A Case of Pellagra Associated with Long Term Alcoholism

Content Source: The Journal of Psychiatry and Neurological Sciences

To the Editor,

Pellagra is a systemic, nutritional disease associated with deficiency of vitamin B3 (niacin) and/or tryptophan and often other B vitamins (1). Pellagra is mostly seen in chronic alcoholics as a result of nutritionally poor diet and malabsorption (2). We present a pellagra case with long history of alcohol use, admitted with psychiatric complaints to our clinic.

Mr. A. was a 44 year old, married, primary school graduate male, who was running a coffeehouse. His socioeconomic status was low. His complaints were irritability, nausea, vomiting and loss of appetite. He had been drinking alcohol every day, for 33 years; its amount had increased to about 100cl for the last 15 years. The longest duration of remission was 3 months, when he was 13 years old. He was experiencing sweating, tremor of hands, insomnia, and irritability as withdrawal symptoms. In the last 2 years, periodically, he had problems in focusing and maintaining attention, delay in reaction time in answering any questions. He had depressive symptoms for 1 year and he had attempted suicide. In the last 2 months, he had diarrhea, vomiting, loss of appetite and erythema, followed by dark discoloration on the dorsal surfaces of his hands. On physical examination, hyper-keratotic plaques with well-defined borders on the dorsal surfaces of both hands, squamous lesions between fingers of both feet, loss of villi and hyperemia on the tongue was detected. He had tremor of both hands and wide-based gait. On psychiatric examination, he was confused, his time orientation was disturbed, self care was poor. Affect was restricted; associations and psychomotor activity were slow. The possibility of pellagra was considered as dermatitis, diarrhea and distortion of cognitive functions were observed. Electrocardiography (ECG), complete blood count, routine blood biochemical tests, routine urine tests, thyroid function tests, VDRL, microscopic stool examination, electroencephalography (EEG), vitamin B12 and folate measurements, cranial MRI, echocardiography, esophago-gastro-duodenoscopy were performed and no significant pathology was detected. As the patient’s symptoms did not respond to oral niacin treatment, niacin malabsorption was considered and a mixture of vitamin B1, B2, B6, B12, nicotinamide and dexpanthenol was given by intramuscular injection and a dramatical recovery was observed.

Pellagra is characterized by photosensitive symmetrical skin lesions, gastrointestinal disturbances, neurologic and psychiatric manifestations. The syndrome is known as “4 D’s”: dermatitis, diarrhea, dementia and death (1). Skin lesions seen in pellagra are photosensitive rash, primarily on the dorsal surfaces of the hands, arms, face and feet. In acute phase, skin lesions are erythema and bullae which resemble sunburn (wet pellagra), but after exposure to sun light, progress to chronic, symmetrical, scaled lesions occurs. Typically they are located on the neck (Casal necklace), hands and forearms (pellagra gauntlet) (3). Irritability, concentration problems, anxiety, fatigue, restlessness, apathy and depression are common psychiatric and neurological manifestations. Even uncommon, psychosis can be seen in pellagra, especially in pellagroid encephalopathy mostly encountered in chronic alcoholics. Confusion and eventually death occurs as the disease progresses (4). Gastrointestinal manifestations are fissures on the tongue and mouth, sourness, loss of appetite, dyspepsia and abdominal pain. Enteritis, which can be severe with nausea, vomitting and diarrhea can also be seen (5). Diagnosis is based on patient’s history and physical examination. There are no chemical tests to definitely diagnose pellagra (6).

In conclusion, low socioeconomic status, long duration of alcohol use, poor diet and characteristic findings should suggest pellagra, although it is a rare disease nowadays. It shouldn’t be considered as a disease that is seen only in undeveloped countries and considering pellagra in the differential diagnosis in chronic alcoholics with psychiatric, dermatologic and gastrointestinal symptoms has vital importance.

REFERENCES

1. World Health Organization. Pellagra and its prevention and control in major emergencies. Geneva, World Health Organization, 2000 (document WHO/NHD/00.10).

2. Stratigos JD, Katsambas A. Pellagra: a still existing disease. Br J Dermatol 1977; 96:99-106.

3. Pipili C, Cholongitas E, Ioannidou D. The diagnostic importance of photosensivity dermatoses in chronic alcoholism: Report of two cases. Dermatol Online J 2008; 14:15.

4. Cook CC, Hallwood PM, Thomson AD. B Vitamin deficiency and neuropsychiatric syndromes in alcohol misuse. Alcohol Alcohol 1998; 33:317-336.

5. Karthikeyan K, Thappa DM. Pellagra and skin. Int J Dermatol 2002; 41:476-481.

6. Hegyi J, Schwartz RA, Hegyi V. Pellagra: Dermatitis, dementia, and diarrhea. Int J Dermatol 2004; 43:1-5.

Spongiosis

Spongiotic reaction pattern is characterised by inter and intracellular oedema of the epidermis and elongation of the intercellular bridges.

Progressive psoriasiform hyperplasia occurs with chronicity.

In the past this reaction pattern was known as ‘eczematous tissue reaction’.

The spongiosis may vary from microscopic foci to grossly visible vesicles.

Inflammatory cells are present in the dermis and their distribution and type may aid in making a specific diagnosis.

Five patterns of spongiosis :

1. Neutrophilic spongiosis (where there are neutrophils within foci of spongiosis)

Example- Pustular Psoriasis

2. Eosinophilic spongiosis (where there are numerous eosinophils within foci of spongiosis).

Example- Bullous Pemphigoid

3. Miliarial (acrosyringial) spongiosis (where edema is related to the acrosyringium).

Example – Miliaria Rubra.

4. Follicular spongiosis (where the spongiosis centered on the follicular infundibulum.

Example – Infundibulofolliculitis

5. Haphazard spongiosis (other spongiotic disorders in which there is no particular pattern).

Example- Spongiotic Drug Reaction


1. Neutrophilic spongiosis (where there are neutrophils within foci of spongiosis)

Examples of Neutrophilic Spongiosis:

Pustular psoriasis;

IgA pemphigus;

Palmoplantar pustulosis;

Dermatophytosis/candidosis;

Acute generalized exanthematous pustulosis.

2. Eosinophilic spongiosis (where there are numerous eosinophils within foci of spongiosis)

Examples of Eosinophilic Spongiosis:

Pemphigus (precursor lesions)

Pemphigus vegetans

Bullous Pemphigoid

Arthropod bites

Allergic contact dermatitis

Eosinophilic folliculitis

Incontinentia pigmenti (first stage)


3. Miliarial (acrosyringial) spongiosis (where edema is related to the acrosyringium).

Example: Miliaria

4. Follicular spongiosis (where the spongiosis centered on the follicular infundibulum

Example: Infundibulofolliculitis, atopic dermatitis

5. Haphazard spongiosis (other spongiotic disorders in which there is no particular pattern).

Other Spongiotic Disorders:  

Irritant contact dermatitis

Allergic contact dermatitis Image

Nummular dermatitis ; Dermatopathology Case 122

Seborrheic dermatitis ;

Atopic dermatitis ;

Pityriasis rosea;

Stasis dermatitis ;

Chronic superficial dermatitis ;

Spongiotic drug reaction.

Histopathological features of some spongiotic diseases:  

Irritant contact dermatitis:  

Superficial ballooning, necrosis and neutrophils; mild irritants produce spongiotic dermatitis mimicking allergic contact dermatitis.

Allergic contact dermatitis:

Variable spongiosis and vesiculation at different horizontal and vertical levels, mild exocytosis, progressive psoriasiform hyperplasia with chronicity.

Superficial dermal oedema and eosinophils in superficial dermal infiltrate.

Seborrheic dermatitis:

Variable spongiosis and psoriasiform hyperplasia depending on activity and chronicity.

Scale crust and spongiosis may localize to follicular ostia.

Atopic dermatitis:  

Mimics other spongiotic diseases. 

There is variable spongiosis, focal parakeratosis, prominence of vessels in the papillary dermis, psoriasiform hyperplasia, exocytosis and perivascular infiltrate of lymphocytes.

Stasis dermatitis:

Mild spongiosis only ; proliferation of superficial dermal vessels, extravasation of erythrocytes, abundant hemosiderin.

Spongiotic drug reaction:  

Spongiosis, conspicuous exocytosis of lymphocytes, rare  apoptotic keratinocytes, eosinophils, plasma cells, lymphocytes in superficial dermis and sometimes in mid dermis.

Sometimes superficial dermal oedema.

Chronic superficial dermatitis:

Mild spongiosis, focal parakeratosis, variable psoriasiform hyperplasia, superficial perivascular infiltrate with upward extension and mild exocytosis.

 

Dermatophytoses

Neutrophils in stratum corneum or compact orthokeratosis should alert observer to perform PAS stain.

Spongiotic vesicles may form on palms and soles.