Unilateral generalized morphea: a case report and literature review

According to Peterson’s classification, linear morphea is one of five subtypes of localized scleroderma (LS) [1]. Unlike systemic sclerosis (SS), linear morphea and other variants of LS are characterized by lack of visceral involvement, Raynaud’s phenomenon, sclerodactyly or nailfold capillary changes [2, 3]. Although extracutaneous manifestations can occur in linear morphea, these are different from the visceral involvement seen in SS [4]. The face, scalp and extremities are usually affected in linear morphea, which can be accompanied by bone involvement, growth retardation and flexion contractures [4, 5]. From single linear indurated plaques and pigmentary changes, through widespread atrophy of skin and muscles, this form may lead to movement abnormalities and poorly healing ulcers. Dermatomal distribution of the mentioned skin lesions has been observed and available data suggest that sclerotic plaques may be localized along the Blaschko lines [6]. Patients may present with elevated titers of one or more autoantibodies, most commonly antinuclear antibodies (ANA). Although no morphea-specific autoantibodies have been reported, anti-single stranded DNA antibodies (anti-ssDNA) and anti-histone antibodies (AHA) are frequently present in linear morphea [7]. Rheumatoid factor (RF) and anti-topoisomerase II antibody can be elevated, but it occurs more often in other forms of morphea, e.g. generalized morphea [8]. Several forms of linear morphea are distinguished, including: linear morphea of the extremities, en coup de sabre, progressive facial hemiatrophy [1], and recently described unilateral generalized morphea (UGM).
A 65-year-old male patient with a history of hypertension and type 2 diabetes mellitus presented with extensive plaques of sclerosis, pigmentary lesions, and atrophy of the skin and muscles on the right side of the body. First signs of the disease were observed at the age of 32. There was no known family history of skin disorders and no history of infection, trauma, drug use or toxic exposure. Despite many years of various treatment, skin lesions localized on the right side of the body were progressing continuously.
Physical examination revealed extensive, unilateral, indurated, hypopigmented and hyperpigmented lesions with a loss of adnexal skin structures, resulting in muscle atrophy of the right upper and lower limbs, right half of the face and torso (Figure 1). The circumferences of the right upper and lower limbs compared...


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