Self-healing juvenile cutaneous mucinosis, a sclerodermoid disorder simulating juvenile dermatomyositis: a case-based review.

Self-healing juvenile cutaneous mucinosis (SHJCM) is a rare childhood disease with characteristic cutaneous and rheumatic manifestations. Cutaneous manifestations include a combination of nodules affecting peri-articular (especially interphalangeal joints) and head and neck areas; and linearly arranged ivory white papules over an erythematous indurated skin. Despite a benign course, an abrupt onset of symptoms with extensive cutaneous involvement often leads to parental anxiety, overenthusiastic evaluation and sometimes aggressive treatment. A peculiar cutaneous distribution in SHJCM including nodular lesions and periorbital edema, arthritis and arthralgia in a few cases, may simulate juvenile dermatomyositis. It is, therefore, important for dermatologists and pediatricians to be aware of this entity. In this report, we describe two cases of SHJCM and briefly review similarly reported cases in children.

Scleromyxedema.

Scleromyxedema is a sclerotic variant of papular mucinosis, in which lichenoid papules and scleroderma-like features are present. We describe a patient with scleromyxedema with IgG type lambda chain paraprotein, a systemic sclerosis-like illness, and myositis. The patient's serum contained Scl 70 antibodies, characteristic of scleroderma. Electromyography showed signs of acute myositis and the creatine phosphokinase (CPK) level was elevated. Multiply passaged fibroblasts from the patient's skin lesions showed altered growth response in vitro. The patient was treated with cyclosporin (4 mg/kg/day) with improvement.

Cutaneous lupus mucinosis: a review of our cases and the possible pathogenesis.

Cutaneous lupus mucinosis (CLM) is a rare variant of lupus erythematosus eruptions. Our 5 cases with CLM were reviewed. All were men with systemic lupus erythematosus (SLE). CLM occurred as asymptomatic cutaneous papules, nodules, or plaques on the trunk, upper and lower extremities, and face. Histopathology of CLM mainly revealed abundant mucin deposits among splayed collagen bundles throughout the dermis. However, some CLM lesions showed discoid lupus erythematosus-like epidermal and dermal changes and/or lupus profundus. Vasculitis was also revealed in the CLM lesions of 2 cases. The pathogenesis of CLM may be closely related to its two important features, the male preponderance and the association with SLE. Vasculopathy may also be involved in the development of CLM.

Reticular erythematous mucinosis syndrome. Report of a case with positive immunofluorescence.

We report a case with the clinical and histological features of the reticular erythematous mucinosis syndrome (REM), in which there was moderate, continuous, fine, granular, IgM deposition along the basal layer. Similar direct immunofluorescence results have been reported in only two previous cases.

Cutaneous focal mucinosis--a histopathological and immunohistochemical analysis of 11 cases.

The histogenesis of cutaneous focal mucinosis (CFM) is controversial. Eleven cases of CFM (5F, 6M; mean age 51 years) from our routine files between 1986 and the present time have, therefore, been examined histopathologically and immunohistochemically. Histology revealed an increased number of fibroblast-like cells in early lesions, whereas they were diminished or predominantly at the margin in advanced ones. The myxomatous areas showed slight to absent reticulum formation. Similarly, elastic fibers were almost absent, and collagen fibers were fragmented and replaced by variable amounts of mucin. One specimen revealed an epithelial component within the lesion reminiscent of a poorly induced trichofolliculoma. Immunohistochemically, vimentin was consistently present and correlated with the number of fibroblast-like cells. A few (< 5%) CD34+ dermal dendritic cells (DDs) were focally seen within CFM. In contrast, FXIIIa+ DDs accounted for up to 30%. Fibroblast-like cells were negative for S-100 protein, Leu7, desmin and alpha-SMA. The epithelial component within one of our specimens seems to have been induced by CFM and is a feature also seen in (angio)-myxomas. CFM appears to be a mesenchymally derived lesion composed predominantly of fibroblasts. DDs do not form the major cell component but rather seem passively incorporated.

Clinical and immunologic studies in reticular erythematous mucinosis and Jessner's lymphocytic infiltrate of skin.

BACKGROUND: Little is understood about reticular erythematous mucinosis and Jessner's lymphocytic infiltrate of skin. OBJECTIVE: Our purpose was to define reticular erythematous mucinosis and Jessner's lymphocytic infiltrate of skin further with focus on immunologic studies. METHODS: In patients with reticular erythematous mucinosis and Jessner's lymphocytic infiltrate of skin, we measured circulating immune complexes before, during, and after therapy. We examined natural killer cells in a functional assay; we performed direct immunofluorescence and T- and B-cell marker studies in skin biopsy specimens. RESULTS: The infiltrate in reticular erythematous mucinosis is composed of helper T cells. Circulating immune complexes are increased in both reticular erythematous mucinosis and Jessner's lymphocytic infiltrate of skin and decrease with hydroxychloroquine therapy and clinical clearing. Natural killer cell function is decreased in reticular erythematous mucinosis and Jessner's lymphocytic infiltrate of skin. CONCLUSION: Changes in circulating immune complex titers accompanying therapy with hydroxychloroquine and clinical clearing, with recurrence of the condition and increase in circulating immune complexes on discontinuation of treatment, point to a possible relation between these events.