Solitary hypertrophic lupus erythematosus of the nose without other cutaneous or systemic involvement
Article first published online: 11 DEC 2012
© 2013 The International Society of Dermatology
International Journal of Dermatology
Volume 53, Issue 1, pages e18–e20, January 2014
How to Cite
Bettoli, V., Minghetti, S., Ferron, P., Borghi, A. and Virgili, A. (2014), Solitary hypertrophic lupus erythematosus of the nose without other cutaneous or systemic involvement. International Journal of Dermatology, 53: e18–e20. doi: 10.1111/j.1365-4632.2011.05349.x
- Issue published online: 18 DEC 2013
- Article first published online: 11 DEC 2012
An otherwise healthy 36-year-old woman presented with a hyperkeratotic plaque over the dorsal surface of her nose. Physical examination revealed multiple verrucous, scaly, and somewhat pearly papules and nodules, together with atrophic and crateriform erythematous areas of scarring (Fig. 1a). The lesions were reported to have been present for about two months and to have progressively enlarged. No further skin, mucosal, or adnexal manifestations were observed. No previous cutaneous diseases or systemic symptoms were reported. The patient had been previously treated with daily topical mometasone furoate 0.1% cream for four weeks without improvement. Laboratory investigations including antinuclear antibody titer and complement level were normal or negative.
Histopathologic examination of a skin biopsy specimen from the dorsal surface of the nose demonstrated a warty, hyperkeratotic lesion, with pseudo-invasive epidermal hyperplasia, mild spongiosis, and a few dyskeratotic keratinocytes (Fig. 2a). Dermal mononuclear lichenoid infiltrate, with heavy involvement of the dermoepidermal junction, was apparent. Alcian blue stain demonstrated mucin deposition (Fig. 2b). Periodic acid-Schiff stain showed the basal membrane to be absent and indicated pronounced hydropic degeneration of the basal cells (Fig. 2c). Orcein stain failed to show transepithelial elimination of the elastic fibers. The clinical and histopathologic features were consistent with a diagnosis of hypertrophic lupus erythematosus (HLE).
Because of the lack of both laboratory abnormalities and clinical manifestations, and the consequent exclusion of systemic lupus erythematosus (SLE), the limited extension of the skin lesion, and the demonstrated efficacy of retinoids in chronic cutaneous lupus erythematosus (CCLE), the patient was treated with daily topical adapalene 0.1% cream, which is associated with sunscreen. Adapalene was well tolerated, and remarkable improvement was seen within four weeks, when the erythema, hyperkeratosis, and thickness were dramatically decreased. Two months later, only depressed scars and no signs of active disease were visible (Fig. 1b). The patient underwent maintenance therapy with adapalene 0.1% cream applied once per day for a further six months without relapse. One year after the discontinuation of treatment, the patient continues to use sunscreen and is still in remission.
First described by Bechet1 in 1940, HLE is a distinct and very uncommon subtype of CCLE. Occurring in approximately 2% of patients with CCLE,2–4 HLE is clinically characterized by dull, hard, erythematous, verrucous, and usually asymptomatic plaques and nodules with hyperkeratotic scales, mainly affecting photoexposed areas.2,3
It frequently occurs together with pre- or coexisting typical discoid lupus erythematosus (DLE), and thus the presence of DLE is a helpful clue to the diagnosis of HLE.2,5,6 It is uncommon in the context of SLE, and patients rarely have positive serologic tests.2,4 Two histologic variants of HLE have been described as lichen planus-like and keratoacanthoma-like patterns.2,3 The distinction of HLE from keratoacanthoma, hypertrophic lichen planus, and squamous cell carcinoma (SCC) may represent a challenge for clinicians and pathologists as these conditions share several morphologic and histologic characteristics and because SCC may arise in HLE. However, in our case, the absence of atypical squamous cells, atypical mitotic figures, and invading cell masses, as well as the finding of a band-like mononuclear infiltrate along the dermoepidermal junction, represented useful histologic clues with which to differentiate HLE from SCC. This was important in order to avoid the instigation of radical therapy.
Therapeutic options include retinoids, intralesional and topical steroids, antimalarials, surgical excision, and thalidomide, associated with sun avoidance.2–4 Retinoids, such as acitretin, etretinate, and systemic and topical tretinoin isotretinoin, have shown efficacy in HLE, even when it is recalcitrant to conventional therapy.2–9
The present case shows that HLE is difficult to diagnose because it is an unusual disease that closely resembles other skin conditions, particularly when it does not occur in a setting of typical CCLE, as in our patient. To our knowledge, only one case of “solitary” HLE has been reported in the literature,3 and thus the present patient represents the first case of HLE to be successfully treated with adapalene.
Furthermore, our report suggests that when HLE involving small surfaces requires a topical approach, a short course of adapalene may represent an efficacious and safe therapy. However, our experience indicates that, as HLE is a chronic disorder, the use of a topical retinoid as a maintenance therapy for six months is advisable.
- 1Hypertrophic lupus erythematosus. Arch Derm Syphilol1940; 42: 211–213..