DERMATITIS GRANULOMATOSA INTERSTICIAL PDF

Mailing address. A year-old female presented with recurrent, widespread, erythematous, painful plaques, over a 3-month period. Skin biopsy was compatible with interstitial granulomatous dermatitis. Additional investigation revealed hypothyroidism and positive anti-thyroid antibodies.

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Mailing address. A year-old female presented with recurrent, widespread, erythematous, painful plaques, over a 3-month period. Skin biopsy was compatible with interstitial granulomatous dermatitis. Additional investigation revealed hypothyroidism and positive anti-thyroid antibodies. Normalization of thyroid function and high-potency topical corticosteroids provided only transitory improvement of the dermatosis. Interstitial granulomatous dermatitis is a histologic inflammatory reaction, with variable cutaneous expression.

It has been reported in association with several drugs, lymphoproliferative diseases and autoimmune disorders, such as rheumatoid arthritis, systemic lupus erythematosus and vasculitis, but association with autoimmune thyroiditis is rare.

Optimal therapy for this condition is yet to be established, but topical corticosteroids have been a mainstay of treatment. In most cases, this disease is characterized by flares and remissions. Keywords: Dermatitis; Granulomatous disease, chronic; Thyroiditis; Thyroiditis, autoimmune. Os restantes exames laboratoriais revelaram hipotiroidismo e anticorpos anti-tiroideus positivos. Interstitial granulomatous dermatitis IGD is an uncommon disease, with characteristic histopathological features but variable clinical expression.

When the clinical picture includes arthritis, it takes the name Ackerman's Syndrome, who first described this entity in We report a case of IGD which occurred as the presenting symptom of an underlying autoimmune thyroiditis.

A year-old Caucasian female presented to the emergency room with 2 erythematous, sharply demarcated, painful, indurated plaques, on her left arm and right thigh, which had appeared in the previous 48 hours.

She denied any systemic complaints, emergency laboratory tests revealed only microcytic anemia and slightly elevated C-reactive protein and, therefore, she was discharged and prescribed only conservative measures. When reevaluted, after 1 week, those lesions had resolved, but 2 new similar plaques had appeared over the right arm and left buttock, and linearly arranged erythematous papules had arisen on the right hand Figure 1. The patient had a history of benign breast nodules, iron deficiency anemia and depression, and had been medicated with fluoxetine, lorazepam and acetylsalicylic acid for over 5 years.

Additional laboratory investigation detected subclinical hypothyroidism free thyroxine 0. She was medicated with high potency topical corticosteroid and evaluated by an endocrinologist who prescribed levothyroxine. However, despite normalization of thyroid function, there was only transitory improvement of individual lesions, and, after a follow-up of 2 years, new lesions keep on occurring Figure 4.

IGD is considered to be a histological inflammatory reaction pattern of the granulomatous type, characterized by collagen necrobiosis, in the context of an internal disease with the ability to produce immunocomplexes. Altough the original description reported subcutaneous indurated linear cords "the rope sign" along the lateral aspect of the trunk, the clinical picture is now known to be quite heterogeneous, as it may also manifest as patches, papules, nodules or plaques, and at different body sites.

The dermatosis is traditionally asymptomatic. However, there have also been reports of occasionally pruritic or tender skin lesions, as was the case of our patient. Arthritis is also an inconsistent feature, which may occur prior to, simultaneously with, after the onset of the cutaneous lesions, or not at all.

The typical microscopic presentation should be that of a granulomatous dermatitis, with palisading or interstitial histiocytes, variable numbers of neutrophils and eosinophils, and scattered areas of degenerating collagen bundles. Accentuation of the process in the reticular dermis is a characteristic feature, although abnormalities can involve the full thickness of the dermis or the superficial hypodermis.

For the time being, IGD is considered to be an idiopathic condition. However, the growing number of reports linking it to a variety of autoimmune disorders, namely rheumatoid arthritis, systemic lupus erythematosus and Churg-Strauss syndrome, strongly support the hypothesis of an underlying autoimmune process.

IGD may also be the heralding symptom for lymphoproliferative diseases, solid organ cancer, or represent an adverse drug reaction, including to the newer biologic agents. Our patient showed decreased levels of free thyroxine and high titers of anti-thyroid antibodies, disclosing a previously unrecognized autoimmune thyroiditis. Autoimmune thyroiditis has already been mentioned once in association with IGD.

The lack of randomized clinical studies has prevented an optimal treatment for IGD to be determined. Topical corticosteroids appear to be only moderately effective, but are still a first line treatment, especially in patients with localized disease. In the case of our patient, topical clobetasol cleared a few established plaques, but did not prevent other lesions from appearing, nor did the normalization of thyroid function.

Thus far, no other treatment has been tried on this patient, due to her poor compliance with treatment and difficult follow-up. In conclusion, IGD is an uncommon skin disease that may be secondary to an underlying systemic condition. Therefore, dermatologists should be aware of this possibility, since they are in a privileged position to diagnose potentially life-threatening diseases, at an early stage. Clues to diagnosis in dermatopathology.

Arch Dermatol. Interstitial granulomatous dermatitis secondary to acute promyelocytic leukemia. Am J Dermatopathol. Interstitial granulomatous dermatitis with arthritis Ackerman syndrome. J Rheumatol. Weedon D. The granulomatous reaction pattern. In: Houston M, Davie B, editors. Weedon's skin pathology.

London: Churchill Livingstone; Myelodysplastic syndrome presenting as generalized granulomatous dermatitis. Interstitial granulomatous dermatitis and paraneoplastic rheumatoid polyarthritis disclosing cancer of the lung. Ann Dermatol Venereol. Interstitial granulomatous dermatitis with plaques. Interstital granulomatous dermatitis Ackerman's syndrome. Eur J Dermatol. Dubey S, Merry P. Instertitial granulomatous dermatitis Ackerman's syndrome in SLE presenting with the 'rope sign'.

Rheumatology Oxford. Egas Moniz Lisboa, Portugal E-mail: joanaferroantunes gmail. Received on Approved by the Advisory Board and accepted for publication on Conflict of interest: None Financial funding: None.

All the contents of this journal, except where otherwise noted, is licensed under a Creative Commons Attribution License. Services on Demand Journal. CASE REPORT A year-old Caucasian female presented to the emergency room with 2 erythematous, sharply demarcated, painful, indurated plaques, on her left arm and right thigh, which had appeared in the previous 48 hours.

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Granulomatous dermatitis

Besides being an uncommon clinicopathological entity, interstitial granulomatous dermatitis, also described as interstitial granulomatous dermatitis with arthritis IGDA , has shown a wide spectrum of clinical manifestations, such as linear and erythematous lesions, papules, plaques and nodules. Histological features include dense dermal histiocytic infiltrate, usually in a palisade configuration, and scattered neutrophils and eosinophils. We describe a middle aged woman with rheumatoid arthritis of difficult management and cutaneous lesions compatible with IGDA. Interstitial granulomatous dermatitis was initially described by Ackerman et al in

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Only comments seeking to improve the quality and accuracy of information on the Orphanet website are accepted. For all other comments, please send your remarks via contact us. Only comments written in English can be processed. Interstitial granulomatous dermatitis with arthritis is a rare rheumatologic disease characterized by the occurrence of inflammatory arthritis in association with large, erythematous, symmetrical cutaneous lesions ranging from typical, but infrequent, cord-like lesions on the flanks to more common violaceous plaques on the trunk and limbs featuring a typical histologic infiltrate mainly constituted of histiocytes. Other search option s Alphabetical list. Suggest an update.

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