title: Coexistence of generalized morphea and lichen sclerosus et atrophicus mimicking systemic disease.
abstract: A 70-year-old white housewife presented with a rare presentation of coexistent generalized morphea and lichen sclerosus et atrophicus with unusual clinical aspects. The patient had disseminated erythematous lesions that evolved into indurated large plaques. Hypopigmentation and hyperpigmentation developed later, in addition to ivory, white, and shiny plaques on the trunk (Figure 1). The skin of the arms and legs showed a wavy contour (Figure 2). Various areas were markedly sclerotic and some had edematous papules (Figure 3). Multiple indurated, ivory, white, shiny, large, and hypochromic plaques were seen on the trunk Laboratory examinations showed increased immunoglobulin A and antinuclear antibodies 1:200 speckled. Scl-70, anti-centromere, anti-ribonucleoprotein, and anti-DNA tests were negative. Esophageal manometry and abdominal and pelvic ultrasound findings were all normal. Complete blood cell count, blood profile, and urinalysis were also within normal limits. Skin biopsy of an arm lesion showed an atrophic epidermis with orthokeratotic hyperkeratosis and follicular plugging. A broad area of homogenization and edema was seen in the papillary dermis with dilated capillaries and a perivascular lymphocytic infiltration. There was also collagen sclerosis throughout the reticular dermis, and thickened, homogenized collagen bundles replaced the subcutaneous fat. Vessel walls showed proliferated intima with mucin deposition and sclerosis, as well as multiplied elastic layers. There was edema of the papillary dermis in some areas and incipient deposition of calcium (Figures 4-7).
authors.affiliation: Sector of Dermatology and Post Graduation Course in Dermatology, HUCFF/UFRJ and School of Medicine, Federal University of Rio de Janeiro, Brazil.
authors.forename: Sueli
authors.initials: S
authors.lastname: Carneiro
authors.forename: Marcia
authors.initials: M
authors.lastname: Ramos-e-Silva
authors.forename: Daniela C
authors.initials: DC
authors.lastname: Russi
authors.forename: Elisa M
authors.initials: EM
authors.lastname: Albuquerque
authors.forename: Maria Auxiliadora J
authors.initials: MA
authors.lastname: Sousa
mesh_terms: D000368:Aged; D001706:Biopsy; D005260:Female; D006801:Humans; D017495:Hyperpigmentation / Q000209:etiology; D017496:Hypopigmentation / Q000209:etiology; D018459:Lichen Sclerosus et Atrophicus / Q000150:complications* / Q000175:diagnosis / Q000473:pathology; D012594:Scleroderma, Localized / Q000150:complications* / Q000175:diagnosis / Q000473:pathology