Erythrokeratoderma en cocardes

B. Rajagopalan, S. Pulimood, S. George and M. JacobDepartment of Dermatology, Christian Medical College Hospital, Vellore, Tamil Nadu, India

Summary The erythrokeratodermas are a distinct but clinically variable group of rare geno-dermatoses, characterized by circumscribed erythematous and hyperkeratotic lesions.All attempts to establish a valid classification have been based on purely clinical andmorphologic criteria. Erythrokeratoderma en cocardes, also known as genodermatoseen cocardes or Degos’ syndrome, was first described by Degos in 1947. The condition ischaracterized by large round plaques with concentric erythema and scaling having atarget configuration, which remit and recur, in addition to scaly plaques as seen inerythrokeratoderma variabilis. A case of this rare genodermatosis is described.

Report

A 30-year-old housewife from South India presentedwith a 13-year history of itchy skin lesions whichinitially began on her neck but later appeared on arms,face, scalp and buttocks. The rash was more severeduring the summer lasting 3–4 weeks followed by partialremission; itching increased on exposure to sunlight andlesions recurred both on previously involved and un-involved sites. Little response had been achieved fromattempts at treatment. She was the fourth child (allfemales) of consanguinous parents with no history ofskin disease or atopy.

On examination the characteristic rosettes were pre-sent only in the right antecubital fossa and glutealregion. They were polycyclic with concentric rings ofscaling erythema. (Fig. 1).

There were multiple dull erythematous scaly plaquesscattered over the face, scalp, trunk and limbs. Thepalms, soles and oral mucosa were normal and nosystemic abnormalities were noted.

Scrapings for fungus were negative on microscopy.Biopsy was nonspecific showing only hyperkeratosis,

focal parakeratosis acanthosis and a light upper dermalperivascular round cell infiltrate.

Discussion

A clinical diagnosis of erythrokeratoderma encocardes1,2 was made, based on: the presence of poly-cyclic plaques with concentric erythema, the scaling thatshowed an ‘en cocarde’ appearance and erythematousscaly plaques suggestive of erythrokertoderma variabi-lis.3,4,6 In view of the concentric appearance of some ofthe lesions, a differential diagnosis of tinea imbricata wasconsidered, but this was ruled out by a negative potas-sium hydroxide preparation. Some of the lesionsappeared suggestive of ichthyosis linearis circumflexabut there were no associated features such as hairshaft defects or typical inflammatory lesions. Lesionssimilar to ichthyosis linearis circumflexa have beendescribed previously in cases of erythrokeratoderma encocardes.6 The morphology and subsequent evolutionwere unlike psoriasis and in the absence of a familyhistory, familial skin peeling syndromes were unlikely.

Symmetrical progressive erythrokeratoderma is char-acterized by fixed geographical plaques unlike those inour patient, in whom lesions faded or migrated todifferent sites. Histopathologically, erythrokeratodermaen cocardes shows hyperkeratosis, papillomatosis andvariable acanthosis. These findings are not specific andare of little aid in the diagnosis.5,6

Although the onset of erythrokeratoderma en

Clinical dermatology • Concise report

q 1999 Blackwell Science Ltd • Clinical and Experimental Dermatology, 24, 173–174 173

Paper No.: 446

Correspondence: S. Pulimood, Department of Dermatology, ChristianMedical College Hospital, Vellore 632 004, Tamil Nadu, India. Tel: þ91 41622102 extn 2054.

Accepted for publication 12 November 1998

cocardes usually occurs at birth, our patient had a lateronset. However, a later onset has been known to occur insome cases of erythrokeratoderma variabilis of whicherythrokeratoderma en cocardes is considered to be avariant.3,6 Yet others have felt that the two are geneti-cally distinct disorders.5

Other typical features of our patient are the normalpalms and the history of aggravation on exposure tosunlight, which contrasts with Cram’s suggestion thatsummer heat is beneficial for these patients.6 Aggrava-tion on exposure to sunlight and remission in winter hasbeen reported previously in erythrokeratoderma varia-bilis occurring in a Jewish Kurdish family.7 Rappaportet al. speculate that the well known sensitivity oferythrokeratoderma lesions to temperature changes,wind and emotional stress to be causally related toincreased numbers of unmyelinated nerves in the papillarydermis.8

Etretinate has been found to be useful in erythrokerato-derma variabilis.3 However, because of financialconstraints and the potential for side-effects with etretinate,our patient was given only topical therapy with akeratolytic (2% salicylic acid) and retinoic acid withsome benefit.

To the best of our knowledge there are no case reportsof genodermatose en cocardes from India and only a fewreports in the world literature.

References

1 Schellander FG, Fritsch PO. Variable erythrokeratoderma.An unusual case. Arch Dermatol 1969; 100: 744–8.

2 Degos R, Delzant O, Morival H. Erytheme desquamatif enplaques, congenital et familial (genodermatose nouvelle?).Bull Soc Fr Derm Syph 1947; 54: 442.

3 Griffiths WAD, Leigh IM, Marks R. Disorders of Keratinisa-tion. In: Champion RH, Burton JL, Ebling FJG, eds. Textbookof Dermatology. London: Blackwell Scientific Publications,1993: 1325–90.

4 Brown J, Kierland RR. Erythrokeratoderma variabilis.Report of three cases and review of the literature. ArchDermatol 1966; 93: 194–201.

5 Phillips SB, Baden HP. Ichthyosiform Dermatoses. In: Fitz-patrick TBEisen AZWolff K et al. eds. Dermatology in GeneralMedicine New York: McGraw Hill, 1993: 531–44.

6 Cram DL. Erythrokeratoderma variabilis and variablecircinate erythrokeratodermas. Arch Dermatol 1970; 101:68–73.

7 Hacham-Zadeh S, Even-Paz Z. Erythrokeratodermia variabi-lis in a Jewish Kurdish family. Clin Genet 1978; 13: 404–8.

8 Rappaport P, Goldes JA, Goltz RW. Erythrokeratodermia vari-abilis treated with Isotretinoin. A clinical, histologic andultrastructural study. Arch Dermatol 1986; 122: 441–5.

Erythrokeratoderma en cocardes • B. Rajagopalan et al.

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Figure 1 Cubital fossa showed concentric rings of erythema andscaling.