Dermal Mucin Deposition Research Articles

The AESOP (Adenopathy and Extensive Skin Patch Overlying a Plasmacytoma) Syndrome

We describe an easily recognizable and previously not individualized clinical syndrome that can reveal solitary plasmacytoma of bone. We report 4 patients with a slowly extending violaceous skin patch overlying a solitary plasmacytoma of bone, associated with enlarged regional lymph nodes. Biopsies of the cutaneous lesion and the lymph nodes were not specific, although increased dermal mucin deposition and vascular proliferation were present in all skin specimens. Three patients had associated polyneuropathy. One patient had POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, and Skin changes) syndrome at the time the plasmacytoma was diagnosed. Another patient developed POEMS syndrome, from which he died, 4 years after excision of the plasmacytoma. The 3 other patients were treated either with irradiation or with a combination of irradiation and surgery, and recovered completely, including from the associated neuropathy and/or POEMS syndrome. We suggest calling this unique and distinctive clinical presentation the for

Lichen myxoedematosus associated with human immunodeficiency virus infection - report of two cases and review of the literature

Lichen myxoedematosus (LM), or papular mucinosis, is an uncommon papular eruption caused by the dermal deposition of mucin. Three clinical forms can be distinguished, namely localised, disseminated (involving more than one site), and generalised LM, the last is called scleromyxoedema, and demonstrates erythema and skin sclerosis.1 Paraproteinaemia, often consisting of an abnormal IgG with lambda light chains, is usually present in patients with LM.2 Visceral involvement has also been documented.3 An association between LM and human immunodeficiency virus (HIV) infection has been reported recently. We now describe two further HIV‐positive patients with LM and present a review of the literature regarding this association.

Lymphomatoid papulosis and cutaneous CD30+ lymphoma.

Lymphomatoid papulosis and cutaneous CD30+ lymphoma are closely related conditions in which large atypical lymphocytes that have similar immunophenotypic features occur. In lymphomatoid papulosis, the lesions are papules and nodules that spontaneously involute. There are two polar histologic patterns, type A and B, in which the large atypical cells resemble those of Hodgkin's disease and mycosis fungoides, respectively, but in many cases, features of both types are present, either separately or in the same lesions. Variants of lymphomatoid papulosis include cases with a perifollicular distribution and those with lymphocytic vasculitis or dermal mucin deposits. Clinical lesions that tend to be stable, a monomorphous cellular composition, and in the case of immunocompromised patients, the presence of Epstein-Barr viral genome characterize cutaneous CD30+ lymphoma. A loss of response to transforming growth factor-beta, which normally dampens cellular proliferation, may differentiate CD30+ lymphoma from lymphomatoid papulosis.

Extracorporeal photochemotherapy: A potentially useful treatment for scleromyxedema

Scleromyxedema, a variant of papular mucinosis, is a rare condition that clinically resembles scleroderma. Itis characterized by a lichenoid papular eruption, induration of the skin, and a paraproteinemia of unknown significance. Histologic examination reveals dermal deposition of mucin; proliferation of large, stellate fibroblasts; and fibrosis. Multisystemic manifestations have also been reported. Scleromyxedema is often difficult to treat. One of the most effective therapies is melphalan or a similar alkylating agent but this carries the risk of acute toxicity and malignancy.l-4 We describe a patient who responded to extracorporeal photochemotherapy (photopheresis) after unsuccessful attempts with other treatment.