may be a mixed population of accessory eyelashes andeyebrows, or the hairs may be originally eyebrowsinstead of eyelashes.

In other reported cases, no positive family history wasnoted, but in our case the same anomaly was reported(although not personally examined by us) in his paternalgrandfather, suggesting the possibility of an autosomaldominant pattern of inheritance. Surgical removal ofectopic cilia is the treatment of choice, although most ofthe reported cases reported no apparentmorbidities withthis anomaly apart from the cosmetic aspect (6).

ACKNOWLEDGMENTS

Authors would like to thank Farzan Institute for Re-search and Technology for technical assistance.

REFERENCES

1. Dalgleish R. Ectopic cilia. Br J Ophthalmol 1966;50:592–594.

2. Owen RA. Ectopic cilia. Br J Ophthalmol 1968;52:280.3. MacQuillanA,HamiltonS,GrobbelaarA.Angiomes, clefts

and eyelashes. Plast Reconstr Surg 2004;113:1400–1403.4. Chen TS, Mathes EFD, Gilliam AE. ‘‘Ectopic eyelashes’’

(ectopic cilia) in a 2-year-old girl: brief report and discussionof possible embryogenic origin. Pediatr Dermatol2007;24:433–434.

5. Peramique L, Barnadas MA, Dalmau J et al. A case ofectopic cilia. J Eur Acad Dermatol 2007;21:249–289.

6. Konas E. Accessory eyelash. Eur J Plast Surg 2008;31:43–44.

7. Guler M, Yilmaz T, Guler O. A case of ectopic cilia. IntOphthalmol 2009;29:297–298.

SHAHRAM BAGHESTANI, M.D.,*SEYYED ALI BANIHASHEMI, M.D.�*Department of Dermatology, Hormozgan University ofMedical Sciences, Banda Abbas, Iran, �Department ofOphthalmology, Hormozgan University of MedicalSciences, Banda Abbas, Iran

FAMILIAL ANNULAR ERYTHEMA—A RARE

DERMATOLOGY DIAGNOSIS

Abstract: We report the second case of autosomaldominant familial annular erythema since its originaldescription in 1966 by Beare et al. A three and a half-year-old boy presented at five days of age with a wide-spread annular urticated erythematous rash which has

persisted despite treatment with various antihistaminesand mast-cell stabilizers. His mother reports an identicaleruption with an absence of systemic symptoms.

A 3-year-old boy was referred with persistent urticariallesions.Hewas born at term following an uncomplicatedpregnancy and delivery. He developed cellulitis of theright foot at 5 days of age and was prescribed intrave-nous flucloxacillin and benzylpenicillin. Within 2 days,he developedurticarial lesions (Figs. 1 and 2)with a clearpalpable edge and central clearing but no scale, on thetrunk, limbs, and face. Examination of the nails andjoints was unremarkable. No evidence existed oflymphadenopathy or hepatosplenomegaly. The eruptionhas since persisted. Itchy annular rings of erythemaoccuron an almost daily basis, slowly enlarge and then settleover the next few days. No history is known of dryness,

Figure 1. Urticarial rash at one week of age.

Figure 2. Urticarial rash on abdomen.

Address correspondence to Shahram Baghestani, M.D., POBox: 13185-1678, Tehran, Iran, or e-mail: swt_f@yahoo.com.

56 Pediatric Dermatology Vol. 28 No. 1 January ⁄February 2011

scaling, bruising, or systemic symptoms. His skin clearswith foreign sun exposure and worsens with intercurrentinfection and teething.

Blood investigations, including ANA, Ro, and La,werenormal.A skinbiopsy (Fig. 3) showedamoderatelydense, superficial, and deep perivascular inflammatorycell infiltrate composed of neutrophils and a smallnumber of eosinophils and histiocytes. A small amountof nuclear dust was seen.

The lesions did not respond well to trimeprazine,chlorpheniramine, cetirizine, or ranitidine individually,or to combinations of cetirizine, piriton, and sodiumchromoglycate. His mother also describes identical per-sistent lesions that started when she was 5 days old. Hersymptoms have lessened over the years, and flares arelocalized to the breast area alone. She is also allergic topenicillin, and her blood investigations are negative.A skin biopsy, taken as an infant, showed a perivascularinfiltrate of lymphocytes, histiocytes, and eosinophils.Family history, aside from his mother’s, dating backthree generations is unremarkable.

DISCUSSION

The main features of previously published cases ofapparently inherited annular erythema are summarizedin Table 1. Our case best fits the original descriptionof autosomal dominant familial annular erythemapublished in 1966 by Beare et al (1). Beare reported fouraffected members in three generations of an Irish family.The clinical and laboratory findings were essentiallysimilar for all subjects. Onset of symptoms occurredeither a few days after birth, or in one family member, aslate as 15 years. In all but one of the familymembers, theannular erythemadidnot appear to settle spontaneously.

The typical lesions reported were erythematous urti-carial spots, which enlarged and cleared centrally leavinga mild pigmentation that then disappeared completely,the cycle occupying 4 or 5 days. Lesions could appearanywhere on the body, and only rarely was the subjectentirely free. Specifically, Beare mentions that dermo-graphism and pruritus associated with lesions weremarked, butnovesiculationor scaling everoccurred.Thelesions appeared to be uninfluenced by seasonal, physi-cal, or psychological factors, or by any local treatment orsystemic antihistamines tried. All members affected wereotherwise fit and well, and routine investigations were allessentially normal, although all affected membersshowed an increase in alpha 2 globulin, on serumproteinelectrophoresis, with low IgA levels.

Biopsies were reported in Beare’s cases to show mildperivascular infiltration with chronic inflammatory cellsin theupper andmiddermis indicating that the skin lesioncould be due to changes in the smaller blood vessels, butoverall the featureswere fairly nonspecific. In conclusion,very few reported cases exist of apparently inheritedannular erythemas, and considerable clinical andpathological overlap occur between these cases. Our caseseems to best fit with the original description of familialannular erythema in terms of its clinical presentation,pathological features, and lack of response to treatment.

Figure 3. Marked superficial and deep perivascular infiltratecomposed of an admixture of cells including eosinophils.

TABLE 1. Familial Annular Erythemas

Disorder Cases Age of onset Duration Clinical manifestations

Familial annularerythema (1)

4 members in3 generations

Infancy in 3 cases;15 yrs in 1 case

Can be lifelong disorder Centrifugally enlarging lesions ontrunk + limbs. No vesiculation orscaling

Erythemagyratumperstans (2)

Brother and sister 3 yrs >15 yrs Annular lesions on face, trunk + limbs.Desquamation + vesiculation

Annularerythema ofinfancy

Daughter andfather (3)

Infancy Can be lifelong disorder.Cleared temporarily whileon antifungal treatment

Erythematous, annular lesions on face,trunk + limbs. No scaling

Identical twins(4)

Childhood Responded to topicalsteroids

Arcuate + polycyclic plaques on trunk.Central scale

Brief Reports 57

REFERENCES

1. Beare JM, Frogatt P, Jones JH,Neill DW. Familial annularerythema: an apparently new dominant mutation. Br JDermatol 1966;78:60–68.

2. Colcott Fox T. Erythema gyratum perstans in the two eldermembers of a family. In: Unna PG, Morris M, Besnier Eet al, eds. International atlas of rare skindiseases.Hamburg,Germany: L Voss, 1891:15–19.

3. Brown S, Parslew R. Erythema annulare centrifugumaffecting father and daughter. Br J Dermatol 2004;151:264.

4. WatskyKL,HansenT.Annular erythema in identical twins.Cutis 1989;44:139–140.

CHRISTINAWILLIAMS, M.A., MRCPWILLIAM J. MERCHANT, FRCPath, DipRCPathSHEILAM. CLARK, MBChB, FRCPDepartment of Dermatology, Leeds General Infirmary,Leeds, England

DISSEMINATED CONGENITAL COMEDONES

Abstract: A 3-month-old boy with congenital andextensive skin comedones without any other extracu-taneous manifestations is reported. This patient doesnot fit with other reported disorders of congenital orchildhood extensive comedones, such as nevuscomedonicus, familial dyskeratotic comedones, idio-pathic disseminated comedones, childhood flexuralcomedones, and acne neonatorum.

Comedones in a newborn are an infrequent finding. Wepresent a child with extensive congenital comedones onhis face, neck, upper trunk, and proximal upper limbs.

CASE REPORT

A 3-month-old boy was referred to our department be-cause of the presence of asymptomatic skin comedonessince birth. Pregnancy and labor had been uneventful,andnopersonalor familial historyofhyperandrogenism,severeacne,orhidradenitis suppurativawasreported.Onexamination,widespread,opencomedoneslocatedontheupper trunk, face, and upper limbs were seen (Figs. 1, 2,and3),with abilateral and symmetric distribution,whichdid not follow the lines of Blaschko or other recognizedtypes of mosaic pattern. The scalp, palms, soles, andmucous membranes were spared. A closer view and der-

moscopy revealed that comedones consisted of keratinplugs obstructing the follicular openings. The remainderof the physical examination was normal.

Figure 2. Open comedones located on the left upper limb.

Figure 3. Magnified image of comedones.

Address correspondence to Christina Williams, M.A., MRCP,Department of Dermatology, Leeds General Infirmary, GreatGeorge Street, Leeds LS1 3EX, England, or e-mail: tinawilliams@doctors.org.uk.

Figure 1. Comedones located on the face and upper trunk,with a bilateral and symmetric distribution.

58 Pediatric Dermatology Vol. 28 No. 1 January ⁄February 2011