urine test failed to confirm proteinuria and theremainder of the workup was negative for renaldisease. The patient had a positive antinuclear anti-body (1:160) and a negative extractable nuclearantibody. She is evaluated regularly for possibleevolving connective tissue disease.
These two cases illustrate the spectrum of primarycutaneous amyloidosis, a long-standing chroniccondition not associated with a systemic disease(case 1) and a new condition associated with possi-ble renal disease and abnormal laboratory studyresults (case 2).
In case1, amyloidfibrils haveanaffinity for calcium,producing secondary calcification.3 Although calcifi-cation, osteogenesis, or both have not been reportedin primary cutaneous amyloidosis, calcification canoccur in systemic amyloidosis, particularly in pulmo-nary amyloidosis, consistent with chronicity.
In case 2, there are two systemic conditionsassociated with primary cutaneous nodular amyloi-dosis: (1) progression to systemic amyloidosis; and(2) Sjogren’s syndrome. Recent studies4-6 show thatsystemic progression is below 10%. There are 15reported cases of nodular primary cutaneous amy-loidosis associated with Sjogren’s syndrome. Theamyloid deposition may be secondary to monoclo-nal plasma cells in infiltrates surrounding exocrineglands in Sjogren’s syndrome. Unknown local factorsmay alter the immunoglobulin light chains leading tonodular amyloidosis in skin.7,8
In summary, close follow-up for progression tosystemic amyloidosis or autoimmune disease isindicated in primary nodular amyloidosis, particu-larly in case 2 with recent onset.
William E. Love, DO,a,b John D. Miedler, MD,a,b
Molly K. Smith, MD,a,b Eliot N. Mostow, MD,e
Kevin D. Cooper, MD,a,b,c,d and Anita C. Gilliam,MD, PhDa,b,c,d
Departments of Pathologya and Dermatology,b
University Hospitals Case Medical Center, Cleve-land, Ohio; Departments of Pathologyc andDermatology,d Case Western Reserve UniversitySchool of Medicine, Cleveland, Ohio; Northeast-ern Ohio Universities College of Medicine,e Roots-town, Ohio
Supported by National Institutes of Health (NIH)National Institute of Arthritis, Musculoskeletaland Skin Diseases (NIAMS) R01 AR 049284, andthe NIH NIAMS Case Skin Diseases ResearchCenter P30-AR 39750 (Dr Gilliam) and Depart-ment of Veterans Affairs, NIH R01, P30 AR39750, R01 AI041766-09A1, R01 AR051498,and T32 AR 07569 (Dr Cooper)
Conflicts of interest: None declared.
Reprints not available from the authors
Correspondence to: Anita C. Gilliam, MD, PhD,Dermatology, Palo Alto Medical Clinic, 795 ElCamino Real, Palo Alto, CA 94301
REFERENCES
1. Santos-Juanes J, Galache C, Curto JR, Astudillo A, Sanchez del
Rio J. Nodular primary localized cutaneous amyloidosis. J Eur
Acad Dermatol Venereol 2004;18:224-6.
2. Steciuk A, Dompmartin A, Troussard X, Verneuil L, Macro M,
Comoz F, et al. Cutaneous amyloidosis and possible association
with systemic amyloidosis. Int J Dermatol 2002;41:127-32.
3. Chan ED, Morales DV, Welsh CH, McDermott MT, Schwarz MI.
Calcium deposition with or without bone formation in the lung.
Am J Respir Crit Care Med 2002;165:1654-69.
4. Vestey JP, Tidman MJ, Mclaren KM. Primary nodular cutaneous
amyloidosiselong-term follow-up and treatment. Clin Exp
Dermatol 1994;19:159-62.
5. Moon AO, Calamia KT, Walsh JS. Nodular amyloidosis. Arch
Dermatol 2003;139:1157-9.
6. Woollons A, Black MM. Nodular localized primary cutaneous
amyloidosis: a long-term follow-up study. Br J Dermatol
2001;145:105-9.
7. Praprotnik S, Tomsic M, Perkovic T, Vizjak A. Is Sjogren’s
syndrome involved in the formation of localized nodular
amyloidosis? Clin Exp Rheumatol 2001;19:735-7.
8. Yoneyama K, Tochigi N, Oikawa A, Shinkai H, Utani A. Primary
localized cutaneous nodular amyloidosis in a patient with
Sjogren’s syndrome: a review of the literature. J Dermatol
2005;32:120-3.
9. Touart DM, Sau P. Cutaneous deposition diseases: part I. J Am
Acad Dermatol 1998;39:149-71.
doi:10.1016/j.jaad.2007.10.488
J AM ACAD DERMATOL
VOLUME 58, NUMBER 2
Letters S35
Intranasal formication correlates withdiagnosis of delusions of parasitosis
To the Editor: Delusions of parasitosis (DP) is anuncommon psychocutaneous syndrome in whichpatients have a false, fixed belief that they areinfested with parasites. The belief is encapsulated,in that other mental functions are generally intact.This primary psychiatric disorder is best classified asa monosymptomatic hypochondriacal psychosis.1 Itmay be the sole psychologic disturbance, may occurin association with other psychiatric or medicaldisorders,2 or may be induced by medication.3 Thepatient with DP may present a variety of signs andsymptoms; erythema, excoriation, and secondaryinfection may be present, and DP may be presentin the context of a primary dermatologic disorder.Patients with DP may reveal their delusion by offer-ing bits of skin and fabric in small containers orplastic bags as evidence of parasites (the ‘‘matchboxsign’’).4 A key feature of DP is the presence offormication, or the sensation of crawling, biting, orstinging.1 This sensation is not specific to DP; nasal
Pregabalin in the treatment of chronicpruritus
To the Editor: Chronic pruritus represents a diagnos-tic and therapeutic challenge demanding continuousevaluation of new therapeutic modalities. We reporton 3 patients responding to treatment with pregaba-lin. The patients presented with a 15- to 25-yearhistory of generalized, severe itch. Diagnostic pro-cedures1 could not detect any underlying causes inone patient. Two patients had aquagenic pruritusassociated with polycythemia vera. The itch intensityas rated by the visual analog scale was 8 points in allpatients. After multiple treatment failures, pregabalinwas commenced at a dose of 75 mg twice daily andincreased to 150 mg twice daily resulting in a morethan 70% reduction of itch 5 to 8 weeks after
J AM ACAD DERMATOL
FEBRUARY 2008
S36 Letters
pruritus is associated with intraspinal administrationof opiates,5 although other features of DP are absentin this setting. Patients with pruritic primary derma-tologic disorders may describe this sensation evenwhile accepting that parasites are absent. Because ofthe myriad ways DP can present, reliable clinicalsigns of the disorder would be of diagnostic utility.
We prospectively interviewed 5 consecutive pa-tients in whom we diagnosed DP (Table I). Thisoffice-based survey of adults without patient-identi-fying data was deemed exempt from institutionalreview. All patients described cutaneous formicationon their face (and elsewhere) that they attributedto nonexistent parasites. As a control group, 10consecutive age-concordant patients with pruriticprimary dermatoses affecting facial skin were inter-viewed. Control patients were only included if,during routine history-taking, they voluntarily usedwords and phrases such as ‘‘crawling,’’ ‘‘creeping,’’‘‘gnawing,’’ ‘‘tingling,’’ or ‘‘like bugs walking acrossmy skin.’’ No control patient believed he or she wasinfested with parasites, and each responded appro-priately when their symptoms were explained in thecontext of the primary dermatosis.
Of the 5 patients with DP, 3 volunteered that theyexperienced a sensation of intranasal formication, asa crawling sensation on the nasal mucosa. A fourthpatient confirmed this finding when asked. All ofthese patients unshakably attributed this sensationto parasitic infestation. Of the 10 control patients,none reported a sensation of intranasal formication(P \ .004 by Fisher exact test; Table I). Three of 5patients with DP and 2 of 10 control patients broughtsamples of skin, hair, and cutaneous debris to clinicin small containers. Patients with DP were notsignificantly more likely to display this time-honored‘‘matchbox sign’’ (P 5 .45). Control patients
Table I. Intranasal formication correlates withdelusions of parasitosis
DP
(N = 5)
Facial dermatoses
(N = 10)*
Age (y) 66.2 6 9.1(range,57-81 y)
62.9 6 11.5(range,49-84 y)
Gender (F:M) 4:1 8:2Paranasal cutaneous
formication5/5 (100%) 10/10 (100%)
Intranasal formicationy 4/5 (80%) 0/10‘‘Matchbox sign’’ 3/5 (60%) 2/10 (20%)
DP, Delusions of parasitosis.
*Seborrheic dermatitis (5), eczema (2), psoriasis (2), postherpetic
neuralgia (1).yP \ .004.
displaying the matchbox sign did not believe theskin debris contained parasites but thought that thesamples might be helpful in diagnosing theircondition.
In conclusion, we report that intranasal formica-tion correlates with the diagnosis of DP. Noting thissymptom in the clinic may assist in distinguishing thisentity from primary dermatologic disorders. It willbe interesting to see whether this observation canbe further validated by studying larger numbers ofpatients with DP.
Hobart W. Walling, MD, PhD,a and Brian L. Swick,MDb
Private practice, West Des Moines, Iowa,a andOmaha, Nebraskab
Funding sources: None.
Conflicts of interest: None declared.
Correspondence to: Hobart W. Walling, MD, 6000University Ave, Suite 450, West Des Moines, IA50266
E-mail: [email protected]
REFERENCES
1. Koo J, Lee CS. Delusions of parasitosis. A dermatologist’s
guide to diagnosis and treatment. Am J Clin Dermatol 2001;
2:285-90.
2. Driscoll MS, Rothe MJ, Grant-Kels JM. Hale MS Delusional
parasitosis: a dermatologic, psychiatric, and pharmacologic
approach. J Am Acad Dermatol 1993;29:1023-33.
3. Swick BL, Walling HW. Drug-induced delusions of parasitosis
during treatment of Parkinson’s disease. J Am Acad Dermatol
2005;53:1086-7.
4. Lee WR. Matchbox sign. Lancet 1983;2:457-8.
5. Scott PV, Fischer HB. Intraspinal opiates and itching: a new
reflex? Br Med J (Clin Res Ed) 1982;284:1015-6.
doi:10.1016/j.jaad.2007.01.029
