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CASE REPORT
Year : 1991  |  Volume : 57  |  Issue : 3  |  Page : 148-149

Squamous cell carcinoma arising in porokeratosis of Mibelli




Correspondence Address:
K R Gautam


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  Abstract 

Multifocal squamous cell carcinoma arose from one of the plaques of porokeratosis just above the right ankle in a 35-year-old female. Histopathological examination from the plaque and the ulcer confirmed the diagnosis of porokeratosis and squamous cell carcinoma. The multifocal origin of the squamous cell carcinoma and its location on a traumatic site are rare.


Keywords: Squamous cell carcinoma, Porokeratosis of Mibelli


How to cite this article:
Gautam K R, Sharma K P, Kar K H, Jain K R, Sharma. Squamous cell carcinoma arising in porokeratosis of Mibelli. Indian J Dermatol Venereol Leprol 1991;57:148-9

How to cite this URL:
Gautam K R, Sharma K P, Kar K H, Jain K R, Sharma. Squamous cell carcinoma arising in porokeratosis of Mibelli. Indian J Dermatol Venereol Leprol [serial online] 1991 [cited 2019 Aug 23];57:148-9. Available from: http://www.ijdvl.com/text.asp?1991/57/3/148/3654


Porokeratosis of Mibelli is a rare autoso­mal dominant disorder described by Mibelli in 1893.[1] Cort and Abdel-Aziz in 1972[2] reviewed the occurrence of malignant degeneration in porokeratosis of Mibelli. They found 13 re­corded cases in the literature and described two of their own cases developing squamous cell carcinomas. Because of the rarity of this complication, we are reporting a case of porokeratosis of Mibelli with extensive and more or less bilaterally symmetrical lesions with squamous cell carcinoma arising from a lesion on the ankle.


  Case Report Top


A 35-year-old female, had a painless ul­cer on the right ankle for the last one year. An initial pustular lesion gradually evolved into an ulcer with oedema of the foot, Besides these, she had multiple, annular skin lesions over the face, trunk, both extremities including the palms and soles since childhood. There was no family history of similar lesions. The lesions were atrophic plaques of varying sizes having elevated margins with prominent grooves and loss of hair. The largest plaque measuring approximately 17x13 cm was present on the right ankle. Similar multiple lesions were present on the dorsum of the tongue and the lower lip. The mucous mem­branes of the nose and genitalia showed no abnormality.

An ulcer, 10 x 8 cm in size having an indurated, raised and everted border was present at the margin of the large sized plaque [Figure - 1]. The floor showed numerous papillomatous growths with keratinous horns at the periphery. There were two crusted lesions present near the ulcer which on removal of the crust revealed small ulcers having everted and indurated margins. All the three ulcers were non-tender. The right inguinal lymph nodes, both vertical and horizontal groups were enlarged, discrete, firm, mobile and non­tender. Four of these nodes in the vertical groups were markedly (2x 1.5 cm) enlarged.

The routine laboratory investigations of the blood were within normal limits. The ra­diological examination of the right ankle re­vealed periosteal elevation of the fibula. X-ray of the chest was normal. A fine-needle aspi­ration cytology from a right inguinal lymph node revealed malignant cells. Biopsy from the inguinal lymph node confirmed metastases. Histopathological examination of the skin lesion revealed keratin-filled invagi­nation in the centre of which was a parakeratotic column - the characteristic cornoid lamella. The granular layer was ab­sent below the parakeratotic column. There was scanty lymphocytic infiltrate around the dilated capillaries in the papillary dermis. The biopsy from the buccal mucosa also revealed a characteristic cornoid lamella. Biopsy from the ulcer showed epidermal cells of variable sizes and shapes in the dermis. There was hyperplasia and hyperchromasia of the nuclei. There was a few cells showing individual cell keratinization. Mitotic figures were prominent. There was absence of keratinization in many areas. No horn pearls were present.


  Comments Top


Porokeratosis of Mibelli is characterized by typical lesions which are usually few and asymmetrical[3]. The development of malig­nancy in porokeratosis of Mibelli is not very frequent and was reported first in the litera­tures by Vigne[4] in 1942. The malignant changes are more likely to occur at the trauma prone sites. The case described by us has many interesting features - numerous and bilaterally symmetrical lesions on the skin and mucous membranes along with multifocal malignant degeneration at three sites of the same plaque located on a trauma prone site i.e. ankle. The development of squamous cell carcinoma at traumatic sites after many years of the appearance of porokeratosis lesions is in confirmity with the findings of other workers except Girla and Bhattacharya[5], they reported its development at a nontraumatic site i.e. trunk.

Since the lesions of porokeratosis of Mibelli have a potential for malignant trans­formation, particularly if they are located on the distal parts of the extremities, a prolonged follow up of such cases is mandatory. Any ulceration appearing on such lesions of porokeratosis should raise the suspicion of malignancy.

 
  References Top

1.Mibelli V : Contributo allo studio della ipercheratosi dei canalo sudoriferi. Giornale Italiano Malattia Venero, 1893; 28 : 313-355 in : Brit J Plast Surg, 1972; 25 : 318-328.  Back to cited text no. 1    
2.Cort DF, Abdel - Aziz AHM : Epithelioma arising in Porokeratosis of Mibelli, Brit J Plast Surg, 1972; 25 : 318-328.  Back to cited text no. 2    
3.Chernosky ME : Porokeratosis, in : Dermatology in General Medicine, Seconded, Editors, Fitzpatrick TB, Eisen AZ, Wolff K et al : Mc Graw-Hill, New York, 1979; P 279-282.  Back to cited text no. 3    
4.Vigne P : Porokeratose de Mibelli trois casfamiliaux trensformation neoplasique chaz deux. Annales Dermatologie Syphiligraphie, Paris, 1942; 2 : 5-15 in, Brit J Plast Surg, 1972; 25 : 318-328.  Back to cited text no. 4    
5.Girla VS and Bhattacharya SK : Clinical study of Porokeratosis. Report of 10 cases, Internat J Dermatol, 1976; 15 : 43-51.  Back to cited text no. 5    


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