Case report

Erythema multiforme: a rare skin manifestation of relapsing

polychondritis

Vijay K. Jain, MD, MBBS, Arshdeep, MD, MBBS, and Sangita Ghosh, MBBS, DDVL

Department of Dermatology, Venereology

and Leprology, Pandit Bhagwat Dayal

Sharma Postgraduate Institute of Medical

Sciences, Rohtak, Haryana, India

Correspondence

Arshdeep, MD, MBBS

Department of Dermatology

Venereology and Leprology

Pt. B. D. S. Postgraduate Institute of

Medical Sciences

Rohtak 124001 Haryana

India

E-mail: arsh_deep2003@yahoo.com

Conflicts of interest: None.

Relapsing polychondritis (RP) is a rare autoimmune dis-ease characterized by inflammation and degeneration ofauricular, laryngotracheal, or nasal cartilaginous tissue,which may progress to involve connective tissue at almostany site.1

Case report

A 25-year-old woman, who was seven weeks pregnant,was referred to our department from the Ear, Nose andThroat Department with a 1-week history of graduallyprogressive, painful, red swellings on the ear (Fig. 1),along with the sudden appearance of multiple, dull pink,asymptomatic, round to oval, raised lesions over theabdomen (Fig. 2) and extremities that had emerged overthe previous three days. She also complained of rhinitisand bilateral painful ankle swelling during the lasttwo weeks, along with low-grade fever and malaise.However, she denied any visual or hearing complaints.The patient reported having experienced a similar epi-

sode of chondritis without any skin or joint symptomsduring the first trimester of her last pregnancy. Herrecent drug and immunization history was negative.There was no past history suggestive of herpes simplexinfection.Cutaneous examination revealed bilateral, erythema-

tous, swollen, and tender ear pinnae sparing the lobules,

and multiple (n = 15) target lesions, varying in size from1.0 9 1.0 to 3.0 9 1.5 cm, asymmetrically distributedover the abdomen and extremities. The oral and genitalmucosae were spared.On general examination, bilateral ankle joint swelling

and tenderness with restricted mobility was observed.Other joints were not involved. No facial deformity(saddle nose or floppy ears) was seen.Based on these findings, the patient was diagnosed with

RP. Histopathology of a representative skin lesionrevealed orthokeratosis, vacuolization of the basal epider-mal layer with tagging of lymphocytes along the dermo–epidermal junction, and a sparse, superficial, perivascularlymphoid infiltrate in the dermis (Fig. 3). Direct immuno-fluorescence findings were negative, confirming the clini-cal impression of erythema multiforme (EM).Further investigations were non-contributory except for

findings of dimorphic anemia (hemoglobin 8 g/dl) andelevated erythrocyte sedimentation rate (130 mm/h).Rheumatoid factor and antinuclear antibody (ANA) werepositive, with an ANA titer of 1 : 160 with a speckledpattern with negative anti-dsDNA antibody and LE(lupus erythematosus) cell phenomena.In view of the severity of the arthritis and chondritis,

the patient was prescribed prednisolone at 0.5 mg/kg(pregnancy category C), which was tapered off withinone week as she was in the early stage of pregnancy.2,3

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The EM lesions also subsided with this short course ofprednisolone.

Discussion

Dermatologic manifestations may represent the presentingfeature in around 12% of RP cases and ultimatelybecome apparent in more than one-third of patients.4 In acase series reported by Frances et al,5 the more frequentlyreported skin lesions were aphthosis, limb nodules, pur-pura, aseptic pustules, erythema nodosum-like lesions,superficial thrombophlebitis, urticarial papules, and livedoreticularis. Pathologic features included leukocytoclasticvasculitis (53%), a Sweet-like infiltrate of polymorphonu-clear neutrophils (15.5%), thrombosis (12.5%), and lym-phocytic vasculitis (6%).Clinical findings in the present patient clearly met the

diagnostic criteria of McAdam et al.6 This unique associ-

ation of EM and RP has not been reported in the derma-tologic literature so far. A study by Tronquoy et al.7

reported the occurrence of tense, fixed, annular urticarialplaques in RP, particularly on the trunk, in male patients,in characteristic association with hematologic abnormali-ties, especially myelodysplastic syndrome. However, theseauthors found characteristic lymphocytic vasculitis onhistology in all cases.7

The pathophysiology of this complex disease is notcompletely understood, but several studies suggest a roleof autoimmunity in RP.8 Hormonal associations have alsobeen suggested.9 Pregnancy does not appear to modifythe course of RP, as concluded by Papo et al.10 However,in the present patient, pregnancy seemed to have precipi-tated the episodes of chondritis, along with the develop-ment of EM lesions, as was evident in both consecutivepregnancies, for both of which the post-partum periodwas uneventful. This suggests a probable hormonal rolein the pathophysiology of the disease.

References

1 Liu CM, Hata TR, Swinyer L, Petersen MJ. Relapsingpolychondritis. Int J Dermatol 2003; 42: 707–708.

2 Fraser FC, Sajoo A. Teratogenic potential ofcorticosteroids in humans. Teratology 1995; 51: 45–46.

(a)

(b)

Figure 3 Histology of an erythema multiforme lesion shows(a) orthokeratosis, vacuolization of the basal epidermal layerand tagging of lymphocytes along the dermo–epidermaljunction, and (b) vacuolization of the basal epidermal layer.(Hematoxylin and eosin stain; original magnification (a)9100, (b) 9400)

Figure 2 Erythema multiforme lesions over the abdomen

Figure 1 Right auricular chondritis with characteristicsparing of the lobule

ª 2013 The International Society of Dermatology International Journal of Dermatology 2014, 53, 1272–1274

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3 Rodríguez-Pinilla E, Martínez-Frías ML. Corticosteroidsduring pregnancy and oral clefts: a case–control study.Teratology 1998; 58: 2–5.

4 Rauh G, Kamilli I, Gresser U, et al. Relapsingpolychondritis presenting as cutaneous polyarteritisnodosa. Clin Invest 1993; 71: 305–309.

5 Frances C, el Rassi R, Laporte JL, et al. Dermatologicmanifestations of relapsing polychondritis: a study of 200cases at a single center. Medicine (Baltimore) 2000; 80:173–179.

6 McAdam LP, O’Hanlan AM, Bluestone R, et al.Relapsing polychondritis: a prospective study of 23patients and a review of the literature. Medicine 1976;55: 193–215.

7 Tronquoy AF, de Quatrebarbes J, Picard D, et al. Papularand annular fixed urticarial eruption: a characteristic skinmanifestation in patients with relapsing polychondritis.J Am Acad Dermatol 2011; 65: 1161–1166.

8 Estes SA. Relapsing polychondritis. Cutis 1983; 32: 471–474.

9 Labarthe MP, Bayle-Lebey P, Bazex J. Cutaneousmanifestations of relapsing polychondritis in a patientreceiving goserelin for carcinoma of the prostate.Dermatol 1997; 195: 391–394.

10 Papo T, Wechsler B, Bletry O, et al. Pregnancy inrelapsing polychondritis: twenty-five pregnancies ineleven patients. Arthritis Rheum 1997; 40: 1245–1249.

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