L44.0 - Pityriasis rubra pilaris

Approximate Synonyms

The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

• Atypical adult pityriasis rubra pilaris
• Atypical juvenile pityriasis rubra pilaris
• Circumscribed juvenile pityriasis rubra pilaris
• Classical adult pityriasis rubra pilaris
• Classical juvenile pityriasis rubra pilaris
• Disorder of skin due to human immunodeficiency virus infection
• Nail dystrophy due to pityriasis rubra pilaris
• Pityriasis rubra pilaris
• Pityriasis rubra pilaris
• Pityriasis rubra pilaris
• Pityriasis rubra pilaris due to human immunodeficiency virus infection

Clinical Information

• Pityriasis Rubra Pilaris-. a chronic skin disease characterized by small follicular papules, disseminated reddish-brown scaly patches, and often, palmoplantar hyperkeratosis. the papules are about the size of a pin and topped by a horny plug.
• Pityriasis Rubra Pilaris-. a rare cutaneous disorder of unknown etiology characterized by the presence of reddish-orange scaly plaques, palmoplantar keratoderma, keratotic follicular papules, and confluent erythematous areas with areas of normal skin between the lesions.

Diagnostic Related Groups - MS-DRG Mapping

The is diagnosis code is grouped in the following groups for version MS-DRG V40.0 What are Diagnostic Related Groups?
The Diagnostic Related Groups (DRGs) are a patient classification scheme which provides a means of relating the type of patients a hospital treats. The DRGs divides all possible principal diagnoses into mutually exclusive principal diagnosis areas referred to as Major Diagnostic Categories (MDC). applicable from 10/01/2022 through 09/30/2023.

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Patient Education

Skin Conditions

Your skin is your body's largest organ. It covers and protects your body. Your skin:

• Holds body fluids in, preventing dehydration
• Keeps harmful microbes out, preventing infections
• Helps you feel things like heat, cold, and pain
• Keeps your body temperature even
• Makes vitamin D when the sun shines on it

Anything that irritates, clogs, or inflames your skin can cause symptoms such as redness, swelling, burning, and itching. Allergies, irritants, your genetic makeup, and certain diseases and immune system problems can cause rashes, hives, and other skin conditions. Many skin problems, such as acne, also affect your appearance.

NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases

Familial pityriasis rubra pilaris

Familial pityriasis rubra pilaris is a rare genetic condition that affects the skin. The name of the condition reflects its major features: The term "pityriasis" refers to scaling; "rubra" means redness; and "pilaris" suggests the involvement of hair follicles in this disorder. Affected individuals have a salmon-colored skin rash covered in fine scales. This rash occurs in patches all over the body, with distinct areas of unaffected skin between the patches. Affected individuals also develop bumps called follicular keratoses that occur around hair follicles. The skin on the palms of the hands and soles of the feet often becomes thick, hard, and callused, a condition known as palmoplantar keratoderma.

Researchers have distinguished six types of pityriasis rubra pilaris based on the features of the disorder and the age at which signs and symptoms appear. The familial form is usually considered part of type V, which is also known as the atypical juvenile type. People with familial pityriasis rubra pilaris typically have skin abnormalities from birth or early childhood, and these skin problems persist throughout life.

Code History

• FY 2023 - No Change, effective from 10/1/2022 through 9/30/2023
• FY 2022 - No Change, effective from 10/1/2021 through 9/30/2022
• FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
• FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
• FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
• FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
• FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
• FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016 (First year ICD-10-CM implemented into the HIPAA code set)