Hairy Cell Leukemia And Aseptic Necrosis Of The Femoral Head

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Hairy cell leukemia

The clinical characteristics of neoplastic associated mucocutaneous syndromes are summarized in Table 2.

4.1. Acanthocytosis Nigricans

This type of dermatosis occurs most commonly on the posterior neck, axilla and groin and presents as confluent small hyperkeratotic papules with hy-perpigmented mass-like appearance. The pathologic features of the lesion are hyperkeratosis and papillomatosis. Although this dermatosis can occur as a benign familial form in children and in endocrinopathies, its appearance in an adult, especially on musocal membranes, is alarming. This type of lesion is frequently associated with abdominal adenocarcinoma, most commonly, gastric [14].

4.2. Acquired Ichthyosis

This type of lesion presents as small white scales with free edges distributed on trunk and extremities. Other features that may be present are moderate hyperkeratosis and mild acanthocytosis. Acquired ichthyosis has been associated with endocrinopathies, acquired immunodeficiency, sarcoidosis and cholesterol-lowering drugs. The malignancies associated with this lesion are mainly Hodgkin's lymphoma, but carcinoma of breast, cervix and lung have also been described.

4.3. Amyloidosis

Light chain amyloidosis (AL-amyloid) is most frequently associated with multiple myeloma. The cutaneous manifestations of AL-amyloidosis included purpura, plaques, scleroderma-like infiltration, alope cia, nail changes and bulbous eruptions. In contrast, secondary amyloidosis (AA), which occurs in association with renal cell carcinoma and other types of solid cancers, presents with parenchymal involvement.

4.4. Bazer Syndrome

This syndrome, also called acrokeratosis paraneoplas-tica, is characterized by three clinical stages: (1) psoriasiform lesions initially appearing on acral areas; (2) palmar and plantar keratoderma associated with nail abnormalities; and (3) generalized psoriasiform plaques.

The detection of cross reactivity between antigens in the tumor and epidermal antigens as well as secretion of growth factors by the tumor cells TGF-a and ILGF-I, have been postulated as pathogenic mechanisms for the dermatosis [15].

4.5. Dermatomyositis

Dermatomyositis is an inflammatory myopathy manifested mainly by proximal muscle weakness as well as other systemic organ involvement such as heart, lung and gastrointestinal tract. The pathognomonic skin lesions are the heliotrope rash (edematous violaceous changes of periorbital areas) and the Gottron's papules (erythematous dermal scaling overlying the metacarpo-phalangeal joints). Other cutaneous manifestations are malignant erythema, photosensitivity, cuticular hypertrophy with erythema and red scaly pruritic scalp rash. About 25% of patients with dermatomyositis, especially those in whom the disease starts at the age of 50 and over, will have cancer. The types of solid tumors associated with this dermatosis seem to approximate those that occur in the general population [16].

4.6. Digital Clubbing and Hypertrophic Osteoarthropathy

Digital clubbing is characterized by thickening of the distal phalanges, increased convexity of the nail plate and thickening of the nail bed. Clubbing can be primary (hereditary or idiopathic) or secondary to systemic diseases such as chronic lung disorders, endocarditis, gastrointestinal disorders or hyperthyroidism. In about 10-20% of patients, the clubbing is associated with hypertrophic osteoarthropathy that includes clubbing, painful swelling and tenderness of the distal phalanges. In this type of lesion, there is subperiosteal edema with new bone formation along the shafts of the tubular bones of the limbs. In 90% of patients with this condition, a nonsmall cell lung carcinoma is usually detected.

Other malignancies that have been noted are intrathoracic tumors and solid tumors with lung metastasis.

4.7. Erythema Gyratum Repens

This type of lesion has a distinctive clinical presentation of an advancing weave-like erythema producing a "striped" appearance of the affected skin. The typical areas affected are the trunk and proximal limbs. In most of the patients an associated malignancy is present or subsequently discovered, most commonly lung cancer and tumors of the genitourinary tract.

4.8. Erythroderma and Exfoliative Dermatitis

The clinical presentation of this lesion involves a widespread redness and skin inflammation. In most of the cases, exfoliation and pruritus are present, as well as other systemic symptoms such as adenopathy, chills and malaise. Erythroderma is often associated with hematological malignancies, especially cutaneous T-cell lymphoma, Hodgkin's disease and leukemias.

4.9. Erythromelalgia

Erythomelalgia is characterized by attacks of erythema, severe burning pain and warmth of the limbs. Symptoms are precipitated by exercise and heat and relieved by cold exposure and elevation of the limb. Erythromelalgia can be idiopathic or secondary to a variety of nonmalignant systemic disorders such as diabetes mellitus, pregnancy, systemic lupus erythematosus, rheumatoid arthritis and drug reaction. In about 20% of patients, an associated myeloproliferative disorder can be detected often presenting with thrombocytosis.

4.10. Extramammary Paget's Disease

This lesion typically appears as an erythematous exudative dermatosis in the groin, perineum and perianal areas. The disease is actually a cutaneous adenocar-

cinoma that is associated with a solid tumor in 50% of patients. The site of the visceral tumor corresponds to the location of the dermatosis adenocarcinoma of the digestive tract for perianal involvement and genitourinary tract malignancy for perineal-groin involvement.

4.11. Florid Cutaneous Papillomatosis

The clinical presentation of the skin lesion includes the sudden appearance of multiple cutaneous verrucous papillomas on the trunk, extremities and finally the face. This type of paraneoplastic manifestation has been associated with malignancy in all reported cases, mostly with adenocarcinoma of the stomach.

4.12. Hypertrichosis Lanugiosa Acquisita

This skin lesion is characterized by the appearance of colorless, fine-textured hair growth which is generalized and spares only the palms and soles. This cutaneous syndrome has been associated with lung and colorectal carcinoma.

4.13. Multicentric Reticulohistiocytosis

Multicentric reticulohistiocytosis is characterized by papunodular skin lesions and polyarthritis affecting hands, knees, shoulders, hips and spine, that progresses to arthritis mutilans in 50% of the cases. The papular and nodular skin lesions involve the upper half of the body, especially the face. In about 30% of the cases, a solid tumor has been discovered with no predominant type.

4.14. Palmar Fasciitis

This syndrome is manifested by thickening of the palmar fascia thus causing flexion contracture of fingers. This type of lesion is often associated with solid tumors, most commonly ovarian and lung cancer, however, its existence is a poor prognostic sign since it manifests only after the tumor is already metastatic.

4.15. Paraneoplastic Pemphigus

This paraneoplastic syndrome is characterized by widely distributed lesions which include erythema tous patches, vesicles, bullae, papules and plaques. The clinical picture can also include mucosal lesions presenting as painful blisters or erosions of the oral cavity and conjuctivas. In addition to the microscopic features, patients with this syndrome have characteristic serum immunoprecipitation findings. This type of lesion is present mainly in patients with chronic lymphocytic leukemia and non-Hodgkin's lymphoma, but can appear also in other neoplasms.

4.16. Pruritus

Although pruritus is a common manifestation that can appear with a variety of systemic diseases, when a patient presents with generalized itching without any apparent disorder, malignancy can be detected in about 10% of cases. The most common malignancies associated with pruritus are Hodgkin's lymphoma and cutaneous T-cell lymphoma. Less commonly, pruritus can occur in leukemia, polycythemia vera and a variety of solid tumors.

4.17. Sweet's syndrome

This syndrome originally described in 1964, is a complex of fever, neutrophilia and erythematous painful, cutaneous plaques of the upper extremities, neck and head. Systemic involvement of eyes, muscles, joints, lungs and liver has also been described. Although most of the cases are idiopathic, malignancy develops in up to 20% of patients [17]. The most commonly associated malignancy is acute myelogenous leukemia. Other less frequently reported neoplasms are solid tumors of the breast, genitourinary tract and colon.

4.18. Vasculitis

The vasculitis associated cutaneous lesions are polymorphous and their pathogenesis is unknown, although it has been suggested that tumors release some cytokines which destroy the vascular bed. Paraneoplastic vasculitis can present pathologically with or without septal panniculitis. About 5-20% of patients with vasculitis have an associated malignancy, most commonly hematologic (hairy cell leukemia, lymphomas and multiple myeloma).

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