CLINICAL HISTORY:

Signs and symptoms:  The patient states that lesions are non-tender and have enlarged slowly over time. The patient also states that new lesions also occur over time. There is no pruritus or discharge from the lesions. They occur only on the upper back and neck.

Previous Treatment:  Topical steroids, topical antibiotic and oral
antibiotics.

Other information:  The patient has been receiving dialysis since 1999 and has a fistula located on her left forearm. The patient moved to the United States from Nigeria in 1997.

PHYSICAL EXAM:

Patient is a well-nourished African American female in no acute distress. Multiple, hyperpigmented, waxy 2-mm to 4-mm dome shaped and flat topped papules located on the upper back and neck. Some of these lesions are arranged in parallel rows. There are also larger, thick, deeply pigmented nodules of irregular size and shapes on the back of the neck. These lesions were non-tender. There were no signs of infection. An AV fistula was noted on the left forearm. No other lesions were noted.

LABORATORY TESTS:

PTH: 1488.9 (10 - 65)
Calcium: 11 (8.5 - 11)
Total Protein: 8.4 (6.5 - 8)
Albumin 8.2 (3 - 5)

DERMATOHISTOPATHOLOGY:

Microscopic description: Histology showed increased numbers of fibroblasts and increased mucin in the reticular dermis.

DIFFERENTIAL DIAGNOSIS:

1.   Amyloidosis
2.   Lichen myxedematosus
3.   Hypertrophic lichen planus
4.   Nodular urticaria pigmentosa
5.  

SCROLL DOWN FOR ANSWER AND DISCUSSION.

CORRECT DIAGNOSIS:

Lichen myxedematosus

DISCUSSION:

Lichen myxedematosus, localized form, affects adults of both sexes and appears from ages 30 - 80. The primary lesions are multiple, waxy, 2-mm to 4-mm dome shaped or flat topped papules that may coalesce into plaques or arrange in a linear arrangement. Other forms such as urticarial nodular annular lesions are not commonly seen. This commonly occurs on the dorsal hands, face, elbows and extensor extremities. Pruritus may occur. Leonine facies may develop due to coalescence of lesions on the forehead and glabella. The disease course is chronic and usually progressive. Treatment for the cutaneous form is ineffective.

The pathogenesis for this disease is unknown. It is associated with
paraproteinemia that consists of myeloma-like homogenous serum globulinemia of IgG type with predominately lambda light chain. The association with multiple myeloma is rare but it is suggested that papular mucinosis represents plasma cell dyscrasia.

Scleromyxedema, a variant, the skin shows erythematous scleroderma-like induration accompanied by rigidity of the lips, hands, arms and legs. It is also associated with systemic manifestations such as severe proximal
myopathy, inflammatory polyarthritis, central nervous system symptoms, acute organic brain syndrome, esophageal parasols and hoarseness.

Pathology shows striking changes in the epidermis which show a horizontal band of mucinous material between collagen bundles. This material is glycosaminoglycan that stains with LCM blue at pH 2.5 and is susceptible to hyaluronidase. There are an increased number of fibroblasts, which appear plump and stellate, and dermatofibrosis.

Clinically, the differential diagnosis should include scleredema,
scleroderma, amyloidosus, disseminated granuloma annulare, malignant lymphoma and dermatomyositis. The more localized form should be differentiated from colloid degeneration, lichen planus, morbus moniliformis and epithelioma
adenoides cysticum.

TREATMENT:

Actual treatment for this patient: Intralesional Kenalog 5-mg per cc at monthly intervals with mild improvement.

Other Treatment options: For cutaneous involvement isotretinoin and
etretinate have been associated with improvement. Also, alpha interferon, cyclosporin, PUVA, electron beam treatment and derm abrasion. Most of these treatments remain unsatisfactory.

REFERENCES:

1. Richard B. Odem, Andrews Diseases of the Skin, 9th Edition, W.B. Saunders Company, 2000.

2. David Elder, Levers Histopathology of the Skin, 8th Edition, Lippincott,
1997.

3. Raul Fleischmajer, Dermatology and General Medicine, 5th Edition,
McGraw-Hill Companies

Additional Comment:

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