Lupus-erythematous-associated interstitial granulomatous dermatitis
- Author(s): Marmon, Shoshana;
- Robinson, Maria;
- Meehan, Shane A;
- Jr, Andrew G Franks
- et al.
Published Web Locationhttps://doi.org/10.5070/D39pz98314
Lupus-erythematous-associated interstitial granulomatous dermatitisThe Ronald O. Perelman Department of Dermatology, New York University School of Medicine, New York, New York
Shoshana Marmon MD PhD, Maria Robinson MD, Shane A Meehan MD, Andrew G Franks Jr MD
Dermatology Online Journal 18 (12): 31
A 41-year-old woman with a prior diagnosis of lupus erythematous presented with a five-year history of small, erythematous, flesh-colored papules and nodules that coalesced into symmetrically-distributed plaques on her upper back. A biopsy specimen showed an interstitial, granulomatous mixed-cell dermatitis with eosinophils. These clinicopathologic findings are consistent with a diagnosis of lupus erythematous-associated interstitial granulomatous dermatitis.
A 41-year-old woman was referred to the Charles C. Harris Skin and Cancer Pavilion with a five-year history of small, erythematous and flesh-colored papules and nodules, which coalesced into symmetrically distributed plaques on her upper back. The eruption had a waxing and waning course, with intermittent burning and pruritus. Associated symptoms of fatigue, arthritis, and hair thinning were of at least the same duration as the eruption. Empiric treatment with potent topical glucocorticoids was unsuccessful and the lesions were never biopsied. The patient also reported a history of lupus erythematous that was diagnosed by a rheumatologist. A use of biologics or other systemic agents had never been attempted.
Multiple, smooth, faintly erythematous, flesh-colored papules and nodules with scattered erosions and coalescence into two well-demarcated, symmetrically-distributed, annular plaques were present on the upper back. Symmetric, erythematous, lichenified plaques that were intermixed with millia-like papules were noted on the retroauricular areas.
An antinuclear antibody titer was positive with a titer >160. A complete blood count, comprehensive metabolic panel, erythrocyte sedimentation rate, thyroid stimulating hormone, anti-Ro/SSA antibody, anti-La/SSB antibody, anti-double stranded DNA antibody, anti-Smith antibody, C-reactive protein, angiotensin-converting enzyme, anti-neutrophil cytoplasmic antibody, and C3/C4 protein levels were normal.
There is a perivascular and interstitial infiltrate of histiocytes, lymphocytes, and eosinophils. There is slight psoriasiform epidermal hyperplasia. A periodic acid-Schiff and diastase stain shows a focally thickened basement membrane and no fungi; a special stain for acid fast bacilli is negative.
Interstitial granulomatous dermatitis (IGD) is a rare dermatologic condition of uncertain etiology. Because of a spectrum of diverse clinical manifestations, the diagnosis of IGD often requires a combination of medical history, histopathologic analysis, and serology. Histopathologic features include an interstitial, histiocyte-rich, granulomatous infiltrate that is localized primarily to the lower dermis . Degenerated collagen, neutophilic debris, and eosinophils are often present, whereas necrotizing vasculitis typically is absent . Clinically, IGD classically presents with large, symmetrical plaques that are located mainly on the extremities and/or trunk . The presence of subcutaneous linear cords that are distributed on the flanks, which also is referred to as the rope sign, was initially thought to be pathognomonic [1, 3]. Subsequently, however, a variety of heterogeneous clinical patterns of expression with common histopathologic findings have been grouped under the diagnosis of IGD. In a case series of 17 patients with IGD that were diagnosed by histopathologic criteria, the majority had erythematous plaques that were largely localized to the skin folds in a bilateral or symmetrical distribution . The clinical presentation of IGD in two of three women was erythematous, annular plaques on the extremities; the third had a papular eruption on the head and neck . A case of IGD presented as annular papules and plaques on the dorsa of the hands and feet .
In the majority of cases of IGD, clinical and serologic evidence of underlying collagen- vascular or autoimmune diseases also were reported. One of the most common findings was concomitant rheumatoid arthritis, which led to the classification of interstital granulomatous dermatitis with arthritis (IGDA) as a distinct entity [1, 2, 5, 6]. Other serologic abnormalities that have often been noted with IGD are an elevated antinuclear antibody, elevated erythrocyte sedimentation rate, and autoantibodies [1, 7, 8].
Reported cases of IGD that are associated with lupus erythematosus (LE) are rare and are marked by highly varied clinical findings. A 26-year-old woman with a long history of LE, who presented with a diffuse, erythematous, macular eruption on the extremities, was diagnosed as IGD based on consistent histopathologic features . In another case, purple, infiltrated plaques on the lower back, flanks, and perianal area were reported as IGD in a patient with adalimumab-induced LE . A case of IGD in a patient with LE was described as annular, erythematous plaques on the limbs . As in our case, the subjects in all of the above reports were women with LE. Despite variations in clinical presentation, all skin lesions showed a common predominance of histiocytic, granulomatous inflammation in the dermis without evidence of vasculitis [9, 10, 11].
Treatment has been challenging, with a variety of therapeutic interventions offering mixed results. In the two cases of LE-associated IGD not induced by an anti-tumor necrosis factor agent, oral glucocorticoids resulted in complete resolution of the cutaneous manifestations of the disease [9, 11].
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