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

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

A 2 mm punch biopsy was taken from the forearm and fixed in alcohol. and distal interphalangeal joints.^^'^^ On initial presentation. A number of the proximal inter- phalangeal joints of botb hands also appeared swollen. proximal interphalangeal. Tbe patient was on a number of medications. shown in Figure 4. Similar lesions were present on the inner thighs. A. X20 magnificantion (hematoxylin and eosion stain). and subcutaneous tophaceous deposits were noted over the metacarpophalangeal. 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 gout. Multiple papules present on an erythematous area of the material surrounded by palisading histiocytes. findings consistent medication when his arthritic fiares resolved. osteoarthritis. as depicted in Figures 2 and 3. Further pathologic examination of the including furosemide. stopping the refractile or polarizable MSU crystals. The dermis showed multiple deposits of Figure 4. 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. XIO magnification. and reported using 200 mg biopsy demonstrated the typical presence of doubly allopurinol inconsistently in the past. with an obvious white chalky material around them.32 Shukla et al Figure 1. arm that appear to contain a material of white to cream color. with gout and displayed in Figure 5. The dermis displays multiple deposits of monosodium urate crystals admixed with eosinophilic to amphophilic amorphous Figure 2. A closer view of the papules present on the arm. A typical presentation of gouty tophi present at the Figure 3. metacarpophalangeal joint of the index finger. . B.

defined as 6 months amount used until the desired results are obtained. was also associated with intradermal tophi The patient should also be reminded of the importance development as uncontrolled access to drugs contain. 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. and is clinical features and risk factors associated with development not contraindicated in patients with urate calculi. Allopurinol administration resulted in were common to our patient and included renal insuffi."^ of intradermal urate tophi. Corticosteroids have also been postulated to enhance tophi formation. converts xanthine and hypoxanthine to uric acid. No further eruptions have occurred. when they form. and a lack 100 to 250 mg/d.6 mg phages may play a key role in maintaining the asymptomatic colchicine twice daily. As the dose of allopurinol increased. with the most recent level being 408 |imol/L. which resulted in a moderate decrease in state in hyperuricemic individuals by clearing crystals as and the number of tophi over his legs during a 4-month period. asymptomatic joints of hyperuricemic individuals.'* papules as the dose increased from 300 to 600 mg daily."' Dalbeth and Haskard suggested that resident tissue macro- The patient was started on 300 mg of allopurinol and 0. Miliarial Gout 33 glucocorticoids. Treatment Discussion The mainstay of medical management of tophaceous gout To our knowledge. of compliance as years of stringent control of hyperuricemia ing steroids.'* Therefore. or more. Six patients with pustule-like concentrations in all patients.mol/L over this time span.'* Tissue macrophages have also been suggested to play a key role in tophi development. the macrophages to differentiate to an end point that does not size and amount of milia-like papules reduced. allopurinol was used successfully to development of hyperuricemia and intradermal urate tophi treat miliarial gout. can be conveniently given once intradermal tophi were studied over 10 years to identify daily.^" Mechanisms that explain tophi develop- His uric acid level significantly decreased from a value of 735 ment susceptibility remain unclear but may include tbe to 520 |J.^" acid also continued to reduce. and self-medication are common practices among the Mexican population studied. respectively. gout with allopurinol reduces uric acid production through We propose the term miliarial gout to describe this new competitive inhibition of xanthine oxidase.^^ Medical management of cutaneous form of gout consisting of milia-like papules. Long-term glucocorticoid use. is appropriate in patients with renal impairment.^" Given that MSU crystals can be found within synovial mononuclear cells in Figure 5. Punch biopsy results revealed the presence of doubly refractile monosodium urate crystals under polarized light. A A case-control study conducted on intradermal tophi in previous report of intradermal gout also indicated success gout also identified the long-term duration of the disease at when treating gout with a dose of allopurinol ranging from the first visit. hypertension. as suggested in the air-pouch model by Rull and colleagues. an enzyme that subtype of intradermal gout. mechanisms explaining intradermal tophi development in peripheral body parts such as the finger and toes have been proposed. The amount of allo- formation of crystals at a rate that exceeds the handling purinol was increased in 4-month intervals to doses of 400 and capacity of tissue macrophages or possibly the failure of 600 mg/d. averaging 9.1 years in the case group." Although the pathogenesis of intradermal gout remains to be elucidated. continual reduction in the size and number of miliarial ciency. nonprofessional recommendation for using may be required for résorption of existing tophi to ^^ . and chronic diuretic therapy. Allopurinol A review of the literature yielded other reported cases of is almost always used initially because it reduces urate intradermal gouty tophi. this is the first description of a remains therapy with allopurinol.'* Factors associated with the In our patient. The level of uric show a proinflammatory response to crystal uptake. we recommend starting of any regular urate-lowering therapy'" as additional risk allopurinol at a low dose and gradually increasing the factors.

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