Pitfalls in the treatment of keloidal scleroderma

Keloidal or nodular scleroderma is a rare variant of localised scleroderma that was first described by Addison in 1854.1 We report a case of keloidal scleroderma with the presence of characteristic histopathological findings and no systemic signs or symptoms. A 65-year-old woman presented with hyperchromic and infiltrated plaques, painful, in both breasts with a 7-year evolution. History of trauma or previous surgery in the affected areas was not reported. Laboratory tests were performed and were negative for antinuclear antibody, anti-Scl-70, anti-dsDNA, anti-SSA and anti-SSB. Complete blood count and metabolic panel did not reveal any abnormalities. The patient did not present sufficient criteria for a diagnosis of systemic sclerosis. Upon physical examination, she showed violaceous and hyperchromic plaques, keloid-like lesions, with large extension in the right and left breasts (Fig. 1A). Histopathological examination showed epidermis without alterations and compact dermis with a decrease between collagen fibres, with characteristics similar to those observed in keloids, favouring the diagnosis of keloidal scleroderma (Fig. 1B). Treatment with methotrexate, clobetasol propionate 0.05% cream and emollients was instituted. Our patient, with a dose of 20 mg per week of methotrexate, presented a stabilisation of the disease, without the appearance of new lesions. However, the pain and the previous keloid plaques persisted, and other treatments were subsequently implemented, such as oral mycophenolate mofetil, monthly intralesional corticosteroid application and daily use of urea cream with colchicine. None of the therapeutic approaches showed a satisfactory response, with no remission of the lesions and with persistence of previous keloid plaques and significant local pain. Keloidal scleroderma is a cutaneous disorder that can occur with or without progressive systemic sclerosis.2 Microtraumas, inherent inflammation of scleroderma, propensity for keloid formation, expression of extracellular protein tenascin and transforming growth factor (TGF)-beta cytokines may be involved in the disease's aetiology.3-5 Unlike our case, the condition generally affects young and middle-aged women. The presentation of nodular scleroderma occurs with asymptomatic or pruritic keloidal nodules and firm plaques, commonly affecting proximal regions and the trunk. The occurrence of pain, reported in our case, is not a common manifestation of the pathology described in the literature.3 Histopathological findings may be characteristic of keloid or scleroderma and may occur in a mixed form in the same biopsy.2 The orientation of collagen bundles varies: in whorl-like patterns in hypocellular areas (keloid-specific) or arranged parallel to the epidermis in the scleroderma pattern. Differential diagnosis with hypertrophic scars, keloids, fibromatosis, dermatomyofibroma and scleromyxedema should be considered.3 In the literature, there is no consensus regarding the treatment of nodular scleroderma. Therapeutic approaches include topical or intralesional corticosteroids, systemic methotrexate, UV light therapy, topical tacrolimus and imiquimod.3, 5 The prognosis is poor, and there is usually no spontaneous remission of the lesions. This case is relevant due to its particularities, such as the patient's age and the presence of pain, and emphasises the difficulty in establishing effective treatment for keloidal scleroderma. Physicians should be aware of this rare condition and consider it in the differential diagnosis of keloid-like lesions. The patient in this manuscript has given written informed consent to the publication of her case details. The data that support the findings of this study are available from the corresponding author upon reasonable request.