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Year : 2020  |  Volume : 21  |  Issue : 3  |  Page : 191-193

Erythrokeratoderma en cocardes – A rare phenotype

1 Department of Dermatology, PSG Institute of Medical Sciences and Research, Coimbatore, Tamil Nadu, India
2 Department of Pathology, PSG Institute of Medical Sciences and Research, Coimbatore, Tamil Nadu, India

Date of Submission24-Jan-2020
Date of Decision10-Feb-2020
Date of Acceptance17-Mar-2020
Date of Web Publication30-Jun-2020

Correspondence Address:
Dr. Reena Rai
Department of Dermatology, PSG Institute of Medical Sciences and Research, Coimbatore - 641 004, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpd.IJPD_16_20

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Erythrokeratodermas are disorders of keratinization with two major types, namely erythrokeratodermia variabilis and symmetrical progressive erythrokeratoderma. Apart from the two main types, rare variants include erythrokeratoderma en cocardes (EEC), erythokeratoderma with erythema gyratum repens-like lesions, erythema annulare centrifugum-like lesions, and reticulate erythrokeratoderma. Herein, we report a rare case of EEC with associated palmoplantar keratoderma, the association which has not been described in previous reports.

Keywords: En cocardes, erythrokeratoderma, palmoplantar keratoderma

How to cite this article:
Arunprasath P, Rai R, Umamaheswari G. Erythrokeratoderma en cocardes – A rare phenotype. Indian J Paediatr Dermatol 2020;21:191-3

How to cite this URL:
Arunprasath P, Rai R, Umamaheswari G. Erythrokeratoderma en cocardes – A rare phenotype. Indian J Paediatr Dermatol [serial online] 2020 [cited 2021 Jun 23];21:191-3. Available from: https://www.ijpd.in/text.asp?2020/21/3/191/288492

  Introduction Top

Erythrokeratodermas are disorders of keratinization which encompass two main subtypes, namely erythrokeratodermia variabilis (EKV) and symmetric progressive erythrokeratoderma (SPEK) and other atypical rare variants.[1] Erythrokeratoderma en cocardes (EEC) is a rare variant characterized by polycyclic, concentric, and targetoid lesions along with erythematous plaques.[2]

  Case Report Top

A 4-year-old boy born out of third-degree consanguineous marriage presented with multiple hyperpigmented scaly lesions involving the arms, forearms, back, gluteal region, thighs, and legs since 6 months of his age. There was a history of aggravation of lesions during cold season and improvement during summer. There was no history of atopy and other systemic symptoms. Milestones were achieved normally. There was no history of similar skin lesions in siblings or other family members.

Cutaneous examination revealed multiple scaly plaques with polycyclic borders involving the arms, forearms, thighs, and legs [Figure 1]a, [Figure 1]b and [Figure 2]. In the posterior aspect of the thigh, there were scaly plaques with polycyclic borders sparing of the popliteal fossa [Figure 3]. There were multiple discrete annular scaly plaques with concentric rings involving the forearms and thighs. Anterior aspect of legs showed diffuse ichthyosis extending to the dorsum of the feet [Figure 4]. Palms and soles revealed diffuse keratoderma with fissuring and scaling [Figure 5]. Examination of hair, nails, and teeth was normal. Systemic examination was essentially normal. Hematological examination did not reveal any abnormality. Scrapings of skin for potassium hydroxide mount were negative for fungal elements.
Figure 1: (a) Multiple scaly plaques with polycyclic borders involving the arm and forearm, (b) Scaly plaques with polycyclic borders involving the arm

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Figure 2: Scaly plaques with polycyclic borders involving the anterior aspect of thighs

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Figure 3: Scaly plaques with polycyclic borders involving the posterior aspect of thighs sparing the popliteal fossa

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Figure 4: Diffuse ichthyosis of legs extending to dorsum of feet

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Figure 5: Diffuse palmoplantar keratoderma

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Histopathology revealed hyperkeratosis, focal mild parakeratosis, conspicuous granular layer, and irregular acanthosis. There were mild spongiosis and lymphocytic exocytosis. The papillary and upper dermis showed pigment incontinence, prominent capillaries, and a mild perivascular lymphohistiocytic infiltrate, a picture compatible with erythokeratoderma [Figure 6].
Figure 6: Epidermis with hyperkeratosis, focal mild parakeratosis, conspicuous granular layer, and irregular acanthosis. Dermis with mild perivascular lymphohistiocytic infiltrates.(H and E, ×100)

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Owing to the distinct clinical morphology with annular lesions, polycyclic borders, and histopathological features, a diagnosis of EEC was entertained.

  Discussion Top

Erythrokeratoderma has two variants: SPEK and EKV. SPEK is characterized by well-demarcated, hyperkeratotic plaques distributed symmetrically over the elbows, knees, dorsal aspect of the hands, feet, and buttocks, sparing the trunk. SPEK has its onset in infancy or early childhood.[3] EKV, on the other hand, manifests as two distinct clinical pictures: with generalized, erythematous, hyperkeratotic plaques in one and localized sharply demarcated hyperkeratotic plaques in the other. EKV has its onset in birth or in neonatal period in 50% of patients and within 1st year of life in 90% of patients.[4]

Rare variants of EKV described include EEC, EKV with erythema gyratum repens-like lesions, EKV with erythema annulare centrifugum-like lesions, and reticulate erythrokeratoderma.[4],[5] Degos et al., in 1947, described EEC in a 13-year-old girl, a rare and atypical variant of EKV with polycyclic plaques, with concentric layers of scaling and erythema giving a targetoid, or “en cocardes” appearance.[6]

The index case described by Degos had an onset at birth, remissions and recurrences, desquamation of palm, similar skin lesions in father and paternal uncle pointing to autosomal-dominant inheritance, and improvement of skin lesions during summer.[7] The present case had onset during 6 months of age, without any family history and improvement during summer. Unlike the index case, the cases reported by Rajagopalan et al. and López-Ferrer et al. did not show improvement of lesions during the summer.[1],[2] Furthermore, the present case showed prominent palmoplantar keratoderma, which has not been described in previous reports.[1],[2],[8]

Apart from EEC, differential diagnoses considered include annular type of epidermolytic ichthyosis, X-linked recessive ichthyosis, and SPEK. Unlike, in annular epidermolytic ichthyosis, there was no history of blisters and erosions at birth, and histopathology did not revealed any epidermolytic hyperkeratosis. X-linked recessive ichthyosis presents with dark brown ichthyotic scales with sparing of popliteal and antecubital fossa with associated cryptorchidism, autism, and deep stromal corneal opacity.[4] Although the present case had ichthyosis of legs with sparing of popliteal fossa, there were no associated systemic abnormalities. Tinea imbricata can be a morphological differential diagnosis, but the presence of skin lesions since 6 months of age and negative test for skin scrapings for fungus preclude the condition.

Histopathology cannot distinguish between the variants of erythrokeratodermas. Genetic studies show mutation in connexin genes in cases of EKV. Connexins are a family of transmembrane proteins that form gap junctions in the cells. These intercellular channels play an important role in cell growth, homeostasis, and epidermal differentiation.[4] Mutations in genes, namely GJB3, GJB4, and rarely GJA1 encoding the connexins, have been identified as the underlying pathogenesis in EKV.[3],[9] In SPEK apart from GJB4 mutation, mutations reported include a frameshift mutation in loricrin gene, homozygous loss of function mutation in KRT83 gene, and mutation in KDSR gene which codes for an enzyme involved in ceramide synthesis.[3] López-Ferrer et al. have reported a case of EEC with missense mutation R32W in GJB3 gene.[1] Similar mutation in GJB3 gene had been reported in patients with palmoplantar involvement and deafness.[10] The present case had clinical lesions analogous to EEC and palmoplantar involvement but without any hearing impairment. However, genetic study for mutation analysis was not done due to resource constraints.

Treatment options include topical emollients, retinoids, salicylic acid, and corticosteroids. Systemic retinoid such as acitretin and isotretinoin shows good improvement, but with recurrence of lesions once the treatment is stopped.[8] Improvement with retinoids with psoralen and ultraviolet A therapy has been reported.[11] Since the disease is a genodermatoses, parents should be counseled regarding the chronic nature of the disease and the need for long-term follow-up. Treatment plan with acitretin was considered, but the patient did not report for follow-up.

The present case is highlighted for its distinct clinical picture and its association with palmoplantar keratoderma.

Declaration of patient consent

Author certify that they have obtained all appropriate patient consent forms. In the form the patients/parents have given their images and other clinical informations to be reported in the journal. The patients & parents understand that their/their children's names and initials will not be published and due efforts will be made to conceal their identity.

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Conflicts of interest

There are no conflicts of interest.

  References Top

López-Ferrer A, Puig L, Nakamura M, Fernández-Figueras MT, Alomar A. Erythrokeratoderma en cocardes with R32W mutation in GJB3. J Clin Exp Dermatol Res 2010;1:113.  Back to cited text no. 1
Rajagopalan B, Pulimood S, George S, Jacob M. Erythrokeratoderma en cocardes. Clin Exp Dermatol 1999;24:173-4.  Back to cited text no. 2
Richard G, Ringpfeil F. Ichthyoses, Erythrokeratodermas, and Related Disorders. In: Bolognia JL, Schaffer JV, Cerroni L, editors. Dermatology. 4th ed. Philadelphia: Mosby Elsevier; 2018. p. 912-6.  Back to cited text no. 3
Fleckman P, DiGiovanna JJ. The Ichthyoses. In: Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ, Wolff K, editors. Fitzpatrick's Dermatology in General Medicine. 8th ed. New York: McGraw Hill; 2012. p. 530-1.  Back to cited text no. 4
Karadag AS, Bilgili SG, Calka O, Bayram I. Erythrokeratodermia variabilis: Two case reports. Indian Dermatol Online J 2013;4:340-3.  Back to cited text no. 5
[PUBMED]  [Full text]  
Degos R, Delzant O, Morival H. Erytheme desquamatif en plaques, congenital et familial (genodermatose nouvelle?). Bull Soc Fr Dermatol Syphiligr 1947;54:442.  Back to cited text no. 6
Cram DL. Erythrokeratoderma variabilis and variable circinate erythrokeratodermas. Arch Dermatol 1970;101:68-73.  Back to cited text no. 7
Srinivas SM, Dhar S. Erythrokeratodermia variabilis and erythrokeratoderma en cocardes: Case series with review of literature. Indian J Paediatr Dermatol 2016;17:202-5.  Back to cited text no. 8
  [Full text]  
Richard G, Brown N, Smith LE, Terrinoni A, Melino G, Mackie RM, et al. The spectrum of mutations in erythrokeratodermias—novel and de novo mutations in GJB3. Hum Genet 2000;106:321-9.  Back to cited text no. 9
Kelsell DP, Wilgoss AL, Richard G, Stevens HP, Munro CS, Leigh IM. Connexin mutations associated with palmoplantar keratoderma and profound deafness in a single family. Eur J Hum Genet 2000;8:469-72.  Back to cited text no. 10
Yüksek J, Sezer E, Köseoglu D Markoç F, Yildiz H. Erythrokeratodermia variabilis: Successful treatment with retinoid plus psoralen and ultraviolet A therapy. J Dermatol 2011;38:725-7.  Back to cited text no. 11


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]


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