A 48-year-old diabetic man presents to the emergency department complaining of blistering of the skin. One week prior, he was seen by his cardiologist, where he was started on a new blood pressure medication. His major complaints are blisters on his arms, chest, and face and pruritus. On physical examination, he is afebrile. The “blisters” are actually shallow erosions associated with erythema, scale, and crust formation, Nikolsky’s sign is present. There is no involvement of the scalp or any mucous membranes. A skin biopsy reveals IgG on the surface of keratinocytes, and there is intraepidermal vesicle formation. Much of the destruction is just beneath the stratum corneum. Basal keratinocytes, and the epidermal basement membrane are spared. Anti-Dsg1 and anti-IgG antibodies are found in the peripheral blood. Which of these statements is true regarding the disease this patient has? (AIIMS May 2012)
|A||An endemic form of this disease is found in Latin America and Tunisia.|
|B||This condition is likely to require plasmapheresis.|
|C||There is high likelihood that this patient has an occult Hodgkin’s lymphoma.|
|D||This patient also probably has a gluten-sensitive enteropathy.|
There is high likelihood that this patient has an occult Hodgkin’s lymphoma
1). This patient has pemphigus foliaceus (PF), an autoimmune cutaneous disorder similar to pemphigus vulgaris (PV).
2). PF is distinguished from PV by the fact that PF is a more superficial disorder than PV.
3). The acantholytic blisters in PF are located high in the epidermis, usually just below the stratum corneum.
4). In PF, mucous membranes are always spared. Fogo selvagem is the endemic form of PF.
5). PF is associated with several autoimmune diseases; however, its association with thymoma and/or myasthenia gravis is most notable.
6). Patients can develop PF after exposure to drugs containing a thiol group (e.g., penicillamine, captopril, enalapril).
7). PF usually responds to topical or systemic glucocorticoids.
8). PV is often associated with lymphoreticular malignant diseases such as lymphoma or Hodgkin’s disease.