10 cm annular plaque in a 62 yo globe trotter

The patient is an otherwise healthy 62 yo man with an eight month history of an anympromatic plaque on his right arm.  His work takes him to places like the Atacama desert of Chile, Baghdad and Mosul, Japan, and Indonesia where he repairs specialized equipment in the field.  He was treated in a number of clinics with prednisone, various topical azoles and 40 days of grizeofulvin to no effect.

O/E:  The is a solitary 10 cm ring-shaped plaque on the right arm.  Sensaton to pinprick is normal.

Clinical Image:

Pathology:  A 4 mm punch biopsy was taken from the border of the ring. 

The specimen exhibits a superficial and deep interstitial proliferation of variably plump to spindle-shaped cells with mildly increased dermal mucin and a superficial and deep perivascular lymphocytic infiltrate with occasional plasma cells. The findings support the diagnosis of Interstitial Granulomatous Dermnatitis associated with a systemic disease.

Diagnosis:  Interstitial Granulomatous Dermnatitis (IGH) vs. Granuloma annulare.

The patient lives in an endemic region for Lyme disease.  His work takes him to Asia, South America, the Mid-East, and all parts of North America. Presently, he is in Iraq and will have a Lyme serology when he returns.  Certainly, the clinical picture could suggest erythema migrans, but an eight month history would be unusual.  There are a host of case reports of IGH in association with arthritis, collagen vascular disease, inflammatory bowel disease and even Lyme disease; but they are hard to make sense of.  (Guilt by association?) Although we see erythema chronicum migrans with regularity in New England, this lesion did not jump out as typical to me; but in retrospect, it could be the interface between IGH, GA and ECM. An old ECM?   Food for thought.  How long will the primary lesion of Lyme disease remain without treatment.  Eight months seems a long time.

References:

1. The expanding spectrum of cutaneous borreliosis.

Eisendle K1, Zelger B. G Ital Dermatol Venereol. 2009 Apr;144(2):157-71.

Abstract:  The known spectrum of skin manifestations in cutaneous Lyme disease is continuously expanding and can not be regarded as completed. Besides the classical manifestations of cutaneous borreliosis like erythema (chronicum) migrans, borrelial lymphocytoma and acrodermatitis chronica atrophicans evidence is growing that at least in part also other skin manifestations, especially morphea, lichen sclerosus and cases of cutaneous B-cell lymphoma are causally related to infections with Borrelia. Also granuloma annulare and interstitial granulomatous dermatitis might be partly caused by Borrelia burgdorferi or similar strains. There are also single reports of other skin manifestations to be associated with borrelial infections like cutaneous sarcoidosis, necrobiosis lipoidica and necrobiotic xanthogranuloma. In addition, as the modern chameleon of dermatology, cutaneous borreliosis, especially borrelial lymphocytoma, mimics other skin conditions, as has been shown for erythema annulare centrifugum or lymphocytic infiltration (Jessner Kanof) of the skin.

2. Interstitial granulomatous dermatitis: a distinct entity with characteristic histological and clinical pattern.  Peroni A et. al.  Br J Dermatol. 2012 Apr;166(4):775-83.

Abstract

BACKGROUND:

Interstitial granulomatous dermatitis (IGD) is a rare disease for which a clinical-pathological correlation is essential to establish diagnosis.

RESULTS:

All cases showed a predominant CD68-positive macrophage infiltrate distributed between collagen bundles of the mid- and deep dermis. Macrophages were also surrounding degenerated collagen fibres. A few neutrophils and/or eosinophils were also present. No vasculitis or significant mucin deposition was observed. Of the 62 cases of IGD reported since 1993, 53 fulfilled stringent diagnostic criteria. Erythematous papules and plaques on the trunk and proximal limbs were the dominant manifestation. Approximately 10% of patients had cord-like lesions. More than 50% of patients with IGD had arthralgia or arthritis, and less commonly other rheumatic disorders. Disease duration is months to years, but long-term prognosis seems favourable.

CONCLUSIONS:
IGD is a distinct entity with a typical histological and clinical pattern. The importance and the nature of the association with extracutaneous diseases remains to be clarified. Patients should be screened for rheumatic and autoimmune diseases.
 

3. Erythema migrans: a spectrum of histopathologic changes.

Wilson TC et. al. Am J Dermatopathol. 2012 Dec;34(8):834-7.

Abstract: Early cutaneous Lyme disease, erythema migrans, manifests as a gyrate erythema at the site of a tick bite. The standard histopathologic description is that of a superficial and deep perivascular lymphocytic infiltrate in which plasma cells are identified at the periphery of the lesion and eosinophils in the center. Deviation from these commonly accepted histopathologic findings may lead to an erroneous diagnosis. Herein, we describe 4 cases of erythema migrans, all biopsied at the periphery of the lesion and confirmed by serologic studies, demonstrating a variety of unconventional histopathologic patterns. These findings include eosinophils and neutrophils at the periphery of the expanding annular plaque of erythema migrans, focal interface change, spongiosis, involvement of the superficial vascular plexus alone, and an absence of plasma cells in all cases. These cases highlight the varied and nonspecific histopathologic changes that can be seen in erythema migrans, including the absence of plasma cells and the presence of focal interface change. Based on these findings, the dermatopathologist should always consider erythema migrans as a diagnostic possibility in a biopsy specimen from an expanding gyrate or annular erythema despite the presence of unusual features. In atypical clinical cases, serologic confirmation may be required for diagnosis in the presence of histopathologic findings considered unconventional for erythema migrans.

Erythema Migrans: Classic Lyme

Presented by Yoon Cohen, D.O. and David Elpern, M.D.

Abstract:  38-year-old man with history of tick bite and expanding annular lesion

HPI: The patient is a 38-year-old man with a 4-day history of and expanding red annular patch on the right inner thigh. He had a tick bite in this area ~ 10 days prior. He has not been treated with any antibiotics at this time and has had denied no flu-like or other associated constitutional symptoms.  He lives in an endemic area and has had many tick bites in the past.

O/E: The skin examination showed a healthy and pleasant man with a well-defined 15 cm pink to red annular patch with a central pink oval shaped patch on his right thigh.  It has a "bull's eye" appearance.  There are no other cutaneous findings.

Clinical Photo:

Diagnosis: Erythema Migrans (Early Lyme Disease)

Discussion: 
Lyme disease is caused by the spirochete Borrelia burgdorferi. Erythema migrans is the most common clinical manifestation of Lyme disease. It typically develops 7-14 days after tick detachment and is characterized by a rapidly expanding, erythematous annular patch or plaque. 

The diagnosis of erythema migrans is based on the clinical presentation and history of recent exposure in the endemic regions. Although the skin lesion cannot be considered pathognomonic of Lyme disease, erythema migrans is so distinctive that serologic testing for antibodies against B.burgdorferi is generally unnecessary. These serologic tests have high false negative results in as many as 60% of cases.

Treatment for Lyme disease depends on the stage. If there is only a tick bite, single dosage of doxycycline 200 mg is considered adequate if administered within 2 - 3 days.  However, if a patient presents with erythema migrans, doxycycline 100 mg twice daily for 14 days (range, 10 to 21 days) is currently advised.  (Amoxicillin is an alternate treatment).  For secondary and tertiary Lyme disease the treatment can be more complex.

The references below give much more detailed information.  Post-Lyme Disease syndrome is controversial and has generated a lot of ink.  See Michael Spector's fine New Yorker piece referenced below.
 

Reference:
1. Early Lyme Disease
Gary P. Wormser, M.D.
N Engl J Med 2006; 354:2794-2801
(This is an extremely helpful article.  Although it is seven years old, little has changed re garding hte literature on chronic Lyme disease.  If you can't get access to the full text of this article, we will send you a pdf.)

2.  Annals of Medicine

The Lyme Wars

The Lyme-disease infection rate is growing. So is the battle over how to treat it.

by Michael Specter The New Yorker July 1, 2013
This is in the current New Yorker as we prepare this post.  It is a level-headed review of Lyme disease from a top science writer.  This article will help the public as well as physicians. 

3.  Patient Friendly Material. 

Diseases Don't Read Textbooks

Abstact: 5 yo girl with enlarging plaque on back.

HPI: The patient is a 5 year old girl seen on February 27, 2009 with a 10 day history of an enlarging plaque on the left back. She had a similar, but less dramatic lesion in April 2008 which was treated with cefuroxime for two weeks. Her family lives in a wooded area and her mother had Lyme Disease last year. The patient feels well, may have had some mild arthralgias according to her mother. No neurological symptoms. She is allergic to penicillin, amocicillin and sulfonamides.

O/E: 17 x 12 cm plaque left back. 2 x 2 cm plaque right arm. These lesions are somewhat urticarial in appearance. The center of the larger lesion is paler than the periphery.

Photos:


Lab: Lyme titers pending

Diagnosis: Presumptive Lyme Disease. She was started on cefuroxime by her pediatrician.
Questions:
1) What else would you consider in the differential diagnosis
2) Can one have ECM more than once? This child had something similar 10 months ago.
3) Presuming this is Lyme -- how long shoud she be treated?

Reasons Presented: Lyme Disease is unusual in the winter. Can one have "primary lesions" with a reoccurence? In a young patient where tetracyclines are contraindicated with a proven allergy to penicillins, what is the best third line drug and how long to administer.