An unusual association of pemphigus vulgaris with hyperprolactinemia

Abstract

A 21‐year‐old unmarried woman presented with oral ulcerations and generalized, itchy, fluid‐filled, skin lesions of 10 days’ duration. The lesions ruptured spontaneously, resulting in extensive denuded areas covered by crusts. One month prior to this, she experienced pain and enlargement of both breasts with galactorrhea. Her menstrual cycles were normal initially, but later she developed menstrual irregularities. No past history suggestive of any other systemic or skin disease, including atopy or drug allergies, could be obtained. Her family history was not contributory. Dermatologic examination revealed multiple, flaccid bullae and extensive denuded areas of skin covered with crusts over the scalp, face, trunk, and upper and lower limbs (Fig. 1). Bulla spread sign and Nikolsky's sign were positive. The oral mucosa, including the lips, buccal surface, tongue, and palate, showed multiple erosions covered with necrotic slough. The rest of the mucocutaneous and systemic examination was within normal limits. 1 Extensive erosions and flaccid bullae over the trunk with breast enlargement The patient's diagnostic work‐up revealed: hemoglobin, 11.2 g%; total leukocyte count, 7400/mm3; differential leukocyte count, P62L34E2M2; erythrocyte sedimentation rate, 34 mm/h. A peripheral blood smear examination, urinalysis, blood sugar, and renal and liver function tests were normal. Venereal Disease Research Laboratory (VDRL) test and enzyme‐linked immunoabsorbent assay (ELISA) for human immunodeficiency virus (HIV) were nonreactive. Antinuclear antibody, lupus erythematosus (LE) cell, rheumatoid factor, and anti‐dsDNA levels were normal. Serum protein electrophoresis demonstrated increased levels of immunoglobulin G (IgG) antibody. The serum prolactin level was significantly raised to 139.49 ng/mL (normal, 3.6–18.9 ng/mL). The sex hormone levels, however, including follicle‐stimulating hormone (FSH), luteinizing hormone (LH), estradiol, and progesterone, were within normal limits. The thyroid hormone profile was also unaltered. Chest X‐ray was normal. Ultrasound of the abdomen and pelvis revealed no visceral abnormality and computerized tomography (CT) scan of the pituitary sella showed no adenoma. Mammography was negative for breast malignancy. A Tzanck smear prepared from the base of the erosion showed multiple acantholytic cells and lymphocytes. Histologic examination from an intact vesicle was suggestive of pemphigus vulgaris (PV), showing a suprabasal cleft with acantholytic cells and the basal layer demonstrating a “row of tombstones” appearance (Fig. 2). Direct immunofluorescence (DIF) revealed the intercellular deposition of IgG and C3 throughout the epidermis in a “fishnet pattern.” Indirect immunofluorescence (IIF) test performed on rat esophagus for circulating IgG antibody was positive in a titer of 1 : 120. 2 Photomicrograph showing suprabasal cleft with “row of tombstones” appearance, suggestive of pemphigus vulgaris (hematoxylin and eosin, × 40) Based on the clinical and immunohistological features, a diag...

Pemphigus in India

“…Mehta et al reported that herpetiform pemphigus clinically resembled bullous pemphigoid, and the diagnosis was confirmed by intracellular deposit of C3 and IgG in the upper epidermis. [39] Different diseases have been described in association with pemphigus and these include cryoglobulinemia and cold agglutinin disease, [40] renal cell carcinoma, [41] hyperprolactinemia, [42] and brain abscesses. [43] Individual reports of single cases cannot rule out pure chance in such associations.…”

Evaluating serum prolactin and serum dehydroepiandrosterone sulfate levels in patients with pemphigus

“…Beneficial effects of bromocriptine in the treatment of pemphigus have been reported by Khandpur and Reddy . They reported a 21‐year‐old woman with idiopathic hyperprolactinemia and extensive mucocutaneous pemphigus in whom complete remission occurred when bromocriptine was added to the conventional therapeutic regimen of prednisolone …”

“…16,17 Beneficial effects of bromocriptine in the treatment of pemphigus have been reported by Khandpur and Reddy. 18 They reported a 21-year-old woman with idiopathic hyperprolactinemia and extensive mucocutaneous pemphigus in whom complete remission occurred when bromocriptine was added to the conventional therapeutic regimen of prednisolone. 18 Accordingly, our present findings of higher levels of prolactin in patients with pemphigus may open the way for further clinical trials to assess the efficacy of dopamine agonist as an adjuvant therapy.…”

Evaluation of prolactin levels in patients with newly diagnosed pemphigus vulgaris and its correlation with pemphigus disease area index

“…Khandpur and Reddy (2002) reported a case of new onset pemphigus vulgaris (PV) and idiopathic hyperprolactinemia that was responsive to steroid and bromocriptine treatment. Other studies have shown a correlation between PRL level and PV (Barzegari et al, 2004, Fallahzadeh et al, 2010, Helmy et al, 2013, Kavala et al, 2006).…”