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Kyrle's Disease (KD) Dr.UwaisRiazUlHasan M.Med et al., AJSRR, 2021; 4:25 Research Article AJSRR (2021) 4:25 American Journal of Surgical Research and Reviews (ISSN:2637-5087) Kyrle’s disease (KD): "An Update with review of literature" A Spongebob Skin pores simulation Dr. UwaisRiazUlHasan* M.Med, Dr. Khathija Hasan M.Med, Dr. Victor Effiong Obong M.B.B.Ch, MWACS, Dr. Okorie Christian Chima M.B.BCh, FWACS, FMCS, Dr. Abdul Aziz Al Nami M.B.B.S, Dr. Abdullah Abdulmonem AlZarra M.B.B.S, Dr. Hassan A Al Wtayyan M.B.B.S, D r . A l i I b r a h i m A l S h a q a q i q M . B . B . S , D r . M o h a m m a d A b d u l M a j e e d A l g h a d e e r M . B . B . S , Dr. ShehlaRiazUlHasan Phd, Dr. Moath AbdulAziz AlMasoud2 M.D, Dr. Noura Al Dossary1 1Department of General Surgery, Al Omran General Hospital, Al Hassa, Kingdom of Saudi Arabia. 2Head of department, 1Hospital Director Al Omran General Hospital. ABSTRACT Kyrle’s disease (KD) is a Chronic skin condition first described *Correspondence to Author: by Austrian pathologist Josef Kyrle in 1916. Kyrle referred to Dr. UwaisRiazUlHasan M.Med this condition as hyperkeratosis follicularis & parafollicularis Assoc.consultant General surgeon, in cutem penetrans. These diseases are characterized by the Department of General Surgery, Al Omran General Hospital, Al Hassa, phenomenon of transepidermal elimination of denatured dermis Kingdom of Saudi Arabia. an acquired form of perforating dermatosis [14]. It is characterized by keratotic crater plugs that develop in hair follicles penetrating How to cite this article: [4] the epidermis and extending into the dermis . This trans UwaisRiazUlHasan, Khathija epithelial migration and elimination of proteinaceous components Hasan, Victor Effiong Obong, Oko- is associated with systemic disorders like renal, liver diseases, rie Christian Chima, Abdul Aziz chronic heart failure and diabetes mellitus. We present two Al Nami, Abdullah Abdulmonem case scenarios of a young Males with multiple chronic papular AlZarra, Hassan A Al Wtayyan, Ali Ibrahim AlShaqaqiq, Mohammad eruptions along with a review of literature for Kyrle’s disease (KD). AbdulMajeed Alghadeer, She- hlaRiazUlHasan, Moath AbdulAziz Keywords: Kyrle’s disease (KD), metabolic disease, perforating AlMasoud, Noura Al Dossary. Kyr- dermatoses, primary disorder le’s disease (KD): “An Update with review of literature” A Spongebob Skin pores simulation.American Journal of Surgical Research and Reviews, 2021; 4:25. eSciPub LLC, Houston, TX USA. Website: https://escipub.com/ AJSRR: https://escipub.com/american-journal-of-surgical-research-and-reviews/ 1 Dr.UwaisRiazUlHasan M.Med et al., AJSRR, 2021; 4:25 Introduction. Systemic disorders are extremely gradually spread to the trunk and face. In a few common in hospital wards. Skin disorders are a months these lesions enlarged to 10 mm in di- reflection of underlying diseases and the associ- ameter. His Laboratory test were as follow se- ation between the two are often missed. Kyrle’s rum creatinine was 15.45 mg/dl, serum uric acid disease (KD) is an example of such chronic pain- was 9.1 mg/dl, and HBA1c was 7.8%. Urine rou- less skin disorder with pruritis characterised by tine and microscopy showed glucose 2 + and migration of either keratin, collagen materials protein 3+, fasting blood sugar 180 mg/dl, Bac- giving rise to intense pruritis. With Obesity and terial and fungi culture were negative. A Derma- diabetes on the rise worldwide It is this intense tological referral revealed depression with kera- itching on a myriad of rashes that every treating totic plugging over his torso and lower limbs (fig- clinician needs to be aware which make Kryles ure 1A, B). A histopathological examination re- disease merits worth of discussion. vealed epidermal invagination into the dermis, CASE PRESENTATION epidermal hyperplasia, and plug formation that The patient was a 50-year-old male with a one were consistent with Kyrle’s disease (KD). He year history of gradually progressive pruritic pap- who had received treated with different topical ules with central dark plugs that started on the and systemic regimens and had a partial resolu- lower legs and spread over to cover his neck, tion of symptoms. face and torso. He had chronic renal failure for 5 The second patient was a 35 year old male with years and was on regular haemodialysis. He had a history of popular rashes all over the trunk and no similar episode in the past and had no family back he had a long history of dermatological history with a similar dermatological disorder. consultation and was referred to us for the eval- The early skin lesions started as hyperkeratotic uation of a focal abscess collection over his right papules of 2-5 mm in the lower limbs that had lower back. Figure 1 (A) Erythematous papules exhibiting a central depression with keratotic craters. (B) A close-up image of pap- ules over front of chest & Abdomen. (C) Hyperpigmented papules lower limb. Fig A. Fig B. Figure 2 (A) Papules with keratotic craters, localised abscess (B) Axillary and truncal distribution AJSRR: https://escipub.com/american-journal-of-surgical-research-and-reviews/ 2 Dr.UwaisRiazUlHasan M.Med et al., AJSRR, 2021; 4:25 Discussion- The incidence of Kyrle’s disease is there is a dislocation of the level of keratinization reported higher in women than men. The com- toward the dermal-epidermal junction [5]. It has mon age at presentation is the third decade. been proposed that in Kyrle disease (KD) kerat- Apart from adults It is reported that it might also inization focally occurs at the basilar layer of the exist in children [2] KD can be a primary or a sec- epidermal skin [3,1] There is a report on the role ondary disorder. It can have to have a genetic of an Infectious aetiology as there appears a re- inheritance [12,4] and is clinically characterised by gression of small lesions with antibiotics like hyperkeratotic parafollicular papules with a cen- clindamycin [7]. These lesions regress over time trally localised plug, It usually involves the exten- leaving behind scars and pigmented spots. sor surface of the extremities [10] The face torso Treatment Itching is relieved with Local treat- and limbs may be involved but the mucous mem- ment with soothing agents, antipruritic lotions branes and palms & soles are spared [9]. These containing menthol and Systemic anti histaminic plugs of keratin may fuse to form coil shaped for severe cases. The recommended duration for plaques. The pathophysiology of Kryles disease use of topical retinoic acid (0.1%) as treatment is is Chronic Itching induces microtrauma the com- six weeks. Tacalcitol is used its for is action on bination of chronic inflammatory mediator’s tis- the process of keratinization [8]. sue hypoxia and altered wound healing, macro The chronic recalcitrant lesions need to be con- and microangiopathy coupled with oxidative sidered for surgical removal of the lesions using stress in chronic metabolic disease states like di- Laser, cryotherapy, electrotherapy [13]. Evidence abetes, and liver disorders all set the stage for an based preference of one modality of treatment acquired dermatosis [16]. over the other are unfortunately lacking. There are currently two views on the reason for Conclusion Chronic dermatological conditions this trans epidermal migration, Pruritis induced like Kryles disease are mirror reflection of the un- repeated microtrauma that injures collagen and derlying body metabolic disease states that elastic tissue that induces transmigration [18]. every Clinician ought to be familiar with. More Metabolic derangements induced micro deposi- studies are required before drawing conclusions tion of calcium salts [19]. The plugs incite an in- on the most effective long term treatment modal- flammatory response and foreign-body giant cell ities for chronic recalcitrant cases. reaction in the dermis adjacent to the lower end References of the keratin plug, increased epithelial migration [1] Acay C, Dogan B, Dogruöz K. Kyrle’s disease a and proliferation, culminating in perforation is as case report. Turkiye Klinikleri J Dermatol 1992;2:83–6. a result of increased fibronectin concentration [17] [2] Alyahya GA, Heegard S, Prause JU. Ocular Eventually there is a transcutaneous migration of changes of Kyrle’s disease. 20-year follow-up. elastic fibres, collagen and keratin plugs. Ac- Acta Ophtalmol Scand 2000; 78: 585-589. PMID: quired KD is found frequently associated with 11037920. systemic diseases like diabetes [6], chronic renal [3] Carter VH, Constantine VS. Kyrle's disease. I. Clinical findings in five cases and review of liter- failure [1,11], liver and Chronic heart conditions. ature. Arch Dermatol. 1968 Jun. 97(6):624-32. A diagnostic criteria called Favers Criteria is de- [Medline]. scribed as meeting all of the following three cri- [4] Cunningham SR, Walsh M, Matthews R, Fulton teria: (i) age of 18 years (ii) clinical presentation R, Burrows D. Kyrle's disease. J Am Acad Der- of umbilicated papules or nodules with a central matol. 1987;16:117–23. [5] Elisabeth Ch, Schreiner W. Kyrle’s disease and adherent keratotic plug; and (iii) histopathologi- other perforating disorders. In: Dermatology in [15] cal elimination of necrotic collagen . Trans ep- General Medicine. Eds. Freedberg IM, Eisen AZ, idermal elimination is characterised with large Wolff K, Austen KF, Katz SI, Goldsmith LA. 6th keratotic and parakeratotic plugs penetrating edition. New York: McGraw-Hill Companies; from the epidermis through the dermis. At times 2003. pp. 537-542. AJSRR: https://escipub.com/american-journal-of-surgical-research-and-reviews/ 3 Dr.UwaisRiazUlHasan M.Med et al., AJSRR, 2021; 4:25 [6] Harman M, Aytekin S, Akdeniz S, Derici M. [13] Rapini RP, Herbert AA, Drucker CR.
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