Journal of Postgraduate Medicine
 Open access journal indexed with Index Medicus & ISI's SCI  
Users online: 721  
Home | Subscribe | Feedback | Login 
About Latest Articles Back-Issues Article Submission Resources Sections Etcetera Contact
 
  NAVIGATE Here 
  Search
 
  
 RESOURCE Links
 ::  Similar in PUBMED
 ::  Search Pubmed for
 ::  Search in Google Scholar for
 ::Related articles
 ::  Article in PDF (1,505 KB)
 ::  Citation Manager
 ::  Access Statistics
 ::  Reader Comments
 ::  Email Alert *
 ::  Add to My List *
* Registration required (free) 

  IN THIS Article
 ::  Abstract
 :: Introduction
 :: Case Report
 :: Discussion
 :: Acknowledgments
 ::  References
 ::  Article Figures

 Article Access Statistics
    Viewed3573    
    Printed89    
    Emailed0    
    PDF Downloaded15    
    Comments [Add]    
    Cited by others 1    

Recommend this journal


 


 
  Table of Contents     
CASE REPORT
Year : 2013  |  Volume : 59  |  Issue : 1  |  Page : 58-60

Keratoacanthoma centrifugam marginatum: An atypical presentation


Department of Dermatology, Venereology and Leprosy, Rajendra Institute of Medical Sciences, Ranchi, Jharkhand, India

Date of Submission21-Sep-2012
Date of Decision12-Oct-2012
Date of Acceptance06-Nov-2012
Date of Web Publication22-Mar-2013

Correspondence Address:
N Rani
Department of Dermatology, Venereology and Leprosy, Rajendra Institute of Medical Sciences, Ranchi, Jharkhand
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0022-3859.109500

Rights and Permissions


 :: Abstract 

Keratoacanthoma centrifugam marginatum (KCM) is a rare variant of keratoacanthoma characterized by a progressive peripheral growth with concomitant central healing. We report a case of a 50-year-old female who presented with multiple, veracious non-tender papules coalescing to form multiple plaques of varied diameter perioral, which was gradually progressive. On the basis of history, clinical presentation and histopathology, diagnosis of KCM was confirmed. We report this case because of atypical presentation and clinical rarity.


Keywords: Keratoacanthoma centrifugam marginatum, perioral, verrucous papules


How to cite this article:
Rani N, Kumar P, Patil J D, Choudhary S S. Keratoacanthoma centrifugam marginatum: An atypical presentation. J Postgrad Med 2013;59:58-60

How to cite this URL:
Rani N, Kumar P, Patil J D, Choudhary S S. Keratoacanthoma centrifugam marginatum: An atypical presentation. J Postgrad Med [serial online] 2013 [cited 2020 Aug 10];59:58-60. Available from: http://www.jpgmonline.com/text.asp?2013/59/1/58/109500



 :: Introduction Top


Keratoacanthomas (KA) are common benign epidermal tumors characterized by the rapid development of a firm, symmetrical, dome-shaped nodules with a horn filled crater in its center and a tendency for spontaneous regression. Solitary KA remains the most common form with its three distinct stages: Proliferation, maturation, and spontaneous involution. [1] Typical cases reach their full size of 1.0-2.5 cm within 6-8 weeks, followed by spontaneous involution within less than 6 months [2] usually located on exposed areas. Keratoacanthoma centrifugam marginatum (KCM) is a rare variant of KA that was initially described in 1962 [3] and characterized by a progressive peripheral expanding border with concomitant central healing. It is often solitary, but multiple KCMs [4],[5] have been described as in eruptive KA of Grzybowski type or in context of familial syndromes like Ferguson-Smith syndrome or the Muir-Torre syndrome. It can be localized to any region of the body but is more frequently seen on the lower legs. We present here a new case of multiple KCM with no signs of spontaneous regression along with progressive destructive growth at an atypical site.


 :: Case Report Top


A 50-year-old woman presented with a history of a small papule that initially appeared on the upper lip and slowly progressed to form a large annular plaque along with multiple small keratotic plaques and papules. The lesions involved the perioral area with its extension up to the nasal orifices and the chin extending laterally beyond the angle of the mouth [Figure 1]. Cutaneous examination showed multiple asymptomatic and confluent plaques with annular, hyperkeratosis, crusted borders along with multiple, veracious, hyperpigmented minipapules of 1-2 mm size at the center [Figure 2]. In some parts, the centers were atrophic with a cicatricle aspect disfiguring both upper lip and lower lip.
Figure 1: Keratoacanthoma centrifugam marginatum located periorally showing distortion of upper and lower lips

Click here to view
Figure 2: Verrrucous papules coalesced together in the center of the lesion

Click here to view


Histological examination of a punch biopsy performed on a papulokeratotic border showed hyperkeratosis and acanthuses with invagination of the entire epidermis into the dermis forming a crater filled with parakeratotic keratin [Figure 3]. Multiple horn pearls were present in the epidermis. The tumor cells had eosinophilia and glassy cytoplasm. The periphery of the tumor cell nests was lined by basophilic non-keratinizing keratinocytes. Cellular atypical was not detected. The adjacent dermis had pronounced infiltration of lymphocytes, neutrophils and eosinophil's. Tissue cultures and stains did not reveal infectious organisms. No granulomas suggestive of tuberculosis were detected.
Figure 3: H and E (×40 magnification) stained section showing invagination of the epidermis containing parakeratotic keratinous material and multiple horn pearls. Dermis infiltrated with multiple polymorphonuclear cells

Click here to view


Considering the clinical and pathological features, KCM was diagnosed.


 :: Discussion Top


Histology remains the gold standard for the diagnosis of classical solitary KA. In KCM, lesions are often too large for excisional biopsy but the characteristic clinical behavior, appearance, histological features (hyperkeratosis and acanthuses) and cytological features (tumor cells with eosinophilia and glassy cytoplasm) lead to the correct diagnosis.

KCM simulates squamous cell carcinoma (SCC) in many aspects but unlike SCC, which develop from epidermal keratinocytes, KA are derived from the supraseboglandular parts of hair follicles.The tendency for spontaneous regression and the extensive degree of keratinization are the most striking features, along with the typical symmetric architecture of the tumor. These develop in three stages. The early phase of proliferation is often difficult to distinguish histologically from SCC due to high mitotic rate and nuclear atypical. [6] The fully developed lesion is normally diagnosed more easily and is followed with evolution phase with frequent detection of apoptotic cells. A recent evaluation showed that a clear cut distinction between SCC and KA is not possible in atypical and difficult cases and that these tumors should be treated like SCC. [7] Histologically, these tumors often show infiltrative behavior. Atypical mitoses may be seen occasionally. [8],[9] This diagnostic difficulty is especially true for the destructive variants of KA such as giant KA, mutilating KA or KCM.

The KCM was first described by Miedzinskain 1962 as a separate entity. It can be localized to any region of the body but is more frequently seen on lower legs. It can be confused with multiple sebaceous neoplasms, Muir-Torre syndrome, SCC, inflammatory dermatomes like hypertrophic lupus erythematous, infectious dermatitis like cutaneous tuberculosis, especially lupus vulgaris, atypical mycobacterial infections or deep fungal infections. The infectious diagnoses are eliminated by tissue culture and histology with special stains.

KCM has no known etiology and has never been described in association with visceral malignancy. It is an acquired disorder of adulthood with no genetic predisposition. The treatment of choice for solitary type of KA is surgical excision with histopathological verification of the diagnosis. If surgery is not possible, as in our case, several other therapeutic options such as intraregional injections of interferon alpha, methotrexate or bleomycin, [10] topical 5-fluorouracil (5-FU) [11] and systemically administered retinoid have been reported to be effective in individual cases. Oral retinoid should be maintained until complete clearance of the lesion, [4],[12] followed by gradual reduction of the dose until termination to avoid recurrence. In our case, treatment was started with topical 5-FU, after considering the cost-effectiveness and convenience of the patient. She showed mild response after 1 month of therapy. After 1 month we started combination therapy with oral isotretinoin (1 mg/kg/day) along with topical 5-FU for better response. The patient was kept on monthly follow-up to see the efficacy of this combination therapy.Though a few cases of multiple KA have been reported, both the presentation of multiple lesions as well as perioral localization is still considered a clinical rarity.


 :: Acknowledgments Top


The authors would like to thank the patient, who allowed us to use clinical photographs and data for publication.

 
 :: References Top

1.Karaa A, Khachemoune A. Keratoacanthoma: A tumor in search of a classification. Int J Dermatol 2007;46:671-8.  Back to cited text no. 1
[PUBMED]    
2.Esche C, Kruse R, Lamberti C, Friedl W, Propping P, Lehmann P, et al. Muir-Torre syndrome: Clinical features and molecular genetic analysis. Br J Dermatol 1997;136:913-7.  Back to cited text no. 2
[PUBMED]    
3.Miedzinski F, Kosakiewicz J. Keratoacanthoma centrifugum: A special variety of keratoacanthoma. Hautarzt 1962;13:348-52.  Back to cited text no. 3
    
4.Ogasawara Y, Kinoshita E, Ishida T, Hamamoto Y, Fujiyama J, Muto M. A case of multiple keratoacanthoma centrifugum marginatum: Response to oral etretinate. J Am Acad Dermatol 2003;48:282-5.  Back to cited text no. 4
[PUBMED]    
5.Cherif F, Mebazaa A, Kort R, Makni N, Haouet S, Mokni M, et al. Multiple keratoacanthoma centrifugum marginatum. Ann Dermatol Venereol 2002;129:413-5.  Back to cited text no. 5
[PUBMED]    
6.Chalet MD, Connors RC, Ackerman AB. Squamous cell carcinoma vs. keratoacanthoma: Criteria for histologic differentiation. J Dermatol Surg 1975;1:16-7.  Back to cited text no. 6
[PUBMED]    
7.Cribier B, Asch P, Grosshans E. Differentiating squamous cell carcinoma from keratoacanthoma using histopathological criteria. Is it possible? A study of 296 cases. Dermatology 1999;199:208-12.  Back to cited text no. 7
    
8.Janecka IP, Wolff M, Crikelair GF, Cosman B. Aggressive histological features of keratoacanthoma. J Cutan Pathol 1977;4:342-8.  Back to cited text no. 8
[PUBMED]    
9.Giltman LI. Tripolar mitosis in a keratoacanthoma. Acta Derm Venereol 1981;61:362-3.  Back to cited text no. 9
[PUBMED]    
10.de la Torre C, Losada A, Cruces MJ. Keratoacanthoma centrifugum marginatum: Treatment with intralesional bleomycin. J Am Acad Dermatol 1997;37:1010-1.  Back to cited text no. 10
[PUBMED]    
11.Yuge S, Godoy DA, Melo MC, Sousa DS, Soares CT. Keratoacanthoma centrifugum marginatum: Response to topical 5-fluorouracil. J Am Acad Dermatol 2006;54:S218-9.  Back to cited text no. 11
[PUBMED]    
12.Street ML, White JW Jr, Gibson LE. Multiple keratoacanthomas treated with oral retinoids. J Am Acad Dermatol 1990;23:862-6.  Back to cited text no. 12
[PUBMED]    


    Figures

  [Figure 1], [Figure 2], [Figure 3]

This article has been cited by
1 A Giant Keratoacanthoma Treated with Surgical Excision
Hyochun Park,Hannara Park,Hoonnam Kim,Hyeonjung Yeo
Archives of Craniofacial Surgery. 2015; 16(2): 92
[Pubmed] | [DOI]



 

Top
Print this article  Email this article
 
Online since 12th February '04
2004 - Journal of Postgraduate Medicine
Official Publication of the Staff Society of the Seth GS Medical College and KEM Hospital, Mumbai, India
Published by Wolters Kluwer - Medknow