Annually Recurring Erythema Annulare Centrifugum: a Distinct Entity?

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Annually Recurring Erythema Annulare Centrifugum: a Distinct Entity? JAM ACAD DERMATOL Brief reports 1091 VOLUME 54, NUMBER 6 Annually recurring erythema annulare centrifugum: A distinct entity? M. Pilar Garcı´aMuret,MD,a Ramon M. Pujol, MD,b Anna M. Gimenez-Arnau, MD,b Carles Barranco, MD,c Fernando Gallardo, MD,b and Agustı´ Alomar, MDa Barcelona, Spain Four patients presenting a peculiar clinical variant of erythema annulare centrifugum are reported. The lesions were clinically and histopathologically indistinguishable from classic superficial erythema annulare centrifugum but constant annual and seasonal recurrences for many years or decades were observed. No clear precipitating factor could be identified. No associated symptoms were present and the eruption regressed spontaneously after a variable period of days to months. Annually recurring erythema annulare centrifugum seems to represent a rare distinct clinical entity that has received little attention in literature. Clinicopathologic features of this peculiar clinical disorder and the differential diagnosis with other recurrent seasonal eruptions are reviewed. ( J Am Acad Dermatol 2006;54:1091-5.) peculiar form of superficial erythema annu- lare centrifugum (EAC) presenting constant Abbreviations used: yearly recurrences for many years was first AR: annually recurring A 1 reported by Christine in 1930. This peculiar variant EAC: erythema annulare centrifugum LDH: lactate dehydrogenase of annually recurring (AR) EAC received little atten- tion in the literature, and only isolated reports in the European literature1 were published afterward. In 1986, Yoshikuni et al,2 pointed out the possible 2000) treated with operation and chemotherapy. association between AR EAC and hereditary lactate She was following treatment with acenocoumarol, dehydrogenase (LDH) M-subunit deficiency. glibenclamide, hydrochlorothiazide, and diltiazem We review the clinical, histopathologic, and bio- chlorhydrate. chemical features of 4 patients presenting with this The patient presented annually relapsing non- peculiar and recurrent form of EAC. pruritic annular lesions located on her chest, back, arms, and legs that constantly appeared in the summer and resolved spontaneously in autumn. CASE REPORTS The eruption began as small erythematous papules Case 1 that coalesced into annular plaques with central A 76-year-old woman was referred for evaluation clearing and centrifugal spread. No precipitating of a relapsing self-healing annular eruption involving factors were identified. No fever, general symptoms, her trunk and extremities that had recurred yearly for or mucosal lesions were present. the last 8 years. Medical history included diabetes Physical examination revealed an apparently mellitus, hyperthyroidism, valvular heart disease, healthy woman with multiple 2- to 8-cm erythema- cholecystectomy, and breast carcinoma (October tous urticariform and annular lesions on her chest, back, arms, and thighs (Fig 1). A peripheral scaling a border was occasionally noted. The lesions regressed From the Department of Dermatology, Hospital de la Santa Creu i Sant Pau and the Departments of Dermatologyb and Patholo- spontaneously 4 months after onset. Neither inguinal gy,c Hospital del Mar, IMAS. nor axillary lymph nodes were present. Funding sources: None. Conflicts of interest: None identified. Reprint requests: M. Pilar Garcı´a Muret, MD, Department of Case 2 Dermatology, Hospital de la Santa Creu i Sant Pau, Avda Sant An 83-year-old man was referred to our depart- Antoni M Claret 128, 08025 Barcelona, Spain. E-mail: ment for evaluation of a 23-year history of an AR [email protected]. 0190-9622/$32.00 pruritic annular eruption that appeared constantly ª 2006 by the American Academy of Dermatology, Inc. during the summer months (May-June) and regres- doi:10.1016/j.jaad.2006.01.025 sed spontaneously in autumn (September-October). 1092 Brief reports JAM ACAD DERMATOL JUNE 2006 Fig 1. Erythematous urticariform and annular plaques on Fig 2. Erythematous purpuric infiltrated plaque on back chest (case 1). aspect of thigh (case 2). Medical history disclosed arterial hypertension and squamous cell carcinoma of the tongue diagnosed in 1988 treated with operation, radiotherapy, and che- motherapy in 1990. During the last 5 years, physical examination had revealed multiple erythematous, purpuric infiltrated papules and plaques with centrifugal extension and central clearing that led to urticarial, nonscaly plaques with arcuate or annular elevated borders and central patchy hyperpigmentation. The lesions involved symmetrically the internal aspects of both arms and legs (Fig 2). Fig 3. Edema of papillary dermis and moderated perivas- Case 3 cular lymphocytic infiltrate in papillary and middermis A 55-year-old man came to our department for (case 2). (Hematoxylin-eosin stain; original magnification: evaluation of a peculiar recurrent summer eruption. 320.) Medical history was unremarkable. For the last 13 years he referred to the development active border, and progressed centrifugally with a of a peculiar and constant skin eruption in summer. discrete scaly peripheral rim and leaving occasional No other precipitating factors were identified. The residual hyperpigmentation. The rest of the physical lesions were pruritic, erythematous, and violaceous examination was unremarkable. annular plaques involving both legs and arms. They progressed centrifugally with fine peripheral scaling, Laboratory studies persisted for 15 days and tended to disappear spon- In all 4 patients a complete routine laboratory taneously. During the last 4 years the disorder was investigation including hematologic, biochemical partially controlled with systemic steroid therapy. No (glucose, hepatic and renal parameters, serum elec- mucosal lesions were present and the rest of the trophoresis), and immunologic (antinuclear factor, physical examination disclosed no abnormalities. rheumatoid factor, A [Ro]/[La], organ-specific anti- bodies, immunoglobulins, radioallergosorbent) tests Case 4 disclosed no abnormalities. Borrelia burgdorferi A 55-year-old woman presented a 15-year history antibodies were constantly negative. Direct potas- of recurrent symmetric violaceous purpuric annular sium hydroxide examination and cultures for fungi erythematous plaques showing a central clearing and bacteria from skin lesions failed to identify and peripheral spread on her arms and legs. Medical micro-organisms. No phototests were performed. history disclosed a goiter treated with surgical exci- Chest radiographs were consistently normal. Elec- sion. The patient was treated with thyroidal substi- trophoretic analysis of erythrocyte LDH isozymes tutive treatment. was not performed (not available in our laboratory). The eruption appeared in the spring and/or summer months and regressed spontaneously after Histopathologic examination 4 months. Physical examination revealed an appar- Histologic examination of 5 skin biopsy spec- ently healthy patient with multiple annular papules imens revealed an inflammatory perivascular lym- and plaques on both arms and legs. The lesions were phohistiocytic infiltrate of variable intensity in the occasionally purpuric and painful, presented an papillary and middermis with occasional eosinophils JAM ACAD DERMATOL Brief reports 1093 VOLUME 54, NUMBER 6 Table I. Annually recurring erythema annulare centrifugum: Review of the literature Author Sex/age, y Affected area Histologic features Associations Treatment Evolution/onset Pin˜ol Aguade´ F/60 Legs, elbows Not done None Spontaneous 7 y/spring- et al 19661 resolution summer in 15 d F/43 Legs, arms Hyperkertosis 1 edema None Spontaneous 17 y/summer dermis, perivascular resolution infiltrate in 20 d Janss F/36 Arms, back Normal epidermis, dense None Spontaneous 1 y previously/ et al 19925 perivascular and resolution not reported periadnexal lymphocytic in 6 wk infiltrate Reported F/76 Chest, back, All cases: perivascular None Spontaneous 8 y/summer cases arms, legs lymphohistiocytic resolution infiltrate dermis 1 in autumn eosinophils M/83 Internal aspect Normal epidermis None Spontaneous 23 y/summer extremities resolution in autumn M/55 Legs, arms No vasculitis None Spontaneous 13 y/summer resolution in 15 d F/55 Legs, arms None Spontaneous 15 y/spring resolution in 4-5 mo F, Female; M, male. (3 biopsy specimens) (Fig 3). Neither fibrinoid viral, fungal, mycobacterial, and parasitic), hormonal necrosis nor vascular damage was detected. Papil- disturbances (menstrual cycle, hyperthyroidism), lary dermal erythrocyte extravasation was observed some food and drugs (salicylate, antimalarial, cimet- in 3 biopsy specimens. Edema of the papillary dermis idine, amitriptyline, gold sodium thiomalate, and was an inconstant feature (two skin biopsy speci- etizolam), and even occult malignant solid or hema- mens). Discrete acanthosis (one biopsy specimen) tologic neoplasms have been incriminated as caus- and mild spongiosis (two biopsy specimens) were ative factors. However, in a large proportion of cases also occasionally noted. no causative agent can be detected (idiopathic EAC). Direct immunofluorecence study from involved Two distinct forms of EAC (superficial and deep) skin in 4 specimens failed to demonstrated IgM-, have been distinguished. In the superficial variant IgG-, IgA-, or C3-specific deposits. the lesions tend to be clinically nonindurated and manifest scaling along the ring-shaped or gyrate bor- DISCUSSION der. Histologically, a well-demarcated ‘‘coat-sleeve’’ ‘‘Erythema annulare centrifugum’’3 is the term superficial perivascular dermal lymphohistiocytic
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