CLINICAL IMAGING Goose skin in a girl with Ehlers Á Danlos syndrome Jared Martı´nez-Coronado, MD, Bertha Torres-Alvarez, MD and Juan Pablo Castanedo-Ca ´ zares, MD* Department of Dermatology, Hospital Central Dr. Ignacio Morones Prieto, Universidad Auto ´ noma de San Luis Potosı ´, San Luis, Me ´ xico Pellagra is a nutritional disorder secondary to niacin deficiency. The classical triad is dermatitis, diarrhea, and dementia. We report the case of a young girl with hypermobility-type Ehlers ÁDanlos syndrome who exhibited the classical pellagra symptoms, despite apparent adequate nutritional intake. Her condition resolved after oral niacin supplements were administered. Although this association has not previously been recognized, an inherited connective tissue disorder may be related to the appearance of pellagra. Keywords: pellagra; Ehlers ÁDanlos syndrome; nicotinamide; goose skin *Correspondence to: Juan Pablo Castanedo-Ca ´zares, Department of Dermatology, Hospital Central Dr. Ignacio Morones Prieto, 2395 Carranza Ave, CP 78290, San Luis, San Luis Potosı ´, Me ´xico, Email: castanju@yahoo.com Received: 25 April 2015; Revised: 13 July 2015; Accepted: 28 July 2015; Published: 1 September 2015 A 7-year-old girl from a rural area located in San Luis Potosı ´, Me ´xico, who exhibited normal food intake, occasionally loose stools and who had been diagnosed with hypermobility-type Ehlers ÁDanlos syndrome 4 years before. She presented with a 1-month history of occasional itching; symmetrical dermatosis located on the face, neck, anterior upper thorax; and external genitalia. The dermatosis was characterized by hyperkeratotic, hyperpigmented, and erythematous pla- ques (Figs. 1 and 2). The patient manifested more than 10 diarrheic evacuations per day, daily episodes of 398C fever, hallucinations, and seizures; thus, her rural health center referred her to our hospital. On physical examina- tion, her stature and weight were inappropriately low for her age (5th percentile), and she presented generalized weakness that was more evident in the upper and lower extremities. Her blood test revealed a normocytic normo- chromic anemia with 5.5 g/dl of hemoglobin. Serum electrolytes and renal function tests were normal, and stool tests were negative to parasites. Electroencephalo- gram revealed generalized dysfunction during physiologi- cal sleep. Pellagra was diagnosed based on the classical triad of dermatitis, diarrhea, and neurologic findings supported by low niacin serum levels below 0.5 ng per milliliter. The patient was treated with a multivitamin intravenous supplement that included nicotinamide 10 mg every 6 hours, blood transfusions, and emollient ointment. The patient showed dermatologic, neurological, and gas- trointestinal improvement and was discharged 5 days later Fig. 1. Cutaneous manifestations of pellagra. Thickened, scaly, and hyperpigmented skin on the photoexposed areas of the face in a girl with Ehlers ÁDanlos disease. JOURNAL OF COMMUNITY HOSPITAL INTERNAL MEDICINE PERSPECTIVES æ Journal of Community Hospital Internal Medicine Perspectives 2015. # 2015 Jared Martı´nez-Coronado et al. This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International License (http://creativecommons.org/licenses/by-nc/4.0/), permitting all non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. 1 Citation: Journal of Community Hospital Internal Medicine Perspectives 2015, 5: 28216 - http://dx.doi.org/10.3402/jchimp.v5.28216 (page number not for citation purpose)