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Pemphigus foliaceus (PF) is a rare autoimmune blistering disease that can be difficult to diagnose and treat. The disease can be endemic or sporadic with a number of different instigating factors. We present a case of a 73-year-old male who developed PF shortly after treatment with 5-fluoruracil for actinic keratosis and with a distant history of ionizing radiation treatment. The patient proved difficult to properly treat due to his transitory and resistant nature. A short review of PF is also provided.

Introduction

Case Report

Pemphigus foliaceus (PF) is a member of the chronic blistering pemphigus family that includes other major forms: pemphigus vulgaris (PV) and paraneoplastic pemphigus.1 PF is a rare disease with low prevalence and incidence worldwide.2 PF can be endemic or sporadic. The endemic form, known as fogo selvagem, is found in most commonly in regions of Brazil but can be found in a number of different locations worldwide. Fogo selvagem is believed to be caused by environmental factors. It presents in genetically susceptible children and young adults who are exposed to the bite of black flies, which are a possible vector that precipitates the disease. 1,2 Fogo selvagem presents clinically the same as the sporadic form of PF. While endemic PF presents in children and young adults, sporadic PF typically presents in adults between ages 40 and 60 years of age.2 Both endemic and sporadic PF tend to present equally in men and women.1,2

Pemphigus can be induced by medications, especially thiol-containing drugs such as penicillamine and captopril.2 Our patient reported his first initial flare of PF after using 5-fluorouracil. The patient also reported a history of ionizing radiation for laryngeal cancer. 5-fluorouracil has been implicated as a possible eliciting factor of PF by at least one author but is otherwise undocumented.3 Ionizing radiation and UV exposure have also been documented as possible instigators of pemphigus.2,4,5

PF is an autoimmune condition in which IgG autoantibodies target desmoglein 1 (Dsg1).6,7 The binding of autoantibodies to Dsg1 leads to loss of intercellular connections known as acantholysis.2 Dsg1 is found in greater abundance towards the upper levels of the epidermis, and in lower levels of the epidermis, co-expression of Dsg3 is found.7,8 For this reason, PF tends to develop more superficial bullae, especially compared to PV, which is predominately caused by Dsg3-directed autoantibodies.7,8

PF presents clinically with shallow cutaneous erosions often with overlying scaly crust. Unlike PV, patients do not have mucosal involvement. The lesions are most often found in a seborrheic distribution. The bullae associated with PF are very fragile, often never seen by the provider intact. The Nikolsky sign is commonly positive. Generally, patients are not severely ill but can experience pain and/or burning. PF can rarely be severe, becoming an exfoliative erythroderma. Also occurring rarely, PF can transition to PV. 1,2,9,10

Pemphigus erythematosus (PE, Senear-Usher syndrome) is a localized variant of PF that presents primarily on the malar face and sun-exposed areas. PE is described as having features of both lupus and pemphigus, with some rare patients having positive anti-nuclear antibodies.10 However, pemphigus erythematosus patients have rarely been reported to have both diseases congruently. PE presents identically to PF histologically. 1,2

The histopathology of PF consists primarily of acantholysis of the upper epidermis in the granular layer. Often the stratum corneum is missing due to the fragility of the bullae, but in early lesions can be split from the underlying epidermis.6,11 The location of acantholysis and separation in the epidermis is helpful in distinguishing from PV. PV usually will develop separation lower in the epidermis. 6,11 Direct immunofluorescence (DIF) of perilesional skin shows IgG and C3 intercellular deposition that is more prominent in upper levels of the epidermis. Pemphigus erythematosus differs from PF with DIF showing intercellular deposition of IgG and C3 as well as at the basement membrane. 10 Indirect immunofluorescence with human skin or guinea pig esophagus substrate shows anti-Dsg1 autoantibodies. 1,11 ELISA is sensitive and specific for anti-Dsg1 without requiring skin biopsy.8 DIF, IIF and ELISA help distinguish PF from other forms of pemphigus, bullous impetigo, subcorneal pustular dermatosis, subacute cutaneous lupus, and seborrheic dermatitis.1 ELISA can also serve as a useful tool monitoring disease response to treatment and to help predict flares.8

Treatment for PF can be similar to PV when the disease is widespread. In cases of widespread disease, systemic corticosteroids remain first line, usually with prednisone at 1 mg/kg/day as the initial dose.12 Tapering the dose is based on clinical improvement as well as monitoring circulating anti-Dsg1 levels. Azathioprine, mycophenolate mofetil, dapsone, and cyclophosphamide as adjuvant therapy with systemic corticosteroids or on their own as steroid-sparing options have shown to reduce lesions and aid in remission. 13-16 Rituximab has proven as an increasingly effective adjuvant therapy to pemphigus. 17 Tetracycline with niacinamide has emerged as a useful corticosteroid-sparing medication, showing 90% efficacy in new flares and 60% control with occasional treatment with topical and/or oral corticosteroids. 18 In localized disease, control can be obtained with super-potent topical corticosteroids.1

Conclusion

Pemphigus foliaceus is a rare disease that can be difficult to diagnose and treat. Our patient would have benefited by additional studies to confirm the diagnosis of PF and to monitor treatment response based on circulating anti-Dsg1 levels such as found with ELISA. It is also important to recognize the possible but uncommon eliciting factors of 5-fluorouracil and radiotherapy. Due to our patient’s refusal for adjuvant immunosuppressive therapy, we will consider treatment with tetracyclines and niacinamide if we are able to regain contact.

References

1. Amagai M. Pemphigus. In: Bolognia JL, Jorizzo JL, Rapini RP, eds. Dermatology. Vol 1. Philadelphia, PA: Elsevier; 2018:494-509.2. James KA, Culton DA, Diaz, LA. Diagnosis and clinical features of pemphigus foliaceus. Dermatol Clin. 2011;29(3): 405–412. doi:10.1016/j.det.2011.03.012.3. Callander J, Ponnambath N. Pemphigus foliaceus induced by topical 5-fluorouracil. Clin Exp Dermatol. 2016;41:443-444. 4. Walker A, Favreau T. Localized pemphigus foliaceus. Cutis. 2017;99:E23-26. 5. Cianchini G, Lembo L, Colonna L, et al. Pemphigus foliaceus induced by radiotherapy and responsive to dapsone. J Dermatolog Treat. 2006;17:244-246. doi: 10.1080/09546630600921197.6. Mahoney MG, Wang Z, Rothenberger K, et al. Explanations for the clinical and microscopic localization of lesions in pemphigus foliaceus and vulgaris. J Clin Invest. 1991;103:461-468.7. Waschke J. The desmosome and pemphigus. Histochem Cell Biol. 2008;130:21-54. doi: 10.1007/s00418-008-0420-0.8. Stanley JR, Amagai M. Pemphigus, bullous impetigo, and the staphylococcal scalded-skin syndrome. N Engl J Med. 2006;355:1800-1810.9. Mihai S, Sitaru C. Immunopathology and molecular diagnosis of autoimmune bullous diseases. J Cell Mol Med. 2007;11(3):462-481. doi:10.1111/j.1582-4934.2007.00033.x. 10. Khachemoune A, Guldbakke KK, Ehrsam E. Pemphigus foliaceus: A case report and short review. Cutis. 2006;78:105-110.11. Ohata C, Ishii N, Furumura M. Locations of acantholysis in pemphigus vulgaris and pemphigus foliaceus. J Cutan Pathol. 2014;41:880-889. doi: 10.1111/cup.12384.12. Atzmony L, Hodak E, Gdalevich M, et al. Treatment of pemphigus vulgaris and pemphigus foliaceus: A systematic review and meta-analysis. Am J Clin Dermatol. 2014;15:503-515. doi: 10.1007/s40257-014-0101-9.13. Martin LK, Werth VP, Villanueva EV, et al. A systematic review of randomized controlled trials for pemphigus vulgaris and pemphigus foliaceus. J Am Acad Dermatol. 2011;65(11):903-908.14. Singh S. Evidence-based treatments for pemphigus vulgaris, pemphigus foliaceus, and bullous pemphigoid: A systematic review. Indian J Dermatol. 2011;77:456-69. doi:10.4103/0378-6323.82400.15. Doukaki S, Platamone A, Alaimo R, et al. Mycophenolate mofetil and enteric-coated mycophenolate sodium in the treatment of pemphigus vulgaris and pemphigus foliaceus. J Dermatolog Treat. 2015; 26(1):67-72. doi:10.3109/09546634.2014.880395. 16. Atzomny L, Hodak E, Leshem YA, et al. The role of adjuvant therapy in pemphigus: A systematic review and meta-analysis. J Am Acad Dermatol. 2015; 73(2):264-27117. Mimouni D, Anhalt JG. In: Lebwohl MG, Heymann WR, Berth-Jones J, Coulson I, eds. Treatment of Skin Disease: comprehensive therapeutic strategies. Philadelphia, PA: Elsevier; 2018: 611-615.18. McCarty M, Fivenson D. Two decades of using the combination of tetracycline derivatives and niacinamide as steroid-sparing agents in the management of pemphigus: Defining a niche for these low toxicity agents. J Am Acad Dermatol. 2014; 71(3):475-479

A 73-year-old male presented to our clinic for evaluation of persistent superficial erosions with overlying scale on his upper extremities, trunk, neck, and lateral face that began 3 months previous (Figure 1). The patient had just recently moved and was a new patient to us. At presentation, the lesions on the patient’s trunk and upper extremities had a thicker scale than those on his neck and face (Figures 2 and 3). Immediately prior to developing the lesions, the patient was treated with 5-fluoruracil for actinic keratosis on the face and scalp. The patient’s medication list included aspirin, clopidogrel, tamsulosin, and atorvastatin. Past medical history is significant for radiation therapy for laryngeal cancer and abdominal aortic aneurysm repair each many years previous.

Three lesional punch biopsies were performed for hematoxylin and eosin staining on chest and upper extremities. Histopathology showed separation of the corneal layer with subtle acantholysis in the granular layer, which favored the diagnosis of pemphigus foliaceus (PF) (Figures 4 and 5). Clinical correlation supported the diagnosis of PF. Direct immunofluorescence, indirect immunofluorescence, and ELISA were not performed due to histological and clinical support of diagnosis, and particularly due to the patient’s resistance to further testing.

The patient was started on high-potency topical corticosteroids and a short course of 20mg prednisone that was tapered over a month. By the end of the first month of treatment, he was cleared of nearly all lesions. However, at the next follow up, the patient had a more localized flare up on the neck and lateral face while continuing the topical corticosteroids. The patient was resistant to starting any immunosuppressant medications other than corticosteroids. The patient was started on 1mg/kg per day of prednisone with the plan to taper. The patient is transitory and was soon lost to follow up after starting the new treatment plan.

The above physicians are part of Aspen Dermatology Residency Program in Springville, Utah.

Craig Parson, D.O.; Nathan Peterson, D.O.

Pemphigus Foliaceus: Case Report and Review

Discussion

Figure 1. Erythematous plaques with erosions and crusting of the patient’s right face, ear and neck.

Figure 2. White scaly plaques on patient’s bilateral dorsal forearms.

Figure 3. Scattered pink eroded plaques with some overlying crust on patient’s chest.

Figure 4. H&E, original magnification 4x Figure 5. H&E, original magnification 10x