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An Itch to Know
The CCA Board of Directors and Professional Advisory Board recently asked us to review the late Dr. J. Alexander Campbell's collection of celiac-related papers and correspondence. One golden nugget uncovered was the original publication of DH. This was the lead article in the Journal of the American Medical Association issued on August 30, 1884. The sole author was Dr. Louis A. Duhring of Philadelphia, Pa. Three months earlier, Dr. Duhring had read his original article to the Section on Practice of Medicine & Materia Medica of the American Medical Association.
Dr. Duhring considered DH rare but managed to accumulate 15 cases in 15 years "to warrant the view that the disease is worthy of a special description and a name ... I propose the name dermatitis herpetiformis." He found "in some cases the lesions (in clusters) were vesicular (herpes-like) and bullous (blebs), in others pustular ... in the majority of cases, bullous and pustular combined ... A varied amount of constitutional disturbance, with violent itching was always present ... repeated attacks ... over years, and was but little influenced by treatment ...The etiology (cause) and pathology of the disease are both obscure" (no elaboration provided).
The clinical features are unchanged today. "All regions are liable to invasion ... the face and scalp, elbows and knees, and palms and soles. The most striking symptom is the itching ... in all cases violent or even intense ...disproportionate in excess of the amount of eruption ... a persistent itching, causing the sufferer to scratch constantly ... generally present for several days before any sign of efflorescence ... when the eruption is profuse, (itching, burning or pricking sensations) are intense, and cause the greatest suffering."
Duhring's article is strictly descriptive. His final sentence states that "(DH) is exceedingly rebellious to treatment." but provides no example of any. Neither does he mention performing skin biopsies or other diagnostic tests. His only available test was his curious power of observation and ability to catalogue these observations until he could recognize a distinctive pattern emerging and publish his findings.
In Duhring's time (1880's), vaccinations and the bacteriological work of Louis Pasteur and others were of paramount emphasis in the medical community and diagnostic laboratory testing was in its infancy. As a dermatologist, Duhring did, however, have the advantage of not requiring postmortem examination to document his observations.
Duhring's publication of DH in 1884 preceded by four years Dr. Samuel Gee's first detailed and accurate description of CD published in 1888. Dr. Gee was senior physician at St. Bartholomew's Hospital in London, UK. His paper was titled 'On the coeliac affection'. He suspected dietary factors were the celiac culprits but another 60 years would pass before gluten toxicity was confirmed.
Suitable therapy for DH developed in 1940 when sulfapyridine was found to clear the rash. In 1950 however, dapsone was found more effective and remains the drug of choice today (in addition to a GFD for life). Dapsone may be required for long-term maintenance in some cases.
In 1966, the suspected connection between DH and CD was published and in 1967, confirmation that both CD and the rash of DH are gluten dependent was achieved.
In 1969, it was recognized that DH could only be diagnosed by a proper skin biopsy (best performed by a dermatologist). This is analogous to CD only being diagnosed by small bowel biopsies, however, it is now accepted that a skin biopsy positive for DH is simultaneously diagnostic of CD without small bowel biopsies being necessary.
Larry T. Diduch, MD, FACS, FRCSC is a member of CCA Board of Directors. Ralph E. Warren, MD, FRCPC, DTM&H is a member of CCA Professional Advisory Board.
Copyright © 2008, Canadian Celiac Association
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