Hematologic Diseases at a Glance
- Mucocutaneous changes (i.e., pallor, jaundice, flushing, erythema, and cyanosis) often predict underlying hematologic pathology.
- Anemia is best differentiated by mean corpuscular volume value.
- Megaloblastic anemia of vitamin B12 deficiency is distinguished from folate deficiency by lack of neurologic signs in folate deficiency.
- Carcinoid tumor results in flushing, typically for 10 minutes or less; in comparison, flushing from mastocytosis lasts 30 minutes or more.
- Polycythemia vera presents with acrocyanosis, aquagenic pruritus, urticaria, Sweet syndrome, and purpura.
- The morphologic diagnosis of purpura begins with three Ps: (1) is the lesion purpuric, (2) is it primary, and (3) is it palpable?
- Leukemia cutis is a localized or disseminated skin infiltration by leukemic cells. It is a sign of dissemination or systemic disease or relapsing leukemia.
Hematology includes the study of blood and blood-forming tissues. Given the number and variety of hematologic disorders, it is not surprising that mucocutaneous manifestations of hematologic disorders are common. Many hematologic malignancies and pseudomalignancies can involve the skin through atypical cell infiltration (specific lesions), and some of these may present preferentially in the skin. These conditions, such as leukemias and lymphomas, plasma cell dyscrasias including myeloma, and Langerhans and non-Langerhans histiocytoses, are discussed in other chapters. Likewise, complications of chemotherapy, radiotherapy, cytokine use, or marrow transplantation are discussed elsewhere.
The first section of this chapter is directed toward the differential diagnosis of purpura with an emphasis on those subsets of purpura with important underlying hematologic abnormalities. The second major section of this chapter is directed toward nonspecific (nontumor-containing) findings that may be associated with hematologic disorders. Although the ready availability of laboratory testing has made early recognition of such conditions as anemia and polycythemia much less dependent on physical findings, cutaneous associations still may aid in the diagnosis of a hematologic condition or complicate its management. A comprehensive listing of these associations is presented in Table 144-1.
Table 144-1 Cutaneous Findings with Hematologic Associations |Favorite Table|Download (.pdf)
Table 144-1 Cutaneous Findings with Hematologic Associations
- Generalized hyperpigmentation
- POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin lesions)3,4
- Hyperpigmentation, 93%–98%; apparent skin thickening, 77%–85%; hypertrichosis, 78%–81%; peripheral edema, ∼90%; digital clubbing, 56%; white fingernails; verrucous angiomata, telangiectasia
- Hemochromatosis may have bronze or grayish pigmentation of the skin
- Megaloblastic anemia of vitamin B12 or folate deficiency
- Fanconi anemia5,6
- Begins age 4–10 years with skin or hematologic presentation
- Generalized hyperpigmentation, especially lower trunk, flexures, neck, 85%
- Scattered darker and lighter macules within hyperpigmented areas
- Progressive hypoplastic anemia with pancytopenia
- Death from leukemia, other neoplasms, or infections 2–5 years after onset
- Dyskeratosis congenita7
- Nail dystrophy, beginning 5–13 years of age
- Fine reticulate gray-brown hyperpigmentation, especially neck, thighs, and trunk
- Atrophic skin with telangiectasis
- Oral leukoplakia
- Mucocutaneous carcinomas, occasional pancreatic carcinoma, Hodgkin disease
- Frequent blood dyscrasias, aplasias, ...