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Brain abscesses with pemphigus vulgaris: A rare association

  • Author(s): Awasthy, Neeraj
  • Chand, Karam
  • Singh, Avninder
  • et al.
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Brain abscesses with pemphigus vulgaris: A rare association
Neeraj Awasthy, Karam Chand, and Avninder Singh
Dermatology Online Journal 11 (3): 35

Departments of Pediatrics and Neurosurgery, Vardhman Mahavir Medical College and Safdarjung Hospital, Delhi,India. n_awasthy@yahoo.com

Abstract

Pemphigus is a rare group of skin disorders characterized by blistering because of loosening of desmosomal links between epidermal cells attributed to immunological attack. Pemphigus vulgaris is characterized by the presence of the split within the epidermis just above the basal layers (suprabasal). Central nervous system complications of the disorder have scantily been described in the English literature. We report what we believe to be the first case of cerebral abscesses in a 38-year-old male associated with pemphigus vulgaris and immunosuppressive therapy.



Introduction

Various forms of pemphigus are a rare group of skin disorders characterized by blistering attributed to loosening of desmosomal links between epidermal cells resulting from immunological attack. Pemphigus vulgaris (PV), the most common form, is characterized by the presence of the split within the epidermis just above the basal layers (suprabasal). Central nervous system complications of the disorder are seldom described in the English literature [1, 2, 3, 4]. These include those related to the disorder itself and also to the treatment and its iatrogenic complications [4]. Abscess formation is rare. We report a case of cerebral abscesses in 38-year-old male associated with pemphigus vulgaris.


Clinical synopsis


Figure 1a Figure 1b

Figure 2a Figure 2b

A 38-year-old male presented with the complaints of weakness of the right side of the body for 5 months, altered sensorium for 7 days, and episodes of right partial motor seizure. He was previously diagnosed with pemphigus vulgaris and being treated with low-dose oral steroids and cyclophosphamide for last 3 years. On examination patient was conscious but drowsy, responding to oral commands. Cranial nerve examination was within normal limits. He had right hemiparesis of grade 3/5 and was dysphasic. He had widespread bullae and erosions and involvement of the oral mucosa. Blisters involved a large area of the trunk and the back but spared the distal parts of the limbs (Fig. 1). He had a large raw area in the left frontotemporal region of scalp with exposed bone. All the laboratory investigations were within normal limits. Contrast enhanced computerized tomography (CECT) of the head revealed multiple brain abscesses in the left frontal and right frontal brain with left frontal subdural empyema and skull osteomyelitis (Fig. 2). The patient underwent excision of left frontal osteomyelitic bone and skin grafting. Brain abscesses were managed conservatively with the IV antibiotics given over 8 weeks. He was also administered diphenylhydantoin for seizures. Steroids and cyclophosphamide were given for pemphigus vulgaris. The patient showed significant neurological recovery on followup with no seizures. Follow up CECT head showed complete resolution of the brain abscesses .


Discussion

Pemphigus is an autoimmune disease involving the skin. Based on the level of blister formation, the pemphigus group is divided into superficial and deep forms. Pemphigus vulgaris forms the least rare amongst the group involving predominantly deep layers [1, 2]. Clinically the characteristic feature of pemphigus vulgaris is the presence of flaccid, weeping bullous lesions, which leave large denuded areas of the skin. Nikolsky sign is frequently present. Cutaneous areas commonly involved include scalp, umbilicus and the intertriginous areas. The mucous membrane are commonly involved including oral cavity, nasal, vaginal, anal mucosa. The disorder may involve any of the age group although generally involving fourth and the fifth decades of life as was observed in our case [1, 2]. Histopathologically it is characterized by suprabasal intraepidermal bulla formation with loss of cohesion of the epidermal cells (acantholysis). A Tzanck preparation is a useful screening cytological procedure for the identification of the acantholytic cells. Complications of the disease include secondary infection, electrolyte imbalances and those arising from the treatment including prolonged immunosuppressive therapy [4, 5]. Although secondary infections including generalized sepsis attributed to corticosteroid therapy occur, localized manifestations have rarely been described [3, 4, 5]. This is particularly true of the brain abscess; our search of the English literature finds no association of brain abscess with pemphigus vulgaris. Formation of the brain abscess seems to follow the intracranial propagation of cutaneous bacteria at the site of the skull osteomyelitis after the involvement of the overlying scalp region with pemphigus lesions and secondary infection. The formation of the abscesses was probably further aggravated on account of the immunosuppressive state of the patient from the corticosteroid and cyclophosphamide. This hypothesis is well supported by the observation of Manfredini et al. of the presence of lung abscess in a pemphigus patient [4]. A change in mental status, focal neurological signs, or seizures in patient with pemphigus should be treated with highest degree of suspicion and investigated with CT scan to rule out intracranial complication. Any patient on chronic immunosuppressive therapy should have periodic and repeated evaluation to evaluate for underlying infection including abscesses in the body.

References

1. Ahmed AR et al : Pemphigus : current concepts . Ann Intern Med 1980;92:396-399

2. Williams DS. Pemphigus. J Insur Med. 2004; 36(3):267-8.

3. Kirtschig G, Khumalo NP. Management of bullous pemphigoid : recommendations for immunomodulatory treatments.Am J Clin Dermatol. 2004;5(5):319-26.

4. Manfredini R, Delli Gatti C, la Cecilia O, Boari B, Gallerani M, Altieri E. Lung abscess as a complication of steroid treatment in pemphigus vulgaris.Arch Intern Med. 2001 Sep 10;161(16):2044-5.

5. Stein KM, Raque CJ, Harrell DD, Samitz MH. Occult lung abscess complicating high-dosage corticosteroid therapy. Postgrad Med. 1972 Apr;51(4):97-101.

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