Keloids in Scleroderma-Keloidal Scleroderma: A Unique Entity

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.

Keloids in patient of scleroderma

Figure 2.

Healed biopsied area, which had a nodule

Figure 3.

Dermis of keloid showing keloidal histopathology (H and E, ×10)

Figure 4.

Increased dermal collagen in biopsied nodule (H and E, ×10)

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.