Adult-onset systemic Langerhans cell histiocytosis mimicking inflammatory bowel disease: the value of skin biopsy and review of cases of Langerhans cell histiocytosis with cutaneous involvement seen at the Mayo Clinic.

BACKGROUND: Langerhans cell histiocytosis (LCH) is frequently known to involve multiple organ systems. However, gastrointestinal involvement by LCH is rare. METHODS: We describe a 68-year-old woman with a 3-year history of intermittent diarrhea initially diagnosed as inflammatory bowel disease. She was subsequently found to have systemic LCH with involvement of the gastrointestinal tract, lungs, liver, and skin after skin biopsy was performed. A retrospective review of patients with cutaneous involvement of LCH seen at the Mayo Clinic over the past 15 years was conducted. The presence of systemic disease as well as specific organ system involvement was reviewed. RESULTS: Twenty-four patients with cutaneous LCH were identified. Besides our case, one other patient with both gastrointestinal and cutaneous involvement was identified. This patient died at six months of age. No other adult-onset cases were identified. CONCLUSIONS: Gastrointestinal involvement with LCH is rare, can be easily misdiagnosed, and likely portends a poor prognosis. In patients with ill-defined systemic symptoms, cutaneous exam and biopsy have the potential to diagnose systemic disease.

Congenital erosive and vesicular dermatosis with reticulated supple scarring: unifying clinical features.

BACKGROUND: Congenital erosive and vesicular dermatosis (CEVD) healing with reticulated supple scarring, a condition usually observed in premature neonates, presents at birth with vesicles and erosions. Lesions typically heal within a few months, leaving behind scarring with a distinctive supple and reticulated texture. OBJECTIVES: We sought to merge existing literature with new cases to further define CEVD. METHODS: We analyzed 19 previous reports of CEVD and added 9 additional patients; we identified unifying characteristics of this cohort. RESULTS: In 28 total cases, notable features included: preterm birth (79%), nail abnormalities (46%), hyperthermia/hypohidrosis (46%), a history of maternal chorioamnionitis (43%), alopecia (43%), neurodevelopmental and ophthalmologic abnormalities (36% each), tongue atrophy (29%), or a combination of these. Patients with CEVD may be prone to postnatal herpetic superinfections. Previously unreported findings included: erosive lichen planus, digital tip gangrene, and hydronephrosis. LIMITATIONS: The small patient sampling makes it difficult to define diagnostic criteria. As certain findings are associated with prematurity, it is unclear to what extent these features result from CEVD, premature birth, or another intrauterine pathology. CONCLUSIONS: Although rare, CEVD should be considered in the differential diagnosis of neonatal vesicles/erosions in the context of a negative infectious workup. This review strengthens the spectrum of CEVD features, thus facilitating its recognition by clinicians.

Henoch-Schönlein purpura associated with solid-organ malignancies: three case reports and a literature review.

Adult Henoch-Schönlein purpura (HSP) is rarely associated with solid-organ malignancies. We describe here three adult patients with HSP diagnosed within 3 months of the diagnosis of associated solid-organ malignancies, including pulmonary, prostate, and renal carcinomas. Two patients had complete remission with a combination of immunosuppressive therapies and treatment of the associated malignancy. The third patient had partial remission with immunosuppressive therapies, but never received treatment for the associated malignancy and did not achieve complete remission before his death 10 months after diagnosis of HSP. These cases suggest that HSP associated with solid-organ malignancies may be resistant to immunosuppressive therapies without treatment of the associated malignancy. Therefore, evaluation for solid-organ malignancies should be considered in adult patients without an identifiable cause of HSP, especially if the disease is not self-limited or does not respond appropriately to treatment.

Bullous acral erythema: an additional consideration in the differential diagnosis of pauci-inflammatory subepidermal bullae*.

Acral erythema is considered a frequent complication of chemotherapy administration. The bullous variant of chemotherapy-induced acral erythema, or bullous acral erythema, occurs less commonly. The condition typically begins with acral dysesthesias and produces symmetric erythema that blisters and eventually desquamates. Overall, 32 cases of bullous acral erythema have been described in the literature, including 21 cases associated with cytarabine administration and 11 cases attributed to methotrexate. We describe a 61-year-old woman with diffuse large B-cell lymphoma in whom bullous acral erythema developed after she received cytarabine and methotrexate. The clinical presentation was unusual, as it was characterized by vesicles in an annular configuration suggestive of linear immunoglobulin A bullous disease. Histopathology revealed a pauci-inflammatory subepidermal bulla that was similar to previously reported cases of bullous acral erythema. We suggest that bullous acral erythema represents an important diagnostic consideration in the differential diagnosis of pauci-inflammatory subepidermal blistering in patients who have recently received chemotherapy.

Cutaneous small-vessel vasculitis associated with solid organ malignancies: the Mayo Clinic experience, 1996 to 2009.

BACKGROUND: Although rare, cutaneous small-vessel vasculitis (CSVV) secondary to solid organ malignancy has been documented. OBJECTIVE: We sought to better understand the frequency, clinical course, therapeutic response, and outcome of CSVV associated with solid organ malignancy. METHODS: We conducted a retrospective chart review of patients seen between 1996 and 2009 with diagnoses of biopsy-proven cutaneous leukocytoclastic vasculitis and solid organ malignancy separated by less than 12 months. RESULTS: Of 17 patients (mean age, 66.5 years), 10 patients (59%) were male. CSVV occurred before (3 patients; 18%), concurrent with (3 patients; 18%), and after (11 patients; 65%) diagnosis of solid organ malignancy. The most common solid organ malignancy was of the lung (n = 4; 24%). Other associated cancers were breast (n = 3); prostate (n = 2); colon (n = 2); renal (n = 2); thyroid (n = 1); bladder (n = 1); gallbladder (n = 1); and peritoneal (n = 1). Three patients had cutaneous vasculitis in association with malignancy recurrence despite having no cutaneous vasculitis associated with their primary malignancy. Vasculitis remission with use of immunosuppressive agents alone occurred in 9 patients (53%). Eleven patients (65%) were alive at last follow-up (mean follow-up duration, 27 months). LIMITATIONS: This was a retrospective study with a relatively small number of patients. CONCLUSION: Solid organ malignancy should be considered as a possible cause of CSVV of unknown origin. In contrast to previous reports, our patients were more likely to respond to immunosuppressive therapies without treatment of the associated malignancy and to be alive at last follow-up.

Marie-Unna hereditary hypotrichosis: case report and review of the literature.

We present an 18-year-old girl with progressively worsening hair thinning and loss since puberty. Her clinical history, physical examination, and pathology results were consistent with Marie-Unna hereditary hypotrichosis, a rare cause of autosomal dominant hereditary hair loss. She had no family history of similar hair loss and represents the first report of a new case of Marie-Unna hereditary hypotrichosis within a previously unaffected family.

Allergic contact dermatitis from exotic woods: importance of patch-testing with patient-provided samples.

Exotic woods from tropical and subtropical regions (eg, from South America, south Asia, and Africa) frequently are used occupationally and recreationally by woodworkers and hobbyists. These exotic woods more commonly provoke irritant contact dermatitis reactions, but they also can provoke allergic contact dermatitis reactions. We report three patients seen at Mayo Clinic (Rochester, MN) with allergic contact dermatitis reactions to exotic woods. Patch testing was performed and included patient-provided wood samples. Avoidance of identified allergens was recommended. For all patients, the dermatitis cleared or improved after avoidance of the identified allergens. Clinicians must be aware of the potential for allergic contact dermatitis reactions to compounds in exotic woods. Patch testing should be performed with suspected woods for diagnostic confirmation and allowance of subsequent avoidance of the allergens.

Aquatic sports dermatoses: part 1. In the water: freshwater dermatoses.

The first of this three-part series on water-related dermatoses involving the athlete will include sports occurring with the majority of time spent in the water. These sports include swimming, diving, scuba, snorkeling and water polo. Numerous authors have described dermatologic conditions commonly seen in swimmers. This series provides an updated and comprehensive review of these water dermatoses. In order to organize the vast number of skin conditions related to water exposure, we divided the skin conditions into groupings of infectious and organism-related dermatoses, irritant and allergic dermatoses and miscellaneous dermatoses. The vast majority of skin conditions involving the water athlete result from chemicals and microbes inhabiting each environment. When considering the effects of swimming on one's skin, it is also useful to differentiate between exposure to freshwater (lakes, ponds and swimming pools) and exposure to saltwater. This review will serve as a guide for dermatologists, sports medicine physicians and other medical practitioners in recognition and treatment of these conditions.

Aquatic sports dematoses. Part 2 - in the water: saltwater dermatoses.

The second part of this three-part series on water-related dermatoses will discuss dermatologic conditions seen in athletes exposed to saltwater. The vast majority of the following dermatoses result from contact with organisms that inhabit saltwater, including bacteria, cnidarians, and echinoderms. This review also will include other dermatoses affecting saltwater athletes and should serve as a guide for dermatologists, sports medicine physicians, and other medical practitioners in recognition and treatment of these dermatoses.

Aquatic sports dermatoses: Part 3. On the water.

The third of this three-part series on water-related sports dermatoses discusses skin changes seen in athletes who participate in sporting activities on top of or nearby water. While also susceptible to several of the freshwater and saltwater dermatoses discussed in parts one and two of the series, these athletes may present with skin changes unique to their particular sports. This updated and comprehensive review details those near-water dermatologic conditions commonly seen in sailors, rowers, fishermen, surfers, windsurfers, rafters, and water skiers, and will serve as a guide for dermatologists, sports medicine physicians and other medical practitioners in recognition and treatment of these conditions.

Chronic recurrent multifocal osteomyelitis (CRMO) and synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome with associated neutrophilic dermatoses: a report of seven cases and review of the literature.

A growing body of literature has identified the association between neutrophilic dermatoses and multifocal, aseptic bone lesions in children, termed chronic recurrent multifocal osteomyelitis (CRMO). Classically, patients present with swelling, pain, and impaired mobility of the affected area, with skin lesions developing concurrently or in the future. Bone biopsy reveals inflammatory changes consistent with infectious osteomyelitis, but cultures and histologic staining invariably fail to identify an infectious source. Patients are refractory to antibiotic therapy, but dramatically respond to systemic steroids and may need to be maintained on low-dose steroids to prevent relapse. Numerous authors have suggested that CRMO and synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome lie along the same clinical spectrum. In fact some believe that CRMO is the pediatric presentation of SAPHO. The two syndromes share numerous characteristics, including osteitis, a unifocal or multifocal presentation, hyperostosis, and pustulosis, which all occur in a generally healthy individual. Our seven patients, five of whom were diagnosed with CRMO, and two of whom were diagnosed with SAPHO syndrome further strengthen the idea that CRMO and SAPHO syndrome do indeed lie along the same clinical spectrum. In addition, we include two rare cases of pediatric Sweet's syndrome with evidence of pathergy.

Neonatal sporotrichosis.

Sporotrichosis is caused by the dimorphic fungus, Sporothrix schenckii. Classically, infection occurs after implantation of the organism into the skin by abrasion of a puncture wound by contaminated thorns, hay, or sphagnum moss. Cats are also a commonly recognized source of sporotrichosis. In children, fixed cutaneous lesions are more common than lymphocutaneous lesions, and the face is a frequent location for infection. We present a neonate with a fixed cutaneous facial lesion and both pre-auricular and cervical lymphadenopathy who developed signs of infection at 3 weeks of age. This patient is currently the youngest reported case of sporotrichosis in the literature. The patient's family denied any trauma and denied contact with flowers, gardens, and cats; thus, the source of infection remains unknown.