Hodgkin's lymphoma, skin manifestations C81.9

Author: Prof. Dr. med. Peter Altmeyer

All authors of this article

Last updated on: 18.11.2021

Dieser Artikel auf Deutsch

Synonym(s)

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

History
This section has been translated automatically.

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

Definition
This section has been translated automatically.

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.

Classification
This section has been translated automatically.

Occurrence/Epidemiology
This section has been translated automatically.

Incidence: 2-3/100.000 inhabitants/year.

Etiopathogenesis
This section has been translated automatically.

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

Manifestation
This section has been translated automatically.

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

Clinical features
This section has been translated automatically.

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

  • Blurred, squamous or nodular infiltrates.
  • Single, multiple or papulo-nodular exanthema, brownish to livid red cutaneous subcutaneous nodules.
  • Tendency to ulceration: Ulcus lymphogranulomatosus, 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, 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.

Autoimmune diseases and Hodgkin's lymphoma: Hodgkin's lymphoma is associated with various autoimmune diseases or diseases from the atopic form group (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.

Laboratory
This section has been translated automatically.

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

Histology
This section has been translated automatically.

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.

Diagnosis
This section has been translated automatically.

Trial excision of an enlarged lymph node.

Differential diagnosis
This section has been translated automatically.

Therapy
This section has been translated automatically.

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

Progression/forecast
This section has been translated automatically.

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

Literature
This section has been translated automatically.

  1. Ambinder R (2003) Infection and lymphoma. N Engl J Med 349: 1309-1311
  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.
  4. Hjalgrim H et al (2003) Characteristics of Hodgkin's lymphoma after infectious mononucleosis. N Engl J Med 349: 1324-1332
  5. Hodgkin T (1832) On some morbid appearances of the absorbent glands and spleen. Medico-Chirurgical Transactions (London) 17: 68-114
  6. Khalifeh I et al. (2009) Solitary plaque on the scalp as a primary manifestation of Hodgkin lymphoma: a case report and review of the literature. J Cutan Pathol 36 Suppl 1:80-85.
  7. Landgren O et al (2006) Autoimmunity and susceptibility to Hodgkin lymphoma: a population-based case-control study in Scandinavia. J Natl Cancer Inst 98:1321-1330.
  8. Listinsky CM et al (2002) A practical approach to the diagnosis of Hodgkin lymphoma. Am J Clin Pathol 117S: S76-94.
  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-.
  18. Winzer M, Müller W, Arnholdt H (1991) Specific skin infiltrates in Hodgkin's disease. Dermatologist 42: 648-651

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

Authors

Last updated on: 18.11.2021