Topical drug-induced subacute cutaneous lupus erythematosus isolated to the hands.

Subacute cutaneous lupus erythematosus (SCLE) is a well-defined subtype of lupus erythematosus, characterised by photosensitivity, annular and/or psoriasiform lesions, variable systemic involvement and presence of circulating SSA/anti-Ro antibodies. SCLE may be idiopathic or drug-induced. Both the idiopathic and drug-induced forms of SCLE are analogous in their clinical, serological and histological features. Drug-induced SCLE has been reported with various oral agents, but to our knowledge this is the first reported case due to a topical medication. A 34-year-old female foot masseuse presented with a 2-month history of scaly, erythematous lesions isolated to the dorsal hands and interdigital spaces. She had used topical terbinafine, a topical antifungal cream, to her clients' feet for a number of years. ANA and anti-SSA/Ro antibodies were positive. Physical examination, serology and histopathology were consistent with SCLE. We propose that our patient's unique presentation of SCLE may be explained by a prolonged occupational exposure to topical terbinafine as a foot masseuse. While oral terbinafine is a drug known to cause drug-induced SCLE, to our knowledge, this is the first topically induced form of the disease.

Massive localized lymphedema: A soft tissue process that may present to dermatologists.

Massive localized lymphedema (MLL) is a clinically and histologically distinct entity seen in morbidly obese patients. We describe two obese patients with MLL in the lower abdomen and suprapubic area. Biopsies showed characteristic histologic features of an expanded dermis with lymphangiectases, fibrotic septae and focal fat necrosis. One patient had long-standing hypothyroidism, an association previously reported. This disease is frequently complicated by recurrent cellulitis and may be amenable to surgery. Patients with MLL may present to dermatologists, and this disease has characteristic histopathologic findings that may mimic liposarcoma to the unaware dermatopathologist.

Acquired perforating calcific collagenosis after topical calcium chloride exposure.

A 24-year-old healthy man presented with a 6-week history of numerous umbilicated coalescing erythematous papules with some scale and crust on his anterior medial thighs. The eruption began 1 to 2 weeks after he spilled calcium chloride rock salts on his pants while salting the sidewalk during a snow storm. The salts dissolved and remained in contact with his skin for at least 4 hours until he was able to change clothes. A skin biopsy shows thick and thin collagen fibers with partial calcification in the papillary and upper reticular dermis associated with a sparse infiltrate of neutrophils, lymphocytes and mononuclear histiocytes. There are foci of transepidermal elimination of calcified fibers with adjacent epidermal hyperplasia and ortho- and parakeratosis. Von Kossa stain highlights calcification of the fibers, and trichrome stain confirms the fibers are collagen. A Verhoeff-van Gieson stain shows no abnormality of elastic fibers. The patient was treated with topical betametasone diproprionate cream twice daily for 3 weeks, as well as a short course of oral levofloxacin and topical gentamicin cream. The lesions resolved over 3 weeks with residual scarring. We report a unique case of acquired perforating calcific collagenosis secondary to topical calcium chloride exposure.

Epidermodysplasia verruciformis in the setting of HIV infection.

A 56-year-old man with human immunodeficiency virus infection presented with pink-to-hypopigmented, thin, flat-topped papules coalescent to plaques on the trunk and extremities for five years. The histopathologic findings were consistent with flat warts resembling epidermodysplasia verruciformis. Typically an inherited condition, this entity has also been observed in the setting of immunosuppression; the risk of developing non-melanoma skin cancers is of concern. Treatment options vary considerably, but often the lesions will recur upon cessation of therapy.

Mastocytosis with urticaria pigmentosa and osteoporosis.

A 76-year-old man with a 52-year history of urticaria pigmentosa was found to have an elevated serum tryptase level as well as osteoporosis. Histopathologic alterations of one his skin lesions showed an infiltrate of mast cells. Urticaria pigmentosa patients are at risk for osteoporosis because of elevated heparin and stem-cell factor levels. These patients should be screened with serum tryptase levels and bone density studies to detect osteoporosis, regardless of their age.

Unilateral nevoid telangiectasia.

A 30-year-old woman, who was six months pregnant, presented with multiple, blanching, asymptomatic telangiectasies on her right upper extremity for two years. At the onset of her pregnancy, her lesions increased in number and redness. Given the unilateral distribution and worsening during pregnancy, a diagnosis of unilateral nevoid telangiectasia was made. This condition is a rare entity that has been most commonly reported in association with puberty, pregnancy, the use of oral contraceptives, and alcoholic cirrhosis. However, there have been case reports in otherwise healthy individuals.

Lichen planopilaris and psoriasis.

A 34-year-old woman presented with large, scaly patches of alopecia with a peripheral rim of violaceous, folliculocentric papules and appreciable pruritus of one-year duration. Histopathologic examination showed changes consistent with lichen planopilaris and psoriasis, which was suggested by neutrophilic spongiosis. Consequently, cyclosporine and betamethasone valerate topical 0.12 percent foam twice daily were initiated. A short time after, there was clinical reduction of perifollicular erythema and attenuation of pruritus. However, there was no decrease of scale. Although LLP is classified in the lymphocytic group of cicatricial alopecias, this case demonstrates a clinical and histopathologic overlap with a psoriasiform dermatosis which may represent a collision of two diseases.

Trichoepithelioma.

A 29-year-old man presented with a long-standing history of asymptomatic, skin-colored, facial papules and nodules. Histopathologic examination of a representative papule demonstrated trichoepithelioma. The patient had a history of a brother with a similar phenotype, which suggests a diagnosis of familial trichoepithelioma. Linkage and mutational analyses support genetic heterogeneity of familial trichoepithelioma, possibly sharing a clinical spectrum with Brooke-Spiegler syndrome and familial cylindromatosis since each entity has been associated with mutations the CYLD gene.

Exogenous ochronosis.

A 55-year-old woman with melasma develops biopsy-proved exogenous ochronosis in the setting of prolonged topical hydroquinone use. A limited number of similar reports exist in the US literature and are the basis for an FDA call to review hydroquinone-based products. This case underscores the difficult therapeutic dilemma which this diagnosis presents to dermatologists.

Mycobacterium marinum infection.

A 64-year-old man presented for evaluation of tender pustules that developed on his right hand and subsequently spread up his arms in a sporotrichoid manner. Owing to the prominent history of recent trauma followed by fish tank exposure, the patient was started on doxycycline for coverage of Mycobacterium marinum. Subsequent tissue culture grew M. marinum and confirmed the clinical diagnosis. M. marinum is an atypical mycobacteria that is ubiquitously found in aquatic environments. Owing to its optimal growth temperature of 25 to 35 degrees C, infections in humans are mostly restricted to the skin. Furthermore, the organism can spread by lymphatic drainage leading to the clinical appearance of sporotrichoid lesions. The preferred treatment for M. marinum is doxycycline, which was started in our patient and has subsequently improved his clinical condition.

Nodular pretibial myxedema.

A 30-year-old man with previously diagnosed and treated Graves disease presented for consultation regarding asymptomatic nodules over his anterior tibias. He was euthyroid at the time of presentation. The nodules arose symmetrically beneath the sites of pressure from his military boots. A biopsy specimen showed an accumulation of acid mucopolysaccharides consistent with pretibial myxedema. The patient had recently stopped smoking and chewing tobacco, which are known risk factors for the development of pretibial myxedema. Following diagnostic punch biopsies, the patient experienced a rapid resolution of the nodule on his right leg and a appreciable reduction in size of the nodule on the left leg. Three months later, the nodules are beginning to enlarge once again.

Cutaneous umbilical endometriosis.

A 35-year-old woman presented with a four-month history of a tender umbilical nodule that bleeds during her menstrual period. Physical examination showed a hyperpigmented umbilical nodule. A biopsy specimen showed fibrotic dermis with increased numbers of blood vessels and scattered glandular structures with areas of hemosiderin deposition consistent with a diagnosis of endometriosis. Cutaneous umbilical endometriosis is rare, with an estimated incidence of 0.5 to 1.0 percent. Although anti-gonadotropin medications, such as danazol, have been used for symptomatic control, but surgical excision is the treatment of choice owing to the possibility of malignant degeneration of cutaneous endometriosis.

Invasive squamous-cell carcinoma and arsenical keratoses.

A 42-year-old man presented with a six-month history of a slowly-enlarging ulcer on his right sole, a 30-year history of altered pigmentation of the trunk and extremities, and hyperkeratotic papules of the palms and soles. Histopathologic examination showed an invasive squamous-cell carcinoma of the right sole and hyperkeratosis with keratinocyte atypia of the left finger and left lateral foot. The clinical and histopathologic findings are consistent with chronic arsenicism, which most commonly occurs in the setting of drinking contaminated water or after occupational exposure. Evaluation should include a physical examination, basic laboratory work-up, and measurement of a 24-hour urine arsenic concentration. Vigilant surveillance for the development of cutaneous malignancies is required. Oral retinoids may be helpful in reducing hyperkeratosis secondary to chronic arsenicism.

Proliferating trichilemmal cyst with focal calcification.

A 64-year-old man presented with a superficial, well-demarcated, skin-colored tumor on the left posterior scalp that measured 4 x 5 x 6 cm. The tumor was nearly hairless, rubbery, non-tender, and mobile over the underlying subcutaneous tissues. The lesion had grown slowly since arising approximately 30 years ago. Treatment options were declined in the past. However, with relatively more rapid growth over the past five years, the nodule began to cause intermittent pain and interfere with the patient's ability to lie on his back. The patient denied a history of similar lesions in himself or his family. A biopsy specimen showed a ruptured proliferating trichilemmal cyst with focal calcification. Complete excision is recommended for all benign proliferating variants owing to their potential for locally aggressive behavior and malignant transformation.

Pretibial epidermolysis bullosa.

A 47-year-old Vietnamese woman presented with dystrophic fingernails and toenails that had been present since infancy. She also had developed, in the third decade, pretibial pruritus with vesicle formation and progressive localized papules and scars. Multiple family members were similarly affected. Physical examination showed lichenoid papules that coalesced into large plaques that were studded with milia over the pretibial areas and 20 nail dystrophy. A biopsy specimen showed milia-like structures and dermal fibrosis. Pretibial epidermolysis bullosa is a rare variant of dystrophic epidermolysis bullosa that shows appreciable clinical overlap with dystrophic epidermolysis bullosa pruginosa. Both disease subsets are characterized by the late age of onset, nail dystrophy, and predominantly pretibial pruritic lichenoid skin lesion; they are associated with glycine substitution mutations in COL7A1.