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Multiple cutaneous squamous cell carcinomas arising in a patient with generalized morphea

European Journal of Dermatology. Volume 16, Numéro 1, 90-1, January-February 2006, Clinical report

Summary

Auteur(s) : M Gréco, L Kupfer-Bessaguet, JF Delahaye, P Plantin , Dermatology Unit, Laënnec Hospital, CHIC, 29107 Quimper Cedex, France, Plastic Surgery Unit, Clinique Mutualiste, 56324 Lorient Cedex, France.

Illustrations

ARTICLE

Auteur(s) : M Gréco¹, L Kupfer-Bessaguet¹, JF Delahaye², P Plantin¹

¹Dermatology Unit, Laënnec Hospital, CHIC, 29107 Quimper Cedex, France
²Plastic Surgery Unit, Clinique Mutualiste, 56324 Lorient Cedex, France

accepté le 15 Juillet 2005

We report a new case of scleroderma associated with the development of multiple squamous cell carcinomas (SCC). A 56-year-old man had been affected by generalized morphea from the age of 37 years. He presented large sclerotic patches on the chest (( figure 1 )) and arms, and numerous confluent sclerotic plaques on the legs, where erosive lesions had appeared after several years, probably due to the fragility of the skin in this area. The treatment during all these years was exclusively local with wood tar preparations and topical corticosteroids. To our knowledge, he had never undergone immunosuppressive systemic treatment and no anomaly in favour of a systemic scleroderma was observed during these years, such as Raynaud’s phenomenon or antinuclear antibodies or anticentromere antibodies. After 18 years evolution, a budding tumour appeared on an ulceration of the left ankle (( figure 2 )). The histology confirmed the diagnosis of SCC. The osseous infiltration of the calcaneum required the amputation of the left leg. Only a year later, two new budding lesions developed on erosive plaques of the right lower limb: on the internal face of the leg and on the heel. Histological examination revealed two other SCC. Amputation of the right leg was required.

Discussion

Many cases of association between scleroderma and malignancies have been reported [1, 2]. Mechanisms of interrelationship are complex and the role of some cytokines as TGF- or 1- 2 integrins implicated at the same time in scleroderma and cancers has been proposed [3]. Most are lung cancers but other tumours have also been observed. All variants of scleroderma (diffuse or limited) are associated with an increased risk of those malignancies. SCC has only been reported in ten cases: with systemic sclerosis, generalized morphea, localized morphea, or pansclerotic morphea (table 1( Table 1 )) [3-14]. SCC occurs frequently in inflammatory chronic dermatoses and in particular on chronic ulcerations [15]. Chronic sun exposure is suggested to be the first cause of SCC. However, the association between SCC and some dermatological lesions is well described: radiodermatitis, burns, lupus, HPV infections and exposure to carcinogenic products like arsenic [16]. The role of immunosuppressive medication can be underlined but cannot explain all the cases reported, such as in our observation, where there were only local treatments like tar preparations which could have been a predisposing factor. It suggests that sclerosis of the skin may indicate a greater risk of developing SCC, especially on the legs and feet. Indeed, as in our observation, the development of three SCC in one year appearing on a young man cannot be considered as fortuitous. Therefore, a follow-up would be justified (as well as information for patients suffering from cutaneous scleroderma), especially in cases with leg morphea and a tendency to ulcerative evolution, more especially as the prognosis of such an association is reserved [13].
Table 1 Squamous cell carcinoma in scleroderma

Case report	Age (years)	Sex	Type of scleroderma	Tumor site	Duration of scleroderma (years)	Reference
(M = Male, F = Female)
Frey et al. 1952	43	F	Generalized morphea	Feet	22	[4]
Duverne et al. 1958	67	M	Generalized morphea	Foot	20	[5]
Michalowski et al. 1967-1969	31	M	Generalized morphea	Feet and leg	24	[6, 7]
Jablonska1975	33	M	Systemic sclerosis	Leg	19	[8]
Laux et al. 1985	65	F	Generalized morphea	Foot	28	[9]
Koeppel et al. 1989	48	F	CREST syndrome	Hand and knee	16	[10]
Nachbar et al. 1993	40	M	Linear morphea	Foot	29	[11]
Ozturk et al. 1998	18	F	Systemic sclerosis	Leg	7	[12]
Parodi et al. 2001	20	M	Pansclerotic morphea	Leg	18	[13]
Wollina et al. 2002-2003	16	M	Pansclerotic morphea	Leg and arm	8	[14, 15]
Present report 2005	55	M	Generalized morphea	Leg and ankle	18

References

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