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Ichthyosiform sarcoidosis

  • Author(s): Rosenberg, Benjamin
  • et al.
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Ichthyosiform sarcoidosis
Benjamin Rosenberg MD
Dermatology Online Journal 11 (4): 15

Department of Dermatology, New York University School of Medicine

Abstract

A 39-year-old woman with a 4-year history of paraparesis developed asymptomatic, ichthyosiform scale on her lower extremities. The lesions consisted of polygonal, pigmented, adherent scales, which were most prominent on the pretibial areas. A biopsy specimen showed granulomatous dermatitis in a patient with pulmonary and neurologic sarcoidosis. Ichthyosiform sarcoidosis is a rare, specific cutaneous manifestation of sarcoidosis, which may be responsive to topical or systemic glucocorticoids.


A 39-year-old woman was referred for evaluation of a scaly eruption on her legs to the Charles C. Harris Skin and Cancer Pavilion during a hospitalization at the Rusk Institute for Rehabilitation for paraparesis. She reported that the eruption had been present for approximately 3 years, was asymptomatic, and did not respond to emollients. The dermatologic history included asymptomatic, skin-colored papules on her eyelids that were excised in 1992. After a biopsy specimen from the right leg was obtained, triamcinolone ointment 0.1 percent and Lac-Hydrin® cream were initiated as treatment. During her hospitalization, she was placed on prednisone and methotrexate, and then a monthly regimen of cytoxan was initiated to treat her underlying condition. She denied shortness of breath or other pulmonary symptoms. Medical history included right eye blindness since childhood and hypertension.

The lower extremities displayed polygonal, pigmented, adherent scales that were most prominent on the pretibial areas.


Figure 1 Figure 2

A complete blood count and chemistry panel were normal. Hepatic panel showed a low total protein of 6.0 g/dL, a low albumin of 2.5g/dL, and an elevated aspartate aminotransferase and alanine aminotransferase of 59 U/L and 87 U/L, respectively. Rheumatoid factor, anti-nuclear antibody, thyroid stimulating hormone, erythrocyte sedimentation rate, C-reactive protein, and angiotensin-converting enzyme levels were normal.

Magnetic resonance imaging (MRI) of the thoracic and lumbar spine was normal. MRI of the brain and orbits showed multiple enhancing miliary nodules diffusely within the subarachnoid/pial spaces of the cerebellum and brainstem as well as small nodules coating the tentorium and dura of the inferior temporal lobes, the basal ganglia, the lateral ventricles, and in the right globe. Computed tomography scan of the chest showed a tiny, calcified, left hilar node as well as numerous non-specific nodules in the lung parenchyma, but no evidence of interstitial lung disease.

Histopathology revealed a mixed-cell infiltrate composed of histiocytes, lymphocytes, and plasma cells in the papillary and reticular dermis. Clusters of epithelial histiocytes are seen. There is no necrosis. A periodic acid-Schiff stain for fungi, a Ziehl-Nielsen stain for acid fast bacilli, and a Steiner stain for spirochetes are negative. The overlying epidermis is normal.


Comment

Sarcoidosis is a multisystem, granulomatous disease of unknown etiology. It can affect any organ of the body but most commonly involves the lungs, lymph nodes, skin, and eyes. Less common but usually severe manifestations also occur in the central nervous system, heart, and skeletal system [1, 2, 3, 4, 5]. Cutaneous manifestations are present in approximately 20-35 percent of patients and are classified as specific or nonspecific based upon the presence or absence of noncaseating granulomas on histopathologic examination [1, 2, 3]. The most common nonspecific lesion of sarcoidosis is erythema nodosum [1, 3, 4]. The most common specific lesions include lupus pernio, infiltrated plaques, macular and papular lesions, and subcutaneous nodules. Less common but still specific manifestations are hypopigmented patches, ulcers, alopecia, verrucous lesions, and erythroderma [1, 2, 3, 4, 5]. Approximately 20 cases of ichthyosiform sarcoidosis that are characterized by non-caseating granulomas have been reported since the initial report in 1981 [1].

Ichthyosiform sarcoidosis typically presents as asymptomatic, adherent, polygonal scales on the lower extremities [1, 3, 5]. The presence of these ichthyosiform scales correlates well with the presence of systemic disease; 95 percent of all reported cases of ichthyosiform sarcoidosis have systemic involvement at the time of diagnosis or develop systemic involvement within several months [1, 2, 5]. Biopsy specimens often show not only non-caseating granulomas but also epidermal changes, which are consistent with ichthyosis vulgaris and which include compact orthokeratosis and a decreased or absent granular layer [1, 2, 5]. The ichthyosiform lesions have been reported to respond to both topical and systemic glucocorticoids [1].

Acquired ichthyosis has been also associated with a number of malignant conditions including lymphomas, especially Hodgkin's disease; multiple myeloma; carcinomas of the lung, breast, and cervix; Kaposi's sarcoma; and leiomyosarcoma. Other systemic diseases that may be characterized by acquired ichthyosis include Hansen's disease, hypothyroidism, phrynoderma, and chronic malnutrition. Medications that interfere with sterol synthesis in epidermal cells have been also associated with acquired ichthyosis; these medications include nicotinic acid, triparanol, butyrophenones, dixyrazine, nafoxidine, cimetidine, and clofazimine [2, 3, 5, 6].

References

1. Cather JC, et al. Ichthyosiform sarcoidosis. J Am Acad Dermatol 1999; 40:862

2. Matarasso SL, et al. Ichthyosiform sarcoidosis: report of a case. Cutis 1991; 47:405

3. Mountcastle EA, et al. An ichthyosiform eruption on the legs. Arch Dermatol 1989; 125:1415

4. Mana J, et al. Cutaneous involvement in sarcoidosis: relationship to systemic disease. Arch Dermatol 1997; 133:882

5. Banse-Kupin L, et al. Ichthyosiform sarcoidosis: report of two cases and a review of the literature. J Am Acad Dermatol 1987; 17:616

6. Griffiths CEM, et al. Acquired ichthyosis and sarcoidosis. Clin Exp Dermatol 1986;11:296

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