Localized pemphigus with vegetative features
The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, New York
We present a 58-year-old Jamaican man with an eight-month history of an isolated, vegetative, eroded, and crusted plaque on the posterior aspect of the scalp. One month prior to referral to the Charles C. Harris Skin and Cancer Pavilion he developed a few, scattered erosions on the extremities. A biopsy specimen showed suprabasal acantholysis along with deposition of C3 and IgG on a direct immunofluorescence test. Localized pemphigus is a rare variant of pemphigus vulgaris. Localized pemphigus presents with a localized plaque that remains localized for an extended period of time without generalized bullae formation. Our patient represents an unusual case of localized pemphigus of the scalp with vegetative features.
A 58-year-old Jamaican man was referred to the Charles C. Harris Skin and Cancer Pavilion in October, 2011, for a nine-month history of an expanding, pruritic, scaly plaque on the posterior aspect of the scalp. He was treated with oral antibiotics without improvement. A biopsy specimen disclosed a diagnosis of syringocystadenoma papilliferum and the patient was referred for surgical excision. The consulting plastic surgeon referred the patient to the Charles C. Harris Skin and Cancer Pavilion for further evaluation and management. The patient noted some lymphadenopathy of the posterior aspect of the neck and also some round erosions and scabs on the extremities over the month prior to the consultation. The patient did not have oral ulcers. He denied fevers, chills, weight loss, night sweats, nausea, vomiting, diarrhea, and arthralgias.
On the posterior aspect of the scalp there was a large, hyperkeratotic, boggy plaque with central alopecia that extended caudally onto the vertex and temporal areas of the scalp with overlying crust and serosanguinous exudate. On the chest, back, and upper extremities there were scattered, hyperpigmented, round patches and superficial erosions. Occipital and posterior auricular lymphadenopathy was present.
Complete blood count, basic metabolic panel, liver function tests, Quantiferon Gold assay for tuberculosis, and hepatitis serologies were normal. Human immunodeficiency virus was absent. A potassium hydroxide preparation of the posterior aspect of the scalp was negative for fungal elements. Indirect immunofluorescence tests showed IgG with a titer of 1:320 on monkey esophagus and 1:160 on human skin. Desmoglein 1 antibody was 90 units and desmoglein 3 antibody 34 units.
There is an intraepidermal blister with suprabasal acantholysis and a superficial, perivascular infiltrate that contains eosinophils. The surface is crusted.
Localized pemphigus is a rare variant of pemphigus vulgaris. Previous cases of localized pemphigus have been reported at sites of trauma, which include surgical sites, burns, and skin lesions of contact dermatitis [1, 2]. Classic pemphigus vulgaris, which is an autoimmune bullous disorder that is characterized by mucosal and cutaneous disease presents locally and may spread to additional skin sites. It is uncommon for pemphigus vulgaris to remain localized for more than five months . However, localized pemphigus has been reported to remain localized for seven years . To date only 36 cases of localized pemphigus have been reported .
Additionally, nearly all published case reports of localized pemphigus occur on the face, scalp, or oral mucosa [3, 5-8]. Localized pemphigus of the oral mucosa or mucosal-dominant type pemphigus is now understood to result as a consequence of autoantibodies to desmoglein 3 . The pathophysiology of localized pemphigus of cutaneous sites is poorly understood. Regional variation of desmoglein antigens and absent or low rates of circulating antibodies to desmoglein have been hypothesized to play a role in localized pemphigus . Some theorize that localized pemphigus of the face and scalp, in particular, may be triggered by ultraviolet radiation. This hypothesis is based on the experimental induction of acantholysis in pemphigus patients and clinical exacerbation of the disease after sun exposure . A case report of Armenian siblings, in each of whom localized pemphigus was present on the face and scalp, suggests that genetic factors, such as specific human leukocyte antigen types also play a role in the pathogenesis of localized pemphigus .
Pemphigus vegetans is a rare variant of pemphigus vulgaris that is characterized by the presence of vegetative plaques. The vegetative plaques of pemphigus vegetans may be localized to the scalp or may occur in two classic forms. The Neumann type generally begins and ends as typical pemphigus and the Hallopeau type usually remains localized [12, 13]. The lesions of pemphigus vegetans may begin as flaccid bullae or pustules and then form vegetations or papillomatous proliferations that favor the scalp and intertriginous areas.
Histopathologic examination of pemphigus vulgaris demonstrates intraepidermal cleavage above the basal layer, with characteristic tombstone-shaped basal keratinocytes that are lined up along the basement membrane. Acantholytic cells may be present within the blister. A direct immunofluorescence test shows that IgG and C3 are deposited within epidermis highlighting intercellular deposits. Pemphigus vegetans has an identical histopathologic and immunofluorescence appearance with the addition of increased acanthosis, papillomatosis, and a dermal, eosinophilic inflammatory infiltrate .
We present a patient with localized pemphigus of the scalp with vegetative features, which was present in isolation for about eight months. Initially, the lesion was diagnosed histopathologically as syringocystadenoma papilliferum. After a repeat biopsy, repeat examination of the original biopsy specimen, and the development of new erosions on the extremities, the diagnosis of localized pemphigus of the scalp with vegetative features was established. Although the scalp plaque is clinically vegetative, the histopathologic features on both the original biopsy specimen and the repeat biopsy specimen were not diagnostic of pemphigus vegetans. The patient’s skin disease is now improving with oral prednisone 60 mg daily, methotrexate 7.5 mg weekly, topical mometasone lotion, and intralesional glucocorticoid injections.
References1. Reichert-Penetrat S, et al. Pemphigus vulgaris on an old surgical scar: Koebner’s phenomenon. Eur J Dermatol 1998; 1:60 [PubMed]
2. Stransky L. Contact Pemphigus vulgaris? Contact Derm 1998; 38:45 [PubMed]
3. Zaraa I, et al. Localized pemphigus: a report of three cases. Int J Dermatol 2010; 49:715 [PubMed]
4. Lapiere K, et al. A case of long lasting localized pemphigus vulgaris of the scalp. Dermatology 2004; 209:162 [PubMed]
5. Baykal C, et al. Pemphigus vulgaris localized to the nose and cheeks. J Am Acad Dermatol 2002; 47:875 [PubMed]
6. Kharfi M, et al. Pemphigus localisé: 3 observations. Ann Derm Vénéréol 2001; 128:S155
7. Kishibe M, et al. Pemphigus foliaceus localized to the nose. Clin Exp Dermatol 2003; 28:560 [PubMed]
8. Termeer CC, et al. Topical tacrolimus (protopic) for the treatment of a localized pemphigus foliaceus. J Eur Acad Dermatol Venereol 2004; 18:636 [PubMed]
9. Ioannides D, et al. Regional variation in the expression of pemphigus foliaceus, pemphigus erythematosus, pemphigus vulgaris antigens in human skin. J Invest Dermatol 1991; 96:159 [PubMed]
10. Yamamoto S, et al. A case of localized pemphigus foliaceus. J Dermatol 1996; 23:893 [PubMed]
11. Gokdemir G, et al. Familial pemphigus vulgaris: two siblings with unusual localized variant. Inter J Dermatol 2006; 45:170 [PubMed]
12. Danopoulou I, et al. Pemphigus vegetans confined to the scalp. Int J Dermatol 2006; 45: 1008 [PubMed]
13. Rackett SC, et al. Treatment-resistant pemphigus vegetans of the scalp. Int J Dermatol 1995; 34: 865 [PubMed]
14. Joly P, et al. Pemphigus group (vulgaris, vegetans, foliaceus, herpetiformis and brasiliensis). Clin Dermatol 2011;29:432 [PubMed]
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