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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 .