Interstitial granulomatous dermatitis with plaques and arthritis

ARTICLE

Auteur(s) : José BAÑULS¹, Isabel BETLLOCH¹, Rafael BOTELLA¹, Maria José JIMÉNEZ², Mar BLANES¹, José Carlos PASCUAL¹, Isabel BELINCHÓN¹, Juan Francisco SILVESTRE¹

Departments of ¹Dermatology and ²Pathology, Hospital General de Alicante, Pintor Baeza S/N, 03010 Alicante, Spain

Article accepted on 18/03/2003

Interstitial granulomatous dermatitis (IGD) is a histopathological condition characterised by an infiltration of the reticular dermis with a predominance of interstitial and palisadic histiocytes with a few areas of degenerating collagen bundles associated with a variable number of polynuclear neutrophils and eosinophils. Since 1993 when Ackerman [1, 2] described a linear form associated with arthritis, different forms of clinical presentation and various aetiologies have been reported. We report a case of IGD with plaques and arthritis.

Case report

A 65-year-old man presented in January 2000 with an asymptomatic inflammatory lesion in his right popliteal fossa, which he had had for two months. He also complained of his hands being painful and swollen for several months. There was no history of fever, general malaise, arthropod bites, injuries or drug use.
On examination there was a large annular erythematous lesion (16 × 14 cm) in the right popliteal fossa. It had a raised border and a slightly scaly, somewhat atrophic centre (Fig. 1). There was a similar but smaller lesion (3 × 3 cm) in the left popliteal fossa. A month later the lesion on the right had enlarged slightly and its edge had become more pronounced.
Two skin biopsies were taken: one of the active edge and the other of the centre of the plaque. Biopsy of the edge showed a diffuse inflammatory infiltrate with a predominance of histocytes surrounding a focus of collagen degeneration, but not forming true palisades (Fig. 2 and 3) and with no mucin present. We also observed moderate numbers of neutrophils and a few eosinophils, but there were no flame figures. There were lymphocytic, perivascular infiltrates but no vasculitis. In the biopsy taken from the centre of the plaque there was an atrophic epidermis with slight hyperkeratosis and a slight epithelioid histiocytic infiltrate in the superficial dermis with frequent phagocytosis of elastic fibres.
Hemogram, biochemistry, proteinogram, HVB and HVC serology, Borrelia burgdoferi serology, antinuclear antibody (ANA), rheumatoid factor, tumour markers and urinary sediment examination were normal.
The Rheumatology Department assessed the patient and seronegative rheumatoid arthritis was diagnosed. Treatment with oral diclofenac started. Although cutaneous plaques were treated with topical corticosteroids, the lesions improved slowly and cleared totally in four months, with a parallel evolution of articular symptoms.
The patient was finally diagnosed as having IGD with plaques associated with seronegative arthritis. The skin lesions and arthritis had disappeared by October 2000. At the present time the patient is completely symptom-free.

Discussion

We report a case of interstitial granulomatous dermatitis with plaques and arthritis. IGD is a histopathological condition described by Ackerman et al. [1, 2]. There are several clinico-pathological types of IGD. Subcutaneous linear cords [1, 2] which occurred on the trunk or adjacent part of the limbs and arthritis was the first variety of IGD described. Subsequently Long [3] described a second clinical form of IGD with annular plaques, which was often found on the limbs and associated with arthritis. Later cases with annular plaques but different sites of the lesions and with or without arthritis were described [4-7]. Isolated cases associated with neoplasia [8] and other systemic diseases have also been reported [5, 10]. Also cases with similar annular characteristics have been reported in association with certain drugs, particularly calcium channel blockers, angiotensin converting enzyme inhibitors, furosemide, fluindione, beta-blockers, lipid-lowering agents, antihistamines, anticonvulsants and antidepressants [11, 12].

In our case there was a chronic inflammatory dermatitis with annular morphology, which suggested various possibilities for a differential diagnosis. From the clinical point of view erythema chronicum migrans could be ruled out because Borrelia burgdorferi serology was negative. Centrifugal annular erythema and mycosis fungoides were easily eliminated by anatomopathological tests.

From the histological point of view differential diagnosis is basically with the interstitial variety of the annular granuloma. Other entities such as Wells and eruptions observed in rheumatoid polyarthritis and autoimmune diseases which can also have histopathological pictures similar to those of Churg Strauss granuloma [13-15] and rheumatoid neutrophilic dermatitis [16], raise nosologic problems with interstitial granulomatous dermatitis.

IGD is a recently described clinical condition which is currently in the process of consolidation and which some authors would like to include within the broad clinico-pathological spectrum of palisaded neutrophilic and granulomatous dermatitis associated with collagen vascular diseases [13, 15].

Nonetheless although their ideas of unifying these entities are interesting, we consider that the histopathology of IGD involves granulomas but not true palisades, maybe because the associated rheumatic diseases are less active, as stated by other authors [7]. We also believe that IGD with plaques is a distinct entity with highly reproducible clinical and histopathological features, and therefore the presence of a “top heavy” interstitial infiltrate and superficial or deep perivascular infiltrates of lymphocytes do not rule out IGD [7] and are not necessarily diagnostic of interstitial granuloma annulare. Moreover, the phagocytosis of elastic fibers by histiocytes found in the biopsy from the centre of the plaque of our patient’s leg was described by Magro [10] in IGD associated with drugs.

In conclusion, the IGD variety with plaques and arthritis is rare and relatively unknown to both dermatologists and rheumatologists, but we believe that it should be considered in the differential diagnosis of cutaneous lesions associated with arthritis. n

References

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