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correspondence

Indian Pediatr 2012;49: 421

Lichen Planus and Nephrotic Syndrome-Coincidence or Causation?


Sriram Krishnamurthy and Sadagopan Srinivasan

Department of Pediatrics, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER),
Pondicherry 605 006, India.
Email: [email protected]



We describe an 8 year old boy who presented with lichen planus (LP) and minimal change nephrotic syndrome (MCNS), and discuss the possible pathogenetic links between the two disorders. A 7 year old boy presented with anasarca for 7 days in association with skin lesions over both the lower limbs. The skin lesions were bilaterally symmetrical violaceous polygonal pruritic papules present over both lower limbs, diagnostic of classical LP; and appeared simultaneously along with periorbital edema progressing to anasarca. The oral, genital mucosa and nails were unaffected. There was no causal relationship of either the skin lesions or anasarca with any drug usage or immunization. There was no jaundice, hepatosplenomegaly, lymphadenopathy or joint involvement. Urinalysis showed proteinuria (urine spot protein: creatinine ratio 3.4). Serum albumin and cholesterol were 1.8 g/dL and 340 mg/dL respectively. Serum creatinine was 0.5 mg/dL. Hepatitis serology profile, chest x-ray and complete blood counts were normal. Anti-nuclear antibody (ANA) were negative and C3 levels were normal. The renal biopsy showed minimal change disease. He was treated with prednisolone as per standard guidelines [1], and went into remission. The skin lesions were treated with 0.05% betamethasone dipropionate cream for 2 weeks and healed with patchy hyperpigmentation. Seven months later, he had a relapse of nephrotic syndrome coincident with flare of the lichenoid lesions over the same sites. He was again treated with systemic and topical steroids. Proteinuria is currently settled. Itching has subsided, however residual postinflammatory hyperpigmentation is present.

LP is a chronic inflammatory dermatological condition usually affecting adults, but rare in children [2]. The diagnosis is essentially clinical [2]. Immunological mechanisms mediate the pathogenesis of LP, as evidenced by dermal infiltrate of T lymphocytes and association with diseases of altered immunity such as vitiligo [3]. An association with hepatitis C is mentioned [2].The immune system including T cells have an important role in the pathogenesis of steroid sensitive MCNS too [4], although the mechanisms are not fully understood [4]. On literature search, only a single similar case of MCNS with LP in a 30 year old Italian woman was found [5].

Considering the temporal association of the two diseases and the flare of the LP coincident with the nephrotic relapse, we believe this may not be a mere coincidence. To our knowledge, this is the first pediatric case of LP in a patient with MCNS, and may reflect common immunological abnormalities, based on altered cell mediated immunity.

References

1. Indian Pediatric Nephrology Group, Indian Academy of Pediatrics, Bagga A, Ali U, Banerjee S, Kanitkar M, Phadke KD, Senguttuvan P, et al. Management of steroid sensitive nephrotic syndrome: revised guidelines. Indian Pediatr. 2008;45:203-14.

2. Kanwar AJ, De D. Lichen planus in children. Indian J Dermatol Venereol Leprol. 2010;76:366-72.

3. Breathnach SM, Black MM. Lichen planus and lichenoid disorders. In: Burns T, Breatnach SM, Cox N, Griffiths C, (eds). Roook’s textbook of Dermatology, 7th ed. Oxford: Blackwell Publishing;2004.p.1-32.

4. Ronco P, Debiec H. Pathophysiological lessons from rare associations of immunological disorders. Pediatr Nephrol. 2009;24:3-8.

5. Mancuso G, Berdondini RM. Coexistence of lichen planus pigmentosus and minimal change nephrotic syndrome. Eur J Dermatol. 2009;19:389-90.

 

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