October 2003 Derm Case of the Month

UPMC Dermatopathology "Case of the Month" Presentations

UPP - Department of Dermatology, Dermatopathology Unit

5230 Centre Avenue (412) 864-3860 Pittsburgh, PA 15213 (412) 864-3890 FAX

Authors: Sarah Harper MS-IV, and Drazen Jukic M.D., PhD.

June /July 2004 CASE OF THE MONTH

FINDINGS

The pemphigus diseases are a family of rare autoimmune blistering diseases affecting skin and/or mucous membranes. Paraneoplastic Pemphigus (PNP) occurs in association with a malignancy, while Pemphigus Vulgaris (PV) usually does not. This web case presentation compares a case of PV with a case of PNP.

CASE 1:
A 50 year-old white female presents with four-month history of eroded bullae that began in the mouth and face, progressed, and spread to the shoulders, legs, inguinal areas, and groin. She was seen in the emergency department two days prior to admission at an outside hospital (OSH) and was treated with azithromycin. She returned with worsening severely painful lesions, inability to eat secondary to mucous membrane involvement, and was admitted to the OSH with a diagnosis of herpes vs. varicella vs. bacterial infection vs. pemphigus. At this point, she was placed on Solu-Medrol at 50 mg IV q. 12. During the hospitalization at the OSH, she a brief period of improvement followed by a worsening of symptoms with spreading of her lesions on her legs, arms, chest, and back. At this point the patient was transferred to UPMC Montefiore.

On admission, two punch biopsies (lesional and non-lesional skin) were performed. Clinical differential diagnosis of the lesion at time of biopsy included pemphigus vulgaris, pemphigus foliaceus, and paraneoplastic pemphigus.

Dermatopathologic exam revealed following features:
Hematoxylin-eosin (H&E) Staining:
H&E staining of a 5.0x3.0 mm punch biopsy demonstrated acantholysis above the basal layer of epidermis (see Figure 1), which extended into the hair follicles seen in this biopsy (Figure 2) and was even more evident on high power (Figure 3).

Immunofluorescence staining of peri-lesional tissues:
IgG +++ (seen as intercellular deposits in the epidermis – Figure 4, also involving the follicular epithelium)
IgM -
IgA + (seen as intercellular deposits in the epidermis in the bulla only)
C3 ++ (seen as intercellular deposits in the lower 1/2 of the epidermis extending into follicles) – Figure 5
Fibrin + (seen as intercellular deposits in the epidermis in the bulla only)
Saline -

CASE 2:
67 yo white male presented with the initial history of multiple bullous and eroded lesions in mouth, arms, and abdomen; clinical differential at that time included an allergic reaction, bullous pepmhigoid, erythema multiforme and tinea. After the initial workup (see below) additional history was requested. This revealed that he also had a recent 20 lb. weight loss and was found to have a mass in his GI tract along with a pancreatic mass; this prompted additional immunoperoxidase stains to be carried out at paraffin-fixed tissue (see below).

Dermatopathologic exam revealed following features:
Hematoxylin-eosin (H&E) Staining:
H&E staining of a punch biopsy of right forearm demonstrates dermatitis with interface, superficial and deep patterns in the background of marked solar elastosis (Figure 6). Interface dermatitis is particularly apparent in Figure 7 (medium power), and some acanthosis in foci (Figure 8). Civatte (colloid) bodies were seen in foci (see Figure 9).

Immunofluorescence staining of peri-lesional tissues:
IgG + (globular aggregations in the superficial dermis)
IgM + (globular aggregations in the superficial dermis)
IgA + (globular aggregations in the superficial dermis)
C3 + - linear pattern at the dermoepidermal junction (see Figure 10);
Fibrin + - at the dermoepidermal junction; cytoplasmic positivity seen in the epidermis
Saline N/A

Immunoperoxidase stains: Both IgG and IgA reveal distinct intracellular deposits, distributed in so-called fish-net pattern; in addition to this pattern, Civatte bodies reacted with IgG that also revealed strong band at the dermoepidermal junction (the IgG pattern is outlined in Figure 11).

Initially, the differential diagnosis included a lichenoid drug eruption, photo-toxic eruption, an unusual variant of lupus. Immunofluorescence studies were compatible with interface dermatitis pattern but not suggestive of a specific disease entity (see again Figures 6 to 10). As this pattern could also be seen in paraneoplastic pepmhigus, additional clinical history was obtained and immunohistochemical stains were carried out (Figure 11). Despite the fact that the immunofluorescent exam was not definitive, the combination of the immunoperoxidase findings, lichenoid dermatitis on H&E (see Figure 12), and additional clinical history, was diagnostic of paraneoplastic pemphigus.

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