Diagnostic criteria for five selected syndromes
| POEMS syndrome*3 . | Schnitzler syndrome†‡16 . | Necrobiotic xanthogranuloma§21 . | Scleromyxedema18 . | TEMPI syndrome23 . |
|---|---|---|---|---|
| Mandatory major criteria | Obligate criteria | Major criteria | 1. Generalized papular and sclerodermoid eruption | Major criteria |
| 1. Polyneuropathy (typically demyelinating) | 1. Chronic urticarial rash | 1. Cutaneous papules, plaques, and/or nodules, most often yellow or orange in color | 2. Evidence of monoclonal gammopathy | 1. Telangiectasias |
| 2. Monoclonal plasma cell-proliferative disorder (almost always λ) | 2. Monoclonal IgM or IgG | 2. Histopathological features demonstrating palisading granulomas with lymphoplasmacytic infiltrate and zones of necrobiosis. Variably present cholesterol clefts and/or giant cells | 3. Microscopic triad associating dermal mucin deposition, thickened collagen, and fibroblast proliferation or an interstitial granuloma annulare–like pattern | 2. Monoclonal gammopathy |
| Major criteria | Minor criteria | 3. Elevated erythropoietin and erythrocytosis | ||
| 3. Castleman disease|| | 3. Recurrent fever¶ | Minor criteria | ||
| 4. Sclerotic bone lesions | 4. Objective findings of abnormal bone remodeling with or without bone pain# | Minor criteria | 4. Absence of thyroid disease | 4. Perinephric fluid |
| 5. Vascular endothelial growth factor elevation | 5. A neutrophilic dermal infiltrate on skin biopsy** | 3. Periorbital distribution of cutaneous lesions | 5. Intrapulmonary shunting | |
| Minor criteria | 6. Leukocytosis and/or elevated CRP†† | 4. Paraproteinemia, most often IgG-λ, plasma cell dyscrasia, and/or other associated lymphoproliferative disorder | 6. Other: venous thrombosis | |
|
| POEMS syndrome*3 . | Schnitzler syndrome†‡16 . | Necrobiotic xanthogranuloma§21 . | Scleromyxedema18 . | TEMPI syndrome23 . |
|---|---|---|---|---|
| Mandatory major criteria | Obligate criteria | Major criteria | 1. Generalized papular and sclerodermoid eruption | Major criteria |
| 1. Polyneuropathy (typically demyelinating) | 1. Chronic urticarial rash | 1. Cutaneous papules, plaques, and/or nodules, most often yellow or orange in color | 2. Evidence of monoclonal gammopathy | 1. Telangiectasias |
| 2. Monoclonal plasma cell-proliferative disorder (almost always λ) | 2. Monoclonal IgM or IgG | 2. Histopathological features demonstrating palisading granulomas with lymphoplasmacytic infiltrate and zones of necrobiosis. Variably present cholesterol clefts and/or giant cells | 3. Microscopic triad associating dermal mucin deposition, thickened collagen, and fibroblast proliferation or an interstitial granuloma annulare–like pattern | 2. Monoclonal gammopathy |
| Major criteria | Minor criteria | 3. Elevated erythropoietin and erythrocytosis | ||
| 3. Castleman disease|| | 3. Recurrent fever¶ | Minor criteria | ||
| 4. Sclerotic bone lesions | 4. Objective findings of abnormal bone remodeling with or without bone pain# | Minor criteria | 4. Absence of thyroid disease | 4. Perinephric fluid |
| 5. Vascular endothelial growth factor elevation | 5. A neutrophilic dermal infiltrate on skin biopsy** | 3. Periorbital distribution of cutaneous lesions | 5. Intrapulmonary shunting | |
| Minor criteria | 6. Leukocytosis and/or elevated CRP†† | 4. Paraproteinemia, most often IgG-λ, plasma cell dyscrasia, and/or other associated lymphoproliferative disorder | 6. Other: venous thrombosis | |
|
CRP, C-reactive protein; IgG-λ, immunoglobulin G-λ; POEMS, polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes; TEMPI, telangiectasias, elevated erythropoietin and erythropoiesis, monoclonal gammopathy, perinephric fluid, intrapulmonary shunting.
POEMS syndrome diagnosis is confirmed when both of the mandatory major criteria, 1 of the 3 other major criteria, and 1 of the 6 minor criteria are present.
Definite diagnosis of Schnitzler syndrome: if IgM, both obligate criteria and at least 2 minor criteria; if IgG, both obligate criteria and 3 minor criteria.
Probable diagnosis of Schnitzler syndrome: if IgM, both obligate criteria and 1 minor criteria; if IgG, both obligate criteria and 2 minor criteria.
For necrobiotic xanthogranuloma diagnosis, both major criteria and at least 1 minor criterion, applicable only in the absence of foreign body, infection, or other identifiable cause.
There is a Castleman disease variant of POEMS syndrome that occurs without evidence of a clonal plasma cell disorder that is not accounted for in this table. This entity should be considered separately.
Must be >38°C and otherwise unexplained. Occurs usually—but not obligatory—together with the skin rash.
As assessed by bone scintigraphy, magnetic resonance imaging, or elevation of bone alkaline phosphatase.
Corresponds usually to the entity described as “neutrophilic urticarial dermatosis,” absence of fibrinoid necrosis, and significant dermal edema.
Neutrophils >10 000/mm3 and/or CRP >30 mg/L.
Because of the high prevalence of diabetes mellitus and thyroid abnormalities, this diagnosis alone is not sufficient to meet this minor criterion.
Approximately 50% of patients will have bone marrow changes that distinguish it from a typical monoclonal gammopathy of undetermined significance or myeloma bone marrow. Anemia and/or thrombocytopenia are distinctively unusual in this syndrome unless Castleman disease is present.