140
SHORT COMMUNICATION
ActaDV ActaDV
Advances in dermatology and venereology Acta Dermato-Venereologica
Light Chain Deposition Disease with Bullous Skin Lesions Mimicking Atypical Bullous Pemphigoid
Marion MALPHETTES 1, Pauline BONNET 1, Pierre SCHNEIDER 2, Marguerite VIGNON 1, Marine BARON 1, Jean-David BOUAZIZ 2 and Maxime BATTISTELLA 3 Departments of 1 Immunology, 2 Dermatology, and 3 Pathology, Hôpital Saint-Louis, Assistance Publique-Hôpitaux, INSERM UMRS _ 1165, Université Paris Diderot, 1 avenue Claude Vellefaux, FR-75010 Paris, France. E-mail: marion. malphettes @ aphp. fr Accepted Aug 23, 2017; Epub ahead of print Aug 23, 2017
Some dermatological entities are strongly associated with the presence of monoclonal gammopathy( MG) and should be referred to as monoclonal gammopathy of cutaneous significance( MGCS), as proposed recently( 1). Acquired bullous dermatosis is a rare complication in this setting. Most reported cases of bullous dermatosis complicating MG are related to systemic light chain( AL) amyloidis( 2). We report here an acquired bullous dermatosis mimicking bullous pemphigoid( BP) presenting as the first manifestation of systemic light chain deposition disease( LCDD).
CASE REPORT
A 77-year-old man was referred to our institution in 2012 for a pruriginous erythematous bullous eruption of the trunk and scalp( Fig. 1). He had multiple, tense, irregular-shaped bullae, either on erythematous skin or in place of recent scars, together with excoriations. There was no Nikolsky’ s sign, and no mucosal involvement. Skin biopsy showed a subepidermal blister associated with an infiltrate of neutrophils and eosinophils, consistent with autoimmune bullous dermatosis. Direct immunofluorescence( DIF) with anti-IgG heavy chain, anti-IgA heavy chain, anti-IgM heavy chain and anti-C3 antibodies was negative. Indirect immuno-fluorescence( IF) on salt-split skin was negative. Serum anti-BP 230 antibodies were slightly positive, while anti-BP180 and anti-desmoglein 1 and 3 antibodies were negative. A diagnosis of atypical BP was suggested. Topical corticosteroids were started and were initially effective, but the cutaneous disease subsequently evolved in a relapsing / remitting manner.
In 2014, flare-up occurred when attempts were made to taper the dosage of topical corticosteroids. A new skin biopsy revealed a subepidermal blister associated with a predominantly lymphocytic moderate infiltrate and rare neutrophils or eosinophils( Fig. S1 1).
Fig. 1. Left thigh, showing tense, irregular-shaped bullae on erythematous skin together with excoriations.
At this time, skin DIF showed no IgG, IgA or IgM heavy chains, nor C3 deposits. Immunoelectrophoresis performed in November 2014 demonstrated a monoclonal IgA lambda paraprotein, and the patient was referred to the haematology outpatient clinic. At the clinic in February 2015, he reported gradual onset of dyspnoea and lower limb oedema, progressing for 3 months. Physical examination revealed symmetrical peripheral oedemas of the inferior limbs. There was no jugular venous distension or lung crackles. Skin examination revealed numerous haemorrhagic tense blisters spread over the trunk and limbs. The rest of the systemic examination was unremarkable.
Laboratory tests revealed a markedly elevated concentration of urine protein( 12 g / 24 h), consisting largely of albumin, with traces of lambda free light chains( FLC). Serum albumin level was 28.9 g / l. Limb oedema was attributed to nephrotic syndrome. Other laboratory tests revealed kidney function to be within the normal range. Immunofixation confirmed the presence of a monoclonal paraprotein IgA-lambda in the serum( 0.7 g / l). Serum lambda FLC were increased, at 1,215 mg / l( normal 5.7 – 26.3 mg / l), while kappa FLC were normal at 11.4 mg / l( normal 3.3 – 19.4 mg / l). Bone mar-
1 https:// www. medicaljournals. se / acta / content / abstract / 10.2340 / 00015555-2775
Fig. 2. Direct immunofluorescence.( a) Abundant granular lambda light chain deposits at the dermoepidermal junction and in the papillary dermis, with( b) no kappa light chain deposits(× 400 magnification). doi: 10.2340 / 00015555-2775 Acta Derm Venereol 2018; 98: 140 – 141
This is an open access article under the CC BY-NC license. www. medicaljournals. se / acta Journal Compilation © 2018 Acta Dermato-Venereologica.