Diffuse systemic sclerosis with bullous lesions without systemic\nmanifestations
Male, aged 61, presenting cutaneous sclerosis involving acute onset, progressive\nevolution, of centrifugal character, for 3 months, associated with functional limitation\nof joint movements and oral opening. The dermatological exam revealed hyperpigmentation\nand diffuse cutaneous thickening, sparing the nipples, an increase in hand volume\nwithout any vasospastic phenomena or sclerodactyly, and tense bullous lesions in the\nlower limbs (, and ). He did not present\ncomorbidity, was a smoker and social drinker, and worked as a general assistant. He\npresented no exposure to chemical products like vinyl chloride, silica, etc. Additional\ntests did not reveal abnormalities, except for elevated ESR and ANA 1:320 with fine\nspeckled cytoplasmatic pattern, negative results for rheumatoid factor,\nanti-topoisomerase, anti-centromere and anti-RNP. Capillaroscopy of the nail bed with\nhairpin-shaped capillary loops, without pathological megacapillaries, ramifications or\nintersections, corresponding to discrete microcirculatory changes of nonspecific\ncharacter. The histopathological analysis showed an area of intense subepidermal edema,\nforming multiocular bullae, moderate perivascular mononuclear infiltrate, collagen\ncompression, incarceration of annexes and lymphangiectasias ( and ). Colloidal\niron staining was weakly positive for mucin in the dermis. Given the rapid evolution of\nthe disease, treatment was started using cyclophosphamide 1g/kg/month and prednisone. In\noutpatient follow-up, after 5 cycles, there was partial improvement in the cutaneous\nsclerosis, without any specific organic involvement.