Coexistence of pemphigus herpetiformis and acrofacial vitiligo

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Pemphigus herpetiformis (PH) is a rare, less severe clinical subtype of pemphigus combining clinical features of dermatitis herpetiformis with histological and immunopathological findings of pemphigus. Herein, a case of PH in a patient with acrofacial vitiligo is reported.

A 19-year-old man presented with complaints of itching and blisters of approximately six weeks’ duration. His personal history was significant for vitiligo, present for several years in a stable pattern. There were no other comorbidities. Treatment with amoxicillin/ clavulanic acid, levocetirizine, and a topical antiseptic spray had been ineffective.

Dermatological examination revealed discrete vesicles and bullae filled with clear yellowish fluid, erosions with crusts, erythematous papules, plaques, and scales, on the neck, trunk, and arms [Figure 1a], and depigmentation of the perioral and periorbital areas [Figure 1b], and tips of fingers [Figure 1c]. Nikolsky’s sign was negative. The mucous membranes, hair, and nails were unaffected.

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Figure 1a: Vesicles and bullae, erosions, crusts, erythematous papules, plaques, and scales, on the neck, trunk, and upper arm. Some vesicles and bullae are indicated with black arrows.

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Figure 1b: Periorbital and perioral depigmentation.

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Figure 1c: Depigmentation of the tips of fingers.

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Histopathology from a fresh vesicle showed eosinophilic spongiosis, intraepidermal vesicles with fibrin and eosinophils, mild acantholysis confined to the superficial epidermis, and mild superficial perivascular infiltrates composed of lymphocytes, histiocytes, and eosinophils [Figures 2a and b]. Direct immunofluorescence (DIF) of perilesional skin showed intercellular continuous deposition of IgG and complement component 3 (C3) in the epidermis [Figure 3]. Serum autoantibodies were not tested. The patient was diagnosed with PH.

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Figure 2a: Histopathology showing intraepidermal vesicle containing eosinophils, spongiosis, mild superficial acantholysis (Haematoxylin & eosin, 50x)

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Figure 2b: Higher magnification showing eosinophilic spongiosis, mild superficial acantholysis, mild perivascular infiltrates composed of lymphocytes, histiocytes, and eosinophils in the dermis (Haematoxylin & eosin, 200x).

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Figure 3: Direct immunofluorescence (DIF) reveals intercellular continuous deposition of IgG and C3 in the epidermis (400x).

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The patient was successfully treated with parenteral corticosteroid with an initial dose of methylprednisolone, 60 mg intravenously, reduced as the patient improved. The treatment resulted in clinical improvement within two weeks. The patient was followed up regularly and is currently in clinical remission, continuing maintenance therapy with 5 mg of oral prednisolone daily.

While the coexistence of vitiligo and other autoimmune diseases is not uncommon, such an association is rare with PH. PH has been reported in association with autoimmune hemolytic anaemia, psoriasis, psoriasis after ultraviolet light treatment, and systemic lupus erythematosus in isolated case reports.^1-5

The coexistence of PH and vitiligo cannot be explained. The immunological mechanisms behind pemphigus involve IgG autoantibodies targeting desmoglein proteins, leading to acantholysis. Many patients with PH have anti-desmoglein 3 autoantibodies, but they do not develop mucosal lesions, and histopathological findings may be with or without acantholysis. The immunological mechanisms in PH differ from those in classical pemphigus vulgaris. Vitiligo is believed to result from immune-mediated destruction of melanocytes by anti-melanocyte-specific cytotoxic T cells.

A clinical manifestation that can be observed in PH and also in vitiligo is pruritus, which is typical of PH and is found in about 20% of patients with vitiligo.⁶

The cytokine IL-31 has been implicated in many pruritic disorders. A study of IL-31 family in the epidermis in pemphigus patients found enhanced in situ expression of IL-31 in PH patients compared with pemphigus vulgaris and pemphigus foliaceus cases.⁷ The serum concentration of IL-31 was measured in vitiligo patients and controls, and the results showed the highest levels of IL-31 in vitiligo patients with pruritus, followed by patients without pruritus, and the lowest in controls.⁶ The increased in situ expression of interleukin-31 in patients with PH and increased serum levels of IL-31 in vitiligo patients may suggest involvement of the IL-31 family in the pathogenesis of both diseases.

In conclusion, the occurrence of PH in a patient with vitiligo is likely coincidental, but common pathological mechanisms can be suggested. To the best of our knowledge, this is the first report on the coexistence of PH and vitiligo.