DOI: http://dx.doi.org/10.18203/issn.2455-4529.IntJResDermatol20203533

A middle-aged male with bullous lesions and co-morbidities

Lavina Mittal, Suparna Madivalara Yallappa, Praveen Kumar Shanmugam Reddy

Abstract


Epidermolysis bullosa acquisita (EBA) is a rare acquired type of mechano bullous disease affecting the dermal-epidermal junction (DEJ) of trauma prone acral surfaces. It manifests as tense vesicles, bullae, and milia and typically heals as atrophic hypo or hyperpigmented scars. Classic noninflammatory mechano bullous EBA typically presents at a mean age of 48 years. A 57 years old male patient, presented with itchy fluid filled lesions over the face since, 2 months. On cutaneous examination, discrete and grouped papulo-vesicles on an erythematous base and areas of erosions present over both eyelids, erythematous annular plaque with vesicles at the border present over the left cheek. Blood investigations revealed an increased total blood count, total bilirubin, direct bilirubin, and positive HCV antibodies. Histopathology showed a sub-epidermal blister and surrounding dermis shows inflammatory infiltrate composed of eosinophils and neutrophils. Direct immunofluorescence revealed linear deposits of IgG and C3 at the DEJ. The patient was started on dapsone.


Keywords


Epidermolysis bullosa acquisita, Blistering disorders, DEJ

Full Text:

PDF

References


Cyr J, Liu A, Ghazarian D, Siddha S. Case Report of a 21 years old man with epidermolysis bullosa acquisita. J Cutaneous Med Surg. 2018;22(3):356-8.

Das JK, Sengupta S, Gangopadhyay AK. Epidermolysis bullosa acquisita. Indian J Dermatol Venereol Leprol. 2006;72:86.

Woodley DT, Chen M. Epidermolysis Bullosa Acquisita. Wollf K, Goldsmith LA, Katz S I, Gilchrest B A, Paller A S, Leffell D J. Fitzpatrick’s Dermatology in General Medicine. 7th ed. Mc Graw Hill Publishers; 2008: 494-499.

Wojnarowska F, Venning VA. Immunobullous diseases. Burns T, Breathnach S, Cox N, Griffiths C. Rook’s textbook of Dermatology. 8th ed. Wiley-Blackwell publishers; 2010: 40.51-40.56.

Chan LS, Woodley DT. Pemphigoid: bullous and cicatricial. In: Lichtenstein LM, Fauci AS, eds. Current Therapy in Allergy, Immunology and Rhematology. St Louis, MO: Elsevier; 1996: 5-93.

Mellot CC, Bodemer C, Caux F. Epidermolysis bullosa acquisita in childhood. Arch Dermatol. 1997;133(9):1122-6.

Lehman JS, Camilleri MJ, Gibson LE. Epidermolysis bullosa acquisita: concise review and practical considerations. Int J Dermatol. 2009;48:227-35.

Gurcan HM, Ahmed AR. Current concepts in the treatment of epidermolysis bullosa acquisita. Expert Opin Pharmacother. 2011;12:1259-68.

Bevans SL, Sami N. The use of rituximab in treatment of epidermolysis bullosa acquisita: three new cases and a review of the literature. Dermatol Ther. 2018;31:12726.