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CORRESPONDENCE
Year : 2012  |  Volume : 57  |  Issue : 2  |  Page : 158-161
Disseminated discoid lupus erythematosus leading to squamous cell carcinoma


1 Department of Dermatology, Father Muller Medical College, Mangalore, Karnataka, India
2 Department of Oncosurgery, Father Muller Medical College, Mangalore, Karnataka, India

Date of Web Publication20-Apr-2012

Correspondence Address:
M Ramesh Bhat
Department of Dermatology, Father Muller Medical College, Mangalore, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.94298

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How to cite this article:
Bhat M R, Hulmani M, Dandakeri S, Kambil SM, Gatti R. Disseminated discoid lupus erythematosus leading to squamous cell carcinoma. Indian J Dermatol 2012;57:158-61

How to cite this URL:
Bhat M R, Hulmani M, Dandakeri S, Kambil SM, Gatti R. Disseminated discoid lupus erythematosus leading to squamous cell carcinoma. Indian J Dermatol [serial online] 2012 [cited 2020 Feb 28];57:158-61. Available from: http://www.e-ijd.org/text.asp?2012/57/2/158/94298


Sir,

Discoid lupus erythematosus (DLE) is a benign disorder of the skin, clinically characterized by red scaly patches which heal with atrophy, scarring and pigmentary changes, and histopathologically by vacuolar degeneration of basal cell layer of epidermis and patchy dermal lymphocytic infiltrate. DLE is subdivided into a localized form in which lesions are confined to the face and neck or a disseminated form in which lesions also occur elsewhere on the body. [1] Malignant transformation is a rare complication of this condition. We report a case of squamous cell carcinoma (SCC) developing over lesions of disseminated DLE. The paucity of reports of this complication in Indian literature warrants its mention.

A 42-year-old male patient came with an asymptomatic growth on the upper lip of 1 year duration. 10 years ago, he was diagnosed to have DLE and was treated with hydroxychloroquine and topical corticosteroids. On examina­tion, there were bilateral, asymmetrically dis­tributed, erythematous plaques of varying sizes over the face, ears, scalp, trunk, back [Figure 1], and extensor aspect of the forearms. Some of the plaques had adherent scaling, depigmentation and atrophy. On the middle of upper lip there was a fungating growth 2 × 3 cm in size [Figure 2]. The submental lymph nodes were not enlarged. Systemic examination was normal. Hematological investigations revealed a raised ESR, positive rheumatoid factor and positive antinuclear antibodies (ANA) (1:80 dilution), otherwise unremarkable including other specific tests of ANA profile. The skin biopsy from a lesion over the forearm was performed and sent for histopathological and direct immunofluorescence studies. Histopatholgy showed hyperkeratosis, follicular plugging, basal cell vacuolation, dermal perivascular lymphocytic infiltrate consistent with DLE [Figure 3]. Direct immunofluorescence result was also consistent with the diagnosis of DLE. Excisional biopsy of the fungating lip lesion was done and sent for histopathological examination which showed features of well differentiated SCC, such as hyperkeratosis, papillomatosis, keratin pearls, and malignant cells invading dermis arranged in cords and sheets [Figure 4]. The patient was treated with oral hydroxychloroquine, topical betamethasone dipropionate, and sunscreen agents [Figure 5].
Figure 1: Lesions of DLE over back

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Figure 2: SCC over upper lip

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Figure 3: Histopathology of DLE, showing hyperkeatosis, follicular plugging, and dermal lymphocytic infiltrate (hematoxylin and eosine staining, original magnification 100×)

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Figure 4: Histopathology of SCC showing keratin pearls under high magification (hematoxylin and eosine staining, original magnification 400×)

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Figure 5: Postoperative photograph (after 3 months)

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In disseminated DLE, characteristic lesions occur in widespread pattern over trunk and limbs. It is most commonly seen in women, tends to be persistent, resistant to therapy, and may be associated with severe psychological upset. [1]

SCC and, less commonly, basal cell carcinoma (BCC) are the most feared complications of CCLE. In one study by Millard et al., the incidence was 3.3% among 120 white patients with CCLE. [2] The scalp is the most common site involved followed by the lips. Malignancy in our patient may be related to his job as a mechanic necessitating frequent and prolonged exposure to sunlight. Heavy smoking could also be a contributing factor. The scalp is the most common site involved, followed by the lips as seen in our patient. [2] Squamous cell carcinoma usually arises in skin damaged by actinic rays. Exposure to chemicals such as coal tar, soot, arsenic and a variety of oils and distillation products is also implicated in its pathogenesis. It occasionally occurs in scars following inflammatory or degenerative processes. It is an end-stage complication of a wide array of inflammatory skin conditions. [3] Due to mutation in p53 tumor suppressor gene, there will be defect in apoptosis of keratinocytes that have sustained UV-radiation-induced DNA damage which ultimately lead to SCC. [4] Usually SCC in DLE develops after about two decades [1] but earlier onset has also been reported. [5],[6] Cutaneous squamous cell carcinomas that arise secondary to inflammatory and degenerative processes have a much higher rate of metastasis than those developing in sun damaged skin. SCC of lower lip also has a higher incidence of metastasis. [7]

There have been sporadic reports of neoplastic change in DLE which range from SCC and basal cell carcinoma to malignant fibrous histiocytoma and atypical fibroxanthoma. [8] The interval between development of DLE and SCC has varied from 4 to 20 years. [3],[5],[9] Precipitating factors for SCC are age more than 40 years, male sex, sun/ultraviolet ray exposure, skin pigmentation, and chronic inflammatory processes. There is an inverse relation between skin pigmentation and development of SCC because of the protective effect of melanin. [4]

The long-term prognosis of such cases is varied. SCC arising in DLE is regarded as a locally aggressive but low-grade carcinoma with recurrences. One study reported local recurrences in about 20% and metastasis in 30% cases. [10] Death has also been reported from multiple metastases. [11]

The risk of a patient with DLE developing systemic lupus erythematosus (SLE) is small. It varies from 1.3% to about 6.5%. The risk is higher with disseminated DLE (22%) than in DLE confined to head and neck (1.2%). [1] Our patient did not have any features of SLE, despite having disseminated DLE for more then 10 years. The presence of laboratory abnormalities in DLE does not itself appear to predispose to the development of SLE. [1]

DLE patients showing signs of nephropathy, presence of arthralgias and elevated ANA titers (> or=1:320) should be carefully monitored, because they may be at risk of developing systemic LE. [12] Anti-single-stranded DNA antibodies in a patient with DLE may indicate an increased risk of development of SLE. [13]

To conclude SCC in a patient with DLE is rare in Indian patients. Our patient had a successful outcome with local excision of the tumor.

 
   References Top

1.Goodfield MJ, Jones SK, Veale DJ. The connective tissue diseases (Discoid Lupus Erythematosus). In: Burns T, Breathnach S, Cox N, Griffiths C, editors. Rook's Textbook of Dermatology. 7 th ed. Oxford: Blackwell Science Ltd; 2004. p. 5-24.  Back to cited text no. 1
    
2.Millard LG, Barker DJ. Development of squamous cell carcinoma in chronic discoid lupus erythematosus. Clin Exp Dermatol 1978;3:161-6.  Back to cited text no. 2
[PUBMED]    
3.Kar BR, Nair V, Ebenezer G, Job CK. Squamous cell carcinoma of the scalp arising from chronic cutaneous lupus erythematosus: report of two Indian patients. Indian J Dermatol Venereol Leprol 2004;70:236-8.  Back to cited text no. 3
[PUBMED]  Medknow Journal  
4.Grossman D, Leffell DJ. Squamous Cell Carcinoma. In: Wolff K, Goldsmith LA, Gilchrest BA, Paller AS, editors. Fitzpatrick's Dermatology in general medicine. 7 th ed. New York: McGrawHill; 2008. p. 1028-36.  Back to cited text no. 4
    
5.Nair VL, Chacko M. Disseminated discoid lupus erythematosus with squamous cell carcinoma. Indian J dermatol Venereol Leprol 1991;57:196-7.   Back to cited text no. 5
  Medknow Journal  
6.Dawn G, Kanwar AJ, Dhar S, Nanda R. Squamous Cell Carcinoma over Disseminated Discoid Lupus Erythmatosus on Non-photoexposed Skin. Ind J Dermatol Venereol Leprol 1994;60:217-8.   Back to cited text no. 6
    
7.Kirkham N. Tumors and cysts of epidermis. In: Elder DE, Elenitsas R, Johnson BL, Murphy GF, editors. Lever's Histopathology of the skin. 10 th ed. Philadelphia: Wolters-Lippincott, 2009. p. 791-849.   Back to cited text no. 7
    
8.Matsushita S, Ishihara T, Kageshita T, Egawa K, Miyake T, Ono T. Multiple squamous cell carcinomas arising in lesions of discoid lupus erythematosus. J Dermatol 2004;31:73-5.   Back to cited text no. 8
[PUBMED]  [FULLTEXT]  
9.Pandhi RK, Gupta R, Kumar SA, Bhutani LK. Discoid Lupus Erythematosus in Northern India. Ind J Dermatol Venereol Leprol 1984;50:97-9.  Back to cited text no. 9
    
10.Sulica VI, Kao GF. Squamous-cell carcinoma of the scalp arising in lesions of discoid lupus erythematosus. Am J Dermatopathol 1988;10:137-41.  Back to cited text no. 10
[PUBMED]    
11.Martin S, Rosen T, Locker E. Metastatic squamous cell carcinoma of the lip. Occurrence in blacks with discoid lupus erythematosus. Arch Dermatol 1979;115:1214.  Back to cited text no. 11
[PUBMED]  [FULLTEXT]  
12.Tebbe B, Mansmann U, Wollina U, Auer-Grumbach P, Licht-Mbalyohere A, Arensmeier M, et al. Markers in cutaneous lupus erythematosus indicating systemic involvement. A multicenter study on 296 patients. Acta Derm Venereol 1997;77:305-8.  Back to cited text no. 12
[PUBMED]    
13.Costner MI, Sontheimer RD, Provost TT. Lupus erythematosus. In: Sontheimer RD, Provost TT, editors. Cutaneous manifestations of rheumatic diseases. 2 nd ed. Philadelphia (PA): Lippincott Williams and Wilkins; 2004. p.15-64.  Back to cited text no. 13
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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