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Three cases of diffuse cutaneous mastocytosis (DCM) were at first incorrectly diagnosed as staphylococcal scalded-skin syndrome. In the first patient, at age 1 day the disease was recognized promptly by simple techniques such as Darier's sign and Tzanck smear. Much delay in making the diagnosis occurred in the other two patients, however: almost 1 year and 15 years, respectively. Bullous manifestations in mastocytosis occur only in the first two or three years. In the first months the disease can be dangerous and life threatening. To distinguish mastocytosis from vesicular and bullous neonatal disorders, one should perform Darier's sign and a Tzanck smear. The diagnosis is confirmed by histopathologic studies. Treatment of the bullous manifestations is symptomatic, with zinc oxide paste and oral antihistamines, which may provide some relief. In addition, cimetidine and sodium cromoglycate may be beneficial. At a later age psoralen plus ultraviolet A therapy may also relieve the symptoms. Particular foods and medicines can liberate histamine and should be restricted as much as possible in extremely affected patients. Special care should be taken when these patients are to undergo anesthesia. The risk of complications during and after anesthesia is also present in other forms of mastocytosis.

A case is described of malignant B-cell lymphoma with scleroderma-like manifestation. Using different monoclonals as B-cell markers the tumor appeared to be positive for surface immunoglobulins (SmIg) and for B2-antigen, but negative for intracytoplasmic immunoglobulin (CIg), BA2- and FMC7-antigens. Therefore, the tumor could be determined as a highly differentiated Sm-positive early B-cell type of B-cell lymphoma. In this clinically rare manifestation of cutaneous B-cell lymphoma aspects of the cell morphology and of cellular mediated immunity are briefly discussed.

A patient developed an exanthem after inhalation of mercury vapors. Clinically and histopathologically the exanthem resembled an erythema exudativum multiformelike drug eruption.

Tissue speciments were taken from three regions in a patient with oral progressive lupus erythematosus (LE): the tongue, the buccal mucosa and clinically uninvolved cutaneous tissue. They were subjected to immunocytochemical studies by use of markers. HLA-DR antigen expression on mucosal epithelial cells was found in association with a predominance of activated helper T-Cells in the lesional tissue. Langerhans cells (OKT-6) and B-cells (Leu-IO, B1 and B2) were nearly absent in the mucosal lesions. The presence of populations of macrophages (MY-4) in the lesional epithelium might suggest an important role for these cells, rather than for Langerhans cells, in the afferent limb of cellular immunity in this case of LE.

In two cases of elastosis perforans serpiginosa (EPS) new clinical and laboratory data are described and discussed. In Case 1, EPS was triggered by penicillamine-D within an unusually short period (about one year). In Case 2, EPS was apparently primarily triggered by a vena puncture. In both cases the light and electron microscopic findings were strictly compatible with EPS. These findings are summarized. In the active lesions of both cases increased numbers of helper T-cells and Langerhans cells were shown, while cytotoxic-suppressor T-cells were nearly completely absent. Leu 3a+ cells and Leu 6+ cells were also present in the inactive central area of the lesions. The data presented may cast doubt on the relationship of immunological findings to pathogenetic events in the disease.