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|Year : 2013 | Volume
| Issue : 1 | Page : 58-60
Keratoacanthoma centrifugam marginatum: An atypical presentation
N Rani, P Kumar, JD Patil, SS Choudhary
Department of Dermatology, Venereology and Leprosy, Rajendra Institute of Medical Sciences, Ranchi, Jharkhand, India
|Date of Submission||21-Sep-2012|
|Date of Decision||12-Oct-2012|
|Date of Acceptance||06-Nov-2012|
|Date of Web Publication||22-Mar-2013|
Department of Dermatology, Venereology and Leprosy, Rajendra Institute of Medical Sciences, Ranchi, Jharkhand
Source of Support: None, Conflict of Interest: None
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
| :: Introduction|| |
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.  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  usually located on exposed areas. Keratoacanthoma centrifugam marginatum (KCM) is a rare variant of KA that was initially described in 1962  and characterized by a progressive peripheral expanding border with concomitant central healing. It is often solitary, but multiple KCMs , 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|| |
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|
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|Figure 2: Verrrucous papules coalesced together in the center of the lesion|
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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|
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Considering the clinical and pathological features, KCM was diagnosed.
| :: Discussion|| |
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.  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.  Histologically, these tumors often show infiltrative behavior. Atypical mitoses may be seen occasionally. , 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,  topical 5-fluorouracil (5-FU)  and systemically administered retinoid have been reported to be effective in individual cases. Oral retinoid should be maintained until complete clearance of the lesion, , 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|| |
The authors would like to thank the patient, who allowed us to use clinical photographs and data for publication.
| :: References|| |
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[Figure 1], [Figure 2], [Figure 3]
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