Erythema elevatum diutinum is a rare, chronic cutaneous leukocytoclastic vasculitis, with prominent fibrosis at its later stage. In this article, we report a case of erythema elevatum diutinum in a 23-year-old woman with well-controlled Crohn disease. To our knowledge, erythema elevatum diutinum has been reported in only three other cases of Crohn disease, in which eruptions of erythema elevatum diutinum were associated with features of active Crohn. Our patient was in clinical remission at the time of erythema elevatum diutinum onset, making this report significant not only for its uncommon presentation, but more importantly, to aid readers. diagnosis and clinical management of similar cases.

Folliculosebaceous cystic hamartoma (FSCH) is a benign lesion that presents as a solitary papule or nodule that typically affects the face and scalp of adults. A few reports have observed a congenital presentation. We present an infant boy exhibiting a tumor with overlap features between an FSCH and fibrofolliculoma in the perianal region that was first noticed at 6 months of age. The histological examination showed a hamartomatous benign proliferation of hair follicles and disordered sebaceous glands, which is consistent with the infundibular structures and radiating sebaceous glands that are typically observed in previously reported cases of FSCH. Sebaceous differentiation is a hallmark feature of FSCH. Folliculosebaceous cystic hamartoma is believed to be a late-stage form of trichofolliculoma (TF). Another consideration is that FSCH and TF are two distinct entities set apart by their degree of sebaceous or follicular differentiation and development of the mesenchymal stroma.

We present an original case report of a 45-year-old woman with a five-month history of sporadic, tender, nodules present on the right upper abdomen, bilateral dorsal wrists, right upper arm, and left flank. Biopsy revealed a mild perivascular infiltrate, increased dermal mucin, and no significant increase in fibroblasts. Presentation and histology were most consistent with nodular lichen myxedematosus (NLM), a rare primary mucinosis. Only four previous cases are reported in the literature to our knowledge. Management of NLM and other subtypes of lichen myxedematosus is not well described. Our patient failed systemic steroids and was unable to tolerate hydroxychloroquine, but subsequently improved with oral methotrexate. This suggests that methotrexate may be of benefit for NLM.

Cutaneous plasmacytosis has <60 cases worldwide, typically characterized by multiple asymmetric facial and truncal cutaneous nodules and plaques. We describe the case of a 68-year-old woman with erythematous plaques on the feet who had a biopsy showing primary cutaneous plasmacytosis and subsequent workup revealing celiac disease. Our patient's clinical presentation of symmetric plaques on the dorsal feet is previously unreported. Additionally, plasmacytosis occurs predominantly in Japanese patients and men younger than 40. Cutaneous plasmacytosis is hypothesized to be reactive from overreaction to stimuli including trauma, infections, or malignancies. The origin of our patient's reactive process could be related to celiac disease or could be unknown. Plasmacytosis in bone marrow has been reported with celiac disease, but to our knowledge, this is the first report of cutaneous plasmacytosis in a patient with celiac disease.

Introduction

Mycosis fungoides (MF), the most common type of cutaneous T-cell lymphoma, can lead to disfiguring lesions, debilitating pruritus and frequent skin infections. This study assessed response to brentuximab vedotin in patients with MF in the phase III ALCANZA study.

Methods

Baseline CD30 levels and large-cell transformation (LCT) status were centrally reviewed in patients with previously-treated CD30-positive MF using ≥2 skin biopsies obtained at screening; eligible patients required ≥1 biopsy with ≥10% CD30 expression. Patients were categorised as CD30_min < 10% (≥1 biopsy with <10% CD30 expression), or CD30_min ≥ 10% (all biopsies with ≥10% CD30 expression) and baseline LCT present or absent. Efficacy analyses were the proportion of patients with objective response lasting ≥4 months (ORR4) and progression-free survival (PFS).

Results

Clinical activity with brentuximab vedotin was observed across all CD30 expression levels in patients with ≥1 biopsy showing ≥10% CD30 expression. Superior ORR4 was observed with brentuximab vedotin versus physician's choice in patients: with CD30_min < 10% (40.9% versus 9.5%), with CD30_min ≥ 10% (57.1% versus 10.3%), with LCT (64.7% versus 17.6%) and without LCT (38.7% versus 6.5%). Brentuximab vedotin improved median PFS versus physician's choice in patients: with CD30_min < 10% (16.7 versus 2.3 months), with CD30_min ≥ 10% (15.5 versus 3.9 months), with LCT (15.5 versus 2.8 months) and without LCT (16.1 versus 3.5 months). Safety profiles were generally comparable across subgroups.

Conclusion

These exploratory analyses demonstrated that brentuximab vedotin improved rates of ORR4 and PFS versus physician's choice in patients with CD30-positive MF and ≥1 biopsy showing ≥10% CD30 expression, regardless of LCT status.

Clinical trial registration

Clinicaltrials.gov, NCT01578499.