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Quiz

Erythematous Papular Lesions on the Neck: A Quiz

Alice Mouchard1, Matthias Tallegas2, Marie-Christine Machet2,3, Laurent Machet1,3 and Hélène Cornillier1,3*

1Department of Dermatology, 2Department of Pathology, CHRU Tours, FR-37044 Tours, and 3PRES Centre, Val de Loire University, University of Tours, Tours, France. *E-mail: drhcornillier@gmail.com

A 14-year-old boy presented with an 8-month history of asymptomatic papules on his neck. The lesions first appeared on the left side and spread to the right side. The patient’s personal and family medical histories were unremarkable, and he was not taking any medication. Clinical examination revealed erythematous papules with umbilicated and keratotic centres, arranged in linear and annular patterns on the neck (Fig. 1).

No other lesions were noted on skin examination and the clinical examination was otherwise normal. Complete blood count, C-reactive protein level, and renal and liver function test results were within normal limits. A skin biopsy was performed (Fig. 2).

What is your diagnosis? See next page for answer.


Fig. 1. (a, b) Erythematous papules with a keratotic centre on the left side of the neck. (c) Papules grouped in an annular pattern on the right side of the neck.


Fig. 2. (a) Intra-epidermal pseudo-cavity connected to the surface, filled with elastin and collagen fibres. Note the thickened epidermis and epithelial mild dermal inflammatory cells infiltrate (hematin-phloxin-saffron, ×200). (b) Higher magnification showing fragmented elastin fibres filling the intraepidermal cavity (orcein ×400).

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Erythematous Papular Lesions on the Neck: A Commentary

Acta Derm Venereol 2018; XX: XX–XX.

Diagnosis: Elastosis perforans serpiginosa (EPS)

A diagnosis of elastosis perforans serpiginosa (EPS) may be suspected if skin examination reveals keratotic papules grouped in an annular pattern (1, 2). Differential diagnoses usually includes folliculitis, prurigo nodularis and lichen planus (1). Dermoscopy may be helpful in differentiating EPS from granuloma annulare (3). Histopathological examination revealed a thickened epidermis and a transepithelial channel filled with basophilic material (Fig. 2a), consisting of degenerated keratinocytes, inflammatory cells and elastic fibres (Fig. 2b). Perforating dermatoses are characterized by a transepidermal elimination of various materials including fibres of collagen, elastin or cell debris. EPS is 1 of the 4 primary acquired perforating dermatoses; the other 3 are reactive perforating collagenosis, perforating folliculitis and Kyrle disease (1, 2).

The prevalence of EPS is unknown; only a few cases have been described in the literature. It appears to be more frequent in young males aged 10–30 years. Three subtypes are described: reactive, drug-induced and idiopathic. Reactive EPS is associated with genodermatoses and connective tissue diseases, such as Down, Ehlers-Danlos, Marfan, and Rothmund-Thomson syndromes; osteogenesis imperfecta; morphea; progeria; cutis laxa; and pseudoxanthoma elasticum (1, 2, 4). Drug-induced EPS can be caused by D-penicillamine treatment of Wilson’s disease (5). So-called idiopathic EPS may have a genetic basis because familial occurrence with autosomal dominant inheritance has been described; however, no gene has been identified (6, 7).

EPS lesions can resolve spontaneously after a few months or years, sometimes leaving atrophic or hypopigmented scars. Several therapies have been proposed: topical or intralesional corticosteroids, topical retinoids, oral isotretinoin, imiquimod, cryotherapy, CO2 laser therapy or photodynamic therapy (1, 8, 9). There are currently no specific recommendations for EPS treatment, probably because of the small number of reported cases, the spontaneous improvement, and a lack of randomized controlled studies. No therapy was initially proposed, as the patient did not report functional or aesthetic discomfort. However, when the eruption progressed and became pruritic, treatment with topical retinoids was initiated (tazarotene 0.1% once/day) (10). Partial remission was noted after 3 and 6 months.

REFERENCES
  1. García-Malinis AJ, Del Valle Sánchez E, Sánchez-Salas MP, Del Prado E, Coscojuela C, Gilaberte Y. Acquired perforating dermatosis: clinicopathological study of 31 cases, emphasizing pathogenesis and treatment. J Eur Acad Dermatol Venereol 2017; 31: 1757–1763.
    View article    Google Scholar
  2. Mehta RK, Burrows NP, Payne CM, Mendelsohn SS, Pope FM, Rytina E. Elastosis perforans serpiginosa and associated disorders. Clin Exp Dermatol 2001; 26: 521–524.
    View article    Google Scholar
  3. Navarrete-Dechent C, Puerto Cd, Bajaj S, Marghoob AA, González S, Jaque A. Dermoscopy of elastosis perforans serpiginosa: a useful tool to distinguish it from granuloma annulare. J Am Acad Dermatol 2015; 73: e7–e9.
    View article    Google Scholar
  4. Lee S-H, Choi Y, Kim S-C. Elastosis perforans serpiginosa. Ann Dermatol 2014; 26: 103–106.
    View article    Google Scholar
  5. Menzies S, Kirby B. Drug-induced elastosis perforans serpiginosa. BMJ Case Rep 2015; 2015. pii: bcr2015212482.
    View article    Google Scholar
  6. Langeveld-Wildschut EG, Toonstra J, van Vloten W A, Beemer FA. Familial elastosis perforans serpiginosa. Arch Derm 1993; 129: 205–207.
    View article    Google Scholar
  7. Rios-Buceta L, Amigo-Echenagusia A, Sols-Candelas M, Fraga-Fernandez J, Fernandez-Herrera J. Elastosis perforans serpiginosa with simultaneous onset in two sisters. Int J Dermatol 1993; 32: 879–881.
    View article    Google Scholar
  8. Vearrier D, Buka RL, Roberts B, Cunningham BB, Eichenfield LF, Friedlander SF. What is standard of care in the evaluation of elastosis perforans serpiginosa? A survey of pediatric dermatologists. Pediatr Dermatol 2006; 23: 219–224.
    View article    Google Scholar
  9. Pola?ska A, Bowszyc-Dmochowska M, ?aba RW, Adamski Z, Reich A, Da?czak-Pazdrowska A. Elastosis perforans serpiginosa: a review of the literature and our own experience. Postepy Dermatol Alergol 2016; 33: 392–395.
    View article    Google Scholar
  10. Outland JD, Brown TS, Callen JP. Tazarotene is an effective therapy for elastosis perforans serpiginosa. Arch Dermatol 2002; 138: 169–171.
    View article    Google Scholar
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