Case Reports

Ichthyosiform Sarcoidosis and Systemic Involvement

Author and Disclosure Information

Sarcoidosis is a systemic disease that can be suspected based on cutaneous findings and confirmed using diagnostic testing such as biopsy and laboratory or radiographic studies. We report the case of a 66-year-old black woman with ichthyosiform sarcoidosis (IS) on the lower extremities. Ichthyosiform sarcoidosis is a rare variant of cutaneous sarcoidosis that is frequently associated with further internal involvement. We review this uncommon presentation of sarcoidosis along with other cases reported in the literature to determine the organ systems most commonly affected.

Practice Points

  • Ichthyosiform sarcoidosis is a rare form of sarcoidosis that presents as polygonal adherent scales.
  • Ichthyosiform sarcoidosis is commonly associated with pulmonary, neurologic, and hepatic involvement.
  • Acquired ichthyosis should warrant further investigation for systemic disease.


 

References

Sarcoidosis is a multiorgan, systemic, granulomatous disease that most commonly affects the cutaneous, pulmonary, ocular, and cardiac organ systems. Cutaneous involvement occurs in approximately 20% to 35% of patients, with approximately 25% of patients demonstrating only dermatologic findings.1 Cutaneous sarcoidosis can have a highly variable presentation. Ichthyosiform sarcoidosis (IS) is a rare form of this disease that has been described as presenting as polygonal adherent scales.2 It often is associated with internal organ involvement. We present a case of IS without any organ system involvement at the time of diagnosis. A review of the English-language literature was performed to ascertain the internal organ associations most commonly reported with IS.

Case Report

A 66-year-old black woman presented to dermatology with dark scaly patches noted by her primary care physician to be present on both of the lower extremities. The patient believed they were present for at least 4 years. She described dark spots confined to the lower legs that had gradually increased in size. Review of systems was negative for fever, chills, night sweats, weight loss, vision changes, cough, dyspnea, and joint pains, and there was no history of either personal or familial cutaneous diseases.

Physical examination revealed cutaneous patches of thin white scale with a sharp edge in arciform patterns on the lower extremities. Several of these patches were hyperpigmented and xerotic in appearance (Figure 1). The patches were limited to the lower legs, with no other lesions noted.

Figure1

Figure 1. Ichthyosis sarcoidosis on the bilateral lower legs with hyperpigmented and xerotic patches (A). Cutaneous patches of thin white scale with a sharp edge in arciform patterns also were present (B).

A punch biopsy of the skin on the right lower leg was performed. Histopathologic analysis showed epidermal compact hyperkeratosis with deep granulomatous infiltration into the subcutaneous tissue (Figures 2A and 2B). At high power, these granulomas were noted to be noncaseating naked granulomas composed of epithelioid histiocytes surrounded by sparse lymphocytic inflammation (Figure 2C). Special stains including acid-fast bacilli, Fite, and periodic acid–Schiff were negative. The diagnosis of IS was made based on clinical presentation and primarily by histopathologic analysis.

Figure2

Figure 2. Histopathology revealed diffuse, predominantly rounded aggregates of epithelioid histiocytes within the deep dermis and subcutaneous tissue (A)(H&E, original magnification ×25). A dense, compact, orthokeratotic stratum corneum with loss of the normal basket-weave pattern also was present (B)(H&E, original magnification ×100). Noncaseating granulomas composed of epithelioid histiocytes were surrounded by sparse lymphocytic inflammation (C)(H&E, original magnification ×200).

The patient’s cutaneous lesions were treated with fluocinonide ointment 0.05% twice daily. Although she did not notice a dramatic improvement in the plaques, they stabilized in size. Her primary care physician was notified and advised to begin a workup for involvement of other organ systems by sarcoidosis. Her initial evaluation, which included a chest radiograph and electrocardiogram, were unremarkable. Despite multiple attempts to persuade the patient to return for further follow-up, neither dermatology nor her primary care physician were able to complete a full workup.

Next Article: