Table 1.

Diagnostic criteria for five selected cutaneous monoclonal gammopathy of clinical significance.

POEMS syndromea) [26]	Scleromyxedema [24]	Schnitzler syndromeb,c) [26]	Necrobiotic xanthogranulomad) [30]	TEMPI syndrome [33]
Mandatory major criteria 1. Polyneuropathy 2. Monoclonal plasma cell-proliferative disorder (almost always l)	Generalized papular and sclerodermoid eruption Evidence of monoclonal gammopathy Characteristic pathologic triad, associating dermal mucin accumulation, increased collagen deposition, and fibroblast proliferation Absence of thyroid disease	Obligate criteria 1. Chronic urticarial rash 2. Monoclonal IgM or IgG	Major criteria 1. Cutaneous papules, plaques, and/ or nodules, most often yellow or orange in color 2. Histopathological features demonstrating palisading granulomas with lymphoplasmacytic infiltrate and zones of necrobiosis. Variably present cholesterol clefts and/or giant cells	Major criteria 1. Telangiectasias 2. Monoclonal gammopathy 3. Elevated erythropoietin and erythrocytosis 4. Perinephric fluid
Other major criteria 3. Castleman disease 4. Sclerotic bone lesions 5. Vascular endothelial growth factor elevation
Minor criteria 6. Organomegaly (splenomegaly, hepatomegaly, or lymphadenopathy) 7. Extravascular volume overload(edema, pleural effusion, or ascites) 8. Endocrinopathy (adrenal, thyroid, pituitary, gonadal, parathyroid, and pancreatic) 9. Skin changes (hyperpigmentation, hypertrichosis, glomeruloid hemangiomas, plethora, lipodystrophy, acrocyanosis, flushing, and white nails) 10. Papilledema 11. Thrombocytosis/polycythemia 12. Other: clubbing, weight loss, hyperhidrosis, pulmonary hypertension/restrictive lung disease, thrombotic diatheses, diarrhea, and low vitamin B12		Minor criteria 3. Recurrent fever 4. Objective findings of abnormal bone remodeling with or without bone pain 5. A neutrophilic dermal infiltrate on skin biopsy 6. Leukocytosis and/ or elevated CRP	Minor criteria 3. Periorbital distribution of cutaneous lesions 4. Paraproteinemia, most often IgGl, plasma cell dyscrasia, and/or other associated lymphoproliferative disorder	Minor criteria 5. Intrapulmonary shunting 6. Other: venous thrombosis

^a)POEMS syndrome diagnosis is confirmed when both mandatory major criteria, one of the other major criteria, and one of the minor criteria are present. ^b)Definite diagnosis of Schnitzler syndrome: if IgM, both obligate criteria and at least2 minor criteria; if IgG, both obligate criteria and 3 minor criteria. ^c)Probable diagnosis of Schnitzler syndrome: if IgM, both obligate criteria and 1 minor criteria; if IgG, both obligate criteria and 2 minor criteria. ^d)Xanthogranuloma diagnosis is confirmed when both major criteria and at least 1 minor criterion are present. It is only applicable in the absence of a foreign body, infection, or other identifiable causes.