Scleromyxedema

Synonyms:

Lichen myxedematosus, papular mucinosis, Arndt-

Gottron syndrome

Etiology:

Unknown

Associations:

Paraproteinemia, multiple myeloma, hepatitis C, HIV

disease, L-tryptophan syndrome

Clinical:

Coalescing indurated waxing papules

Histology:

Increased dermal mucin with increased fibroblasts

IHC repertoire:

N/A

Staging:

N/A

Prognosis:

Slowly progressive—increased mortality rate

Adverse variables:

Monoclonal gammopathy

Treatment:

Careful screening

Scleromyxedema is a cutaneous condition that is associated with paraproteinemia in more than 80% of cases (1). In the vast majority of cases, the paraprotein is an IgG heavy chain and a y light chain. Rare cases of K light chains have been reported, and polyclonal hypergammopathy is also seen in some cases. It has been reported that only 10% of patients with scleromyxedema and associated monoclonal gammopathy will progress to multiple myeloma (1). Overall, scleromyxedema is associated with an increased mortality rate, probably due to the manifold systemic associations.

Less commonly, scleromyxedema has been seen in association with Hodgkin's lymphoma, non-Hodgkin's lymphoma, leukemia, and Waldenstrom's macroglobulinemia (1,2). In about one quarter of patients, proximal muscle weakness may be encountered. Less commonly, peripheral neuropathies and central nervous system aberrations may be seen (3). Associated joint disease is characterized as a seronegative polyarthritis. Table 22.1 summarizes less common systemic associations.

Scleromyxedema is an unusual dermatosis that presents as the insidious onset of multiple firm, waxy, skin-colored papules that progress to confluence (Figure 22.1).

Middle-aged adults are most frequently affected (mean age 53) and there is no gender predilection (4,5). They are most common on the face, upper trunk, and extremities, and in the extreme form give rise to a leonine facies. The individual papules are only several millimeters in diameter and minimally elevated. Papules may be linearly aligned. When the glabella is involved, deep furrowing is apparent. When the eyelids are involved, ectropion may be a problem. In some cases, erythema and/or a brown discoloration may be present. The eruption tends to be relatively asymptomatic to minimally pruritic. When extensive, there may be diffuse thickening of the skin, resulting in decreased mobility of the fingers, joints, and mouth. Scleromyxedema is regarded by some authors as the generalized, lichenoid form of lichen myxedematosus (6).

The histologic features include a normal to somewhat flattened epidermis. The papillary dermis is characterized by increased neutral mucopolysaccharides and an increased number of fibroblasts (Figures 22.2 and 22.3).

There is minimal associated inflammatory response. In most cases, the increased dermal mucin is apparent with routine stains, though in some cases, it may be difficult to discriminate from dermal edema (Figure 22.4).

In these cases, colloidal iron or alcian blue stains done at a pH of 4.5 are helpful in identifying the increased amounts of mucin (Figure 22.5).

Table 22.1. Systemic Conditions Associated with Scleromyxedema (4)

Condition

Percent of Cases of Scleromyxedema with This Association

Paraproteinemia

83.2

Multiple myeloma

10

Proximal muscle weakness

27

Central or peripheral

neuropathies

15

Joint involvement

10.5

Carpal tunnel syndrome

9.6

Raynaud's phenomenon

8.8

Dyspnea

16.7

Dysphagia

31.6

Renal disease/coronary

artery disease

Reported

Ophthalmologic disorders

(corneal opacities,

ectropion, lagophthalmos)

18%

Hepatitis C

AIDS

Scleromyxedema Papules

The increased cellularity and mucin deposition is centered in the upper portions of the dermis and rarely extends deeply into the reticular dermis. Collagen bundles have also been reported to be thickened in this condition (4).

The histologic differential diagnosis includes scler-edema and pretibial myxedema. In scleredema, the

Figure 22.1. Multiple linearly arrayed waxy papules in scleromyxedema.

increased ground substance is located primarily in the deeper reticular dermis and is not accompanied by a concomitant increase in cellularity. Pretibial myxedema demonstrates mucin within the papillary and superficial reticular dermis, similar to scleromyxedema, but rarely has a significant proliferation of fibroblasts.

There is a less common histologic variant in which pools of mucin are surrounded by fibroblasts and histio-cytes. In these cases, the distinction from granuloma

Figure 22.2. Low power photomicrograph depicting diffuse dermal fibroplasia.

Figure 22.2. Low power photomicrograph depicting diffuse dermal fibroplasia.

Pretibial MyxedemaScleromyxedema
Figure 22.3. Medium power photomicrograph depicting increased number of spindled fibrocytes with interspersed lymphocytes.

annulare may be difficult and is most easily made based upon the clinical presentation.

The pathogenesis of scleromyxedema remains an enigma. Paraprotein levels do not correlate with extent or progression of disease (7).

Scleromyxedema is very difficult to treat effectively. A wide range of treatments including intralesional corticosteroids, PUVA, electron beam therapy, and plasmapheresis have demonstrated slight improvements. Low doses of melphalan and cyclophosphamide are effective, but may

Scleromyxedema
Figure 22.4. High power detail of spindled cells. Note stringy gray mucin deposits.

Figure 22.5. Colloidal iron diffuse dermal blue staining corresponding to the presence of increased dermal mucin (hyaluronic acid) deposits.

Figure 22.5. Colloidal iron diffuse dermal blue staining corresponding to the presence of increased dermal mucin (hyaluronic acid) deposits.

Colloidal Iron Mucin Lupus

be related to subsequent development of hematologic malignancies.

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  • adelmio
    How To Treat Pretibial Myxedema?
    7 years ago

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