2026 ICD-10-CM Diagnosis Code L10.81Paraneoplastic pemphigus

AHRQ’s CCSR groups this code into broader clinical categories.

• Desmoglein 1

  a desmosomal cadherin that is an autoantigen in the acquired skin disorder pemphigus foliaceus.

• Pemphigus

  group of chronic blistering diseases characterized histologically by acantholysis and blister formation within the epidermis.

• Pemphigus, Benign Familial

  an autosomal dominantly inherited skin disorder characterized by recurrent eruptions of vesicles and bullae mainly on the neck, axillae, and groin. mutations in the atp2c1 gene (encoding the secretory pathway ca2++/mn2++ atpase 1 (spca1)) cause this disease. it is clinically and histologically similar to darier disease - both have abnormal, unstable desmosomes between keratinocytes and defective calcium-transporting atpases. it is unrelated to pemphigus vulgaris though it closely resembles that disease.

• ATP2C1 wt Allele|ATP2C1A|ATPase Secretory Pathway Ca2+ Transporting 1 wt Allele|ATPase, Ca(2+)-Sequestering Gene|ATPase, Ca(2+)-Transporting, Type 2c, Member 1 Gene|ATPase, Ca++ Transporting, Type 2C, Member 1 Gene|BCPM|Benign Chronic Pemphigus (Hailey-Hailey Disease) Gene|HHD|HUSSY-28|KIAA1347|PMR1|PMR1, Rat, Homolog of Gene|SPCA1|Secretory Pathway Ca(2+) ATPase 1 Gene|Secretory Pathway Ca2+/Mn2+ ATPase 1 Gene|hSPCA1

  human atp2c1 wild-type allele is located in the vicinity of 3q22.1 and is approximately 166 kb in length. this allele, which encodes calcium-transporting atpase type 2c member 1 protein, plays a role in the transport of ions into the golgi to regulate the trafficking of newly synthesized proteins via the secretory pathway. mutation of the gene are associated with familial benign pemphigus.

• Desmoglein-1|Cadherin Family Member 4|DESMOGLEIN 1|DG1|DGI|DSG1|Desmoglein 1|Desmosomal Glycoprotein 1|Pemphigus Foliaceus Antigen

  desmoglein-1 (1049 aa, ~114 kda) is encoded by the human dsg1 gene. this protein is involved in cell-cell adhesion and keratinization.

• Desmoglein-3|130 kDa Pemphigus Vulgaris Antigen|Cadherin Family Member 6|DESMOGLEIN 3|DSG3|Desmoglein 3|PVA

  desmoglein-3 (999 aa, ~108 kda) is encoded by the human dsg3 gene. this protein plays a role in desmosome-mediated cell-cell adhesion.

• Desmoplakin|250/210 kDa Paraneoplastic Pemphigus Antigen|DP

  desmoplakin (2871 aa, ~332 kda) is encoded by the human dsp gene. this protein is involved in the maintenance of desmosome structure.

• DSG3 Antibody Positive|Anti-DSG3 Antibody Positive|Anti-Desmoglein 3 Antibody Positive|Anti-Desmoglein-3 Antibody Positive|Anti-PVA Antibody Positive|CDHF6 Antibody Positive|Cadherin Family Member 6 Antibody Positive|Desmoglein 3 Antibody Positive|Desmoglein-3 Antibody Positive|PVA Antibody Positive|Pemphigus Vulgaris Antigen Antibody Positive

  an indication that antibodies that recognize desmoglein-3 (dsg3) have been detected in a sample.

• DSG3 wt Allele|ABOLM|CDHF6|Desmoglein 3 (Pemphigus Vulgaris Antigen) Gene|Desmoglein 3 wt Allele|PVA|Pemphigus Vulgaris Antigen Gene

  human dsg3 wild-type allele is located in the vicinity of 18q12.1 and is approximately 31 kb in length. this allele, which encodes desmoglein-3 protein, is involved in the modulation of cell-cell adhesion.

• Familial Benign Pemphigus|Benign Familial Pemphigus|Hailey-Hailey Disease

  an autosomal dominant disorder characterized by a history of multiple relapses and remissions of pemphigus lesions.

• Mucosal-Dominant Pemphigus Vulgaris|Mucosal Dominant Pemphigus Vulgaris|mPV

  a subtype of pemphigus vulgaris in which lesions are predominantly found on mucosal membranes with relative sparing of cutaneous tissues.

• Pemphigus

  a blistering skin disorder. morphologically it is characterized by acantholysis and intraepidermal blister formation.

• Pemphigus Vulgaris

  an autoimmune blistering disorder. it is characterized by the presence of painful blisters and erosions in the skin and mucous membranes.

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