|Year : 1991 | Volume
| Issue : 3 | Page : 148-149
Squamous cell carcinoma arising in porokeratosis of Mibelli
KR Gautam, KP Sharma, KH Kar, KR Jain, Sharma
K R Gautam
Source of Support: None, Conflict of Interest: None
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 2020 Aug 10];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. Cort and Abdel-Aziz in 1972 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|| |
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|| |
Porokeratosis of Mibelli is characterized by typical lesions which are usually few and asymmetrical. The development of malignancy in porokeratosis of Mibelli is not very frequent and was reported first in the literatures by Vigne 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, 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|| |
|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. |
|2.||Cort DF, Abdel - Aziz AHM : Epithelioma arising in Porokeratosis of Mibelli, Brit J Plast Surg, 1972; 25 : 318-328. |
|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. |
|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. |
|5.||Girla VS and Bhattacharya SK : Clinical study of Porokeratosis. Report of 10 cases, Internat J Dermatol, 1976; 15 : 43-51. |
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