Bullous lichen sclerosus et atrophicus of the penis

ARTICLE

Auteur(s) : Mariana V Hoffner¹, Antonio Rodríguez-Pichardo¹, Begoña García-Bravo¹, Juan José Ríos Martín², Francisco M Camacho¹

¹Department of Dermatology, Virgen Macarena University Hospital, Av. Dr. Fedriani s/n 41009 Seville, Spain
²Department of Pathology, Virgen Macarena University Hospital, Seville, Spain

Lichen sclerosus et atrophicus (LSA) is a rare, chronic, mucocutaneous disease of unknown aetiology, commonly affecting the anogenital area in postmenopausal women, first described by Hallopeau in 1887. Clinically it presents with ivory-white papulous elements, causing intense pruritus and soreness, with an increased risk of malignant transformation [1]. Bullous LSA is an unusual variant of the disease, with haemorrhagic bullae in genital and/or extragenital areas [2].

We report a 59-year-old man operated for urethral stenosis. He was subject to periodic urethral stretchings that worsened the genital lesions, so his urologist sent him for a dermatological consultation. On physical examination the patient had numerous, tense, haemorrhagic, pruritic bullae, vesicles and crusts on the prepuce and glans, surrounded by porcelain-white atrophic changes, present for the last year, with no lesions on other sites (figures 1A and B). A biopsy, with a negative immunofluorescence study, confirmed the diagnosis of bullous LSA (figures 1C and D). Treatment with clobetasol dipropionate cream 0.05% twice daily for three weeks, was partially effective.

The exact prevalence of the bullous variant of LSA is uncertain [2]. Several cases of extragenital bullous LSA have been described, on single locations as well as generalized affections including the genital area. Cases of genital or perineal lesions in female patients were described by Ristic et al. [2], however, exclusively genital lesions in men, as in our case, have not been described to our knowledge.

The underlying cause of LSA remains unknown and many factors may be involved, such as a genetic susceptibility, low sex hormone output and autoimmunity with type II diabetes mellitus [1]. However, the laboratory studies carried out in our patient (complete blood count, biochemical parameters including basal glycemia, and coagulation studies) were normal. Koebner phenomenon has also been associated with LSA, with lesions occurring in previously injured skin, such as after surgical procedures, severe sunburn, thermal burn, or vaccination, which probably explains why our patient worsened after the urologist’s genital manipulations during the periodic stretchings of the uretha [3]. Histologically, both bullous LSA and LSA arising on a scar show typical features of LSA (a hyperkeratotic and thinned epidermis with vacuolar degeneration in the basal layer, telangiectases, homogenization of the collagen in the papillary dermis and a perivascular and interstitial lymphohistiocytic infiltrate), but the former shows as sub-epidermal blisters caused by extensive vacuolar degeneration of the epidermal basal layer, as in our case, while the latter shows findings consistent with a scar in the underlying mid and deep dermis. However, Allan et al. [4] suggest that these histological findings may represent “LSA-like changes occurring within a long-standing scar” rather than true “LSA”.

Other atypical forms of LEA include blaschkoid zosteriform linear lichen sclerosus et atrophicus [5]. The differential diagnosis of bullous LSA may include cicatricial or localized bullous pemphigoid, bullous morphea, bullous lichen planus, circumscribed lymphangioma and leukoderma [2].

There is no definitive treatment for LSA, although numerous medical therapeutic modalities have been used, including topical corticosteroids, estrogen or testosterone ointments, topical retinoids, 0.1% tacrolimus ointment and UVA-phototherapy, alone or combined [1-6]. The treatment of choice for anogenital LSA seems to be topical application of a potent corticosteroid for a limited period of time. Surgical therapy is indicated when the lesions present signs of malignant transformation or if there is failure of medical treatment. The main surgical procedures are vulvectomy, circumcision, cryosurgery and laser ablation [2].

Bullous LSA is a rare chronic disease that, to our knowledge, has not been described previously as a unique localization in male genitalia. We believe it is important to have this diagnosis in mind when seeing a male patient with a bullous dermatosis in the genital area.

Acknowledgments

References

2 Ristic B, Divic J, Belic LJ, Zdelar D. Bullous lichen sclerosus atrophicus. J Eur Acad Dermatol Venereol 2004; 18: 108-9.

3 Weiss G, Shemer A, Trau H. The Koebner phenomenon: review of the literature. J Eur Acad Dermatol Venereol 2002; 16: 241-8.

4 Allan A, Andersen W, Rosenbaum M, Bhawan J. Histologic features of lichen sclerosus et atrophicus in a surgical scar. Am J Dermatopathol 1999; 21: 387-91.

5 Cabanillas González M, Monteagudo B, de las Heras C, Cacharrón JM. Blaschkoid, zosteriform linear lichen sclerosus et atrophicus. Actas Dermosifiliogr 2009; 100: 155-7.

6 Valdivielso-Ramos M, Bueno C, Hernanz JM. Significant improvement in extensive lichen sclerosus with tacrolimus ointment and PUVA. Am J Clin Dermatol 2008; 9: 175-9.