Response of Schoenlein-Henoch syndrome to ACTH; report of a case with serial skin biopsies.

The Schoenlein-Henoch syndrome, a condition of unknown aetiology, is defined as the clinical combination of gastro-intestinal symptoms, articular symptoms, and a characteristic exanthema which is usually purpuric. Nephritis may be an associated finding. The few available histological studies stress inflammation of the minute vessels as the dominant pathological feature (Gairdner, 1948). Because of the similarity of these manifestations of systemic disease to those of rheumatic fever, disseminated lupus erythematosus, polyarteritis nodosa, and serum sickness, it was of interest to note whether ACTH would effect as striking symptomatic relief in the Schoenlein-Henoch syndrome as it does in these clinically similar conditions. In the present study, ACTH was employed in the treatment of a case with the classical features of the Schoenlein-Henoch syndrome, including nephritis. Although the administration of ACTH was followed by symptomatic improvement, exacerbations occurred after the cessation of each course of therapy, and he nephritis persisted. The study of seven serial skin biopsies afforded the opportunity of observing what, if any, histological changes took place in consequence of ACTH therapy. Case Report Clinical History.-M.B., a 15-year-old schoolgirl, was admitted to the Massachusetts General Hospital on May 11, 1950, complaining of malaise, severe anorexia, joint pains, and a rash. She had been well and gave no history of infection or drug administration preceding the appearance of a pruritic maculopapular exanthema on the dorsum of the ankles and the extensor surfaces of the elbows and wrists 5 weeks previously. The rash extended progressively up the legs to the knees, thighs, and buttocks. Some of the maculopapular lesions on the extensor surfaces of the lower legs became haemorrhagic bullae, then dried and became scabbed. Simultaneously with the appearance of the exanthema, she had developed malaise, severe anorexia, and generalized abdominal pain which tended to localize in the right iliac fossa. Appendectomy had been performed at another hospital on April 12, 1950, several days after the onset of these symptoms. The surgeon reported a " subacute appendix " though no histological examination was made. Colicky pain accompanied by vomiting had persisted intermittently after the operation. Two and a half weeks after the onset of symptoms, the knees, elbows, and