Response of Schoenlein-Henoch Syndrome to ACTH : Report of a Case with Serial Skin Biopsies
Authors: J. E. Levinson, M. Horwitz, J. P. Kulka, L. Page, W. Bauer
Journal: Annals of the Rheumatic Diseases (1951)

wrists became painful, and painless swelling of the ankles developed.The patient was therefore admitted to the hospital.She had lost 18 lb. in weight since her illness began.The past history and family history were irrelevant.Physical Examination.-Thepatient appeared thin, pale, and ill, but in no acute distress.The rectal temperature was 100.20 F.; pulse 110 per minute; blood pressure 160/120.The lower eyelids were slightly puffy and the dorsum of the feet and ankles showed slight pitting oedema.The abdomen was not tender.The liver was palpable one fingerbreadth below the right costal margin and was firm.The spleen was palpable two fingers-breadth below the left costal margin.No abnormalities were noted on examination of heart, lungs, fundi, and central nervous system.Both knee joints exhibited slight soft tissue thickening and small effusions.Movements of the elbows were painful.On the extensor surfaces of the lower legs there were numerous lesions of the following types: red maculopapules, purple papules, petechiae, and raised purple scabbed areas, 3 to 10 mm. in diameter (see Fig. la, p. 259).There were red and purple maculopapules on the arms, thighs, and buttocks.Laboratory FindingsBloocl.-Haemoglobin 12-2 g. per 100 ml., white-cell count 12,700 with 81 per cent.neutro- phils, eosinophil count 70-125 per c.mm., no red-cell abnormalities, platelets present in usual numbers.Corrected sedimentation rate (Rourke-Ernstene) 1 0 mm.per min.(haematocrit of 40 per cent.).Bleeding and clotting times normal.Prothrombin activity 61 per cent.Rumpel-Leede tourniquet test positive.Urine.-Examination revealed macroscopic haematuria; sediment contained many red cells and occasional red-cell and granular casts per high-power field.Protein present in large amounts.Specific gravity 1-025.Stools.-Guaiac reaction repeatedly positive during first 2 weeks in hospital.Some stool specimens contained flecks of red blood.Serum albumin 3 55 g. per 100 ml.Serum globulin 2-27 g. per 100 ml.Serum sodium 135-9 mEq, potassium 4-5 mEq, carbon dioxide content 29-4 mEq per litre.Non-protein nitrogen 21 mg.per 100 ml.Cephalin flocculation test negative after 48 hours.Bromsulphalein retention 1 per cent.after 45 minutes.Phenolsulphonphthalein test 25 per cent.excretion of dye after 15 minutes and 75 per cent.after 2 hours.Electrocardiogram.-Normal.X-Ray Examination.-Chest,abdomen, and joints no abnormalities. Clinical CourseMay 11 to May 24.-During the first week in hospital her general condition was un- changed.Rectal temperatures 1000 to 1010 F. Tachycardia, anorexia, malaise, splenomegaly, hepatomegaly, hypertension, proteinuria, and haematuria persisted.The eyelids remained puffy but the pitting oedema of the ankles subsided.The joint pains and the slight soft tissue swelling continued, though the knee effusions decreased in size.She received 300,000 U. penicillin every 12 hours for 4 days because the throat culture revealed,8-haemolytic streptococci.Biopsy (May 16) of two maculopapular lesions on the legs was consistent with the Schoenlein-Henoch syndrome.On May 18, severe abdominal cramps recurred, the joint pains increased, and she looked much worse.New maculopapular lesions appeared on the legs, forearms, and buttocks on May 24.The liver increased in size to two fingersbreadth below the right costal margin.ACTH therapy was begun at this time and the sodium in the diet was simultaneously reduced to less than 20 mEq daily.May 25 to May 29 (First Course ofACTH Therapy).-25mg.ACTH were administered at 6-hourly intervals for 4 days.Symptomatic improvement commenced 1 day after the institution of therapy.She felt well, her appetite returned, the abdominal cramps ceased 256