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E-IJD CASE REPORT
Year : 2015  |  Volume : 60  |  Issue : 4  |  Page : 423
Kyrle's disease presenting in an extensive distribution along lines of Blaschko


1 Command Hospital, Pune, Maharashtra, India, India
2 Department of Pathology, Command Hospital, Pune, Maharashtra, India

Date of Web Publication10-Jul-2015

Correspondence Address:
Dr. Biju Vasudevan
Department of Dermatology, Command Hospital (Southern Command), Wanowrie, Pune - 411 040, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.160542

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   Abstract 

A 24-year-old lady presented with the complaints of asymptomatic dark, raised lesions over her body since past 4 years. Dermatological examination revealed hyperkeratotic, hyperpigmented papules in a Blaschkoid distribution over trunk and extremities. The hyperkeratotic lesions could be removed leaving behind a small crater. Skin biopsy revealed hyperkeratosis, parakeratosis with epidermal invagination, focal cornified plug with basophilic degenerated material and dyskeratotic keratinocytes. Dermis showed dilated capillaries with moderate amount of perivascular lymphocytic infiltrate and granulomatous lymphocytic infiltrate in the deep dermis. A diagnosis of Kyrle's disease was made and patient was started on oral acitretin 25 mg daily with satisfactory response to therapy. This is the first reported instance of Kyrle's disease occurring in an extensive Blaschkoid distribution.


Keywords: Blaschkoid, Kyrle′s disease, perforating disorders, retinoids


How to cite this article:
Verma R, Vasudevan B, Kakkar S, Mishra P, Pragasam V, Dabbas D. Kyrle's disease presenting in an extensive distribution along lines of Blaschko. Indian J Dermatol 2015;60:423

How to cite this URL:
Verma R, Vasudevan B, Kakkar S, Mishra P, Pragasam V, Dabbas D. Kyrle's disease presenting in an extensive distribution along lines of Blaschko. Indian J Dermatol [serial online] 2015 [cited 2021 Jul 31];60:423. Available from: https://www.e-ijd.org/text.asp?2015/60/4/423/160542

What was known?

  • Kyrle′s disease is one of the rare perforating disorders usually associated with renal disorders and diabetes.
  • Kyrle′s disease can occur in a linear distribution as part of Koebner′s phenomenon.



   Introduction Top


Kyrle's disease is an acquired perforating disorder usually associated with diabetes and renal failure. It classically presents on the limbs as hyperkeratotic, umbilicated papules with central keratotic crusts. It is histopathologically characterized by the transepithelial elimination of dermal material. Retinoids and ultraviolet B (UVB) constitute the first line of therapy. We herein present a case of Kyrle's disease in an extensive Blaschkoid distribution, which has not been reported earlier in the literature.


   Case Report Top


A 24-year-old lady presented with the complaints of asymptomatic dark, raised lesions over her body since past 4 years. The lesions initially appeared over both thighs and subsequently over a period of few months spread to involve the legs, chest, neck and forearms. She gave history that the lesions could be scraped off with her nails.

Her general physical and systemic examination was normal. Dermatological examination revealed hyperkeratotic, hyperpigmented papules in a Blaschkoid distribution over trunk and extremities [Figure 1] and [Figure 2]. The hyperkeratotic lesions could be removed leaving behind a small crater. Hair, nails, palms, soles and mucosa were normal. A differential diagnosis of linear epidermal verrucous nevus, naevoid porokeratosis and Kyrle's disease was considered.

Hemogram, blood sugar, renal and liver function tests were normal. Skin biopsy revealed hyperkeratosis and parakeratosis with epidermal invagination [Figure 3]a. A focal cornified plug was seen with basophilic degenerated material in small collections throughout the plug [Figure 3]b. Dyskeratotic keratinocytes were present in pockets inside the dermis [Figure 4]a. Dermis showed dilated capillaries with moderate amount of perivascular lymphocytic infiltrate and granulomatous lymphocytic infiltrate in the deep dermis [Figure 4]b. A diagnosis of Kyrle's disease was therefore made based on clinical and histopathological findings. She was started on oral acitretin 25 mg daily as she had completed her family and is showing satisfactory response to therapy.
Figure 1: Hyperpigmented hyperkeratotic papules in Blaschkoid distribution. (a) Linear lesions on lateral trunk. (b) Linear lesion on right thigh. (c) Similar lesions on posterior aspect of left lower limb and both buttocks

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Figure 2: (a) Curvilinear lesions following blaschko lines on right abdomen. (b) Close up view of the hyperpigmented hyperkeratotic lesions

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Figure 3: Histopathology of skin lesions showing: (a) Epidermal invagination (H and E, ¡¿10). (b) Focal cornified plug with basophilic degenerated material in small collections throughout the plug (H and E, ¡¿40)

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Figure 4: Histopathology of skin lesions showing: (a) Dyskeratotic keratinocytes below site of perforation (H and E, ¡¿40). (b) Dilated capillaries with granulomatous lymphocytic infiltrate in the deep dermis below an area of epidermal perforation (H and E, ¡¿10)

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   Discussion Top


Kyrle's disease is an acquired perforating dermatosis and is synonymous with hyperkeratosis follicularis et parafollicularis in cutem penetrans. The condition was first described in a young female diabetic by Kyrle in 1916. [1] It is characterized by the transepithelial elimination of certain dermal material, this feature being common to all acquired perforating disorders. [2] The exact etiology is not known, but the condition is most commonly associated with renal failure and diabetes. [3] Cases without diabetes and renal disease have been reported mainly in young siblings, pointing toward a familial pre-disposition. [4],[5] Our patient had primary Kyrle's disease with no evidence of any secondary metabolic cause.

In Kyrle's disease, dyskeratotic cells develop at multiple points within the epidermal invaginations. These cells have limited capacity to proliferate, but retain accelerated rates of keratinisation. This causes a defect in the epidermis and the rapid keratinisation perforates into the dermis eliciting a granulomatous inflammatory response. Subsequent re-epithelization from adjacent epidermis covers the entire process from base upward. Dermal connective tissue along with inflammatory and keratotic debris degenerates to form the basophilic debris, which corresponds to the keratotic plug. This is extruded from the invagination seen in the fully evolved form of the lesion. Our patient had all the classical histopathological features of Kyrle's disease namely: Follicular or extrafollicular cornified plug embedded in an epidermal invagination, basophilic degenerated material identified in small collections throughout the plug, dyskeratotic keratinization of the epithelial cells, irregular epithelial hyperplasia and inflammatory component that is typically granulomatous.

Kyrle's disease should be differentiated from the other acquired perforating disorders. In perforating folliculitis, there is epidermal invagination related to vellus hair, which was not present in our patient. Elastosis perforans serpiginosa and reactive perforating collagenosis were ruled out by absence of features on elastin and collagen stains respectively.

Blaschko's lines are cutaneous patterns considered as representing physiological development pathways of skin during the period of embryogenesis. Mutated skin cells have been proposed to move along these lines as a part of genetic mosaicism. First described in 1901 by Blaschko, the lines form V shape over spine, S shape on abdomen and inverted U shape on chest and upper back. Most of the conditions reported in Blaschkoid distribution are congenital nevoid lesions and genodermatoses such as hypomelanosis of Ito, linear and whorled nevoid hypermelanosis and inflammatory linear verrucous epidermal nevus. Acquired disorders have also been rarely described along Blascko's lines. They include lichen striatus, linear lichen planus, seborrheic keratosis, morphea, granuloma annulare, fixed drug eruption, erythema multiforme and vitiligo. The pathogenesis of acquired conditions following Blascko'slines may involve formation of mutated skin cells causing Blaschkoid pattern developing during embryogenesis and the exposure of such cells by triggers after birth.

Koebner's phenomenon in Kyrle's disease secondary to scratching leading to linear lesions has been reported in many patients. [6] Though linear lesions have been mentioned, a Blaschkoid distribution of lesions could not be found despite extensive search of literature.

Treatment of perforating disorders is difficult. Prognosis depends on the underlying diseases and they need to be treated first. In idiopathic cases and treatment resistant secondary cases, topical steroids, topical and oral retinoids along with UVB therapy constitute first line therapies. [7],[8] Alternatively, few case reports also mention satisfactory results with psoralen with UVA, doxycycline [9] and allopurinol. [10] Our patient is showing good response to oral retinoids.

Our case was unique for many reasons. Incidence of this disease in adults without a metabolic disorder is itself very rare. Linear Kyrle's disease without Koebnerization has hardly been reported. This is also the first reported case of Kyrle's disease in an extensive Blaschkoid distribution.



 
   References Top

1.
Bhambri S, Del Rosso JQ, Mobini N, Janda P. Kyrle's disease. Cosmet Dermatol 2008;21:26-8.  Back to cited text no. 1
    
2.
Sehgal VN, Jain S, Thappa DM, Bhattacharya SN, Logani K. Perforating dermatoses: A review and report of four cases. J Dermatol 1993;20:329-40.  Back to cited text no. 2
    
3.
Carter VH, Constantine VS. Kyrle's disease. I. Clinical findings in five cases and review of literature. Arch Dermatol 1968;97:624-32.  Back to cited text no. 3
    
4.
Weiner J. Kyrle's disease: Hyperkeratosis follicularis et parafollicularis in cutem penetrans in siblings. Arch Dermatol 1967;95:329-32.  Back to cited text no. 4
    
5.
Viswanathan S, Narurkar SD, Rajpal A, Nagpur NG, Avasare SS. Rare presentation of Kyrle's disease in siblings. Indian J Dermatol 2008;53:85-7.  Back to cited text no. 5
[PUBMED]  Medknow Journal  
6.
Saray Y, Seçkin D, Bilezikçi B. Acquired perforating dermatosis: Clinicopathological features in twenty-two cases. J Eur Acad Dermatol Venereol 2006;20:679-88.  Back to cited text no. 6
    
7.
Bayramgürler D, Apaydin R, Cetiner D, Zincirci C. Narrow-band ultraviolet B phototherapy for acquired perforating dermatosis. Australas J Dermatol 2003;44:76-8.  Back to cited text no. 7
    
8.
Gambichler T, Altmeyer P, Kreuter A. Treatment of acquired perforating dermatosis with narrowband ultraviolet B. J Am Acad Dermatol 2005;52:363-4.  Back to cited text no. 8
    
9.
Brinkmeier T, Schaller J, Herbst RA, Frosch PJ. Successful treatment of acquired reactive perforating collagenosis with doxycycline. Acta Derm Venereol 2002;82:393-5.  Back to cited text no. 9
    
10.
Shih CJ, Tsai TF, Huang H, Ko WC, Hung CM. Kyrle's disease successfully treated with allopurinol. Int J Dermatol 2011;50:1170-2.  Back to cited text no. 10
    

What is new?

  • Kyrle′s disease can occur in an extensive Blaschkoid distribution.
  • Kyrle′s disease can also occur in adults without an underlying metabolic disorder.


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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    Abstract
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   Case Report
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