CASE REPORT

Miliarial Gout (A New Entity)

Rahul Shukla, Ronald B. Vender, Ayman Alhabeeh, Samih Salama, and Frank Murphy

Background: Tophaceous gout typically presents as a subcutaneous, nodular collection of monosodium urate crystals sharply
circumscribed from surrounding tissues. Although intradermal cutaneous manifestations of gout have been described, no reported
cases of miliarial gout remain.
Objective: We describe the first known presentation of miliarial gout and list other uncommon cutaneous manifestations of gouty
tophi. The treatment of miliarial gout is discussed, as well as risk factors predisposing an individual to the development of intradermal tophi.
Results: Miliarial gout is an intradermal phenomenon consisting of multiple tiny papules containing material of a white to cream
color scattered on an erythematous base that responds to allopurinol administration. Risk factors predisposing an individual to the
development of intradermal gout include renal insufficiency, hypertension, chronic diuretic therapy, long duration of disease, and
lack of consistent use of urate-lowering therapy.
Conclusion: Miliarial gout is a unique intradermal manifestation of tophaceous gout.

Antécédents: La goutte tophacée se présente généralement sous forme d'une collection sous-cutanée et nodulaire de cristaux
d'urate de monosodium nettement circonscrite par rapport aux tissus avoisinants. Malgré ses manifestations intradermiques
rapportées, aucun cas de goutte miliaire n'a été signalé.
Objectif: Nous décrivons la première occurrence connue de goutte miliaire et listons d'autres manifestations cutanées rares de
tophus. Nous discutons par la suite du traitement de la goutte miliaire et présentons les facteurs de risque qui prédisposent une
personne à développer un tophus intradermique.
Résultats: La goutte miliaire est un phénomène intradermique qui consiste en de multiples petites papules contenant du matériel de
couleur blanche à crémeuse, dispersées sur une base érythémateuse et qui est sensible à l'administration de l'allopurinol. Les facteurs de
risque qui prédisposent un individu au développement de goutte intradermique comprennent l'insuffisance rénale, l'hypertension, une
thérapie diurétique chronique, la longue durée de la maladie, et le manque de cohérence dans l'utilisation du traitement visant la baisse
des niveaux d'urate.
Conclusion: La goutte miliaire est une manifestation intradermique unique de tophus.

OUT is an infiammatory arthritis characterized
G by hyperuricemia (serum urate > 450 |imol/L or
7.0 mg/dL in men and 350 pmol/L or 6.0 mg/dL in
women) and recurrent attacks of acute arthritis provoked
by the release of monosodium urate (MSU) crystals into
synovial spaces.' Currently, gout afflicts 2.7% of
Americans,^ progressing through a sequence of clinical
stages consisting of asymptomatic hyperuricemia, acute or
recurrent gout, intercritical gout, and chronic tophaceous
gout."^''* Three percent of gout patients develop the chronic
tophaceous manifestation of the disease, generally those
Vkiho have not received treatment with uricosuric agents.^
From the Departments of Medicine and Pathology, McMaster University,
Hamilton, ON.
Address reprint requests to: Ronald B. Vender, MD, FRCPC, Dermatrials
Research, 132 Young Street, Hamilton, ON L8N 1V6; e-mail: drvender@
dermatrials.com.
DOI ¡0.2310/7750.2007.00002
Tophi are granulomas of mono- and multinucleated
macrophages surrounding a core of debris and MSU crystals.*^
Tophi are commonly located in bursae, subcutaneous tissue
overlying tendons, cartilage, and periarticular sites localized to
the feet, ankles, knees, and fingers, as shown in Figure 1.^"'
Typical gouty tophi characteristically appear asfirm,yellowish
white, subcutaneous, irregular nodules sharply circumscribed
from surrounding tissues.'" A number of unusual manifesta-
tions of tophaceous gout have been reported and include
btillous," fungating,'^ papular,'^''* tilcerative," pustular,'^''*
post-traumatic,'* and nodular'"* tophaceous skin lesions. In
the following case report, we describe the first reported
manifestation of muiarial gout, a unique presentation of
tophaceous gout consisting of müia-like papules.
Case Presentation
Ahyperuricemic (735 pmol/L; normal range 230-480 pmol/L),
obese, 73-year-old type 2 diabetic male with hyperten-
sion, chronic renal insufficiency, alcohol abuse, psoriasis.

Journal of Cutaneous Medicine and Surgery, Vol 11, No 1 (January/February), 2007: pp 31-34 31
32 Shukla et al

Figure 1. A typical presentation of gouty tophi present at the Figure 3. A closer view of the papules present on the arm.
metacarpophalangeal joint of the index finger.

osteoarthritis, and a 10-year history of gout presented crystals admixed with eosinophilic to amphophilic amor-
with a 24-month history of a milia-like presentation of phous material surrounded by palisading histiocytes, as
gout. Tbe patient was on a number of medications, shown in Figure 4. Further pathologic examination of the
including furosemide, and reported using 200 mg biopsy demonstrated the typical presence of doubly
allopurinol inconsistently in the past, stopping the refractile or polarizable MSU crystals, findings consistent
medication when his arthritic fiares resolved. with gout and displayed in Figure 5.^^'^^
On initial presentation, multiple tiny papules were
found on erythematous areas of the anterior aspects of the
forearms and shins that appeared to contain material of a
white to cream color, as depicted in Figures 2 and 3.
Similar lesions were present on the inner thighs, and
subcutaneous tophaceous deposits were noted over the
metacarpophalangeal, proximal interphalangeal, and distal
interphalangeal joints. A number of the proximal inter-
phalangeal joints of botb hands also appeared swollen,
with an obvious white chalky material around them.
A 2 mm punch biopsy was taken from the forearm and
fixed in alcohol. The dermis showed multiple deposits of

Figure 4. The dermis displays multiple deposits of monosodium urate

crystals admixed with eosinophilic to amphophilic amorphous
Figure 2. Multiple papules present on an erythematous area of the material surrounded by palisading histiocytes. A, XIO magnification;
arm that appear to contain a material of white to cream color. B, X20 magnificantion (hematoxylin and eosion stain).
 Miliarial Gout
Figure 5. Punch biopsy results revealed the presence of doubly
refractile monosodium urate crystals under polarized light.
The patient was started on 300 mg of allopurinol and 0.6 mg
colchicine twice daily, which resulted in a moderate decrease in
the number of tophi over his legs during a 4-month period.
His uric acid level significantly decreased from a value of 735
to 520 |J,mol/L over this time span. The amount of allo-
purinol was increased in 4-month intervals to doses of 400 and
600 mg/d, respectively. As the dose of allopurinol increased, the
size and amount of milia-like papules reduced. The level of uric
acid also continued to reduce, with the most recent level being
408 |imol/L. No further eruptions have occurred.
Discussion
To our knowledge, this is the first description of a
cutaneous form of gout consisting of milia-like papules.
We propose the term miliarial gout to describe this new
subtype of intradermal gout.
A review of the literature yielded other reported cases of
intradermal gouty tophi. Six patients with pustule-like
intradermal tophi were studied over 10 years to identify
clinical features and risk factors associated with development
of intradermal urate tophi.'* Factors associated with the
development of hyperuricemia and intradermal urate tophi
were common to our patient and included renal insuffi-
ciency, hypertension, and chronic diuretic therapy.'*
A case-control study conducted on intradermal tophi in
gout also identified the long-term duration of the disease at
the first visit, averaging 9.1 years in the case group, and a lack
of any regular urate-lowering therapy'" as additional risk
factors. Long-term glucocorticoid use, defined as 6 months
or more, was also associated with intradermal tophi
development as uncontrolled access to drugs contain-
ing steroids, nonprofessional recommendation for using
33
glucocorticoids, and self-medication are common practices
among the Mexican population studied. Corticosteroids
have also been postulated to enhance tophi formation, as
suggested in the air-pouch model by Rull and colleagues."
Although the pathogenesis of intradermal gout remains
to be elucidated, mechanisms explaining intradermal tophi
development in peripheral body parts such as the finger and
toes have been proposed. Reduced urate solubüity and
enhanced crystal precipitation owing to decreased tempera-
ture in peripheral body parts and enhanced crystal
deposition in sites subjected to repetitive trauma have been
suggested to play a role in intradermal tophi development.'*
Tissue macrophages have also been suggested to play a
key role in tophi development.^" Given that MSU crystals
can be found within synovial mononuclear cells in
asymptomatic joints of hyperuricemic individuals,"'
Dalbeth and Haskard suggested that resident tissue macro-
phages may play a key role in maintaining the asymptomatic
state in hyperuricemic individuals by clearing crystals as and
when they form.^" Mechanisms that explain tophi develop-
ment susceptibility remain unclear but may include tbe
formation of crystals at a rate that exceeds the handling
capacity of tissue macrophages or possibly the failure of
macrophages to differentiate to an end point that does not
show a proinflammatory response to crystal uptake.^"
Treatment
The mainstay of medical management of tophaceous gout
remains therapy with allopurinol.^^ Medical management of
gout with allopurinol reduces uric acid production through
competitive inhibition of xanthine oxidase, an enzyme that
converts xanthine and hypoxanthine to uric acid. Allopurinol
is almost always used initially because it reduces urate
concentrations in all patients, can be conveniently given once
daily, is appropriate in patients with renal impairment, and is
not contraindicated in patients with urate calculi."^
In our patient, allopurinol was used successfully to
treat miliarial gout. Allopurinol administration resulted in
continual reduction in the size and number of miliarial
papules as the dose increased from 300 to 600 mg daily. A
previous report of intradermal gout also indicated success
when treating gout with a dose of allopurinol ranging from
100 to 250 mg/d.'* Therefore, we recommend starting
allopurinol at a low dose and gradually increasing the
amount used until the desired results are obtained.
The patient should also be reminded of the importance
of compliance as years of stringent control of hyperuricemia
may be required for résorption of existing tophi to ^^
34 Shukla et al
Morphological manifestations
of tophaceous gout
Fungating'^ Nodular'""
Figure 6. A morpbologic summary of the reported cutaneous manifestations of tophaceous gout.
Surgery is an uncommon but important therapeutic option
and is indicated to decompress nerves,^^'^'' permit restoration of
10.
motion and stabilization of joints, control infection status, and
improve cosmetic appearance.^^ Curettage and débridement 11.
are the conventional surgical approach for treating tophaceous
gout. The soft tissue shaving system has also shovwn promise for 12.
the surgical treatment of tophaceous gout.^^
13.
Conclusion
14.
In conclusion, a number of morphologic variants of
tophaceous gout have been reported and are summarized 15.
in Figure 6. We report and describe the first case of miliarial
gout, a subtype of intradermal gout consisting of milia-like
16.
papules on an erythematous base. Although the pathogenesis
of this intradermal manifestation remains uncertain, treat-
17.
ment occurs successfully wdth allopurinol.
18.
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