Myelodysplastic Syndrome Presenting as Generalized Granulomatous Dermatitis

Balin, Samuel J.; Wetter, David A.; Kurtin, Paul J.; Letendre, Louis; Pittelkow, Mark R.

doi:10.1001/archdermatol.2011.39

Cited by 37 publications

(31 citation statements)

References 16 publications

(23 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Successful treatment of MDS with 5-azacytidine led to a complete clearance of IGD, which might indicate a pathogenetic relationship of both diseases in this patient. To our knowledge, only one previous report exists on the coincidence of IGD and MDS (9). Our report further suggests to screen for MDS or other haematologic malignancies in patients with IGD.…”

supporting

confidence: 56%

See 1 more Smart Citation

Interstitial Granulomatous Dermatitis Associated with Myelodysplastic Syndrome – Complete Clearance Under Therapy with 5-azacytidine

Patsinakidis¹,

Susok²,

Hessam³

et al. 2014

Acta Derm Venerol

View full text Add to dashboard Cite

Granulomatous skin diseases comprise a heterogenous spectrum of dermatoses that are characterised by a dermal infiltrate of predominantly histiocytes (1). Among this group, interstitial granulomatous dermatitis (IGD) is a term introduced by Ackerman in 1993 (2). It describes a rare form of granulomatous, non-infectious, reactive dermatosis that is frequently associated with rheumatoid arthritis (3, 4). However, several reports of drug-induced IGD and IGD associated with haematological malignancies exist as well (5-8). We report a case of IGD with underlying myelodysplastic syndrome (MDS). Successful treatment of MDS with 5-azacytidine led to a complete clearance of IGD, which might indicate a pathogenetic relationship of both diseases in this patient. To our knowledge, only one previous report exists on the coincidence of IGD and MDS (9). Our report further suggests to screen for MDS or other haematologic malignancies in patients with IGD. CASE REPORTA 73-year-old man presented at our institution with symmetrical, erythematous, infiltrated, annular, and partially urticarial plaques on the thighs, lateral aspects of the trunk, as well as on both arms (Fig. 1a). All lesions rapidly cleared with topical corticosteroid treatment, but relapses occurred shortly after finishing therapy. The patient's medical history included a complete gastrectomy at the age of 68 because of gastric cancer, heart insufficiency, a mitral valve replacement, atrial fibrillation under anticoagulation, diabetes mellitus type 2, chronic obstructive lung disease, and Hashimoto thyreoiditis. Laboratory work-up revealed a normal white blood count but a pronounced, megalocytic, hyper chromic anaemia (haemoglobin: 9.6 g/dl [normal 14-18 g/dl], mean corpuscular volume: 102.2 fl [normal 85-95 fl], and mean corpuscular haemoglobin: 35.3 pg [normal 27-33 pg]). Vitamin B12 deficiency as a result of lack of intrinsic factor after gastrectomy (pernicious anaemia) was suspected as cause for the anaemia (vitamin B12: 99.79 pg/ml [normal 197-866 pg/ml]). Furthermore, a discrete but persistent elevation of lactate dehydrogenase (280 U/l [normal 135-225 U/l]) and an elevation of beta-2 microglobulin (4.0 mg/l, [normal 0.8-2.2 mg/l]) was detected. A blood smear test showed an anisocytosis with macrocytosis, which was ascribed to the known vitamin B12 deficiency. Folic acid, iron, bilirubin and serum protein electrophoresis were all normal. Rheumatoid factor, anti-cyclic citrullinated peptide antibodies, antinuclear antibodies, antibodies against extractable nuclear antigens, as well as antibodies against double-stranded and anti-neutrophil cytoplasmic antibodies were unremarkable. Histopathological evaluation of several skin biopsies showed a superficial and deep perivascular, as well as interstitial dermatitis. The superficial perivascular infiltrate mostly consisted of lymphocytes with only occasional neutrophiles and eosinophils, whereas the inflammatory infiltrate in the middle and deep dermis consisted of compact histiocytic and lymphocytic infiltrates, s...

show abstract

supporting

confidence: 56%

“…However, several reports associate IGD with haematological malignancies, e.g. chronic myelomonocytic leukaemia or acute myeloid leukaemia (8,9). In the present case of IGD, an underlying MDS was finally detected.…”

Section: Discussionmentioning

confidence: 47%

Interstitial Granulomatous Dermatitis Associated with Myelodysplastic Syndrome – Complete Clearance Under Therapy with 5-azacytidine

Patsinakidis¹,

Susok²,

Hessam³

et al. 2014

Acta Derm Venerol

View full text Add to dashboard Cite

show abstract

“…[1][2][3][4] In addition, disseminated cutaneous granulomatous eruptions have been described in patients with MDS. [38][39][40] Vestey et al 39 described 2 cases of widespread papular granulomatous eruptions containing epithelioid granulomata with variable numbers of giant cells. The first case resembled granuloma annulare with transformation to acute myeloblastic leukemia and the second case of cutaneous sarcoidosis.…”

Section: Discussionmentioning

confidence: 99%

A Cutaneous Interstitial Granulomatous Dermatitis-Like Eruption Arising in Myelodysplasia With Leukemic Progression

Cornejo

Lum²,

Izumi³

2013

The American Journal of Dermatopathology

View full text Add to dashboard Cite

Cutaneous manifestations associated with myelodysplastic syndromes (MDS) are uncommon and can occur as specific or nonspecific lesions. Recognizing these cutaneous manifestations is important as they can precede blood or bone marrow transformation to leukemia. Granulomatous reactions have rarely been described as nonspecific lesions of MDS. These rare cases histologically resembled granuloma annulare, sarcoid, and a generalized dermal interstitial granulomatous dermatitis (IGD) which were not associated with leukemic infiltration. The authors report an interesting case of an IGD-like eruption evolving over the course of MDS with eventual progression to systemic leukemia. IGD is an inflammatory reaction that refers to a varied spectrum of histologic patterns and is associated with a variety of systemic illnesses and hypersensitivity reactions, including lymphoma and leukemia. In patients with MDS, surveillance for leukemia is a critical component of their follow-up care. Normally, this surveillance occurs through serial peripheral blood smears and bone marrow studies. IGD-like eruptions are a cutaneous reaction pattern that may serve as an additional clinical indicator of leukemic progression in patients with MDS. Although primarily a reactive pattern, this entity can rarely harbor leukemic blasts.

show abstract

“…There remain types of reactive cutaneous granulomatous dermatitis that are not encapsulated by the aforementioned entities, including granuloma annulare and sarcoidosis-like cutaneous reactions, which are beyond the spectrum of this review. However, there exist clinical eruptions that resemble PNGD, IGD, or IGDR, either clinically or histologically, and occur in response to inciting systemic processes similar to those entities, such as generalized/erythrodermatous granulomatous dermatitis in the setting of myelodysplastic syndrome, 77 or generalized granuloma-annulare-like polymorphic eruptions (Fig. 7), which may warrant consideration within the umbrella of diseases described in this review.…”

Section: Reactive Granulomatous Dermatitismentioning

confidence: 99%

Reactive Granulomatous Dermatitis

Rosenbach

English

2015

Dermatologic Clinics

109

View full text Add to dashboard Cite

Myelodysplastic Syndrome Presenting as Generalized Granulomatous Dermatitis

Abstract: Background: Granulomatous dermatitis has rarely been reported as a manifestation of leukemia or myelodysplastic syndrome (MDS). We describe a case of widespread granulomatous dermatitis that preceded the diagnosis of MDS by 2 years.

Cited by 37 publications

References 16 publications

Interstitial Granulomatous Dermatitis Associated with Myelodysplastic Syndrome – Complete Clearance Under Therapy with 5-azacytidine

Interstitial Granulomatous Dermatitis Associated with Myelodysplastic Syndrome – Complete Clearance Under Therapy with 5-azacytidine

A Cutaneous Interstitial Granulomatous Dermatitis-Like Eruption Arising in Myelodysplasia With Leukemic Progression

Reactive Granulomatous Dermatitis

Contact Info

Product

Resources

About