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Granulomatous pigmented purpuric dermatosis revisited: Lessons learned from a rejected manuscript


DII small banner By Warren R. Heymann, MD
May 14, 2018


  pigmented-purpuric-dermatosis.jpg
Nonpalpable pigmented purpuric eruption seen on lower aspect of right leg.
Credit: JAAD
In 1982, I was a second-year dermatology resident fascinated by a woman with a history of hyperlipidemia who developed a pigmented purpuric dermatosis (PPD) on her lower extremities. A biopsy demonstrated a granulomatous infiltrate admixed with extravasated erythrocytes. I submitted the case report to Archives of Dermatology detailing the case — I was not sure if the foamy histiocytes were just innocent bystanders or if the lipids incited the inflammatory response. The reviewer stated that the paper “strains my credulity”; the manuscript was rejected. The rest of this publication saga will follow an update on granulomatous pigmented purpuric dermatosis (GPPD).

The fact is, just as in medical records, if it’s not documented, it’s not done.

Every dermatology resident can describe the clinical variants of PPDs:  Schamberg disease (progressive pigmentary dermatosis); pigmented purpuric lichenoid dermatosis of Gougerot‐Blum; purpura annularis telangiectodes (Majocchi disease); lichen aureus, and the eczematoid‐like purpura of Doucas and Kapetanakis. PPD has also been associated with comorbid conditions such as diabetes, autoimmune disease, and hypersensitivity to medications.

The initial report of GPPD did not appear until 1996 when Saito and Matsuoka reported two cases in a 61-year-old woman with a positive ANA, and a 53-year-old woman with hepatitis C. The granulomatous inflammation was in the papillary dermis. The authors stated that the pathogenesis was unknown but considered that serum proteins could play a role. (1)
 
Huang et al surveyed the histologic spectrum of 107 cases of PPD. Five major pathological patterns were identified: lichenoid (45/107, 42.1%), perivascular (40/107, 37.4%), interface (11/107, 10.3%), spongiotic (7/107, 6.5%) and granulomatous (4/107, 3.7%). Lymphocytic vasculitis was present in 17 patients (15.9%), and Langerhans cell microabscess was seen in 4 (3.7%). Nine patients had partial features mimicking mycosis fungoides but none were confirmed. The lichenoid, perivascular and spongiotic patterns correlated to lichen aureus, Schamberg and eczematoid clinical variants, respectively. (2)

In a review of 27 cases of GPPD have been reported in the English literature, patient age ranged from 9 to 76 years, and, unlike other variants of PPD, there was a female predominance. Although the disease was initially described in Asian patients, Caucasian individuals represent more than one-half of reported cases. The disease duration showed great variability, ranging from 3 weeks to 20 years, with a mean of 4.5 years. Most patients were treated with topical corticosteroids, with either slight or no response. Dyslipidemia (DLP) was the most commonly associated abnormality, reported in 15 of 27 patients. It was suggested that DLP could give rise to an insufficient Th1 response, leading to granuloma formation. Alternatively, other conjecture that underlying vascular injury, induced by lipid deposition in endothelial cells, might result in subsequent erythrocyte extravasation and a granulomatous response. (3)
 
When considering the diagnosis of GPPD, it may be valuable to perform a deeper punch biopsy as cases have been reported where the granulomatous inflammation is in the mid to deep dermis. (4). It has also been suggested that patients with GPPD should have a lipid profile to assess cardiovascular risk. (5)

With the exception of controlling hyperlipidemia, treatment for GPPD is the same as for other PPDs — managing underlying aggravating factors if relevant (compression for venous insufficiency, discontinuation of suspected PPD-inducing drugs), and consideration of the following therapies (none of which are especially effective in my experience) — topical corticosteroids, calcineurin inhibitors, phototherapy (narrow band UVB, PUVA), rutoside and vitamin C.

The late Frederick Malkinson MD, DMD, was editor of Archives of Dermatology from 1979 to 1983. Aside from the reviewers’ comments, Dr. Malkinson offered his personal critique, handwritten in red ink, as though I was in my high school English class. His suggestions were more about syntax, expression, and grammar than scientific content. I was stunned — but grateful. What a remarkable man to care about the writing skills of a resident, not just the content submitted to his journal. I took his suggestions seriously and have since strived to write in the manner that Dr. Malkinson preferred — personal, precise, and crisp.
 
I rewrote the article per Dr. Malkinson’s suggestions. At the time of resubmission in 1983, Kenneth Arndt was the new editor, and the paper was rejected again (I assume for content rather than syntax). Why didn’t I submit it to another journal? I cannot recall. Perhaps I was frustrated and tired of the process. I was a newlywed chief resident about to take my board exam and embark on my career – there were more important issues to focus on.

The world has come to know GPPD. It is no tragedy that the entity was not recognized 14 years earlier (although a few patients may have had their lipids checked when they might not have done so otherwise). I personally benefited by having Dr. Malkinson hone my writing skills – his efforts have immeasurably enhanced my career. The most important lesson I learned is not to get discouraged when others think your ideas are implausible. Science, and life, does not progress unless credulity is strained.

Point to remember: Check a lipid profile in any patient diagnosed with a granulomatous pigmented purpuric dermatosis.

1. Saito R, Matsuoka Y. Granulomatous pigmented purpuric dermatosis. J Dermatol 1996; 23: 551-556.
2. Huang Y-K, et al. The pathological spectrum and clinical correlation of pigmented purpuric dermatosis – A retrospective review of 107 cases. J Cutan Pathol 2018; 45: 325-332.
3. García-Rodino S, et al Granulomatous variant of pigmented purpuric dermatosis: Report of two cases and review of the literature. J Dtsch Dermatol Ges 2017; 15: 565-9.
4. Morrissey K, et al. Granulomatous changes associated with pigmented purpuric dermatosis. Cutis 2014; 94; 197-202.
5. Allan A, et al. Granulomatous pigmented purpuric dermatosis. Cutis 2017; 100: 256-8.

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