A Split Decision: Unmasking Immunobullous Skin Disease

1Lenox Hill Hospital, New York, NY
2Lenox Hill Hospital, New York, NY
3Lenox Hill Hospital, New York, NY

Meeting: Hospital Medicine 2013, May 16-19, National Harbor, Md.

Abstract number: 261

Case Presentation:

This is a 93‐year‐old woman who presented with a 1‐day history of dyspnea and was subsequently found to have pneumonia. Her medical history was significant for diastolic congestive heart failure, atrial fibrillation, and dementia. The patient had recently been started on furosemide due to worsening dyspnea. A review of systems was positive for a pruritic rash on her chest and abdomen. Skin exam revealed a warm, vesicular rash with an erythematous base along the anterior chest bilaterally and left upper abdomen. There were around 20 vesicles, 2–4 mm in diameter that covered about 5% of the patient's body. Some ruptured vesicles had crusted. The patient denied any skin pain or burning. Examination of her oral cavity revealed white lesions on her tongue and buccal mucosa, initially treated as candidiasis. As her pneumonia resolved, the rash worsened, extending to her back and upper and lower extremities with formation of bullae. Ultimately, these tense bullae ruptured and evolved into areas of skin desquamation. Perioral erythema and desquamation were also present. These symptoms were concerning for drug‐induced Stevens–Johnson syndrome (SJS)/toxic epidermal necrolysis (TEN), prompting discontinuation of furosemide. Pruritus had resolved, but the patient started experiencing intense pain from involved areas of her skin and mouth. Because of the clinical appearance of TEN, with around 30% of her body affected, intravenous immunoglobulins and glucocorticoids were initiated. A punch biopsy was obtained, and direct immunofluorescence showed continuous linear deposits of IgG and C3 along the dermal‐epidermal junction, a common finding in bullous pemphigoid. Salt split technique was used to further differentiate between the subtypes of immunobullous diseases. It revealed a significant pattern of floor immunoreactivity, not supportive of bullous pemphigoid but more consistent with antiepiligrin pemphigoid and epidermolysis bullosa acquisita. Intravenous glucocorticoids were continued and the patient's skin lesions slowly improved with complete re‐epithelialization.

Discussion:

Immunobullous diseases are autoimmune blistering disorders mainly affecting the skin. Clinically distinguishing between their subtypes can be challenging due to minor variations in their clinical presentations. The rarer subtypes that include mucosal involvement and desquamation can be mistaken for findings in the SJS/TEN spectrum.

Conclusions:

By reporting this case, we hope to shed light on a rare presentation of immunobullous skin disease presenting as SJS/TEN because of rapidly evolving cutaneous and oral involvement. This case also emphasizes the utility of the salt split technique in differentiating bullous pemphigoid disease from other immunobullous disease subtypes.

Skin desquamation of hand.

To cite this abstract:

Heivand N, Wong J, Dungarani T. A Split Decision: Unmasking Immunobullous Skin Disease. Abstract published at Hospital Medicine 2013, May 16-19, National Harbor, Md. Abstract 261. Journal of Hospital Medicine. 2013; 8 (suppl 2). https://www.shmabstracts.com/abstract/a-split-decision-unmasking-immunobullous-skin-disease/. Accessed November 22, 2019.

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