NOTES — Synonyms & Subdivisions


From the Editor…

I have been working on a revision of the PRP Report in the Rare Disease Database hosted by the National Organization of Rare Disorders. One of the topics is SYNONYMS.

NORD PRP REPORT SYNONYMS

NORD guidelines require “a list of other names for pityriasis rubra pilaris. The current PRP Report offers six “synonyms. PRP was actually listed last. We begin this revision by moving PRP to the top of the list.

When published, only the synonym will appear (NORD format). The additional commentary will not appear,

❏½ PRP – the acronym for pityriasis rubra pilaris. Duh. Not to be confused with Plasma Rich Platelets which is what you will find when you Google, Yahoo or Bing the acronym PRP.

❏½ Devergie’s Disease – Misspelled in NORD PRP Report (missing ‘s). Marie Guillaume Alphonse Devergie, a dermatologist and forensic doctor at St. Louis Hospital in Paris, published the most complete description of PRP. In fact, it was considered to be the “original description” of PRP. Devergie’s article was published in the Gazette Hebdomadaire de Medecine et de Chirurgie in 1856. Claudius Tarral, a French dermatologist, wrote about the case in “Traite theorique et pratique des maladies de la peau” (Treatise on Skin Diseases) in 1835. Tarral saw it as a variant of psoriasis. Might have been called Tarral’s Disease if Claudius had been a better writer.

❏½ lichen acuminatus – Synonym for lichen planus. A primary disorder of the skin resulting in violaceous, polygonal, flat skin lesions that often pruritic (itchy). Seen commonly on the wrists, shins, lower back and genitalia. Involvement of the scalp may lead to hair loss. The cause of lichen planus is unknown, but may occur after the use of a drug (thiazide diuretics…
Found on http://www.encyclo.co.uk/local/20973

❏½ lichen psoriasis – This synonym doesn’t return any results when you Google, Yahoo or Bing pityriasis rubra pilaris. Suggest that this acronym be dropped. We defer to ANY dermatologist who wants to put it back on the SYNONYM list.

❏½ lichen ruber acuminatus – A chronic skin disease characterized by small follicular papules, disseminated reddish-brown scaly patches, and often, palmoplantar hyperkeratosis. The papules are about the size of a pin and topped by a horny plug.

❏½ pityriasis pilaris – Similar to “lichen psoriasis”, this synonym fails to return any results when you Google, Yahoo or Bing pityrasis rubra pilaris. Suggest that this acronym be dropped. We once again defer to ANY dermatologist who wants to put it back on the SYNONYM list.

❏½ Lichen ruber (Hebra) – no other references found where “lichen ruber” stands alone rather than as a preface for lichen ruber planus and “lichen ruber acuminatus”, the latter is already a listed synonym.

❏½ Fr., Pityriasis rubra pilaire – a Google search of “pityriasis rubra pillare” generates 13,200 results. This seems to be a legitimate synonym.

SOURCE: http://www.doctortreatments.com/Diseases_Of_The_Skin/Class_II_Inflammations_Pityriasis_Rubra_Pilaris.htm


❏½ Gronbled-Strandberg syndrome
(Pseudoxanthoma Elasticum) – From a lay person’s perspective, this disease does not appear to resemble pityriasis rubra pilaris in any way. It’s inclusion in the list of synonyms is not recommended.

SOURCE: NORD Guide to Rare Disorders, 2003, p133

❏½ lichen ruber pilaris – According to DoveMed “lichen ruber pilaris” is a synonym, or alternative name, or is closely related to the medical condition pityriasis rubra pilaris.

SOURCE: DoveMed

Subdivisions of PRP

The NORD Pemphigus Report includes subdivisions of that disease. This would be the appropriate place to identify the six variations (types) of pityriasis rubra pilaris. There is NO requirement to explain the details of each type here. Details will be provided in another epart of the NORD PRP Report.

❏½ Type 1 – Classical Adult Onset
❏½ Type 2 – Atypical Adult Onset
❏½ Type 3 – Classical Juvenile Onset
❏½ Type 4 – Circumscribed Juvenile Onset
❏½ Type 5 – Atypical Juvenile Onset
❏½ Type 6 – HIV-associated

 

Synonyms & Subdivisions