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Pemphigus foliaceus occurring with adenocarcinoma of prostate
2 Department of Pathology, Dr. D.Y. Patil Medical College and Hospital, Pimpri, Pune, Maharashtra, India
Queenstown Society, M-704, Behind Chinchwad Railway Station, Chinchwad, Pune, Maharashtra - 411 033
|How to cite this article:
Wadhokar M, Sharma YK, Deo K, Buch A, Gupta A. Pemphigus foliaceus occurring with adenocarcinoma of prostate. Indian J Dermatol Venereol Leprol 2015;81:525-526
A 73-year-old man presented with painful and burning vesiculo-bullous lesions that had developed sequentially over scalp, face, trunk and extremities, eroding after a few days and recurring episodically for the preceding one year despite treatment from private practitioners. Examination revealed multiple crusted erosions intermixed with hyperpigmented patches on these sites with a few intact vesicles and bullae [Figure - 1]a and b. General physical and systemic examination were normal.
|Figure 1: Multiple crusted erosions and postinflammatory hyperpigmented patches over (a) front, (b) back, of trunk and upper arms|
Histopathological examination revealed a subcorneal bulla containing a few acantholytic cells. A few plasma cells and lymphocytes were present in the dermis [Figure - 2]a and b. Direct immunofluorescence of perilesional skin revealed IgG deposition in the intercellular spaces in the upper epidermis [Figure - 2]c suggestive of pemphigus foliaceus. Absence of mucosal involvement, lack of vacuolar degeneration at the interface and absence of apoptotic/dyskeratotic keratinocytes ruled out paraneoplastic pemphigus.
|Figure 2: (a) Subcorneal bulla (H and E, ×100), (b) (H and E, ×400) (c) Immunofluorescence showing IgG positivity in the intercellular spaces of the upper epidermis (DIF, IgG, ×400) (d) Prostatic biopsy showing nests of neoplastic cells (H and E, ×400)|
The patient responded poorly to daily oral prednisolone, 1 mg/kg given for 1 month. Detailed enquiry revealed that he had hesitancy and dribbling of urine. Urological work-up revealed elevated levels of prostate specific antigen (PSA) at 104.9 ng/mL. Histopathological examination of transurethrally resected prostatic tissue confirmed adenocarcinoma, Gleason score 4 [Figure - 2]d. Bilateral orchidectomy was done and the urologist instituted oral leuprolide, a luteinizing hormone-releasing hormone agonist. Administration of exactly the same dosage of steroids as previously now led to prompt response of pemphigus within a fortnight. Steroids were gradually tapered off over 2 months after introducing dapsone as a steroid sparing agent. Follow-up after 6 months revealed normal skin and prostate specific antigen (PSA) levels returned to normal.
The malignancy most commonly reported with pemphigus foliaceus is thymoma.  Less commonly, mycosis fungoides, hepatocellular carcinoma, Kaposi sarcoma and non-Hodgkin lymphoma have been reported  and in one previous report, adenocarcinoma of prostate.  Paraneoplastic pemphigus is a subtype of pemphigus commonly associated with lymphoid neoplasms including non-Hodgkin lymphoma, chronic lymphocytic leukemia and Castleman disease. It is characterized clinically by severe mucosal erosions, polymorphic cutaneous eruptions, particularly on the upper body and palmo-plantar target lesions and serologically by the presence of antibodies to desmoplakin-1, envoplakin, periplakin and plectin and organ antigens. 
Of the epidermal and subepidermal immunobullous disorders, concurrent malignancy has been reported least commonly in pemphigus foliaceus.  Younus et al., in a study undertaken before paraneoplastic pemphigus was defined, reported 60 cases of malignancies in patients belonging to the pemphigus group; thymic malignancies being equally prevalent among pemphigus foliaceus and pemphigus vulgaris patients.  A recent report described 19 neoplasms in patients of pemphigus vulgaris: 12 skin cancers, 2 cervical cancers, 2 prostatic cancers and 1 each of breast, thyroid, and thymic cancers. 
The co-occurrence of adenocarcinoma of prostate with pemphigus foliaceus in our patient seems a chance association, as Curth′s criteria are not met. However, the improved response to treatment for pemphigus after surgery for prostatic adenocarcinoma suggests that tumor-induced immune dysregulation may have contributed to the initial recalcitrance of the disease.
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