Kyrle's Disease

From Anvita Health Wiki

Contents 1 Introduction 2 Etiology 3 Epidemiology 4 Pathology 5 Clinical-manifestations 6 Laboratory 7 Differential-diagnosis 8 Management 9 More General Terms 10 References

Introduction

• A disorder of keratinization characterized keratotic papules on the extremities & trunk, most commonly associated with diabetes mellitus & renal failure, but also without associated disease.

Etiology

• associated disorders

• diabetes mellitus & renal failure (most common)
• hepatic insufficiency
• congestive heart failure
• hypothyroidism
• hyperlipoproteinemia

• idiopathic

Epidemiology

• uncommon
• 3rd to 7th decade, rarely in infants & children
• females > males

Pathology

• invaginating epidermal keratotic plug

• may perforate epidermal basement membrane into the dermis

• focal parakeratotic & basophilic debris
• marked thinning or perforation of the epidermis
• neutrophil infiltrating in the dermis beneath keratotic plugs
• lymphocyte & histiocyte dermal infiltrate around the lesions

Clinical-manifestations

• insidious, asynchronous development of lesions
• early lesions may be asymptomatic to slightly pruritic
• older lesions may be painful, especially with applied pressure
• traumatized lesions may become secondarily infected
• initial lesion is small hyperkeratotic papule
• lesions enlarge to form papules, nodules & plaques with hyperkeratotic caps/plugs
• removal of the hyperkeratotic plug leaves a crater within a depressed nodule or papule
• resolution of lesions generally leaves atrophic scars
• larger lesions may become hyperpigmented
• lesions are scattered or clustered
• enlarging lesions may coalesce
• distribution

• extensor surface of extremities
• trunk
• buttocks
• mucous membranes are not involved

Laboratory

• skin biopsy

Differential-diagnosis

• prurigo nodularis
• multiple keratoacanthomas
• perforating folliculitis
• elastosis perforans serpiginosa
• reactive perforating collagenosis
• atypical mycobacterial infection
• verruca vulgaris

Management

• topical agents

• tretinoin gel or cream
• keratolytic agents such as salicylic acid

• oral etretinate

• high dose
• need for chronic admistration precludes practicality

• cryo-, electro- or CO2 laser surgery

• resultant scar formation
• may prevent recurrence of lesions at the treatment site

• prognosis

• chronic disorder
• lesions may heal with atrophic scar
• local recurrence is common

More General Terms

• skin disease (dermatologic disorder, dermatopathy, dermatosis)

References

Color Atlas & Synopsis of Clinical Dermatology, Common & Serious Diseases, 3rd ed, Fitzpatrick et al, McGraw Hill, NY, 1997, pg 108-109

Kyrle's disease (hyperkeratosis follicularis et parafollicularis in cutem penetrans)