Hodgkin's lymphoma, skin manifestations C81.9

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 17.12.2022

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Hodgkin M.; Hodgkin's disease; Hodgkin's sarcoma; Lymphogranuloma papulosum disseminatum; Lymphogranulomatosis; lymphogranulomatosis maligna; lymphogranulomatous ulcer; Paltauf Steinberg disease; Paltauf-Steinberg disease

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Hodgkin, 1832; Paltauf, 1897; Sternberg, 1898; Reed, 1902

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A malignant disease of the lymphoreticular system histologically characterized by atypical mononuclear (Hodgkin cells) or polynuclear (Sternberg-Reed cells) atypical cells in a granulomatous tissue. Clinically unilocular onset, spread via adjacent lymph node stations and later hematogenic dissemination.

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Incidence: 2-3/100.000 inhabitants/year.

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Unclear, possible virus infection (Epstein-Barr virus).

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Especially 3rd to 4th decade of life, possible at any age. Men are 1-2 times more frequently affected than women.

Clinical features
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Specific skin lesions (0.5-3.5% of patients):

  • Blurred, squamous or nodular infiltrates.
  • Single, multiple, or papulo-nodular exanthema-impressing, brownish to livid-red, cutaneous-subcutaneous nodules.
  • Tendency to ulceration: lymphogranulomatous ulcer, especially on the trunk, lower abdomen, inguinal and thigh regions, and head.
  • Specific infiltrates in the lymphatic pharyngeal ring with tendency to ulceration.

Nonspecific skin lesions (in 30-50% of patients):

  • Pruritus: Excruciating pruritus with scratching effects, impetiginization, eczematization and lichenification, erythroderma, blistering, which can hardly be influenced by medication,
  • Chronic prurigo (old name: prurigo lymphogranulomatotica)
  • Hyperpigmentation: Diffuse, morbus-Addison-like hyperpigmentation on skin and mucous membranes.
  • Ichthyotic skin lesions
  • Zoster generalisatus
  • Pyoderma
  • Molluscum contagiosum (dissemnierts occurrence possible, see Fig.)

Autoimmune diseases and Hodgkin's lymphoma: Hodgkin's lymphoma is associated with various autoimmune diseases or diseases from the atopic form circle (Landgren O et al. 2006). These include:

General symptoms of Hodgkin's lymphoma:

  • Initial symptoms are often lymph node enlargement, especially in the neck and axillae. Furthermore B symptoms: weight loss, night sweats.
  • In about one third of patients, lymph node pain a few minutes after alcohol consumption, relapsing lymphogranulomatous fever (Pel-Ebstein fever) with recurrent character.
  • In early stage: Few days of fever alternating with fever-free intervals. In the late stage: appearance of Pel-Ebstein fever with retroperitoneal lymphoma. For detailed clinic and diagnosis see below. Hodgkin's lymphoma.

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Absolute lymphocytopenia in 3/4 of the patients in the phase of generalization. SPA elevation, increase in γ globulins.

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Depending on the cell infiltrate, a distinction is made between 5 different forms (see also under Hodgkin's lymphoma):

  1. Diffuse lymphocytic and/or histiocytic form (approx. 5% of cases).
  2. Nodular lymphocytic and/or histiocytic form (approx. 80% of cases)
  3. Mixed cell form (approx. 10-15% of cases)
  4. Diffuse fibrosis (approx. 1% of cases)
  5. Lymphocyte-poor form (approx. 1% of cases)

In the early phase, reticulum cell proliferations are seen in the lymph nodes, then an interspersion with neutrophil and eosinophil granulocytes, scarring, mononuclear (Hodgkin cells) and multinucleated (Sternberg-Reed giant cells), and atypical reticular cells. Skin biopsies are often less characteristic. Hodgkin and Sternberg-Reed cells are CD30/MIB1 positive.

An assignment of the specific skin infiltrates to the individual Hodgkin subtypes has not yet been made.

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Trial excision of an enlarged lymph node.

Differential diagnosis
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Polychemotherapy or radiotherapy by haematologists or radiotherapists. For further details see below. Hodgkin's lymphoma.

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10-year survival rate of 60%. The prognosis will be less favourable one to five years.

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  2. Browne P et al (2003) The B-cell transcription factors BSAP, Oct-2, and BOB.1 and the pan-B-cell markers CD20, CD22, and CD79a are useful in the differential diagnosis of classic Hodgkin lymphoma. Am J Clin Pathol 120: 767-777
  3. Durmus Ö et al (2020) Mucous membrane pemphigoid in a patient treated with nivolumab for Hodgkin's lymphoma. Dermatol Ther 33:e14109.
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  9. Macaya A et al (2003) Cutaneous granulomas as the first manifestation of Hodgkin's disease. Eur J Dermatol 13: 299-301
  10. Paltauf R (1889) On the relations of the thymus to sudden death. Vienna Klin Wschr 46: 877-881
  11. Paltauf R (1897) Lymphosarcoma (lymphosarcomatosis, pseuloleukemia, myeloma, chloroma). Results of general pathology and pathological anatomy of man and animals 3: 652-691.
  12. Rahman A et al (2007) Hodgkin's lymphoma in systemic lupus erythematosus. Rheumatology (Oxford) 46:830-832.
  13. Rafiq M et al (2020) Allergic disease, corticosteroid use, and risk of Hodgkin lymphoma: A United Kingdom nationwide case-control study. J Allergy Clin Immunol 145:868-876.
  14. Reed D (1902) On the pathological changes in Hodgkin's disease, with especial reference to its relation to tuberculosis. Johns Hopkins Hospital Reports (Baltimore) 10: 133-196.
  15. Serra-García L et al (2021) Chronic prurigo as an onset of Hodgkin's lymphoma. Med Clin (Barc) 156:47.
  16. Sternberg C (1898) Ueber eine eigenartige, unter dem Bild der Pseudoleukämie verlaufende Tuberkulose des lymphatischen Apparates. Journal of Medical Science (Prague) 19: 21-90.
  17. Wang N et al (2015) Paraneoplastic pemphigus as the first manifestation of non-Hodgkin's lymphoma: a case report and literatures review. Zhonghua Xue Ye Xue Za Zhi 36:702-.
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Last updated on: 17.12.2022