UPMC Dermatopathology "Case of the Month" Presentations
UPP - Department of Dermatology, Dermatopathology Unit
Case Authors: P.C.Parham-Vetter, MD,MPH Drazen Jukic, MD
MARCH 2004 CASE OF THE MONTH
DISCUSSION & DIAGNOSIS
DIAGNOSIS
Given these findings a diagnosis of interstitial granuloma annulare was made.
DISCUSSION
Granuloma annulare (GA) is a self-limited, benign process. In a small subset of patients it is associated with glucose intolerance. The two most common types are localized GA and generalized GA. Localized GA is the most common type and typically presents with large annular and arcuate lesions typically isolated to the hands and arms or legs and feet. It usually occurs in people younger than 30 years old, and occurs twice as often in women compared to men. Generalized GA comprises 15% of all cases and characteristically occurs in children younger than 10 and adults older than 40. The annular plaques in this variant are typically only 1-2 cm in size with a widespread distribution involving the trunk, neck, forearms and legs. In this case the age at presentation and distribution of the lesions were typical of generalized GA; however, the large size of the plaques was more characteristic of localized GA.
The exact etiology of GA is unknown. Proposed inciting factors include trauma, insect bite, TB skin testing, sun exposure, and viral infections. It is considered by many to be a delayed-type hypersensitivity reaction to an unknown antigen and it appears to be a Th1 mediated inflammatory reaction with lymphocytes producing interferon-gamma that results in dermal matrix degradation. Laboratory tests in the research setting have shown an increase in heparin precipitable cryofinbrinogen, fibronectin, serum lysozyme, and benzylamine monoamine oxidase. Electron microscopy shows degeneration of both collagen and elastic fibers. Histiocytes have a high content of primary lipsomes with release of lysozymes into the extracellular space. Increased levels of interstitial heparin sulfate have also been postulated to play a role.
Histopathologically granuloma annulare can present in three patterns. The interstitial pattern is the most common, comprising 70% of all cases. As in this case, this pattern presents as scattered histiocytic infiltrate between collagen bundles with a variable amount of mucin and an inflammatory infiltrate. Occasionally Giant cells may be seen. , Another common pattern is the palisaded granuloma pattern. This appears as a focal degeneration of collagen and elastic fibers with feathery blue mucin deposition surrounded by a palisade of histiocytes. A perivascular and interstitial lymphyhistiocytic infiltrate in the upper and mid dermis is also seen. , Both of these two patterns have variable vascular changes of fibrinoid deposition in vessel walls and occasionally vascular lumina with immunoflorescence showing perivascular deposition of IgM, C3, and fibrinogen. , The third pattern, epithilioid histiocytic nodular pattern, is quite rare.
Fifty percent of cases will spontaneously resolve within two years; however, there is a 40% recurrence rate. Numerous topical therapies have been reported as beneficial including topical corticosteroids, intralesional steroid injections with or without interferon-gamma, topical vitamin E, topical immiquimod, cryotherapy, PUVA, and UVA-1 therapy. Systemic therapies that have been used include nicotinamide, niacinamide, isotretinoin, anti-malarials, cyclosporine, chlorambucil, dapsone, potassium iodide, thyroxine, dipyridamole, and pentoxifylline. -
Initially, our patient was prescribed a moderate potency topical steroid cream for one month, but showed no response. A high potency topical steroid was also used for one month, again showing no response. The number of lesions precluded the use of cryotherapy and intralesional steroid injections. Additionally, the patient lived in a rural area and was unable to travel several time a week for light therapy. The patient was anxious to have improvement of the skin but did want to take oral medications with potentially harmful side effects. Based upon the above factors a regimen was prescribed to include Nicomide 500 mg per day combined with topical Elidel cream in the morning and Tazorac cream at night. The patient reported some initially irritation from the Tazorac but after one month showed no further development of plaques and partial resolution of the lesions on the neck, chest and arms. The patient was asked to return to clinic if the lesions persisted or new ones developed. As of nine months later, she not returned with new or persistent lesions.
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All photos Copyright 2004 P.C. Parham-Vetter, MD, MPH
