Mouth and Genital Ulcers with Inflamed Cartilage Syndrome: Case Report and Review of the Published Work.

Mouth and genital ulcers with inflamed cartilage (MAGIC) syndrome are disease that fulfilled criteria for diagnosis of Behcet's disease (BD) and relapsing polychondritis (RP). We report a 22-year-old Japanese woman presented with MAGIC syndrome and we described the clinicopathological characteristics of MAGIC syndrome based on a review of published cases from July 1985 to December 2015. In our case, the patient with oral aphthae, erythema nodosum, acne-like eruptions, uveitis, and polyarthritis fulfilled criteria for diagnosis of incomplete form of BD. The patient with uveitis, polyarthritis, and histological confirmation of chondritis also fulfilled criteria for diagnosis of RP. The patient was successfully treated with oral colchicine followed by prednisolone. The symptoms of MAGIC syndrome gradually disappeared, and the prednisolone dosage was gradually decreased and stopped. She has been in remission without active medication for a further 8 months. In the previous reports, some authors suggested that MAGIC syndrome was not a disease entity and might be RP occurring secondary to BD, another association of an autoimmune disease, or vasculitis with RP. However, the pathogenic association between MAGIC syndrome, BD, and RP is still unclear, and the number of reported cases of MAGIC syndrome is insufficient to establish a clear explanation. Therefore, further accumulation of data and careful observation of the clinical course are required to improve the understanding of MAGIC syndrome.

Clinical usefulness of Mohs' chemosurgery for palliative purposes in patients with cutaneous squamous cell carcinoma with risk factors or without indication for surgery: three case reports.

Mohs' chemosurgery is a technique of chemical fixation of a cutaneous tumor using 20% zinc chloride, and the surgeon performs a sequential series of excisions until no residual tumor can be found in the bottom layer of the specimen by microscopic examination. This technique has also been gradually accepted as one of the palliative treatment options in patients with cutaneous tumors with risk factors for surgery. We report three cases of cutaneous squamous cell carcinoma that were treated with Mohs' chemosurgery for palliative purposes. In case 1, Mohs' chemosurgery could prevent rapid tumor growth and control the bleeding, exudation and offensive odor from ulcerated tumors. The patient was able to live enjoyably at home with her family before she was transferred to a palliative care hospital. In case 2, Mohs' chemosurgery followed by debridement of residual tumor and a full-thickness skin graft contributed to an early hospital discharge, which prevented the progression of cognitive decline. In case 3, remission of the tumor was achieved by concurrent treatment with local radiotherapy and Mohs' chemosurgery. Mohs' chemosurgery for palliative purposes is not a radical treatment, but contributes to the patient's quality of life. It has the possibility of becoming a radical treatment if eligible patients and concurrent treatment are carefully selected. An increase in the number of skin cancer patients with risk factors for surgery has been predicted because of the progression of the aging society. Mohs' chemosurgery may become one of the treatment options for such patients.

Acute generalized exanthematous pustulosis caused by dihydrocodeine phosphate in a patient with psoriasis vulgaris and a heterozygous IL36RN mutation.

IMPORTANCE: Acute generalized exanthematous pustulosis (AGEP) is a rare and severe type of drug eruption. Dihydrocodeine phosphate is a semisynthetic opioid analgesic. Recently, recessive mutations in IL36RN have been identified in generalized pustular psoriasis (GPP). To date, 4 cases of AGEP and IL36RN mutation without previous history of psoriasis vulgaris (PV) have been reported. OBSERVATIONS: A woman in her 60s with PV presented with diffuse erythema, nonfollicular pustules, and fever. She had been treated with dextromethorphan hydrobromide hydrate, amoxicillin hydrate, clarithromycin, dihydrocodeine phosphate, tipepidine hibenzate, and tulobuterol tape for a cough and common cold. Based on histopathologic results and a positive result in a drug provocation test with dihydrocodeine phosphate, she was diagnosed with AGEP. A heterozygous IL36RN mutation c.28C>T (p.Arg10X) was also confirmed by mutation analysis. CONCLUSIONS AND RELEVANCE: This is the first report of dihydrocodeine phosphate-induced AGEP. In this case, helper T cells, type 17, might have been activated because of morphine and underlying PV, followed by increased production of interleukin (IL) 36. However, because of the IL36RN mutation, IL-36 signaling was uncontrolled, which might have resulted in the occurrence of AGEP. An IL36RN mutation might underlie several different pustular skin eruptions, including AGEP and GPP, and further accumulation of patient data is required.

Rheumatoid arthritis/methotrexate-associated primary cutaneous diffuse large B-cell lymphoma, leg type.

This report concerns a 62-year-old man with primary cutaneous diffuse large B-cell lymphoma (PCLBCL), leg type that developed during methotrexate (MTX) treatment for rheumatoid arthritis (RA). Several tumors were observed on the left lower leg. A histological analysis showed diffuse proliferation of large neoplastic B-cells that were immunophenotypically CD10-/MUM1+/BCL6-/BCL2+ and cytogenetically had IgH/c-MYC translocation without translocation involving BCL6 or IgH/BCL2. No evidence of Epstein-Barr virus (EBV) infection was found. The discontinuation of MTX resulted in a 20-month disease-free period. No previous cases of PCLBCL, leg type associated with RA or MTX therapy have been reported. The phenotypes of our patient were partly different from those of typical PCLBCL, leg type or RA/MTX-associated lymphoma.

Mycosis fungoides palmaris et plantaris successfully treated with radiotherapy: case report and mini-review of the published work.

Mycosis fungoides palmaris et plantaris (MFPP) is a rare variant of cutaneous T-cell lymphoma limited to the palms and soles that is not widely recognized because of its uncommon occurrence. We report a 73-year-old Japanese man who presented with an erosion on the left dorsal hand, a reddish tumor on the right palm, and hyperkeratotic erythematous plaques on the right sole. Skin biopsy showed histological features of mycosis fungoides (MF) with invasion into the deeper layers of skin. There was no visceral or lymph node invasion. We diagnosed this case as MFPP. External beam radiotherapy (EBRT) was performed to treat the hand lesions. Combination treatment with topical steroids and topical psoralen plus ultraviolet light therapy was performed to treat the right sole lesion, but was ineffective. Therefore, sequential EBRT was performed. Complete remission of all lesions was obtained. This is the first report of MFPP with a locally advanced tumor for which the efficacy of radiotherapy is described in detail. MFPP lesions occur on the dorsal aspect of hand or foot, and here we propose a classification of MFPP as hand and foot MF. The pathogenesis of MFPP is still unclear and further accumulation of data is required.