Joshua O. Podjasek

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

Abstract:  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.

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.

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.

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.

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.

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

Langerhans cell histiocytosis (LCH) is frequently known to involve multiple organ systems. However, gastrointestinal involvement by LCH is rare.

Histopathological findings in cutaneous small-vessel vasculitis associated with solid-organ malignancy

Histopathological findings in biopsy specimens from patients with cutaneous small‐vessel vasculitis (CSVV) secondary to solid‐organ malignancy have not been previously reported.

Painful erosions induced by patch testing in a patient with Hailey–Hailey disease

Editor, We present a 40-year-old woman with a history of biopsy-proven Hailey–Hailey disease. She had a remote history of irritation from adhesive tapes. She presented with pruritic, eczematous patches on her periocular skin and back that were suspicious for allergic contact dermatitis. Patch testing to our standard/preservative series and topical corticosteroid series was performed with paper tape because of her history of adhesive sensitivity. After 48 hours, the patient began to experience significant pain and irritation along her back in the area of the patches. The patches were removed, revealing extensive, urticariallike plaques and a few superficial erosions in areas beneath the paper tape, in addition to likely irritant reactions to lanolin alcohol and benzalkonium chloride (Fig. 1). Four days after placement, multiple erosions and scattered, fluid-filled vesicles were present (Fig. 2). A biopsy was offered to confirm our clinical impression of patch test-induced Hailey–Hailey disease, but the patient declined. Polymerase chain reaction (PCR) testing for herpes simplex virus (HSV) and varicella zoster virus (VZV) from a representative vesicle was negative. Bacterial culture revealed growth of Staphylococcus aureus sensitive to methicillin. The erosions improved with dilute acetic acid wet dressings over topical clobetasol cream applied twice daily for two weeks. There are several reports of allergic contact dermatitis occurring in patients with Hailey–Hailey disease. However, patch testing may be associated with complications in these patients, secondary to their inherent skin fragility. One group described a 43-year-old man with a known diagnosis of Hailey–Hailey disease who experienced a flare-up of disease secondary to infection with HSV type 1. Patch tests were applied to rule out a coexistent allergic contact dermatitis, and the patient subsequently developed severe blistering, which the authors attributed to a combination of sweating and frictional forces associated with patch testing. Another group reported two cases of Hailey–Hailey disease diagnosed when the patients developed severe, painful erosions after patch testing. A review of our database showed that we have patch tested over 5000 patients in the last 10 years, including several patients with Hailey–Hailey disease. This is the first case of patch test-induced exacerbation of Hailey– Hailey disease that we have encountered at our institution. Our case adds further evidence for the occurrence of this rare reaction in patients with Hailey–Hailey disease and indicates that patch testing must be performed with caution in these patients. In order to prevent this complication, some authors have recommended that patches should be removed at 24 hours rather than 48 hours in patients with Hailey–Hailey disease. We would add that, if patch testing is necessary, the least adherent tape possible should be applied to secure the patches, and the patient should be warned of this rare but potential reaction.