Ann Dermatol.  2011 Dec;23(Suppl 3):S390-S392. 10.5021/ad.2011.23.S3.S390.

Bullous Pemphigoid in an Elderly Patient with Myelodysplastic Syndrome and Refractory Anemia Coupled with Excess of Blast

• Lee YY
• Bee PC
• Lee CK
• Naiker M
• Ismail R

• KMID: 2171880
• DOI: http://doi.org/10.5021/ad.2011.23.S3.S390

Abstract

Bullous pemphigoid (BP) has a recognized association with solid organ tumors, but is relatively rare in hematological malignancies. We report a 67-year-old male who developed BP after being diagnosed with myelodysplastic syndrome and refractory anemia with excess of blast (RAEB). Skin biopsy elucidated sub-epidermal bulla using direct immunofluorescence, revealing linear C3 and IgG deposits along the basement membrane. His BP was recalcitrant to the conventional treatment and only responded to a combination of high dose oral prednisolone and azathioprine. The relative refractory nature of his condition and concurrent RAEB supports a paraneoplastic nature.

Keyword

Anemia; refractory; with excess blast; Myelodysplastic syndromes; Paraneoplastic syndromes; Pemphigoid; bullous

MeSH Terms

Aged
Anemia
Anemia, Refractory
Anemia, Refractory, with Excess of Blasts
Azathioprine
Basement Membrane
Biopsy
Blister
Fluorescent Antibody Technique, Direct
Hematologic Neoplasms
Humans
Immunoglobulin G
Male
Myelodysplastic Syndromes
Paraneoplastic Syndromes
Pemphigoid, Bullous
Prednisolone
Skin
Azathioprine
Immunoglobulin G
Prednisolone

Figure

• 

  Fig. 1 Multiple hemorrhagic tense bullae on erythematous and normal skin with scattered dried erosions (hand and foot).

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  Fig. 2 Presence of subepidermal bullae with surrounding perivascular and periadnexal lymphocytic inflammation (H&E, ×4).

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