The disorder exhibits a highly variable penetrance among family members. Hereditary fibrinogen Aα-Chain amyloidosis shows variable penetrance among family members, a distinctive histological appearance, proteinuria, progressive renal impairment, and markedly better survival rates than other forms of systemic renal amyloidosis. Treatment
Treatment of hereditary fibrinogen Aα-Chain amyloidosis has relied on chronic maintenance hemodialysis and, where possible, kidney transplantation. While recurrence of amyloidosis in the transplanted kidney occurs and is to be expected, transplant survival rates for this form of amyloidosis are significantly better than those for transplants in other forms of systemic renal amyloidosis. Relatively healthy individuals with hereditary fibrinogen Aα-Chain-related renal amyloidosis may be considered for kidney and liver bi-transplantation with the expectation that survival of the transplanted kidney will be prolonged by replacing the fibrinogen Aα-Chain-producing liver with a non-diseased donor liver. Acquired dysfibrinogenemia
Presentation
Acquired dysfibrinogenemia commonly present with signs, symptoms, and/or prior diagnoses of the underlying causative disease or drug intake in an individual with an otherwise unexplained bleeding tendency or episode. Bleeding appears to be more prominent in acquired compared to congenital dysfibrinogenemia; pathological thrombosis, while potentially occurring in these individuals as a complication of their underlying disease, is an uncommon feature of the acquired disorder. Pathophysiology
Acquired dysfibrinogenemia occurs as a known or presumed consequence of an underlying disease which directly or indirectly interferes with the clotting function of fibrinogen. Individuals with acquired dysfibrinogenemias have a greater tendency for bleeding complications than those with congenital fibrinogenemia.
Symmetrical drug-related intertriginous and flexural exanthema (SDRIFE), more popularly known as baboon syndrome because of its resemblance to the distinctive red buttocks displayed by female baboons, is a systemic contact dermatitis characterized by well-demarcated patches of erythema distributed symmetrically on the buttocks. The cause of the syndrome may be drug-related, i.e. induced by systemic administration of hydroxyzine penicillin, iodinated radio contrast media and others. Symptoms and signs
The typical rash commonly appears on buttocks. This then resembles the colour of a baboons buttocks. Other areas like upper inner thigh and armpits, may be affected by the rash. The rashes are red and well-defined. The presentation is typically symmetrical and not associated with systemic symptoms. Cause
Diagnosis
Treatment
Treatment of symmetrical drug related intertriginous and flexural exanthema involves identifying and stopping the causative agent. Topical steroids can help to reduce the redness. Epidemiology
Baboon syndrome affects both sexes equally, and can occur at any age, but seems to be more common in childhood than in adulthood. See also
Airbag dermatitis
List of cutaneous conditions


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