A Diagnostically Challenging Case of Febrile Ulceronecrotic Mucha-Habermann Disease in an Adult Female Successfully Treated with Methotrexate and Cyclosporine.

Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is a rare and severe variant of pityriasis lichenoides et varioliformis acuta (PLEVA) characterized by intermittent pyrexia, acute onset of generalized ulceronecrotic lesions, and histopathology suggestive of PLEVA. Prompt diagnosis and treatment are necessary to halt the progression of this potentially fatal disease; however, the widely variable presentation of FUMHD in addition to its rarity poses a diagnostic challenge. We report the case of a previously healthy 43-year-old woman who presented to the emergency department with 1 month of generalized rash and intermittent fevers. Her only reported new exposure were elective intravenous vitamin infusions received at a medi-spa 1 week prior to onset of lesions. Initial evaluations were inconclusive, and confluent ulceronecrotic, hemorrhagic lesions appeared on approximately 90% of her body despite steroid, antibiotic, and cyclosporine therapy. Repeat histopathology was consistent with PLEVA, and in the context of her clinical presentation she was diagnosed with FUMHD. The patient rapidly attained remission with methotrexate therapy but sustained residual scarring.

The histological prevalence and clinical implications of folliculotropism and syringotropism in mycosis fungoides.

BACKGROUND: Mycosis fungoides (MF), the most common form of cutaneous T-cell lymphoma, remains a challenge for clinicians to stage and manage. Classically, MF is determined through histopathologic evidence of a neoplastic infiltrate within the epidermis. In certain patients, however, the infiltrate extends into the hair follicles and sweat glands. The objective of this study is to determine the utility of expanding the analysis of histopathology reports to include the reporting of folliculotropism and syringotropism. METHODS: This is a prospective, observational study conducted in a single facility from 2012-2018. All patients with MF, excluding those treated at this facility for less than six months with the exception of those with incomplete pruritus documentation, or absence of initial biopsy analysis were studied. Modified severity weighted assessment tool (mSWAT) quantified body surface area and treatments attempted per patient were continuously charted. Patients were surveyed for presence and degree of pruritus and pain. Evaluation of these parameters were charted at the initial patient visit and correlated with their primary biopsy for presence or absence of folliculotropism and syringotropism. RESULTS: Of the 87 patients examined, 70 patients (80%) exhibited syringotropism in their original biopsy and 68 patients (78%) exhibited folliculotropism. Presence of both findings concurrently was present in 56 patients (64.4%), while neither finding was present in 5 patients (5.8%). The singular finding of folliculotropism was found in 12 patients (13.8%), while the singular finding of syringotropism was exhibited in 14 patients (16.1%). A significant association between the presence of folliculotropism and pruritus was established (P=0.043, α=0.05). The general trend towards increase in mSWAT score and pruritus in patients in regard to the mean and median values suggest that increasing the sample population of the study might yield a significant value in the future. CONCLUSIONS: These presentations are more prevalent than previously recognized and have findings indicative of more severe disease. We propose that MF histopathology reports document the presence of folliculotropism and syringotropism and that these findings be added to the NCCN guidelines as they may aid in predicting severity and progression risk.

Camouflaging Agents for Vitiligo Patients.

Vitiligo is an acquired condition resulting in patches of depigmented skin that is cosmetically disfiguring and can subsequently be psychologically disturbing. For patients seeking to mask their vitiligo, camouflage options have historically been limited and been designated as a cosmetic, rather than a medical, concern. As research has indicated that proper concealment of vitiligo lesions can vastly improve quality of life, we believe it is essential that dermatologists become aware of all the options available to their patients and that discussions of camouflage options be broached from the first visit. Methods for concealment include cosmetic tattoos, dihydroxyacetone, general cosmetics, and various topical camouflage agents, including the newest product, Microskin™. We conducted a literature review of all of the available options for vitiligo concealment and evaluated their advantages and disadvantages. Ultimately, temporary methods of concealment are recommended; but the particular agent used can come from discussion with the patient based on the location of the lesions, degree of concealment desired, cost, and availability.

Crosstalk between Desmoglein 2 and Patched 1 accelerates chemical-induced skin tumorigenesis.

Aberrant activation of Hedgehog (Hh) signaling is causative of BCCs and has been associated with a fraction of SCCs. Desmoglein 2 (Dsg2) is an adhesion protein that is upregulated in many cancers and overexpression of Dsg2 in the epidermis renders mice more susceptible to squamous-derived neoplasia. Here we examined a potential crosstalk between Dsg2 and Hh signaling in skin tumorigenesis. Our findings show that Dsg2 modulates Gli1 expression, in vitro and in vivo. Ectopic expression of Dsg2 on Ptc1(+/lacZ) background enhanced epidermal proliferation and interfollicular activation of the Hh pathway. Furthermore, in response to DMBA/TPA, the Dsg2/Ptc1+/lacZ mice developed squamous lessons earlier than the WT, Ptc1(+/lacZ), and Inv-Dsg2 littermates. Additionally, DMBA/TPA induced BCC formation in all mice harboring the Ptc1(+/lacZ) gene and the presence of Dsg2 in Dsg2/Ptc1(+/lacZ) mice doubled the BCC tumor burden. Reporter analysis revealed activation of the Hh pathway in the BCC tumors. However, in the SCCs we observed Hh activity only in the underlying dermis of the tumors. Furthermore, Dsg2/Ptc1(+/lacZ) mice demonstrated enhanced MEK/Erk1/2 activation within the tumors and expression of Shh in the dermis. In summary, our results demonstrate that Dsg2 modulates Hh signaling, and this synergy may accelerate skin tumor development by different mechanisms.