[Schamberg's disease].
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A 65-year-old male developed progressive dry cough and digital clubbing after starting rituximab-CHOP chemotherapy for non-Hodgkin lymphoma. A lung biopsy showed loose non-necrotic granulomas in a background of mild fibrosis and rare eosinophils, compatible with a drug-induced hypersensitivity pneumonia. Associated manifestations of this hypersensitivity reaction were a high eosinophil count, elevated serum levels of immunoglobulin E, and a skin rash consistent with pigmented purpuric dermatitis (Schamberg disease). Corticosteroids were marginally efficacious in treating this reaction. Few similar reactions have since been described, 2 of them ultimately fatal, but none was associated with pulmonary hemorrhage. A 2.5:1 ratio between the interstitial alveolar T4/T8 lymphocytes in our case is similar to the findings in methotrexate-induced pneumonitis and farmer lung disease. This report documents the serologic and immunohistologic findings associated with a pulmonary interstitial reaction to rituximab. A review of the pertinent literature is provided. The possible pathogenetic mechanisms, including the role of cytokines, cytotoxic T-lymphocytes and CD 20 positive T-cells in relation to the administration of rituximab are discussed.
A 13-year-old boy presented with progressive pigmented purpura of Schamberg in an unusual zosteriform distribution. He recently has taken methylphenidate which has not been described as a cause of Schamberg disease. Many different skin diseases can present in a zosteriform distribution. They are reviewed systematically and sorted by pathogenetic criteria.
The clinical and histological findings in a case of pigmented purpuric lichenoid dermatitis of Gougerot-Blum is presented, and the relationship of this disease to Schamberg's disease, Majocchi's disease, and angioma serpiginosum of Hutchinson is reviewed.
The pigmented purpuras comprise a group of dermatoses that are most commonly located on the lower extremities and share related clinical and histopathologic appearances. Included is a discussion of the following entities: progressive pigmentary disease of the skin (Schamberg's disease), purpura annularis telangiectodes (Majocchi's disease), pigmented purpuric lichenoid dermatosis (Gougerot and Blum's disease), and lichen aureus.
Using a modified Raji cell radioimmunoassay, IgA- and IgG-containing circulating immune complexes (IgA-CIC, IgG-CIC) were examined in the sera of 10 patients with Henoch-Schönlein purpura (HSP) and compared with those in the sera of patients with systemic lupus erythematosus (SLE), erythema multiforme (EM), erythema nodosum (EN) and Schamberg's disease. The mean level of IgA-CIC in HSP (233 micrograms/ml) was statistically higher than that in samples from healthy blood donors (14 micrograms/ml; p less than 0.05). In the other diseases, the mean values of IgA-CIC (SLE, 72 micrograms/ml; EM, EN, 0 microgram/ml; Schamberg's disease, 5 micrograms/ml) were not statistically different from that of normal controls. The IgA-CIC level in HSP rose markedly during the active phase of the cutaneous manifestation (233 micrograms/ml), declining to the normal range during remission (4 micrograms/ml; p less than 0.05). On the contrary, IgG-CIC did not increase during either phase. There was an isolated patient in whom IgA-CIC seemed to correlate with the clinical events of cutaneous and systemic involvement. The timing was such that a change in the level of IgA-CIC generally preceded disease activity. These findings suggest that IgA-CIC may be an etiologic factor in the cutaneous and systemic involvements of HSP, and the laboratory test for IgA-CIC is not only useful as a research tool, but also valuable as a predictive indicator of disease activity.
Five patients with chronic pigmented purpura associated with odontogenic infection are described. Four patients had Schamberg's disease, and one had itching purpura. These patients were resistant to topical corticosteroid treatment, but appearance of purpuric spots ceased after treatment for periodontitis, pulpitis, or both. No circulating immune complexes were detected, and neither immunoglobulin nor complement was deposited in the papillary vessels of the skin. Odontogenic infection might be one of the precipitating factors for chronic pigmented purpura.
We have investigated the immunoperoxidase staining pattern of the epidermis in several dermatoses characterized by exocytosis of mononuclear cells into the epidermis. We found that HLA-DR antigens showed an intercellular distribution in localized areas of the epidermis in nine of ten cases of pityriasis rosea, and in all four cases of spontaneously regressing flat warts, two cases of pityriasis lichenoides chronica, two of Schamberg's disease, and one case of lichen striatus. Lichen planus and mycosis fungoides cases were used as positive controls. OKT6 antigen was recognized only on the dendritic cells of the epidermis in all these cases. Judging from the distribution of Langerhans cells, the epidermal intercellular HLA-DR antigen seems to be expressed on the keratinocytes in such diseases, and this feature was confirmed by immunoelectron microscopy. These findings support the hypothesis that the expression of HLA-DR antigen on keratinocytes in these dermatoses is linked to cellular immune reactions involving the epidermis.
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