Inflammatory Morphea Profunda Carlo Tomasini, MD

A 56–year-old woman was admitted to our department with a six-month history of multiple erythemato-violaceous and brownish plaques symmetrically distributed on the upper and lower extremities, and on the trunk. The skin appeared appeared atrophic and there was a remarkable induration of the subcutaneous tissues. All laboratory investigations including serology for Borrelia Burgdorfery, ANA and ENA, were within normal range. No peripheral eosinophilia was present. Chest X-ray demonstrated a moderate lung fibrosis, confirmed by CT scan. Involvement of other internal organs was excluded. A deep incisional biopsy including the subcutaneous fat was performed on a lesion of the thigh. Histopathology Slide 1 Click to view with ImageScope Click to view with a Web-Based Viewer Histopathology showed marked thickening of the septa of the subcutaneous fat with deposits of mucin in the deep portion of the dermis with a slight superficial and deep perivascular infiltrate of lymphocytes. The epidermis was unaffected. The changes in the hypodermis were characterized by thickened, edematous and fibrotic septa and a moderately dense inflammatory infiltrate consisting mainly of neutrophils with variable amounts of eosinophils, lymphocytes, and plasmacells. Mucin deposits were also copiuos. Small nodular aggregates of lymphocytes resembling lymphoid follicles were also seen at the periphery of the fat lobules. The lining of the vessels was focally thickened with plump endothelial cells. No fibrin in the vessel walls or thrombi were seen. Direct immunofluorescence was negative for Ig deposits at the dermo-epidermal junction or within vessels. The diagnosis of inflammatory morphea profunda was made on the basis of clinical and histopathologic findings and the patient was treated with prednisolone at 1mg/kg/die with a subjective improvement of symptoms and a partial disappearing of cutaneous lesions after two months' therapy with progressive tapering of the steroid. Comment The term "scleroderma" is applied to a spectrum of disorders characterized by thickening of the skin and/or subcutaneous tissues [1]. Two different clinico-laboratoristic subsets can usually be identified: systemic scleroderma, in which vesceral involvement is present, and localized scleroderma, or morphea, in which the lesions are limited to the skin. Transition from morphea to systemic sclerodermia may occur. According with a recent classification, morphea may appear in 5 main forms : plaque, generalized, bullous, linear and deep, or subcutaneous [2]. All the subtypes of morphea have a common histopathologic denominator, i.e, the sclerosis of the connective tissue, in company with a variable inflammatory infiltrate. The cause and pathogenesis of morphea are unknown. Some cases are claimed to be a consequence of infection by Borrelia burgdorferi, but that has yet to be proven. In morphea profunda, patients have multiple lesions on the upper and lower extremities, rarely on the torso. A deep, bound-down induration of the skin is most characteristic, with often a cobblestone or dimpling appearance of the overlying skin surface. The induration may cause a decreased range of motion and, in severe cases, joint contractures. Occasionally, systemic involvement is present with mild pulmonary and esophageal impairment. Histopathologically, morphea profunda is fundamentally a septal panniculitis characterized by variable thickening and hyalinization of collagen bundles in the deep dermis, the subcutaneous septa, and sometimes fascia. A moderate to marked lymphocyte-predominant infiltrate is present interstitially and around small vessels in the panniculus. Aggregates of lymphocytes and plasmacells are also seen at the periphery of the lobules. In the early, evolving or acute inflammatory stages of morphea, septa are widened by copious amounts of mucin with subtle, if any, thickening in bundles of collagen, and eosinophils and neutrophils may predominate overwhelmingly within the inflammatory infiltate. In foci, however, crowded, thickened collagen bundles are detectable [3] . In the case described herein the presence of copious amounts of mucin throughout the deep dermis and subcutaneous septa and nodular aggregates of lymphocytes at the periphery of the fat lobules could evocate a diagnosis of lupus profundus. This condition may present alone or in association with other clinico-pathologic expressions of lupus erythematosus. Histopathologically, in lupus profundus inflammatory changes involve mainly the lobules (lobular panniculitis), with a prominent lymphocytic infiltrate sometimes forming lymphoid follicles in concert with plasma cells e mucin deposits. In time, hyaline sclerosis may replace necrotic lobules and extend into the septa. References Doyle JA, Connolly SM, Winkelmann RK. Cutaneous and subcutaneous inflammatory sclerosis syndrome. Arch Dermatol 1982; 118: 886-90 Peterson LS, Nelson AM, Su WPD. Classification of morphea (linear scleroderma). Mayo Clin Proc 1995; 70: 1068-76. Su WPD, Person JR. Morphea profunda. A new concept and a histopathologic study of 23 cases. Am J Dermatopathol 1981; 3: 251-60.