Cerebriform Cutaneous Lesions in Pemphigus Vegetans.

Pemphigus vegetans is an autoimmune bullous disorder characterized by vegetating lesions commonly over the flexures. A 42-year-old female patient came with pemphigus vegetans presenting with interesting cerebriform morphology of the cutaneous lesions over the flexures. Cerebriform tongue, a morphology with typical pattern of sulci and gyri over dorsum of the tongue is a well-known sign seen in pemphigus vegetans. Interestingly, we noticed the typical sulci and gyri pattern in the skin lesions of pemphigus vegetans over the flexures of the body. This clinical sign can be used as a clue in the diagnosis of pemphigus vegetans. Morphology and physical characteristics are important for the diagnosis of the disease. Clinical signs always give a clue to the probable or possible diagnosis in most of the dermatological conditions.

What was known?

Cerebriform tongue, a morphology with typical pattern of sulci and gyri over dorsum of tongue is a well-known sign seen in pemphigus vegetans.

Introduction

Pemphigus vegetans, a rare variant of pemphigus, is an autoimmune bullous disorder. There is a production of immunoglobulin G (IgG) antibodies against desmoglein, an intercellular adhesion protein leading to acantholysis.[1] This disorder is characterized by vegetating lesions more commonly over flexures. Cerebriform tongue, a morphology with typical pattern of sulci and gyri over dorsum of the tongue, is a well-known sign seen in pemphigus vegetans.[234] Interestingly and for the first time in our setting, to the best of our knowledge, we noticed typical sulci and gyri pattern in the flexural cutaneous lesions of pemphigus vegetans. Hence, we report this case.

Case Report

A 42-year-old female, a homemaker, came to our Dermatology OPD with c/o itchy, painful fluid-filled lesions over genitals and groin for 3 months. The lesions gradually progressed in size and number over 2 weeks and following itching, the lesions burst open to leave behind painful, itchy raw malodorous oozy areas over 2–3 days with no tendency to heal. A month later, lesions progressed to involve both axillae and perianal region. The patient also developed raw areas in the mouth. Another month later, the patient developed dark raised lesions over the raw areas which were coalescing to form larger lesions. New fluid filled lesions continued to develop over the neck, trunk, abdomen, both the lower limbs with sparing of upper limbs, scalp, palms, and soles. On examination, multiple hyperpigmented vegetative malodorous oozy plaques distributed over bilateral groin, labia majora, axillae, and perianal region with few erosions around the plaques were present. Most of the plaques had coalesced to form large plaques with a pattern of sulci and gyri [Figures 1 and 2]. Few discrete lesions covered with crusting were present over trunk, neck, bilateral popliteal fossae, and extensor aspect of right thigh. Marginal Nikolsky sign was positive. Oral cavity showed multiple erosions over bilateral buccal mucosa. Crusted plaques were present over bilateral angle of the mouth. The pattern of gyri and sulci was present over tongue suggestive of cerebriform tongue [Figure 3]. Nail findings included edema of lateral nail folds of bilateral thumb and ring finger with right index finger suggestive of paronychia. With this clinical background, diagnosis of pemphigus vegetans, Neumann's type was made. This was later confirmed with skin biopsy which showed suprabasal clefting with prominent papillomatosis and irregular acanthosis. Cleft showed acantholytic cells admixed with neutrophils and eosinophils. Papillary dermis showed prominent vessels with neutro-eosinophilic infiltrate [Figures 4 and 5]. Direct immunofluorescence showed intercellular deposition of IgG and C3. Other investigations performed were complete blood count, HIV, and hepatitis B surface antigen, which were within normal limits. Venereal Disease Research Laboratory was also performed since the possibility of secondary syphilis was kept in mind, which came nonreactive. The patient was started on oral prednisone 40 mg, dapsone 100 mg, and topical steroids. The patient improved well within a week with flattening of lesions to leave behind postinflammatory hyperpigmentation.

Figure 1

Cerebriform cutaneous lesions over the groin and labia majora

Figure 2

Cerebriform cutaneous lesions over the left axilla

Figure 3

Cerebriform tongue

Figure 4

Low power view showing suprabasal bulla (H and E, ×10)

Figure 5

High power view showing acantholytic cells admixed with neutrophils and eosinophils (H and E, ×40)

Discussion

Pemphigus vegetans, a rare variant of pemphigus was first described by Neumann in 1876. Two subtypes recognized are Neumann and Hallopeau types. They are differentiated based on clinical occurrence, course, response to treatment, and prognosis. They occur as vegetating or verrucous plaques more commonly over flexures. They can occur over the lesions of pemphigus vulgaris or over normal skin. Initially, they can develop as vegetative papules and plaques which later coalesce to form larger plaques. The large plaques seen in our patient had fissuring pattern which resembled typical pattern of sulci, gyri over the flexural lesions as seen in cerebriform tongue, a well-known sign described in pemphigus vegetans as “Premalatha sign.” In a study done by Premalatha et al. during 1968–1979, out of 12 confirmed cases of pemphigus vegetans, 6 cases of Neumann type (50%) showed cerebriform tongue, and two cases had a cerebriform scalp.[34] However, to the best of our knowledge, no cases have been reported on this morphology over the flexures. The papillary hyperplasia occurring in pemphigus vegetans could be the cause of the cerebriform morphology.[2] This could be the possible cause for cerebriform cutaneous lesions over the flexures too. Morphology and physical characteristics are important for the diagnosis of disease. This is achieved by careful observation by clinicians. Clinical signs always give a clue to the probable or possible diagnosis in most of the dermatological conditions.

Financial support and sponsorship

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Conflicts of interest

There are no conflicts of interest.

What is new?

The typical sulci and gyri pattern as seen in cerebriform tongue was seen in the skin lesions over the flexural areas in our patient of pemphigus vegetans.