|
Indian Pediatr 2012;49: 4 21 |
|
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.
|
|
|
|