Indian Journal of Dermatology
CORRESPONDENCE
Year
: 2013  |  Volume : 58  |  Issue : 2  |  Page : 153--154

Keloids in scleroderma-keloidal scleroderma: A unique entity


Sumit Sen, Tanusree Biswas, Gautam Banerje, Saugato Biswas 
 Department of Dermatology, IPGMER and SSKM Hospitals, Kolkata, India

Correspondence Address:
Sumit Sen
Department of Dermatology, IPGMER and SSKM Hospitals, Kolkata
India




How to cite this article:
Sen S, Biswas T, Banerje G, Biswas S. Keloids in scleroderma-keloidal scleroderma: A unique entity.Indian J Dermatol 2013;58:153-154


How to cite this URL:
Sen S, Biswas T, Banerje G, Biswas S. Keloids in scleroderma-keloidal scleroderma: A unique entity. Indian J Dermatol [serial online] 2013 [cited 2021 Apr 17 ];58:153-154
Available from: https://www.e-ijd.org/text.asp?2013/58/2/153/108066


Full Text

Sir,

Scleroderma with multiple keloids is a rare finding. The disorder may present as nodules or keloidal plaques and may occur on normal skin or on thickened sclerodermatous skin. The morphology of these lesions often does not correlate with the histologic findings.

A 26-year-old female patient presented to the dermatology outdoor with progressive tightening of skin of face and fingers, constricted oral aperture, sclerodactyly, and digital pitted scarring of second and third fingers of left upper limb since last 8 years. Flat swellings had erupted over the sternal region, upper extremities [Figure 1] and trunk since the last 6 years were itchy, progressive and could clinically be diagnosed as keloids. There was no previous history of keloid formation nor was there any such suggestive family history. The person developed arthalgia of the great toe and wrist since last 2.5 years, and Raynauds phenomenon was present for the last 1 year. At this time, she observed two small, non-itchy nodules on her abdomen. She presented a mask-like facies and thickened skin over abdomen and upper extremities. Examination of other systems and routine investigations were normal. ANA was positive at a titer of 1:40 with speckled pattern. Anti-Smith antibody was equivocal, and antibody against Scl 70 was negative. Anti-centromere antibody showed a negative result. Biopsy was taken from a keloidal lesion over the upper extremity and a nodule near the umbilicus, which soon healed [Figure 2]. Histopathology of the keloidal lesion was more suggestive of a keloid though it did not have the whorled pattern characteristic of keloids [Figure 3]. The biopsied nodular lesion over the abdomen showed hyperkeratosis, acanthosis, and increased pigmentation of the basal layer. Dermal collagen was increased. Features were suggestive of nodular scleroderma [Figure 4].{Figure 1}{Figure 2}{Figure 3}{Figure 4}

Nodular scleroderma is a rare type of cutaneous scleroderma and may present as countless lesions. [1] Patients often develop plaques that are clinically indistinguishable from a keloid; however, the disorder is different from keloids, occurring in otherwise healthy people. Histopathological findings are more variable. [2] Nodules may histologically show the presence of keloidal collagen. [3] Focally increased tenascin expression, thought to be a marker for tissue remodeling, [4] differing from that in the surrounding scleroderma skin was found in these nodules on histopathological examination. Others [5] reported the occurrence of acid-fast bacilli and non-acid fast coccifrom the histologic sections of nodular scleroderma, but this role of bacteria have not been substantiated.

Keloidal nodules in scleroderma is the result of dermal injury caused by the inflammatory process of scleroderma, which triggers a keloidal process, possibly in persons having a fibrotic diathesis. [6] Recent studies suggest that connective tissue growth factor (CTGF) is involved in this fibrotic process, and its expression is increased in fibroblasts from affected skin. [7] Rencic et al.[8] have identified 2 clinical variants: [1] keloidal or nodular lesions arising from sclerodermatous skin with histological findings of keloid or scleroderma, [2] typical keloids, clinically and histologically, arising in normal skin in patients with a family history of keloids. Our case demonstrated the sudden appearance of such plaques and nodules on affected thickened skin.

References

1Krell JM, Solomon AR, Glavey CM, Lawley TJ. Nodular scleroderma. J Am Acad Dermatol 1995;32:343-5.
2Kauer F, Simon JC, Sticherling M. Nodular morphea. Dermatology 2009;218:63-6.
3Wriston CC, Rubin AI, Elenitsas R, Crawford GH. Nodular scleroderma: A report of 2 cases. Am J Dermatopathol 2008;30:385-8.
4Mizutani H, Taniguchi H, Sakakura T, Shimizu M. Nodular scleroderma: Focally increased tenascin expression differing from that in the surrounding scleroderma skin. J Dermatol 1995;22:267-71.
5Cantwell AR Jr, Rowe L, Kelso DW. Nodular scleroderma and pleomorphic acid-fast bacteria. Arch Dermatol 1980;116:1283-90.
6Akintewe TA, Alabi GO. Scleroderma presenting with multiple keloids. Br Med J (Clin Res Ed) 1985;291:448-9.
7Yamamoto T, Sawada Y, Katayama I, Nishioka K. Nodular scleroderma: Increased expression of connective tissue growth factor. Dermatology 2005;211:218-23.
8Rencic A, Brinster N, Nousari CH. Keloid morphea and nodular scleroderma: Two distinct clinical variants of scleroderma? J Cutan Med Surg 2003;7:20-4.