Thymoma-associated multiorgan autoimmunity with cutaneous only presentation: A case report.

Thymoma-associated multiorgan autoimmunity disease can be seen in patients with thymomas and presents with features of graft versus host disease. Here, we report a case of a 52-year-old woman with a complex medical history including malignant thymoma, myasthenia gravis, Good's syndrome, and T cell large granular lymphocytic leukemia who presented with a diffuse pruritic rash ultimately found to be compatible with a cutaneous presentation of thymoma-associated multiorgan autoimmunity disease. The eruption heralded the recurrence of the malignant thymoma and the rash was ultimately found to be resistant to a multitude of therapies except for oral corticosteroids.

Narrowband ultraviolet B therapy for refractory immune-related lichenoid dermatitis on PD-1 therapy: a case report.

Treatment with programmed cell death 1 inhibitors is associated with a wide range of cutaneous immune-related adverse events, with lichenoid eruptions representing one of the major cutaneous toxicities. We describe the case of an 81-year-old man with metastatic melanoma treated with pembrolizumab who subsequently developed a delayed-onset generalized lichenoid dermatitis. After failing multiple lines of systemic immunosuppression, narrowband ultraviolet B (NBUVB) phototherapy three times per week for 17 sessions resulted in a significant clinical response in his cutaneous eruption and was well tolerated. NBUVB is a safe, lower-cost modality that induces local, skin-specific immunosuppression without the toxicities of traditional systemic immunosuppressive agents. To date, this is the first report of use of NBUVB in immune-related lichenoid dermatitis resistant to multiple standard therapies.

Small Cell Variant of T-Cell Prolymphocytic Leukemia with Acquired Palmoplantar Keratoderma and Cutaneous Infiltration.

T-cell prolymphocytic leukemia (T-PLL) is a rare and aggressive post-thymic malignancy that is characterized by the proliferation of small- to medium- sized prolymphocytes. The classic clinical features of T-PLL are lymphocytosis, lymphadenopathy, hepatosplenomegaly, and skin lesions. Skin involvement varies clinically from diffuse infiltrated erythema. Infiltration is localized to the face and ears, nodules, and erythroderma. We present a case of small cell variant of T-PLL in a patient who presented with unusual cutaneous manifestations of acquired palmoplantar keratoderma (PPK) followed by diffuse erythematous infiltrated papules and plaques involving the trunk. When the etiology of acquired PPK is not clear, the physician should consider the possibility of an underlying malignant disease. In this case, the diagnosis of T-PLL was subsequently confirmed by laboratory and cytological findings, as well as by the immunophenotyping of leukemic cells in skin biopsy. Since paraneoplastic acquired PPK may be the initial evident sign of malignancy, the physician's awareness of this manifestation may be crucial for early diagnosis and treatment. Our case emphasizes the importance of accurate evaluation of skin lesions and early skin biopsy in the diagnosis of some hematological malignancies.

Hypopigmented segmental Darier disease.

BACKGROUND: Darier disease is a genodermatosis caused by a mutation in the ATP2A2 gene. It classically presents as hyperkeratotic greasy papules in a seborrheic distribution. Several variants have been reported, notably the hypopigmented variant, which predominantly targets dark-skinned individuals, and a segmental variant that often follows the lines of Blaschko. METHODS: We report a case of a 41-year-old African-Canadian female with a long-standing history of macular hypopigmented pruritic eruption following the lines of Blaschko on her back. The eruption was persistent and recalcitrant to various treatments. Dyskeratosis with corps ronds and grains, acantholysis, and parakeratosis were observed on histopathology. Those findings were consistent with the diagnosis of segmental hypopigmented Darier disease. RESULTS AND CONCLUSIONS: To our knowledge, this is the first case reporting a combined segmental and hypopigmented variant of Darier disease. We further present a literature review for hypopigmented and segmental variants of Darier disease.

Hiccups, eructation, and other uncommon prodromal manifestations of herpes zoster.

Although the most frequent presentation of herpes zoster involves sensory neurons, motor and autonomic symptomatology is also known to occur in this disease. An unusual symptom of hiccups is described here. Other infrequent manifestations of this common illness, including the Ramsay Hunt syndrome, herpes zoster ophthalmicus, urinary and fecal retention, sexual dysfunction, and zoster sine herpete, are reviewed. Greater awareness of unusual presentations of herpes zoster is necessary for proper diagnosis and timely management of complications that may otherwise lead to disability and serious long-term sequelae.

Leukemia cutis presenting with fingertip hypertrophy.

BACKGROUND: Patients with leukemia often manifest cutaneous findings, which include nonspecific lesions and specific leukemic infiltrates termed leukemia cutis. OBJECTIVE: A case of leukemia cutis involving distal finger pads is reported and literature describing hand involvement of specific leukemic infiltrates is reviewed. METHODS AND RESULTS: An 80-year-old woman with a 10-year history of chronic lymphocytic leukemia developed painful symmetric tumors of her distal finger pads. Histopathological examination of the biopsy specimen revealed infiltration by neoplastic lymphocytes. Only a few cases of leukemia cutis involving the hands have been reported in the literature, none with this particular presentation. The clinical and histopathologic features of leukemia cutis are reviewed. CONCLUSION: This case emphasizes the importance of obtaining a biopsy specimen for histopathological examination of any suspicious skin lesion in a patient with leukemia.

Use of CO2 laser in the treatment of periungual fibromas associated with tuberous sclerosis.

BACKGROUND: Tuberous sclerosis complex is an autosomal dominant disorder with variable clinical expression. In severe forms it may involve almost any organ system. Periungual fibromas are a common cutaneous manifestation and a frequent source of cosmetic and functional concern. Current treatment involves surgical excision of the lesions. OBJECTIVE: To evaluate laser vaporization of periungual fibromas and to compare results to the standard surgical treatment. METHODS: A patient with tuberous sclerosis and recurrent periungual fibromas was treated with a continuous wave CO2 laser in sequential focused and defocused modes at 10-15 W. RESULTS: Laser treatment required approximately 10 seconds per lesion and produced no intraoperative bleeding. The wounds healed well with good cosmetic results. CONCLUSION: Though similar to conventional surgery in terms of cosmetic satisfaction, CO2 laser presents a viable alternative to surgery through significantly reduced treatment time and amount of bleeding.