in improving the appearance of lesions.4 Figure 1 Figure 2 Figure 3 Figure 3. Low-power (10x) view showing cup- shaped invagination with hyperkeratotic plug and fragmented elastic fibers in the epidermis. Figure 4 Figure 4. Higher-power (40x) field showing Figure 2. Hyperpigmented papules with epidermal hyperplasia surrounding areas of Figure 1. Hyperpigmented papules with hyperkeratotic central plaques on right lower neutrophilic debris and fragmented elastic hyperkeratotic central plaques on right forearm. extremity. fibers in the epidermis. SKINNER, SABZEVARI, HURD Page 33 Case Presentation scaly crust and central hyperkeratotic plug.6 In efficacious therapy and appropriate referrals for A 43-year-old African American male presented some instances, the lesions may coalesce to form the management of underlying disease. Future to our clinic with a chief complaint of bilateral larger plaques. Although the lower extremities are studies are needed to further investigate the role pruritic papules on his upper and lower the primary sites of involvement in the majority of renal disease and diabetes in the development extremities of two to three months’ duration. of cases, the trunk may also be affected. Typically of Kyrle’s disease. there is sparing of the mucous membranes, He initially treated the lesions using a topical 2 antifungal cream prescribed by his primary care palms, and soles. The lesions are usually painless, References physician, which was unsuccessful. His past but can be intensely pruritic and cosmetically 1. Freedberg I. Fitzpatrick’s Dermatology in medical history consisted of end-stage renal bothersome. General Medicine. New York, NY: McGraw- disease, diabetes mellitus, hypertension, thyroid Histologically, the lesions are characterized by Hill; 1999. disease, anxiety, and depression. Dialysis was a keratotic plug with basophilic cellular debris 2. Joseph D, Papali C, Pisharody R. Kyrle’s disease: initiated for five months prior to this cutaneous 7,8 filling epithelial invaginations. Additionally, a cutaneous marker of renal disorder. Indian J event due to worsening renal failure. there may be parakeratosis in some parts of the Dermatol Venereol Leprol. 1996;62:222-5. On clinical exam, the patient had numerous plug as well as abnormal keratinization involving 3. Sybert V. Genetic skin disorders. New York: hyperpigmented, excoriated papules, with the entire epidermal thickness, leading to contact 9 Oxford University Press; 1997. occasional hyperkeratotic central plaques, on the between keratinized cells and dermis. In some bilateral upper and lower extremities (Figures cases, there are neutrophils located where the 4. Saray Y, Seckin D, Bilezikci B. Acquired keratinized cells contact the dermis. Lymphocytic perforating dermatosis: clinicopathological 1 and 2). A punch biopsy was obtained from 10 a lesion on the right forearm, and pathology and histiocytic infiltrates may also be seen. features in twenty-two cases. J Eur Acad of revealed epidermal hyperplasia surrounding areas Treatment targets the presumed underlying Dermatol Venereol. 2006;20(6):679-88. of neutrophilic debris and fragmented elastic associated disease processes, such as adequate 5. Viswanathan S, Narurkar S, Rajpal A, Nagpur fibers in the epidermis and dermis (Figures 3 blood glucose control for diabetes mellitus and N, Avasare S. Rare presentation of Kyrle’s disease and 4). The appearance of the lesions, co-existing dialysis for end-stage renal disease, as well as in siblings. Indian J Dermatol. 2008;53(2):85. medical conditions, and pathologic findings direct treatment to the lesions and symptom confirmed a diagnosis of Kyrle’s Disease. The relief with topical agents. One study showed that 6. Abele D. Hyperkeratosis Penetrans (Kyrle’s patient was given potent topical corticosteroids, with better glycemic control, where fasting blood Disease). Arch Dermatol. 1961;83(2):277. and narrowband UVB therapy was recommended, glucose was < 75 mg/dL, post-prandial plasma 7. Nair P, Jivani N, Diwan N. Kyrle′s disease in but the patient failed to follow up. glucose was < 131 mg/dL, and HbA1c was < 7.5, a patient of diabetes mellitus and chronic renal the lesions completely healed in about eight weeks failure on dialysis. J Family Med Prim Care. and left behind only a hyperpigmented scar.10 2015;4(2):284. Discussion Other documented effective therapies include Kyrle’s disease is classified as one of the 8. Cunningham SR, Walsh M, Matthews R, topical salicylic acid, oral isotretinoin, tretinoin perforating dermatoses along with elastosis Fulton R, Burrows D. Kyrle’s disease. J Am Acad cream, and hypoallergenic emollients to soften perforans serpiginosa, perforating folliculitis, 11,12 Dermatol. 1987;16:117–23. and reactive perforating collagenosis. It the skin. To control pruritus, topical lotions 9. Constantine V. Kyrle’s Disease. Arch Dermatol. was first described by Austrian pathologist including methanol or camphor may be soothing, 1968;97(6):633. and dermatologist Josef Kyrle in 1916 as and oral antihistamines like hydroxyzine, which can be sedating, may aid in symptom control “hyperkeratosis follicularis et parafollicularis in 11 10. Swetha C, Sathweek S, Srinivas B. A case cutem penetrans.”1 Despite efforts to identify a at night. UV therapy can be helpful for those of Kyrle’s disease with diabetes and renal cause for the disease, it has yet to be determined with diffuse skin involvement and individuals insufficiency. J Diabetol. 2014;2:6. with renal or hepatic disease.12 The proposed whether there are any underlying hereditary links 11. Saleh H, Lloyd K, Fatteh, S. Kyrle’s Disease or if it is idiopathic in nature.1,2 The strongest mechanism of UV therapy is modification of sensitivity to pruritogens presenting in the skin.12 effectively treated with isotretinoin. J Fla Med associations thus far are with chronic renal failure Assoc. 1993;80(7):467. and diabetes mellitus; it is estimated that about Carbon dioxide laser or cryosurgery can be used 10% of patients on hemodialysis will eventually for smaller lesions; however, there are risks of 12. Krause R. Vitamin D and UV exposure in develop Kyrle disease.2 In a study by Papali et skin hypopigmentation in individuals with chronic kidney disease. Dermatoendocrinol. al., of 21 patients with Kyrle’s disease, 12 were darker skin, as well as the possibility of inducing 2013;5(1):109-16. diabetic foot infections in patients with diabetes found to have diabetic nephropathy as well 11 as elevated serum phosphorus levels.3 They mellitus or peripheral vascular disease. There proposed that because the skin lesions appeared is no randomized data to indicate a primary to improve following dialysis, it is possible that modality of treatment, and a variety of therapies hyperphosphatemia and uremic toxin buildup can be implemented with special attention to the could be contributing factors to development of underlying comorbidity, if one is present. the lesions; however, further investigation of this relationship is needed.3 Conclusion Kyrle’s has also been linked to various other In this report, we describe the case of a patient diseases, including, but not limited