Annular atrophic lichen planus: A rare clinical entity

(1)

Correspondence

Clinical Letter

831

Clinical Letter

Annular atrophic lichen planus: a rare clinical

entity

DOI: 10.1111/ddg.13258 Dear Editors,

A 28-year-old male patient presented with a six-week his-tory of a pruritic eruption on arms and legs. He reported that similar lesions had appeared two years earlier and that they had spontaneously resolved with postinfl ammatory hyperpigmentation. The past medical history and family history were both unremarkable. Dermatological examina-tion revealed hyperpigmented macules on both sides of the trunk. Moreover, he exhibited erythematous, violaceous plaques with a hyperpigmented, depressed, and atrophic center symmetrically distributed on the dorsal aspects of the hands, forearms, legs, and feet (Figure 1 a, b). We per-formed a skin biopsy from the active border of a plaque on the leg to arrive at a defi nitive diagnosis. Histopathological examination revealed focal parakeratosis, hypergranulo-sis, acanthohypergranulo-sis, exocytosis of lymphocytes, vacuolar dege-neration of basal keratinocytes, apoptotic keratinocytes, a perivascular and interstitial lymphohistiocytic infi ltra-te, melanophages, and elastolysis (Figure 2 a, b). Based on these fi ndings, we made the diagnosis of annular atrophic lichen planus.

Lichen planus is a chronic infl ammatory disease cha-racterized by mucocutaneous lesions [ 1 ] . Although the ex-act etiopathogenesis remains unknown, it has been sugge-sted that autoreactive cytotoxic T lymphocytes may play a role in keratinocyte destruction [ 2 ] . Moreover, given that

anti-interleukin-17 (IL-17) agents have been shown to be ef-fective in infl ammatory skin diseases, IL-17 has been impli-cated as the main cytokine in the pathogenesis of such disor-ders, including lichen planus. Recently, it has been suggested that the differentiation of regulatory T cells to IL-17-produ-cing cells may play a key role in infl ammation [ 3 ] .

Lichen planus has several clinical variants including hy-pertrophic, vesiculobullous, actinic, annular, atrophic, and linear forms; lichen planopilaris and lichen planus pigmen-tosus [ 4 ] . A rare subtype that can result in cicatricial alope-cia, lichen planopilaris can be classifi ed into three distinct variants: classic type, frontal fi brosing alopecia, and Gra-ham-Little-Piccardi-Lassueur syndrome. Recently, Mein-hard et al. retrospectively evaluated 104 patients with lichen planopilaris; only one individual had Graham-Little-Piccar-di-Lassueur syndrome [ 5 ] .

Figure 1 Erythematous papules and plaques with a

hyperpig-mented atrophic center on the dorsal aspects of hands and feet (a, b).

Figure 2 Focal parakeratosis, hyper-granulosis, acanthosis, exocytosis of lymphocytes, vacuolar degeneration of the basal keratinocytes, apoptotic keratinocytes, perivascular and inters-titial lymphohistiocytic infiltrate, and melanophages (hematoxylin-eosin stain, original magnification x 100) (a). Decreased elastic fibers in the upper dermis (orcein stain, original magnification x 200) (b).

(2)

Correspondence

Clinical Letter

Funda Tamer

1

_{, Ozge Mine Orenay}

2

_,

Evren Sarifakioglu

2

_{, Reyhan Bayrak}

3

(1) Department of Dermatology , Ufuk University Faculty of Medicine , Ankara , Turkey

(2) Department of Dermatology , Turgut Ozal University Faculty of Medicine , Ankara , Turkey

(3) Department of Pathology , Turgut Ozal University Faculty of Medicine , Ankara , Turkey

Correspondence to Funda Tamer, MD

Ufuk University Faculty of Medicine, Department of Dermatology

Kizilirmak mahallesi, Ufuk universitesi caddesi No:1, 06510 Cankaya

Ankara, Turkey

E-mail: [email protected] References

1 Kim BS , Seo SH , Jang BS et al. A case of annular atrophic lichen planus . J Eur Acad Dermatol Venereol 2007 ; 21 : 989 – 90 . 2 Domingues R , de Carvalho GC , da Silva Oliveira LM et al. The

dysfunctional innate immune response triggered by Toll-like receptor activation is restored by TLR7/TLR8 and TLR9 ligands in cutaneous lichen planus . Br J Dermatol 2015 ; 172 : 48 – 55 . 3 Gatzka M , Scharffetter-Kochanek K . T-cell plasticity in

inflam-matory skin diseases-the good, the bad, and the chameleons . J Dtsch Dermatol Ges 2015 ; 13 : 647 – 52 .

4 Gorouhi F , Davari P , Fazel N . Cutaneous and mucosal lichen planus: a comprehensive review of clinical subtypes, risk factors, diagnosis, and prognosis . ScientificWorldJournal 2014 ; 2014 : 742826 .

5 Meinhard J , Stroux A , Lünnemann L et al. Lichen planopilaris: Epidemiology and prevalence of subtypes – a retrospective analysis in 104 patients . J Dtsch Dermatol Ges 2014 ; 12 : 229 – 36 . 6 Sugashima Y , Yamamoto T . Letter: Annular atrophic lichen

planus of the lip . Dermatol Online J 2012 ; 18 : 14 .

7 Ponce-Olivera RM , Tirado-Sánchez A , Montes-de-Oca-Sánchez G et al. Annular atrophic lichen planus . Int J Dermatol 2007 ; 46 : 490 – 1 .

8 Kim JS , Kang MS , Sagong C et al. Annular atrophic lichen planus associated with hypertrophic lichen planus . Clin Exp Dermatol 2008 ; 33 : 195 – 7 .

9 Manousaridis I , Manousaridis K , Peitsch WK , Schneider SW . Indi-vidualizing treatment and choice of medication in lichen planus: a step by step approach . J Dtsch Dermatol Ges 2013 ; 11 : 981 – 91 . 10 Morales-Callaghan A Jr , Martínez G , Aragoneses H et al. Annular

atrophic lichen planus . J Am Acad Dermatol 2005 ; 52 : 906 – 8 .

Nevertheless, annular atrophic lichen planus is the ra-rest clinical variant of lichen planus. It was fi rst described by Friedman and Hashimato in 1991. Since then, only few cases have been reported. Clinically, annular atrophic lichen pla-nus presents with multiple violaceous, annular papules and plaques with a hyperpigmented atrophic center and raised borders [ 1 ] . Previously reported sites of occurrence of this unusual variant of lichen planus include the lower lip, hands, forearms, legs, feet, knees, elbows, trunk, back, and epi-gastric area [ 6, 7 ] . Histopathologically, the lesions are iden-tical to lichen planus. However, the atrophic center shows a thinned epidermis, with loss of rete ridges and elastolysis [ 8 ] . Topical corticosteroids, calcipotriol, calcineurin inhibi-tors, and photochemotherapy are the treatments of choice. First-line systemic therapies include acitretin, isotretinoin, methotrexate, systemic corticosteroids, hydroxylchloroqui-ne, and dapsone. Cyclosporihydroxylchloroqui-ne, azathioprihydroxylchloroqui-ne, and myco-phenolate mofetil represent second-line treatment options [ 9 ] . The clinical course of annular atrophic lichen planus is chronic, and the disease is usually resistant to treatment [ 1, 6 ] . While Morales-Callaghan et al. reported slight clinical improvement after three months of topical tacrolimus the-rapy [ 10 ] , Sugashima et al. described suffi cient improvement only after six months of such treatment [ 1, 6 ] . It has been suggested that early treatment may result in good clinical outcome [ 6 ] .

The patient presented herein is quite exemplary of an-nular atrophic lichen planus. The clinical disease course was chronic and marked by remission and fl are-ups. Predilection sites of annular atrophic lichen planus include the trunk and extremities. Similarly, our patient developed lesions at acral sites. Although the disease has been reported to primarily occur in middle-aged individuals, our patient was only 26 at the onset of the disease. He was started on topical corti-costeroids and oral antihistamine therapy but, unfortunately, lost to follow up. We were therefore unable to evaluate the therapeutic response.

Annular atrophic lichen planus is very rare and only few cases have been reported to date. Therefore, characteristic disease features have, as yet, not been clearly defi ned. This case report is intended to highlight the characteristic clinical features of this unusual entity.

Conflict of interest None.