Papuloerythroderma associated with monoclonal gammopathy of undetermined significance

Abstract

We describe a 73-year-old Japanese man with papuloerythroderma overlapped with monoclonal gammopathy of undetermined significance (MGUS). Clinically, prominent erythroderma was associated with disseminated pruriginous papules, which were characteristically spared on the axillary and inguinal regions, the cubital and popliteal fossae as well as abdominal and small positional folds. Histopathologically, there was a significant perivascular infiltrate of lymphohistiocytic cells intermingled with eosinophils in the upper dermis. A biochemical profile revealed the presence of immunoglobulin G kappa chain type monoclonal protein in the serum but the absence of hematological neoplasms. We diagnosed the patient as papuloerythroderma with MGUS, and treated him with narrow-band ultraviolet B and topical steroid. His skin changes were improved, but the sharp gamma-globulin peak remained in the electrophoresis of serum protein. This case suggests an association between papuloerythroderma and MGUS.

Papuloerythroderma 2009: Two New Cases and Systematic Review of the Worldwide Literature 25 Years after Its Identification by Ofuji et al.

“…Cancer was associated with PEO in 37 cases (21.76%; 24 Japanese and 13 non-Japanese), among which 25 were solid malignancies and 12 hematological neoplasms (a case of monoclonal gammopathy of undetermined significance [50] and one of myelodysplastic syndrome [40] were also included even if they are actually preneoplastic conditions; table 2). In 3 cases, cancer treatment induced remission of PEO [34,49,52].…”

“…Seventy papers belonged to the English literature, 25 to the Asian literature and 17 to the non-English/non-Asian Western literature. One hundred twenty-five [1,2,3,4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22,23,24,25,26,27,28,29,30,31,32,33,34,35,36,37,38,39,40,41,42,43,44,45,46,47,48,49,50,51], 25 [52,53,54,55,56,57,58,59,60,61,62,63,64,65,66,67,68,69,70,71,72,73], and 18 cases of PEO [74,75,76,77,78,79,80,81,82,83,84,85,86,87] were identified, respectively, for a grand total of 170 cases (including the present 2) as of July 31, 2009. Six case reports were excluded as redundancy with later articles was suspected [88,89<...>…”

“…Nail and buttock infarcts [2], hypothyroidism [7], depression [10], coronary artery disease [11,15,21,50], Quincke’s edema [11], thrombocytopenia [12], arterial hypertension [12,21,74], chronic obstructive pulmonary disease [15,16,21], diabetes mellitus [15,64], benign prostatic hyperplasia [15], cholesteatoma [19], hypercholesterolemia [19], hepatic cysts [21], arthritis [3,21], atrial fibrillation [21], duodenal ulcer [21], goiter [41] and gallstones [26,50] were not considered significant. Seven cases of drug-induced PEO have been reported (4.11%) [23,40,45,47,48,58,67,69], including one in which the name of the medication could not be obtained [58].…”

“…Synchronous or diachronical coexistence of PEO and malignancy or preneoplastic syndromes (2 cases) [40,50] was reported in 21.76% of patients, almost two thirds of whom were Japanese (relative risk 1.69). It should also be noted that the frequency of gastric cancer (40.54%) and hematological neoplasms (32.43%) – found most often in Japanese and non-Japanese subjects, respectively – is much higher than expected.…”