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    OpenPayerBilling CodesBullous disordersICD-10-CM l10-pemphigus
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    ICD-10-CM Code For Pemphigus: Group Overview

    The International Classification of Diseases, Tenth Revision, Clinical Modification (ICD-10-CM) code group L10 includes autoimmune blistering disorders such as pemphigus vulgaris and related pemphigus variants that cause intraepidermal blister formation affecting skin and mucous membranes. These ICD-10-CM diagnosis codes are used to document clinical scenarios including presence of mucosal involvement, extent of skin involvement, and complications such as secondary infection or failure to respond to therapy, capturing disease severity and complexity. Accurate coding within the ICD-10-CM L10 group supports proper claim adjudication, complete clinical documentation, and appropriate reimbursement.

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    ICD-10-CM L10: Pemphigus Overview

    The ICD-10-CM L10 group captures autoimmune blistering disorders characterized by intraepidermal acantholysis leading to flaccid blisters and erosions of the skin and mucous membranes. These conditions primarily affect the skin and mucosal surfaces and can cause significant morbidity due to pain, infection risk, and fluid loss. Accurate coding matters for clinical continuity and reimbursement because it documents disease severity, mucosal involvement, and specific pemphigus subtypes that influence treatment intensity.

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    When to Use

    Use a code from this group when a clinician documents a diagnosis of pemphigus or a specific pemphigus subtype based on clinical evaluation, biopsy, or immunopathology. Assign codes that reflect mucosal involvement, extent of skin disease, and any documented complications to accurately represent clinical complexity.

    ICD-10-CM Codes

    9 rows

    ICD-10-CM CodeDescription
    L10.0Pemphigus vulgaris
    L10.1Pemphigus vegetans
    L10.2Pemphigus foliaceous
    L10.3Brazilian pemphigus [fogo selvagem]
    L10.4Pemphigus erythematosus
    L10.5Drug-induced pemphigus
    L10.81Paraneoplastic pemphigus
    L10.89Other pemphigus
    L10.9Pemphigus, unspecified

    DRG Codes

    2 rows

    DRG CodeDescription
    595Major Skin Disorders with MCC
    596Major Skin Disorders without MCC

    Typical Clinical Scenarios

    • An older adult presents to the emergency department with sudden onset of widespread flaccid blisters and painful erosions on the trunk, limbs, and oral mucosa after several days of progressive skin detachment; Nikolsky sign is positive and the patient is dehydrated from fluid loss and unable to tolerate oral intake. A diagnosis from this group is assigned because the acute, diffuse mucocutaneous blistering is consistent with a severe autoimmune blistering disorder requiring urgent dermatology evaluation and inpatient management. Typical codes: L10.0, L10.1
    • A middle-aged patient followed in dermatology clinic with a months-long history of recurrent tense or flaccid blisters primarily affecting the skin with intermittent oral ulcers; disease activity is monitored while on chronic immunosuppressive therapy and periodic relapses occur when medications are tapered. A diagnosis from this group is assigned to document the chronic autoimmune bullous condition guiding long-term treatment and follow-up. Typical codes: L10.2, L10.9
    • A hospitalized patient with known autoimmune blistering disease develops extensive secondary bacterial infection of large epidermal erosions with systemic signs of sepsis and requires IV antibiotics and wound care; the infection complicates the underlying bullous disorder and prolongs hospitalization. A diagnosis from this group is assigned to identify the underlying pemphigus as the primary dermatologic condition that predisposed to the infectious complication. Typical codes: L10.3, L10.89

    Related Codes

    L11-OTHER-ACANTHOLYTIC-DISORDERS

    ICD-10-CM Code For Other Acantholytic Disorders: Group Overview

    L12-PEMPHIGOID

    ICD-10-CM Code For Pemphigoid: Group Overview

    L13-OTHER-BULLOUS-DISORDERS

    ICD-10-CM Code For Other Bullous Disorders: Group Overview

    L14-BULLOUS-DISORDERS-IN-DISEASES-CLASSIFIED-ELSEWHERE

    ICD-10-CM Code For Bullous Disorders In Diseases Classified Elsewhere: Group Overview

    Frequently Asked Questions

    References

    1. 1.CPT® Codes and Descriptions. AAPC. Retrieved Aug 11, 2026.Maintains the CPT code set's official descriptions and guidelines, licensed from the American Medical Association, used as the definitional source for CPT codes on this page.
    2. 2.HCPCS Quarterly Update. Centers for Medicare & Medicaid Services (CMS). Retrieved Aug 11, 2026.CMS's quarterly release of HCPCS Level II codes and short/long descriptors, the source for HCPCS code definitions and update history.
    3. 3.ICD-10-CM Full Code List. Centers for Medicare & Medicaid Services (CMS). Retrieved Aug 11, 2026.CMS's complete ICD-10-CM diagnosis code list, used to cross-reference diagnosis codes associated with this procedure or service.
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