kyrle 's disease ( kd ) is a rare skin disorder characterized by transepidermal elimination of abnormal keratin .
it is classified as a subtype of acquired perforating dermatosis along with reactive perforating collagenosis , elastosis perforans serpiginosa and perforating folliculitis .
it is commonly seen in patients with diabetes , renal disease and rarely in liver disease .
kd is regarded as a genetically determined disease with onset during adulthood usually between the age of 30 and 50 years but onset as early as age 5 years and as late as 75 years has been reported with female - to - male ratio of up to 6:1 .
it presents as multiple discrete , eruptive papules with a central crust or plug , often on the lower extremities .
the first line therapy of kd is keratolytics ( salicylic acid and urea ) followed by electrocautery or radiocautery .
a 64-year - old male presented to skin department with complaints of lesions over body since 1 month with severe itching .
the lesions initially began over right leg which progressed to involve both lower limbs , back , hands , face and scalp .
he was a known case of diabetes and hypertension since 25 years on regular treatment .
patient was on hemodialysis since 1 year ( twice a week ) for chronic renal failure . on investigations
his serum creatinine was 15.45 mg / dl , serum uric acid was 9.1 mg / dl , and hba1c was 7.8% .
urine routine and microscopy showed glucose 2 + and protein 3 + , fasting blood sugar 180 mg / dl and post - prandial blood sugar 200 mg / dl . ultrasonography of abdomen and pelvis revealed right kidney size to be 8  3 cm and left kidney size to be 8.5  3 cm .
multiple , large , discrete hyperpigmented , hyperkeratotic papules with central crusted keratotic plugs over bilateral extremities [ figure 1 ] , back , neck , face and scalp were seen .
linear distribution of lesions was seen over bilateral thighs showing koebnerization [ figure 2 ] .
biopsy taken from one of the papule from right leg showed irregular epithelial hyperplasia with follicular cornified plug and focal parakeratosis .
multiple discrete hyperkeratotic papules on bilateral lowerlimbs koebnerization over right lateral thigh follicular cornified plug with focal parakeratosis ( h and e , 4 ) the patient was seen after 15 days with symptomatic improvement in the form of reduced intensity of itching .
kyrle first described this disorder in 1916 under the name hyperkeratosis follicularis et parafollicularis in cutem penetrans .
perforating disorders ( pd ) are a heterogeneous group of disorders which are categorized into four types based on physical and pathological investigations sharing the common characteristics of transepidermal elimination as elastosis perforans serpiginosa , reactive perforating collagenosis , perforating folliculitis and kd .
kasiakou et al . , suggested an infectious etiology while abnormal keratinization been proposed by tappeiner et al .
detmar et al . , suggested that defective differentiation of the epidermis and the dermoepidermal junction owing to alteration of the underlying glycosylation process may be responsible for the lesions .
elevated serum and tissue concentrations of fibronectin may be responsible for inciting increased epithelial migration and proliferation , culminating in perforation was suggested by morgan et al .
it has been found to be associated with renal failure and diabetes , shown by the report of saray et al . , in which among 22 patients with pd , 72% had chronic renal failure and 50% had diabetes . among patients with diabetes and pd , 90% had chronic renal failure .
in a report involving 21 cases of kd , 19 cases presented with a renal disorder and 12 cases had diabetes . the pathogenesis associated with diabetes mellitus
is unknown , it may be an outcome of changes in the epidermis or dermis leading to metabolic derangements and a cutaneous response to the superficial trauma and vasculopathy arising from products of oxidative damage or endoplasmic stress like advanced glycation end products and oxidized low - density lipoprotein ( ldl ) .
the mode of inheritance of kd is unclear as both autosomal dominant and autosomal recessive patterns have been reported .
it is characterized by hyperkeratotic papules and nodules , with a central keratotic plug most commonly on the lower extremities with marked disposition for calf , tibial region and posterior aspect .
arms , head and neck region are also involved while palms and soles are involved rarely .
koebner phenomenon has been noted in some cases like in our case . along with diabetes mellitus and chronic renal failure ,
it is also seen with tuberculosis , pulmonary aspergillosis , scabies , atopic dermatitis , aids , neurodermatitis , malignancy , hepatic disorders , congestive heart failure and endocrinological disorders .
it occurs in 10% of dialysis patients like our case nine cases of kd among 200 patients underwent hemodialysis because of chronic renal failure a keratotic plug is seen histologically in an atrophic epidermis penetrating papillary dermis with underlying dermal histiocytic and lymphocytic infiltrate , which constitutes the foreign body granulomatous reaction .
transdermal elimination of collagen , elastic fibers and degenerated follicular contents with or without collagen or elastic fibers is seen in rpc , eps and pf , respectively while transdermal elimination of keratotic material with no collagen or elastic fibers is observed in kd .
the first line therapy for treatment of kd is keratolytics ( salicylic acid and urea ) followed by electrocautery , cryotherapy or co2 laser surgery .
other options include ultraviolet irradiation after curetting the hyperkeratosis along combination of oral retinoids and psoralen plus ultraviolet a radiation .
emollients and oral antihistamines are useful in relieving pruritus . oral clindamycin ( 300 mg )
kd is a rare entity which has potential to adversely affect the quality of life .
therefore physicians need to have high index of suspicion for kd in patients with diabetes along with concomitant renal disease .
any suspicious lesion should be biopsied , sent for histopatholgy , to confirm the diagnosis and start the treatment at the earliest .
 kyrle 's disease ( kd ) is an acquired perforating dermatosis associated with an underlying disorder such as diabetes mellitus or chronic renal failure . 
 it presents as multiple discrete , eruptive papules with a central crust or plug , often on the lower extremities . 
 a keratotic plug is seen histologically in an atrophic epidermis and may penetrate the papillary dermis with transepidermal elimination of keratotic debris without collagen or elastic fibers . 
 various therapies have been reported that include cryotherapy , laser therapy , narrow - band ultraviolet b and use of topical or systemic retinoids . 
 hereby a case of 64-year - old male , a known case of diabetes mellitus , hypertension and chronic renal failure who developed kd is presented . 
Introduction
Case Report
Discussion
Conclusion