Congenital erythropoietic porphyria. A case report with discussion of different porphyrin excretion patterns.

A 5-year-old girl with the classical clinical features of congenital erythropoietic porphyria (CEP) is described. Different types of CEP are discussed based on varying excretion patterns of porphyrins.

The occurrence and features of hemorrhagic skin lesions in 115 cases of systemic meningococcal disease.

Hemorrhagic skin lesions (SH) are common in systemic meningococcal disease (MCd) and are very importance in differential diagnosis. The occurrence of SH in 115 MCd cases in relation to outcome (sequelae or death) and diagnostic category of MCd is described as well as the occurrence of SH in 61 control patients with similar referral diagnoses. We found SH in 77% of the MCd patients and about 20% of the control patients. The SH in the latter group tended to be small, localized and few. Multiple SH, a larger size and a generalized distribution was found in MCd patients with a graver prognosis. Health workers and particularly the public should learn more about SH in MCd and their significance. This may improve the care of MCd patients in promoting earlier diagnosis and therapy.

Effect of treatment on antibody activity against Mycobacterium leprae antigen 7 in tuberculoid leprosy.

Anti-Mycobacterium leprae antigen 7 antibody activity was determined by radioimmunoassay during treatment in a longtime study of individual patients with newly diagnosed borderline tuberculoid (BT) leprosy and in BT leprosy patients who were suspected from their case histories to have dapsone (DDS) resistant leprosy. There was a strong correlation between clinical and antibody activity, and clinical improvement following treatment led to a marked decrease in antibody activity in most cases. A characteristic pattern of rapid and marked increase in antibody activity shortly after the initiation of treatment was observed in patients with newly diagnosed BT leprosy. This pattern may become of practical importance in the evaluation of patients with BT leprosy as an indicator that the therapy is effective, even though this pattern was associated with a transient increase in inflammatory activity in the skin lesions. The association of inflammatory activity with increased antibody activity strongly indicates that the underlying processes are associated with the stimulation of both humoral and cellular immune responses.

Pathogenesis of hemorrhagic skin lesions in meningococcal disease.

The pathogenesis of purpuric skin lesions in meningococcaemia is still not clear. The histopathology of biopsy specimens from human purpura resembles that of the local Shwartzman reaction in rabbits. However, the early histopathological picture of purpuric lesions shows a resemblance to the experimental epinephrine-endotoxic reaction produced in rabbits. Furthermore, immunological reactions may play an important role in the pathogenesis since deposits of immunoglobulins, complement and fibrinogen can be demonstrated in the walls of capillaries and small vessels in the dermis of patients with acute meningococcaemia.

Mixed bullous disease with labile erythrocyte sedimentation rate.

A woman, aged 66, fulfilled all the usual criteria of dermatitis herpetiformis. Subsequently, she developed circulating IgA and IgM basement membrane zone antibodies, a labile erythrocyte sedimentation rate (ESR), and the clinical picture changed to one of bullous pemphigoid. Her labile ESR was obviously caused by a factor related to the erythrocytes. Direct Coombs test was positive.