“Dyssebacia”—A Fifth D of Pellagra

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PRACTICAL GASTROENTEROLOGY • MARCH 2003 41 A ttention history buffs and aficionados of medical arcana. Here’s something for you. .............. .. Pellagra (pelle agra, rough skin) is tradition- ally portrayed as a niacin-deficiency disorder resulting from a diet of three Ms—meat (fatback), meal (corn- meal), and molasses—and manifesting clinically as four Ds (dermatitis, diarrhea, dementia, and death). Approximately one-fifth of pellagrins, however, mani- fest a fifth D—“dyssebacia” (1). In this report, I sum- marize the essential features of “dyssebacia” and illus- trate this finding in three patients. “Dyssebacia” is the name coined to describe numer- ous plugs of inspissated sebum projecting from dilated orifices of sebaceous glands (Figures 1–4). Histologi- cally, the glands show hyperplasia with sebaceous material plugging dilated follicles. On palpation, the plugs feel like sharkskin or sandpaper. They first appear along the alae nasi, then spread over the nose, and in advanced cases, involve the forehead, lips, and chin. When small, the plugs reflect light and mimic sulfur flakes. The larger plugs range in color from white to grayish yellow, occasionally with darker tips. Their “Dyssebacia”—A Fifth D of Pellagra PEARLS OF GASTROENTEROLOGY Herbert L. Fred, M.D., Professor of Internal Medicine, The University of Texas Health Science Center, Hous- ton, Texas. Herbert L. Fred Figure 1. Nasal area of a 65-year-old woman with pellagra showing diffuse sebaceous plugging, especially prominent on the left. Figure 2. Side view of the patient in Figure 1 showing plugs over the nose, cheek, forehead, and scalp.

Transcript of “Dyssebacia”—A Fifth D of Pellagra

Page 1: “Dyssebacia”—A Fifth D of Pellagra

PRACTICAL GASTROENTEROLOGY • MARCH 2003 41

A ttention history buffs and aficionados of medicalarcana. Here’s something for you. .............. ..

Pellagra (pelle agra, rough skin) is tradition-ally portrayed as a niacin-deficiency disorder resultingfrom a diet of three Ms—meat (fatback), meal (corn-meal), and molasses—and manifesting clinically asfour Ds (dermatitis, diarrhea, dementia, and death).Approximately one-fifth of pellagrins, however, mani-fest a fifth D—“dyssebacia” (1). In this report, I sum-marize the essential features of “dyssebacia” and illus-trate this finding in three patients.

“Dyssebacia” is the name coined to describe numer-ous plugs of inspissated sebum projecting from dilatedorifices of sebaceous glands (Figures 1–4). Histologi-c a l l y, the glands show hyperplasia with sebaceous material plugging dilated follicles. On palpation, theplugs feel like sharkskin or sandpaper. They first appearalong the alae nasi, then spread over the nose, and inadvanced cases, involve the forehead, lips, and chin.When small, the plugs reflect light and mimic sulfurflakes. The larger plugs range in color from white tograyish yellow, occasionally with darker tips. Their

“Dyssebacia”—A Fifth D of Pellagra

PEARLS OF GASTROENTEROLOGY

Herbert L. Fred, M.D., Professor of Internal Medicine,The University of Texas Health Science Center, Hous-ton, Texas.

Herbert L. Fred

Figure 1. Nasal area of a 65-year-old woman with pellagrashowing diffuse sebaceous plugging, especially prominenton the left.

Figure 2. Side view of the patient in Figure 1 showing plugsover the nose, cheek, forehead, and scalp.

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“Dyssebacia”—A Fifth D of Pellagra

prevalence increases with age of the patient and ishigher in blacks than in whites.

In contrast to pellagral dermatitis, which isinflammatory and typically develops on sun-exposedsurfaces (Figure 5), “dyssebacia” is not inflammatory,is usually confined to the face, and is unrelated to sun-light. Furthermore, it sometimes precedes the dermati-tis by weeks to months (2). With niacin therapy, the

sebaceous plugs fall out within several days, and thelesions clear completely within a week or so.

COMMENTThe three patients shown here came under my care 40years ago. Since that time, pellagra has virtually disap-peared in the United States. Nevertheless, it still occurselsewhere—even in epidemic proportions (3)—and westill need to know what it looks like. ■

References1. Smith SG, Smith DT, Callaway JL. Dysfunction of the sebaceous

glands associated with pellagra. J Invest Dermat, 1941; 4:23-42.2. Smith DT. Nicotinic acid deficiency (pellagra). M Clin North

America, 1943; 27:379-397.3. Outbreak of pellagra among Mozambican refugees—Malawi,

1990. MMWR Morb Mortal Wkly Rep 1991 (April 5); 40 (issue13): 209-213.

Figure 3. Right paranasal and infraorbital area of a 63-year-old pellagrin with typical sebaceous plugging.

Figure 4. Nasal area of patient in Figure 5 showing diffusesebaceous plugging.

Figure 5. A 47-year-old man with classic pellagral dermati-tis—scaly, reddened, and hyper-pigmented lesions distrib-uted symmetrically over sun-exposed surfaces.