Prurigo Pigmentosa

Prurigo Pigmentosa

Accepted Manuscript Title: Prurigo Pigmentosa Author: Daisuke Yamada, Tatsuya Fujikawa PII: DOI: Reference: S0002-9343(17)30985-3 https://doi.org/doi...

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Accepted Manuscript Title: Prurigo Pigmentosa Author: Daisuke Yamada, Tatsuya Fujikawa PII: DOI: Reference:

S0002-9343(17)30985-3 https://doi.org/doi:10.1016/j.amjmed.2017.08.043 AJM 14290

To appear in:

The American Journal of Medicine

Please cite this article as: Daisuke Yamada, Tatsuya Fujikawa, Prurigo Pigmentosa, The American Journal of Medicine (2017), https://doi.org/doi:10.1016/j.amjmed.2017.08.043. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.

Prurigo pigmentosa

Daisuke Yamada, #1 M.D., Tatsuya Fujikawa, #2 M.D., Ph.D. #1, The Post-graduate Education Center, #2, Department of General Internal Medicine, Mitoyo General Hospital, 708 Himehama Toyohama, Kanonji, Kagawa 769-1695, Japan Phone: +81-8-7552-3366 Fax: +81-8-7552-4936 E-mail: [email protected]

Corresponding author: Tatsuya Fujikawa Department of General Internal Medicine, Mitoyo General Hospital 708 Himehama Toyohama, Kanonji, Kagawa 769-1695, Japan Phone: +81-8-7552-3366 Fax: +81-8-7552-4936 E-mail: [email protected]

Daisuke Yamada contributed to patient care, wrote manuscript, and contributed to discussion. Tatsuya Fujikawa reviewed and edited manuscript and contributed to discussion. We do not have any funding sources in the writing of the manuscript or the decision to submit for publication. I, Tatsuya Fujikawa, had full access to all the data in the study

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and the final responsibility for the decision to submit for publication. We do not have any conflict of interest.

To the editor: A 49-year-old man with type 2 diabetes was admitted to our hospital because of impaired consciousness. The patient was fatigued for 10 days at home. In ER, he was unconscious with a Glasgow Coma Scale of 6; his breathing pattern was classified as Kussmaul breathing. Red papules and reticulate pigmentation were observed on the chest (Figure 1), abdomen, and back (Figure 2) of the patient; this condition did not appear the previous day while the patient was at home. Laboratory data revealed dehydration, electrolytic imbalance, hyperglycemia (862 mg/dl), acidosis (pH 6.80), and urinary ketones. The patient was diagnosed with ketoacidosis due to cessation of diabetes therapy. Prurigo pigmentosa was diagnosed by a dermatologist based on the presence of red papules and reticulate pigmentation. After the treatment of blood glucose levels and dehydration, consciousness was regained. The red papules and reticulate pigmentation mostly resolved without administration of specific medication. The patient was discharged after his blood glucose level stabilized on day 17.

Prurigo pigmentosa is a rare inflammatory dermatosis of unknown etiology and is characterized by a rash that consists of itchy, reddish papules coalescing to form a reticulated pattern1. It commonly presents on the back, chest, and neck1,2. Disease progression is divided into three stages: early, fully developed, and late. Each stage is

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distinguished by unique clinical and histologic features1,2. The mechanism of pathogenesis of prurigo pigmentosa may be related to neutrophil-mediated inflammation1,2. Alternatively, mechanical stimulation, contact allergy, or climate may contribute to its development1,2. Some studies have indicated underlying diseases, such as adult-onset Still’s disease, atopy, Helicobacter pylori infection, and Sjögren's syndrome, in patients with prurigo pigmentosa1. Patients with conditions that can potentially lead to ketosis, such as anorexia nervosa and diabetes, may also be at a higher risk of developing prurigo pigmentosa1,2,3. Although glycemic control along with disappearance of ketonuriacontrol can be used to resolve prurigo pigmentosa3, dapsone or minocyclin also may be effective treatments1,2; both agents inhibit migration and/or function of neutrophils2. Physicians should consider prurigo pigmentosa when unconscious patients with diabetes present with characteristic exanthema patterns. If such a case arises, ketoacidosis should be managed appropriately.

Reference: 1. Beutler BD, Cohen PR, Lee RA. Prurigo Pigmentosa: Literature Review. Am J Clin Dermatol. 2015; 16(6):533-43. 2. Satter E, Rozelle C, Sperling L. Prurigo Pigmentosa: An under-recognized inflammatory

dermatosis

characterized

by

an

evolution

of

distinctive

clinicopathological features. J Cutan Pathol. 2016; 43(10):809-14. 3. Hanami Y, Yamamoto T. Bullous prurigo pigmentosa in a pregnant woman with hyperemesis gravidarum. J Dermatol. 2015; 42(4):436-7.

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Figure legend: Red papules and reticulate pigmentation were observed on the chest (Figure 1), and back (Figure 2) of the patient.

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