Cerebriform Toungue, The History Behind the Sign

Sir,

I would like to thank the author Dr. Sentamilselvi Ganapati and the editorial team of IJD^® for the article entitled “Eponymous dermatological signs in bullous dermatoses” published in Indian Journal Dermatol 2014:59(1):21-23. “Cerebrifom tongue – a clinical sign in pemphigus vegetans” published by me in 1981 has been quoted as an eponymous sign.

The history behind this eponymous sign is interesting. One fine morning about 35 years ago, a middle-aged housewife attended our skin outpatient clinic at the Govt. General Hospital and Madras Medical College. She had flaccid bullae mainly over the flexural areas. Tzanck smear showed acantholytic cells with few eosinophils and the Nikolsky sign was positive. She was admitted to our female skin ward. A provisional diagnosis was made of pemphigus vulgaris or early pemphigus vegetans. During ward rounds on that day, I wanted to check if she had developed oral mucosal lesions. When she opened her mouth, I was surprised to see the dorsum of her tongue showing “sulci and gyri” pattern simulating the surface of a brain! [Figure 1].

Figure 1.

Sulci and gyri pattern-cerebriform tongue in pemphigus vegetans. (Published in Br J Dermatol 1981;104:587-91)

Biopsy of one of the bullous lesions of the skin showed suprabasal bulla containing Tzanck cells and few eosinophils. She was started on oral steroids. While resolving, some of the bullous lesions in the axillae and groin transformed into vegetating plaques in favor of pemphigus vegetans. The cerebriform appearance of the tongue showed only partial resolution. I was curious to do a biopsy from the tongue lesion to find out the reason behind the cerebriform appearance. Fortunately, the patient agreed for a biopsy from the tongue lesion. The histopathology showed suprabasal lacunae containing acantholytic cells and few eosinophils, in additition to acanthosis and papillomatosis consistent with the clinical diagnosis of pemphigus vegetans [Figure 2].

Figure 2.

H and E section from the cerebriform tongue showing suprabasal lacuna containing acantholytic cells, papillomatosis, and villi lined by a single layer of basal cells projecting into the lacuna

By coincidence, I came across a typical case of pemphigus vegetans, a 46-year-old male with skin lesions of vesicles, bullae, and vegetating plaques on the scalp, axillae, and groin. Over the scalp the plaques of pemphigus vegetans resembled cutis parietalis and verticis gyrata giving the cerebriform appearance [Figure 3]. Secondary cutis verticis gyrata in pemphigus vegetans has been already mentioned in the literature.[1]

Figure 3.

Cerebriform scalp with cutis parietalis gyrata in a case of pemphigus vegetans

The histological presence of papillomatosis would have been the basis for the morphological appearance of both cerebriform scalp and cerebriform tongue. Retrospectively, I traced the cases of pemphigus vegetans for about a decade (1968-1979) from the “pemphigus register” maintained in our inpatients ward and from the old case sheets stored in the medical record department of our hospital.

Out of 108 cases of pemphigus, 12 cases belonged to pemphigus vegetans of Neumann type. It was found that about 50% of those cases presented with tongue lesions were mentioned as scrotal tongue or lobulated tongue in the records. Those 12 cases were reviewed for follow-up in person to confirm the presence of this tongue sign in pemphigus vegetans and this important clinical sign was published in 1981.[2]

This morphological sign was observed by me during routine ward rounds, described in detail and published by me as the first author.[2]

In that article, I included Dr. S. Jayakumar as one of my co-authors, to inspire a post-graduate student of our department as he has helped me to get the old case sheets from the Medical Record Department.[2]

I included Dr. Patrick Yesudian and Dr. AS. Thambiah also as my co-authors in that article as a gesture to show my respect and gratitude to my teachers.[2]

In all cases of pemphigus and in any case of bullous dermatosis with lesions especially over the flexural areas, the tongue should be evaluated for this sign – “cerebriform tongue” which if present may be a clue for the diagnosis of pemphigus vegetans. This important tongue sign in pemphigus vegetans may precede, coincide, or follow the skin lesions of pemphigus vegetans and shows a slow response to therapy. Isolated pemphigus vegetans of the tongue persisted for seven years also has been reported.[3]

Now, after about 17 citations in the literature since my original publication, references in standard text books and after being quoted by members of the specialty on various occasions, the morphological sign – “cerebriform tongue in pemphigus vegetans” described by me has become eponymous.[4]

The old B and W photographs of this correspondence speak the “history behind Cerebriform tongue in pemphigus vegetans.”