We observed 5 patients with subcutaneous panniculitis-like T-cell lymphoma (SPTCL) who were unusual, in that they also exhibited features of lupus erythematosus (LE). This observation is in keeping with a recent study that reported an increased rate of autoimmune disease, including systemic lupus erythematosus (SLE), among patients with SPTCL. In all cases, attributes indicating SPTCL included an infiltrate of lymphocytes with pleomorphic nuclei involving subcutaneous lobules exhibiting a cytotoxic T-cell (CD3/CD8/betaF1) immunophenotype. Additionally, a high proliferation rate and a monoclonal T-cell receptor-gamma gene rearrangement were observed in most cases. The manifestations of LE in these patients included a spectrum of clinical and histopathological abnormalities. Clinical manifestations of LE included, in some patients, morphologic evidence of lupus erythematosus panniculitis (LEP) with subcutaneous nodules that healed with lipoatrophy on the face. In addition, all the patients exhibited serologic and/or extracutaneous end-organ abnormalities seen in patients with SLE, with 2 patients having sufficient findings to meet American College of Rheumatology criteria for SLE. Histopathological evidence of LE included vacuolar change at the dermal-epidermal interface in 3 patients, 2 of whom also showed interstitial deposition of mucin in the reticular dermis. One of these patients also had findings of LEP in the subcutaneous lobules with clusters of CD20 B cells partially arranged within germinal centers. In 2 patients in which neither the epidermis nor dermis was available for review, histopathological features of LE included, in one patient, a few small clusters of CD123 plasmacytoid dendritic cells within the adipose tissue and, in the other patient, a positive direct immunofluorescence test (lupus band) on clinically uninvolved and lesional skin. Our study shows that some patients show overlap between SPTCL and LE. We suspect that these patients may suffer from both diseases concomitantly. Furthermore, patients with LE, particularly LEP, should be monitored for evolution into SPTCL with biopsy of any subcutaneous lesion that is not typical of LEP. Additionally, screening for cutaneous LE and SLE could be considered in patients with SPTCL.
Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is characterized by panniculitic infiltrates that may be difficult to distinguish from inflammatory disorders, particularly lupus erythematosus profundus (LEP). We report on 11 patients (M:F=5:6; median age: 49 y; range: 20 to 75 y) presenting with lobular panniculitic infiltrates showing histopathologic features of both SPTCL and LEP in different parts of the same biopsy specimen. The areas showing aspects of SPTCL revealed dense infiltrates of small and medium-sized, atypical α/β T-cytotoxic lymphocytes with focal rimming of the adipocytes and high proliferation. In other areas the infiltrate was composed of nodules of B lymphocytes arranged characteristically at the periphery of the fat lobules and in the septa and showing a low proliferation rate. CD123-positive plasmocytoid dendritic cells arranged in small clusters could be observed in 3 cases. Our observation raises an important question concerning the relationship between SPTCL and LEP. A simple chance overlap of 2 unrelated pathologies seems unlikely, as we could observe these unusual features in 11 cases, much more than mere chance would justify. Three other hypotheses may explain the features observed in our patients: (1) these are examples of SPTCL with focal histologic features mimicking those of LEP; (2) these are examples of LEP with focal atypical histologic features mimicking those of SPTCL; (3) SPTCL and LEP may represent 2 ends of a spectrum, a hypothesis that may be supported by the frequent association of the 2 diseases.
Amyloidosis cutis dyschromica (ACD) is a rare form of primary cutaneous amyloidosis (PCA). There is a paucity of information in the dermatology literature to guide its diagnosis, investigation and treatment. We present two siblings with ACD and summarise the epidemiology, clinical features, natural history and treatments in 48 cases of ACD from the literature. Familial cases were more common (37) than sporadic cases. ACD is predominantly reported in those of East and South-East Asian ethnicity (63%). The mean age of onset was 6 years in familial cases, and 23 years in sporadic cases. The clinical features of familial and sporadic ACD do not differ substantially. Pruritus was the only symptom, and was reported in 19% of all cases. There were no reported ACD cases with systemic amyloidosis. Acitretin was reported to result in improvement in seven of 10 patients treated. Routine investigation for systemic involvement is not necessary. Acitretin may be helpful.
The association of necrotizing fasciitis, often due to group A streptococcus and primary varicella (chicken pox), is unusual but recognized in children. The association in adults is rare but one report in the literature describes a previously healthy man with the two disorders. We now describe a case of disseminated cutaneous herpes zoster complicated by subacute necrotizing fasciitis in an elderly woman taking low dose methotrexate and prednisone for rheumatoid arthritis. Staphylococcus aureus was isolated. Localized debridement and split skin grafting were required.
Abstract— A 10‐year‐old male Labrador Retriever dog presented with alopecia and scaling that was extensive in the head and limb regions and patchy over the trunk. The major histopathological feature was degeneration of the outer root sheath with disruption of keratinocytes and formation of cystic spaces filled with mucin. A diagnosis of follicular mucinosis was made. This case report represents the first case of this syndrome in the canine species. Résumé— Un Labrador, mâle de 10 ans est présenté pour une alopécie squameuse, extensive, de la tête, des membres, et en “taches” sur le tronc. La lésion histopathologique principale est une dégénérescence de la gaine externe du poil avec dislocation des kératinocytes et la formation d'espaces kystiques remplis de mucine. Un diagnostic de mucinose folliculaire a été fait. Ce caqs est le premier décrit chez le chien. [Bell, A., Oliver, F. Alopecia mucinosa (follicular mucinosis) in a dog (Alopecia Mucinosa (mucinose folliculaire) chez un chien).
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