Stevens-Johnson syndrome

Description

Stevens-Johnson syndrome is an immunological skin conditions, often secondary to drugs or infection. Initial presentation is usually a feverish prodromal period followed by a blistering erythematous skin condition – characterised by mucous membrane involvement


History

1844Ferdinand Ritter von Hebra (1816 – 1880)  [Translated archive text] Hebra separated from the ill defined group of erythemas an entity which he called “erythema multiforme exudativum.” – a febrile erythema occasionally involving mucous membranes

1922Albert Mason Stevens (1884-1945) and Frank Chambliss Johnson (1894-1934) published the case reports of two boys aged 7 and 8 at Bellevue hospital, New York. This report was also published later that year in the Lancet. “A New Eruptive Fever Associated with Stomatitis and Ophthalmia” in children. They could find no description of a similar cutaneous eruption and thought that no known diagnosis could be made from the symptoms and course of the disease.

Two cases have been observed of a generalized cutaneous eruption, not conforming to any recognized dermatologie condition. Both cases occurred in boys, one aged 7, the other 8, coming from widely separated parts of New York City, with no possibility of contact. Both cases manifested a purulent conjunctivitis, in Case 2 going on to panophthalmitis and total loss of vision, and in Case 1 responding to treatment, but leaving a severe corneal scar. The pus showed pyogenic organisms only; no gonococci.

Stevens-Johnson 1922 Case 1. Nature and extent of eruption on the thirteenth day of the illness.
1922: Case 1. Nature and extent of eruption on the thirteenth day of the illness.

Stevens and Johnson were the first to draw attention to the conjunctival involvement of the syndrome which was the distinguishing feature at that time

1929 – CM Rutherford

1930 – John M Wheeler

Wheeler 1930 Stevens-Johnson Syndrome case
Day 17. Marked swelling of eyelids with profusion of pus. Swelling of lips, with black crusts covering part of upper lip and all of lower. Brown spots on face and chest. Wheeler 1930

To two cases previously reported by Stevens and Johnson, and one of probably similar nature reported by Rutherford, the author adds a new case. The condition is febrile, with a general skin eruption not corresponding to any exanthematous or cutaneous disease previously identified. The disease is serious only as regards the eyes, but the incidental membranous conjunctivitis seems to tend to more or less complete destruction of sight, by the development of extremely severe corneal ulceration.

Wheeler 1929

1935 – George J. Ginandes (1904 – 1988) presented a case, reviewed the literature and suggested the term atypical erythema exudativum multiforme (Stevens-Johnson).

1946 – The Commission on Acute Respiratory Diseases (1946) considered that “The term ‘Stevens-Johnson’ has been applied with increasing frequency in this country although it has no historical justification”

The Commission published six cases of severe erythema multiforme with evidence of a pulmonary involvement in three, and recognized as a non-bacterial pneumonia. The chief purpose of the report was to emphasize the relationship of pneumonia to erythema exudativum multiforme, since lung involvement was a feature not stressed enough in previous descriptions.

1950 – The condition of Stevens-Johnson seemed to be more severe than Hebra. Later authors dividing erythema exudativum multiforme into major (Stevens-Johnson) and minor (Hebra) types


Associated Persons

Alternative names
  • There is overlap in the presentation with Toxic Epidermal Necrolysis and erythema multifome exsudativum majus.
  • They are all on the spectrum of the same pathogenetic disease process
  • Erythema exudativum multiforme (Hebra disease)

References

eponymictionary CTA

eponymictionary

the names behind the name

Emergency physician MA (Oxon) MBChB (Edin) FACEM FFSEM with a passion for rugby; medical history; medical education; and informatics. Asynchronous learning #FOAMed evangelist. Co-founder and CTO of Life in the Fast lane | Eponyms | Books | vocortex |

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