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Open Access Publications from the University of California

About

Dermatology Online Journal is an open-access, refereed publication intended to meet reference and education needs of the international dermatology community since 1995. Dermatology Online Journal is supported by the Department of Dermatology UC Davis, and by the Northern California Veterans Administration.

Volume 20, Issue 11, 2014

Review

Detailed protocol for administration of intralesional IL-2 for the treatment of Stage IIIc and IV M1a metastatic melanoma based on current NCCN guidelines

Melanoma claims approximately 9,000 lives in the United States annually [1].  Patients who present with satellite, in-transit, or distant cutaneous metastases have limited treatment options and the prognosis for patients with metastatic disease remains poor. Surgical excision remains the most common treatment modality for cutaneous metastases, but may not address concurrent subclinical in-transit metastases. Other palliative treatment options include Bacillus Calmette–Guérin (BCG) and isolated limb perfusion (ILP).  Although intravenous IL-2 has been used for treatment of metastatic melanoma since 1998, intralesional IL-2 has only now been included in the most recent National Comprehensive Cancer Network (NCCN) guidelines after case series and phase I/II clinical trials have shown promising results against Stage IIIc and IV M1a melanoma. Intralesional IL-2 protocols have varied markedly from study to study and there are no consensus guidelines available to help direct treatment.  Herein, we present a detailed protocol for the administration of intralesional IL-2 that has been successfully used at two different institutions for treatment of cutaneous melanoma metastases.

Case Presentation

A case of Griscelli syndrome

A hallmark of Griscelli syndrome, a rare autosomal recessive disorder, is hair hypopigmentation characterized by a silver-gray sheen and the presence of large clusters of pigment unevenly distributed in the hair shaft. Either a primary neurological impairment or immune abnormalities are associated with this phenotype. We report the case of a 10-year-old child of consanguineous parents. He presented with abdominal pain and fever and was noted to have silvery hair, eyelashes, and eyebrows. Bone marrow studies indicated hemophagocytosis, whilst microscopic examination of the hair showed irregular agglomerations of pigment in hair shafts. The prognosis, treatment, and genetic counseling needs differ considerably among the various forms of Griscelli Syndrome.

Necrolytic acral erythema masquerading as cellulitis

Necrolytic acral erythema (NAE) is a rare cutaneous sign of hepatitis C virus infection and has recently been linked to zinc deficiency. It presents as well-demarcated erythematous plaques in a sandal-like distribution on the dorsal feet with psoriasiform epidermal hyperplasia on histology. Our patient reported a 9-month history of progressive bilateral lower extremity erythema, swelling, erosions, and nail dystrophy that failed to improve despite multiple courses of antibiotics for presumed lower extremity cellulitis. Serum studies revealed zinc deficiency. This case supports the association of NAE with both HCV infection and zinc deficiency and highlights the pitfalls in the diagnosis of chronic unrecognized NAE. Suspected cases of NAE should prompt evaluation for underlying HCV and zinc deficiency to avoid treatment delay and associated complications.

Deep dermatofibrosarcoma protuberans in a ninety-year old-woman

A 92-year-old woman was referred for the assesment of an asymptomatic subcutaneous tumor that developed after an accidental fall. The mass clinically and radiologically simulated a subcutaneous hematoma. Finally, the histological study was consistent with subcutaneous dermatofibrosarcoma protuberans.

Hydroxychloroquine-induced fatal toxic epidermal necrolysis complicated by angioinvasive rhizopus

The majority of toxic epidermal necrolysis (TEN) cases are provoked by “high risk” medications (e.g. allopurinol, aromatic anticonvulsants, nevirapine, oxicam non-steroidal anti-inflammatory agents, and sulfonamides). TEN usually occurs 1 to 8 weeks after initial administration of the offending agent, but re-administration can evoke TEN within hours to days [1]. Hydroxychloroquine has rarely been associated with TEN, with one case proving fatal [2-4]. Herein, we report a case of hydroxychloroquine-induced fatal TEN complicated by angioinvasive Rhizopus. To our knowledge, this is the first case report of angioinvasive Rhizopus in a TEN patient. Initial misidentification of the offending agent causing TEN also serves as an important teaching point worth highlighting.

Unusual CD8 positive lymphomatoid papulosis in childhood

Lymphomatoid Papulosis (LyP) is a rare disorder characterized by a self-healing eruption of papules and small nodules with histopathologic features mimicking a cutaneous T-cell lymphoma CD 30+. We report a 15-year-old girl with CD8+ T-cells, an unusual phenotype in this disease. The clinical and pathological differential diagnoses are discussed.

Unusual case of frontal mucocele presenting with forehead ulcer

Paranasal mucoceles are benign slow-growing paranasal sinus lesions, which usually develop following the obstruction of the sinus ostiu. They most frequently occur in the frontal sinus. Frontal mucoceles are expansive lesions usually causing visual clinical signs and symptoms such as diminution of vision, visual field defects, diplopia, orbital swelling, retroorbital pain, displacement of eye globe, ptosis, and proptosis. When the frontal mucocele extends intracranially, it can manifest with meningitis, meningoencephalitis, intracranial abscess, seizures, or cerebrospinal fluid fistula. Very rarely it can cause forehead swelling. We report an 80-year-old woman presenting with a forehead skin ulcer and painless subcutaneous forehead induration. Histopathologic examination revealed mucin deposition and inflammation. Computerized tomography (CT) and magnetic resonance imaging (MRI) scans showed a mass originating from the frontal sinus with frontal bony defect and frontocutaneous fistula. Surgical excision of the mass confirmed the mucocele diagnosis. In this article, we present a case of frontocutaneous fistula and skin ulcer, which is an unexpected complication of frontal mucocele.  We propose that in the case of a localized non-healing ulcerated forehead skin lesions, mucocele should be considered in the differential diagnosis.

A case report of primary cutaneous histoplasmosis requiring deep tissue sampling for diagnosis

Importance: This is a case of primary cutaneous histoplasmosis, without any systemic involvement, that occurred without a history of trauma. Due to its rarity and varied clinical presentation, there is much difficulty in diagnosis of this disease entitiy, especially in differentiating it from pyoderma gangreosum. This patient required deep tissue sampling and a DNA probe for Histoplasma to establish a time-sensitive diagnosis as multiple superficial biopsies are nondiagnositic.

An eschar and violaceous nodules as the presenting signs of lymphomatoid granulomatosis

Lymphomatoid granulomatosis (LYG) is a rare B-cell lymphoproliferative disorder associated with infection by Epstein-Barr virus (EBV). The lung is the most common site of involvement, but LYG may initially manifest in the skin. LYG has been associated with immune dysregulation. Treatment regimens are not well-defined, but clinical trials targeting EBV have been successful. We report a 31-year-old male with LYG who presented with cutaneous symptoms. The skin biopsy was devoid of B-cells and non-reactive for EBV. We present this case to emphasize the role of dermatologists in the diagnosis of LYG and to caution clinicians that cutaneous lesions may lack diagnostic evidence of EBV infection.

Aggressive meningioma presents as innocuous forehead lesion: a case report

Cutaneous meningiomas are very rare neoplasms. In this case report we document a type III (anaplastic meningioma) presenting as a subcutaneous forehead mass. Anaplastic meningiomas arise from the neuraxis. They are biologically aggressive neoplasms that extend into the dermis or subcutaneous tissue via direct extension through the bone.

Eccrine poromatosis in a patient with acute myeloid leukemia following chemotherapy

Eccrine poromas are rare, benign adnexal tumors that often occur as solitary papules. Rarely, eccrine poromas can present as multiple lesions, which is referred to as eccrine poromatosis. We report a case of eccrine poromatosis occuring on the palms and soles occuring after chemotherapy in a patient with a history of acute myeloid leukemia.

Letter

Erythema ab igne: evolving technology, evolving presentation

We present a case of a 49-year-old woman with erythema ab igne on her posterior thighs owing to 2-4 hours per day of seat heater use in her car. Erythema ab igne is caused by prolonged exposure to a heat source. It used to be caused mainly by wood stoves used to heat homes. Erythema ab igne is now more often related to other heat sources, including heating pads, laptop computers, and car seat heaters, as in our case. As technology changes, so does the presentation of skin conditions that are related to technology.

Photo Vignette

Congenital onychodysplasia of the index finger presenting as a congenital bifid nail

Congenital onychodysplasia of the index finger (COIF) is a rare disorder characterized by various nail dystrophies, often with underlying bony deformity. We report a case of a COIF presenting as a congenital bifid nail deformity with underlying bone deformity.

Erythematous nodule of the ear

An elderly may with eccrine porocarcinoma of the ear is presented. This rare tumor in an unusual location has the potential for metastasis and local recurrence.

Linear, pruritic red to brown papules on the left chest

A 28-year-old woman presented with a 12-year history of red to brown papules in a linear distribution on the left lateral chest associated with recent flares of pruritus. She had previously been clinically diagnosed with lichen planus. A punch biopsy was performed, and histopathologic exam revealed Darier-like acantholysis. The patient was diagnosed with type 1 segmental Darier disease and her symptoms improved with topical tretinoin.

Rapidly involuting congenital hemangioma (RICH): a brief case report

Congenital hemangiomas (CH) are benign vascular neoplasms that proliferate in utero and have completed development by birth. Two subtypes of CH are recognized: rapidly involuting congenital hemangiomas (RICH) and non-involuting congenital hemangiomas (NICH). Involution of the RICH subtype often begins in the first weeks of life. NICH does not involute, allowing the distinction between RICH and NICH. We report a case of an infant with RICH occurring on the scalp, examined at birth and followed for 26 weeks.

Unilateral and pruritic papules: segmental Darier-White disease

Darier-White disease is an uncommon disorder, which presents in a localized pattern in about 10% of patients, usually without nail, mucosa, or acral involvement. Type-1 is the most common of the segmental Darier-White disease types: papules have unilateral distribution along Blaschko lines. A 36-year-old woman diagnosed with type-1 segmental Darier-White disease is reported herein.

Zosteriform lichen planus after herpes zoster: report of a new case of Wolf’s isotopic phenomenon and literature review

The Wolf’s isotopic phenomenon corresponds to the occurrence of a skin disease at a body site affected previously by a different, already healed dermatosis. We report a 74-year-old man who presented with a zosteriform eruption consisting of erythematous-scaly, slightly pruritic papules on the right flank some weeks after herpes zoster (HZ) had healed on the same area. Histologic examination showed typical changes of lichen planus, confirming the diagnosis of post-HZ zosteriform lichen planus (ZLP). The lesions regressed with topical steroid treatment. Zosteriform lichen planus (ZLP) is one example of Wolf’s isotopic phenomenon appearing after HZ. So far, only 17 cases have been reported in the literature. The precise pathogenesis of ZLP is not well known, although persisting viral proteins could be responsible for the hypersensitivity reaction. We review and discuss the salient clinicopathologic features of this rare entity based on a thorough literature review.

Commentary

Proposed classification for koebner, wolf isotopic, renbok, koebner nonreaction, isotopic nonreaction & other related phenomen.

Students of skin diseases have long noted a variety of disease responses and non-responses to trauma and the presence of structural abnormalities. This article will review the series of these responses including: Koebner phenomenon, Wolf isotopic response, Renbök response, Koebner nonreaction, isotopic nonreaction, and other related skin reactions. Because most of these reported phenomena have similar morphological features the diagnosis is often made on the basis of differences in the clinical presentation. Note that some of the cutaneous reactions of similar phenomena have been described using varied nomenclature, further adding to the confusion. In view of this, we believe that at present there is a robust need to define each cutaneous reaction accordingly and classify the various types of these phenomena under a broad universal term or terms. Respecting the idea of differences might facilitate a better understanding of the pathophysiology of these entities. We then will propose an alternative classification system.