Faculty Bibliography

We report a 51-year-old female with a 3-year history of recalcitrant annular elastolytic giant cell granuloma (AEGCG) who was effectively treated with the anti-tumor necrosis factor (TNF)-alpha antibody, adalimumab. Her disease was refractory to topical glucocorticoids, intralesional glucocorticoids, narrow-band ultraviolet light (UV)-B phototherapy and cyclosporine. During her treatment with adalimumab she developed a positive anti-nuclear-antibody and double-stranded-DNA antibody and her treatment was terminated. Our findings suggest that adalimumab is an efficacious therapeutic alternative for the treatment of annular elastolytic giant cell granuloma unresponsive to standard therapies, however drug-induced lupus is a potential side effect that clinicians must be cognizant of. To our knowledge, this is the first time adalimumab has successfully been used in the treatment of AEGCG. <p><em>J Drugs Dermatol. 2017;16(2):169-171.</em></p>.

Kikuchi-Fujimoto's disease (KFD) and adult-onset Still's disease (AOSD) are rare inflammatory conditions with some overlapping features. We encountered a 22-year-old male patient who presented with daily fevers, neck discomfort, and sore throat and subsequently developed rash, arthritis, and cervical lymphadenopathy. Biopsy of the skin rash was consistent with KFD skin involvement. Given that the patient also met criteria for AOSD, a final diagnosis of KFD/AOSD co-occurrence was made. Anti-IL-1beta therapy with anakinra resulted in rapid resolution of all symptoms. A literature search identified eight more cases of KFD/AOSD. Fever, rash, arthritis, and lymphadenopathy were present in all patients. No case report demonstrated an association of rash eruption clearly associated with fever spikes. Duration of symptoms ranged from 3 weeks to 10 years. Seven patients had leukocytosis, six had anemia, and five demonstrated elevated ferritin and/or decreased glycosylated ferritin. Seven patients had elevated erythrocyte sedimentation rate (ESR), and seven had transaminitis. Eight of nine patients had no evidence of infectious disease. Autoantibodies were absent from all patients. KFD and AOSD are very rare diseases, yet they may overlap. The two conditions not only share several clinical and laboratory characteristics but also differ in characteristic ways. Given the rapid response observed with anakinra in the index patient, IL-1beta likely plays a role in both diseases.

BACKGROUND: The histopathologic diagnosis of mycosis fungoides (MF) has classically relied on the presence of atypical epidermotropic T-lymphocytes predominating over spongiosis. However, in some cases of MF, prominent epidermal mucinosis in a spongiosis-like pattern mimics a spongiotic dermatitis. To our knowledge, only one series in the literature has thus far recognized the presence of epidermal mucinosis in MF. METHODS: We evaluated 30 skin biopsies from 18 patients with the clinical diagnosis of MF, which fulfilled all histopathologic criteria for patch- or plaque-stage MF, but also showed epidermal mucinosis in a spongiosis-like pattern. Fifteen specimens were studied by immunohistochemistry, and 7 were tested for T-cell receptor (TCR) gene rearrangements. Twenty biopsies of spongiotic dermatitides were included as controls. RESULTS: We confirmed the presence of epidermal mucinosis in all 30 cases of MF with a spongiosis-like pattern based on histopathologic criteria and the colloidal iron stain for mucin. Immunohistochemistry in 14 out of 15 specimens showed marked loss of pan-T-cell antigens CD5 and CD7; and TCR clonality was detected in 7 specimens from 6 patients, supporting the diagnosis of MF CONCLUSIONS: We report helpful histopathologic criteria for distinguishing MF with epidermal mucinosis in a spongiosis-like pattern from spongiotic dermatitis.

We present a 76-year old woman with a five-year history of asymptomatic, white papules that were grouped on the lateral and posterior aspects of the neck, inferior axillae, and central mid-back. The histopathologic findings showed thickened collagen bundles. A diagnosis of white fibrous papulosis of the neck was made, which is believed to be a manifestation of intrinsic aging. There are no treatments for white fibrous papulosis of the neck. One hypothesized approach is the application of a topical anti-oxidant to reduce free-radical induced aging.

A 68-year-old woman presented for evaluation of a large, red-brown plaque on her left buttock with irregular borders and prominent overlying verrucous changes. The plaque had been present since childhood but over a three-year period had been enlarging with increasing nodularity and easy bleeding with trauma. Histopathologic examination demonstrated an enlarged papillated and polypoid heterogeneous lesion. In part of the specimen, there are bulbous aggregates of small squamous cells with foci of eccrine ductal differentiation. There are other areas with horn pseudocysts, hypergranulosis, and compact orthokeratosis with parakeratosis. There are scattered enlarged heavily pigmented melanocytes, some of which have long and thick dendrites. This collision tumor consisted of an eccrine poroma, a seborrheic keratosis, and a viral wart. The clinical and histopathologic features of collision tumors and poromas are reviewed.

Palpable migratory arciform erythema is a T-cell pseudolymphoma with no well-established treatment. The disease is rarely reported in the literature, perhaps because it is difficult to diagnose. We present a case of a variant of PMAE in a 30-year-old healthy man with no history of medication use and erythematous to violaceous annular and arciform plaques on his face, scalp and trunk. This case is of particular significance because gene rearrangement studies and histopathologic findings are concerning for folliculotropic mycosis fungoides while the clinical course does not support this diagnosis. The authors' emphasize that clinical history is imperative for definitive diagnosis of palpable migratory arciform erythema as it can clinically and histopathologically resemble other cutaneous lymphocytic diseases

J Drugs Dermatol 2014;13(10):1288-1289.

A 64-year-old man presented with a three-year history of an enlarging, pruritic, linear, verrucous plaque on his left lower extremity. Histopathologic examination was consistent with a verrucous epidermal nevus, which is a benign epidermal hamartoma, most commonly observed in the pediatric population. Verrucous epidermal nevi are often refractory to treatment and have high rates of recurrences, causing them to be therapeutic challenges. We review the treatment modalities reported to be effective in verrucous epidermal nevi.

Lichen planus pigmentosus (LPP) is an uncommon variant of lichen planus that tends to occur in middle-aged individuals with darker pigmented skin. Clinical findings include hyperpigmented, brown to gray-brown macules and patches in sun-exposed areas, typically on the head and neck. Histopathologic features include epidermal atrophy, vacuolar degeneration of the basal layer of the epidermis, perivascular lymphohistiocystic infiltrate in the upper dermis, and dermal melanophages. We present a unique case of LPP that was characterized by an atypical initial inflammatory phase and subsequent circinate lesions with central clearing.

We present a case of chronic graft-versus-host disease in a 61-year-old woman with a history of B-cell chronic lymphocytic leukemia that was treated with an allogeneic bone marrow transplant from an unrelated donor. The patient initially presented with erythematous patches on the trunk and extremities that evolved into reticulated, hyperpigmented patches and lichenified plaques.