Coexistence of lichen planus pigmentosus and minimal change nephrotic syndrome

ARTICLE

Auteur(s) : Giuseppe Mancuso, Renza Maria Berdondini

Department of Dermatology, Municipal Hospital of Lugo (RA), Italy

We describe a patient with lichen planus pigmentosus (LPP) [1-3], a relatively rare variant of lichen planus, associated with minimal change nephrotic syndrome (MCNS) [4-6] and discuss the relationship between these two immunologically mediated diseases.

In January 2006, a 30-year-old Italian Caucasian woman with histologically confirmed MCNS, which had arisen about two months earlier, was referred to our attention for evaluation of asymptomatic brown macules localized exclusively on both axillae and the groin (figure 1). The skin lesions had appeared at the same time as the nephrotic syndrome. Further cutaneous examination was otherwise unremarkable, except for pretibial edema. In particular, oral, genital mucosa, and nails were unaffected. There was no causal relationship of either disease with the use of drugs. Moreover, no association was found with lymphoid or other malignancies with regard to her personal history, basic physical examination or 3-year clinical follow-up. A chest X-ray and an ultrasound study of the abdomen, lymph nodes and thyroid gland were normal. Urinalysis revealed proteinuria (3.2 g/day). Total serum protein, albumin and total cholesterol were 4.5 g/dL, 2.1 g/dL and 380 mg/dL, respectively, and blood urea nitrogen and creatinine were 12 and 0.4 mg/dL respectively. A hepatitis serology profile was negative. A skin biopsy specimen from the right axilla showed histopathological features consistent with LPP (figure 2). On the basis of the intertriginous location, we diagnosed the patient as having LPP-inversus [2, 3]. The patient was treated with prednisolone 60 mg/day for six weeks, followed by 40 mg/48 h for the next six weeks with complete remission of the proteinuria. Treatment with prednisolone was tapered and then discontinued the following month. The skin lesions were treated with a topical corticosteroid (betamethasone dipropionate 0.05% cream) for around two weeks and all local therapy was then discontinued. A dermatological check-up, about two months later, showed that the lesions had healed with patchy hyperpigmentation. In February 2007, about eight months after discontinuation of the systemic steroid treatment, the patient suffered a relapse of the nephrotic syndrome, followed by a flare of the lichenoid lesions and the appearance of new pigmented lichenoid lesions on the forearms, legs and buttocks, with itching. The relapse of MCNS was successfully treated with prednisolone according to the therapeutic protocol previously used. Topical treatment of the skin lesions, first for about 2 weeks with a topical corticosteroid (betamethasone dipropionate 0.05% cream) and then for the following month with tacrolimus 0.1% ointment (twice daily), led to complete resolution of the lesions and disappearance of the itching. Eleven months after the nephrotic relapse, the patient has residual post-inflammatory hyperpigmentation and proteinuria values within the normal ranges.

Immunological mechanisms are almost certainly involved in lichen planus, as shown by the dermal infiltrate of T lymphocytes, the occurrence of lichenoid eruptions concomitant with chronic graft-versus-host disease and the association with diseases of altered or disturbed immunity [1]. Immune cell disorders appear to play a role in the pathogenesis of steroid sensitive MCNS [4, 5]. The occurrence of MCNS with hemopathies, particularly those of lymphoid origin or involving lymphoid cells, supports the role of T cells in the pathogenesis of proteinuria, although the characteristics of those T cells have still to be established and the glomerular permeability factor(s) identified [5]. MCNS has also been reported as a late complication of graft-versus-host disease after hematopoietic stem cell transplantation [6].

Given the contemporary appearance of the two diseases and the flare of the LPP immediately after the nephrotic relapse, we think that our case substantiates the possibility of common immunological abnormalities, based on an altered cell-mediated immune response. To our knowledge, this is the first reported case of LPP that developed in a patient with MCNS.

Acknowledgement

References

2 Pock L, Jelinková L, Drlik L, Abrhámová S, Vojtechovská S, Sezemská D, Borodácová I, Hercogová J. Lichen planus pigmentosus -inversus. J Eur Acad Dermatol Venereol 2001; 15: 452-4.

3 Kashima A, Tajiri A, Yamashita A, Asada Y, Setoyama M. Two Japanese cases of lichen planus pigmentosus-inversus. Int J Dermatol 2007; 46: 740-2.

4 Audard V, Lang P, Sahali D. Pathogénie du syndrome néphrotique à lésions glomérulaires minimes. Med Sci 2008; 24: 853-8.

5 Ronco P, Debiec H. Pathophysiological lessons from rare associations of immunological disorders. Pediatr Nephrol 2008 Oct 14 (epub ahead of print).

6 Colombo AA, Rusconi C, Esposito C, Bernasconi P, Caldera D, Lazzarino M, Alessandrino EP. Nephrotic sindrome after allogeneic hematopoietic stem cell transplantation as a late complication of chronic graft-versus-host disease. Transplantation 2006; 81: 1087-92.