Annular Elastolytic Giant Cell Granuloma Successfully Treated With Adalimumab Subsequently Complicated by Drug-Induced Lupus.

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.

J Drugs Dermatol. 2017;16(2):169-171.

Mycosis fungoides with epidermal mucinosis: a variant of mycosis fungoides with a spongiosis-like pattern.

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. A total of 15 specimens were studied by immunohistochemistry, and seven 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 15 specimens showed significant loss of pan-T-cell antigens CD5 (10/15) and CD7 (14/15); 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.

Co-occurrence of Kikuchi-Fujimoto's disease and Still's disease: case report and review of previously reported cases.

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-1ß 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-1ß likely plays a role in both diseases.

White fibrous papulosis of the neck.

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.

Collision tumor of eccrine poroma, seborrheic keratosis, and a viral wart.

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.

A variant of palpable migratory arciform erythema.

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.

Verrucous epidermal nevus.

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.

Graft-vs-host disease.

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.

Fluoroscopy-associated radiation dermatitis.

We present a 67-year-old man with an ulcerated, indurated plaque on the right mid back with a presumed diagnosis of morphea that was complicated by an allergic contact dermatitis. Further clinical and histopathologic data elucidated the diagnosis of fluoroscopy-induced radiation dermatitis. We present a brief review of the common locations, clinical characteristics, pathophysiology, and management options for fluoroscopy-induced radiation dermatitis.

Lichen planus pigmentosus.

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.

Trauma-related papular granuloma annular.

Granuloma annulare (GA) is a benign, granulomatous disease with several clinical manifestations, which include localized, generalized, perforating, subcutaneous, patch, papular, and linear forms. We report a case of papular GA of the dorsal aspects of the hands that arose after repeated, direct trauma to the site of subsequent involvement. Although multiple etiologies for GA have been proposed, which include ultraviolet light, arthropod bites, trauma, tuberculin skin tests, viral infections, and PUVA photochemotherapy, the underlying pathogenesis of the disorder remains unclear. However, owing to the key histopathologic findings of focal collagen and elastic fiber degeneration and mucin deosition in GA, it is not surprising that cutaneous trauma may have played a role in connective tissue injury, subsequent degeneration, and the production of a granulomatous response with increased mucin deposition.

Steatocystoma.

Steatocystoma multiplex is a rare condition that is characterized by cutaneous cysts and may be inherited in an autosomal dominant manner or may occur sporadically. The pathogenesis is hypothesized to involve mutations in the keratin 17 gene. There are no internal manifestations. The lesions are usually asymptomatic. However, a suppurative variant exists in which the lesions become inflamed and suppurative after minor trauma. Treatments include cryosurgery, aspiration, surgical excision, laser therapy, and modified surgical incision techniques. This report presents a case of steatocystoma multiplex, the suppurative variant, in a 26-year-old woman with involvement of rare locations on the buttocks, groin, and extremities.

Single versus multiple primary melanomas: old questions and new answers.

BACKGROUND: In patients with multiple primary melanomas (MPM), mean tumor thickness tends to decrease from the first melanoma to the second melanoma, and prognosis may be improved compared with the prognosis for patients who have a single primary melanoma (SPM). In this study, the authors compared the clinicopathologic features of patients with MPM and SPM to better characterize the differences between these 2 groups and to determine whether or not there is an inherent difference in tumor aggression. METHODS: In total, 788 patients with melanoma who were enrolled prospectively in the Interdisciplinary Melanoma Cooperative Group database from 2002 to 2008 were studied. Patients with SPM and with MPM were compared with regard to clinical and primary melanoma characteristics. RESULTS: Of 788 patients with melanoma, 61 patients (7.7%) had 2 or more primary melanomas. The incidence of developing a second primary melanoma 1 year and 5 years after initial melanoma diagnosis was 4.1% and 8.7%, respectively, and most of the risk accumulated within the first year. The incidence of MPM was greater in patients aged ≥60 years than in those aged ≤60 years. The absence or presence of mitosis and other tumor characteristics did not differ significantly between patients with SPM and patients with MPM (P = .61). CONCLUSIONS: No difference was observed in the presence or absence of mitoses, a marker of tumor proliferation, in SPM and MPM. Because it has been demonstrated that the presence of mitosis is a powerful prognostic marker, the current findings suggested that the tumors behave similarly in patients with SPM and patients with MPM. The authors concluded that differences in tumor thickness and prognosis between SPM and MPM more likely are caused by factors other than tumor biology, such as increased surveillance.

Eruptive basal-cell carcinomas in the setting of human immunodeficiency virus infection.

Patients with human immunodeficiency virus are known to have an increased risk for development of cutaneous malignant conditions. We present a 55-year-old man with a history of an isolated squamous-cell carcinoma five years prior, who presented with 27 cutaneous lesions, which had developed over the course of the preceding year. This occurred in the context of a new diagnosis of human immunodeficiency virus (HIV) infection and acquired immune deficiency syndrome (AIDS). The initiation of anti-retroviral therapy and subsequent restoration of CD4 counts was associated with an increase in the number of lesions. Histopathologic examination of representative tumors showed basal-cell carcinomas, which lead to the diagnosis of eruptive basal-cell carcinomas in the setting of HIV-related immunosuppression. The incidence and epidemiology of malignant conditions that are related and non-related to AIDS are reviewed as well as discussion of the current literature regarding presentation, treatment, and prevention of non-melanoma skin cancers in patients with HIV infection.

Poikilodermatous mycosis fungoides.

Poikilodermatous mycosis fungoides (MF) is a variant of MF, formerly referred to as poikiloderma vasculare atrophicans. The lesions are classically characterized by large plaques of hypopigmentation and hyperpigmentation with atrophy and telangiectases. The plaques may be asymptomatic or mildly pruritic and typically involve the major flexural areas and trunk. Poikilodermatous MF has an early stage (IA-IIA) at diagnosis and a male predominance. Poikilodermatous MF shows an atypical T-cell infiltrate in the papillary dermis with evidence of epidermotropism, epidermal atrophy, dilated blood vessels in the dermis, melanophages, and melanin incontinence. Recent studies suggest a predominance of a CD8+, CD4- immunophenotype. Treatment modalities are similar to classic MF with phototherapy being the most common first-line therapy. Poikilodermatous MF has an excellent prognosis.

Purpura annularis telangiectoides.

Purpura annularis telangiectoides of Majocchi (PATM) is a clinical variant of pigmented purpuric dermatoses. It is characterized by asymptomatic or mildly pruritic, erythematous, annular patches that vary in size. The patches exhibit central clearing and peripheral cayenne pepper petechiae. It classically occurs on the lower legs but may be more generalized. Histopathologic features include a lymphocytic capillaritis with subsequent capillary leakage and extravasated erythrocytes, which is typical of all pigmented purpuric dermatoses. The pathogenesis of PATM is unknown, but it may uncommonly be associated with underlying diseases such as hematologic disorders and rarely cutaneous T-cell lymphoma. There is no standard effective treatment.

Eruptive vellus hair cysts.

Eruptive vellus hair cyst (EVHC), described initially in 1977, is a benign dermatologic condition that is characterized by the sudden appearance of monomorphic, follicular, asymptomatic, small papules in children and young adults. The diagnosis is based on the histopathologic findings of stratified-squamous epithelium with a granular layer that surrounds a cystic space filled with laminated keratin and a variable number of vellus hair cysts. EVHC can be associated with steatocystoma multiplex. A current hypothesis suggests that EVHC originates from a cystic change at the insertion of the pilosebaceous duct. EVHC is primarily treated for cosmesis with retinoids, surgery, and lasers.

Dissecting cellulitis of the scalp.

Dissecting cellulitis of the scalp is a chronic, relapsing, inflammatory disease of the scalp that results in scarring alopecia. We present a case of a 32-year-old man with recalcitrant disease who is now responding to treatment with isotretinoin. The pathogenesis, clinical presentation, disease associations, and histopathological findings are reviewed. Treatment can be challenging. The literature on medical and surgical therapeutic options is reviewed.

Perifollicular Langerhans cell histiocytosis.

A 25-year-old man presented with a 13-year history of an erythematous, papular eruption of his face and trunk, which was treated in the past as acne and psoriasis with isotretinoin and methotrexate, respectively. Histopathologic examination demonstrated an infiltrate of Langerhans cells, which was consistent with Langerhans cell histiocytosis. The epidemiology, clinical presentation, and treatment options of this disease are reviewed.