Abstract
We describe a clinical case concerning a 36 year old man with a recalcitrant dermatosis involving the face and neck to demonstrate how multiple pathogenic mechanisms may ultimately prohibit disease resolution. This patient’s disease persisted despite multiple standard treatments for the leading differential diagnoses early in the disease course including: topical/systemic corticosteroids for an initially suspected facial dermatitis followed by minocycline and oral ivermectin for granulomatous rosacea with high Demodex burden. These failed therapies prompted the use of oral prednisone and topical pimecrolimus that resulted in some improvement but worsening flares if therapy was discontinued. The leading differential shifted toward rosacea fulminans or an unusual manifestation of immune reconstitution inflammatory syndrome (IRIS) in the setting of possible HIV or iatrogenic immunosuppression. An extensive diagnostic workup was completed and showed isolated IgM deficiency (49 mg/dl, normal range 60 to 300 mg/dl), low levels of 25 hydroxyvitamin D (15 pg/mL, normal range 18 to 64 pg/mL), and low ascorbic acid (0.3 mg/dl, normal range 0.6 to 2.0 mg/dl). The rash finally resolved following a tapering course of cyclosporine and vitamin repletion through supplements and dietary alteration. Our case is one with multiple confounding variables that may have contributed to the recalcitrant nature of this dermatosis: (1) presence of Demodex; (2) iatrogenic immunosuppression due to prolonged systemic and topical steroid use; and (3) vitamin deficiency. It is unclear exactly what role each of these factors played but the purpose of our case is to illustrate these variables can be encountered in regular practice and that sometimes the physician must explore and correct all potential vectors of pathogenesis in order to successfully treat recalcitrant dermatoses.
Highlights
Our patient is a 36-year-old otherwise healthy male referred to Indiana University (IU) Department of Dermatology for an 8-month history of a recalcitrant dermatosis involving the face and neck
The clinical manifestations of rosacea are hypothesized to be the result of a dysregulation of the innate immune system that begins with increased toll like receptor 2 (TLR2) expression [1]
A higher concentration of mites are found in the skin of patients with papulopustular rosacea (PPR) and it has been hypothesized that Demodex may contribute to its pathogenesis
Summary
He was initially treated for 5 months by his primary care physician for a facial dermatitis of unknown etiology with multiple courses of steroids including: 5 courses of oral prednisone, 2 cortisone intramuscular injections, and a 3-week course of a topical steroid. A higher concentration of mites are found in the skin of patients with papulopustular rosacea (PPR) and it has been hypothesized that Demodex may contribute to its pathogenesis. One proposed mechanism is that Demodex infection is due to an initial immune defect, innate or acquired, that allows for organism proliferation.
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