Kalman L. Watsky

Skin and bones. I

Skin disorders in which a radiograph may detect associated bony changes or abnormalities of calcification are discussed. They are grouped into eight categories: (1) inherited diseases (e.g., alkaptonuria, neurofibromatosis); (2) congenital disorders (e.g., Sturge-Weber and Proteus syndromes); (3) inflammatory conditions (e.g., dermatomyositis, sarcoidosis); (4) infections (e.g., dental sinus, syphilis); (5) neoplasias (e.g., histiocytosis, mastocytosis); (6) drug- and environment-induced (e.g., acroosteolysis, retinoid toxicity); (7) calcinosis cutis; and (8) osteoma cutis. Part I of our review discusses the first two categories.

Cross-comparison of patch test and lymphocyte proliferation responses in patients with a history of acute generalized exanthematous pustulosis

An adverse cutaneous reaction to a systemically administered drug may rarely manifest as acute generalized exanthematous pustulosis (AGEP). Several recent reports have documented positive patch test results in patients with a history of AGEP, while two have demonstrated drug-specific in vitro lymphocyte proliferative responses. These findings suggest that drug-specific T cells mediate AGEP. We describe two patients with a history of AGEP who each demonstrated positive patch test results specific for the inciting drug: Patient #1 to the antibiotic metronidazole, and Patient #2 to the calcium channel-blocker diltiazem. Histologic examination of biopsy specimens taken from the patch test sites of these patients revealed spongiotic dermatitis and perivascular lymphocytes consistent with a delayed-type hypersensitivity reaction, rather than demonstrating subcorneal neutrophilic pustules more typical of AGEP. In vitro testing by measuring peripheral T cell proliferative responses to chemically purified drug correlated with the clinical response. In a direct cross-comparison, patch test results were shown to correlate with in vitro lymphocyte proliferative responses in two patients with a history of AGEP to different drugs. These findings provide additional evidence that the pathogenesis of AGEP involves a T cell-mediated immune response.

Erosive perianal lichen planus responsive to tacrolimus.

A 46‐year‐old man presented with bleeding and painful sores around the anus of 2–3 years’ duration. The lesions had improved with a course of oral antimicrobials prescribed by a surgeon, who had performed a biopsy reported as showing nonspecific changes. Topical corticosteroids had also led to improvement, accompanied by the development of painful “pimples.” The patient had a history of recurrent oral herpes simplex, but denied other medical problems, persistent oral lesions, or human immunodeficiency virus risk factors.

Airborne allergic contact dermatitis from pine dust

BACKGROUND Airborne contact dermatitis is a challenging entity that requires comprehensive patch testing and exposure review to diagnose accurately. OBJECTIVE Four patients with airborne contact dermatitis and positive patch tests to colophony were evaluated. METHODS Patch testing was performed on all patients using a modified European standard series plus additional applicable allergens. RESULTS All patients had positive patch tests to colophony and exposure to pine dust. CONCLUSION Patch testing is a critical element in the evaluation of patients presenting with an airborne pattern of contact dermatitis.

Pediatric Contact Dermatitis Registry Inaugural Case Data

BackgroundLittle is known about the epidemiology of allergic contact dermatitis (ACD) in US children. More widespread diagnostic confirmation through epicutaneous patch testing is needed. ObjectiveThe aim was to quantify patch test results from providers evaluating US children. MethodsThe study is a retrospective analysis of deidentified patch test results of children aged 18 years or younger, entered by participating providers in the Pediatric Contact Dermatitis Registry, during the first year of data collection (2015–2016). ResultsOne thousand one hundred forty-two cases from 34 US states, entered by 84 providers, were analyzed. Sixty-five percent of cases had one or more positive patch test (PPT), with 48% of cases having 1 or more relevant positive patch test (RPPT). The most common PPT allergens were nickel (22%), fragrance mix I (11%), cobalt (9.1%), balsam of Peru (8.4%), neomycin (7.2%), propylene glycol (6.8%), cocamidopropyl betaine (6.4%), bacitracin (6.2%), formaldehyde (5.7%), and gold (5.7%). ConclusionsThis US database provides multidisciplinary information on pediatric ACD, rates of PPT, and relevant RPPT reactions, validating the high rates of pediatric ACD previously reported in the literature. The registry database is the largest comprehensive collection of US-only pediatric patch test cases on which future research can be built. Continued collaboration between patients, health care providers, manufacturers, and policy makers is needed to decrease the most common allergens in pediatric consumer products.

Patch Testing for Evaluation of Hypersensitivity to Implanted Metal Devices: A Perspective From the American Contact Dermatitis Society.

The American Contact Dermatitis Society recognizes the interest in the evaluation and management of metal hypersensitivity reactions. Given the paucity of robust evidence with which to guide our practices, we provide reasonable evidence and expert opinion–based guidelines for clinicians with regard to metal hypersensitivity reaction testing and patient management. Routine preoperative evaluation in individuals with no history of adverse cutaneous reactions to metals or history of previous implant-related adverse events is not necessary. Patients with a clear self-reported history of metal reactions should be evaluated by patch testing before device implant. Patch testing is only 1 element in the assessment of causation in those with postimplantation morbidity. Metal exposure from the implanted device can cause sensitization, but a positive metal test does not prove symptom causality. The decision to replace an implanted device must include an assessment of all clinical factors and a thorough risk-benefit analysis by the treating physician(s) and patient.

A Pragmatic Approach to Patch Testing Atopic Dermatitis Patients: Clinical Recommendations Based on Expert Consensus Opinion

Allergic contact dermatitis (ACD) may complicate the clinical course of atopic dermatitis (AD), and patch testing remains the criterion standard for diagnosing ACD. To date, there have been no guidelines or consensus recommendations on when and how to patch test individuals with AD. Failure to patch test when appropriate may result in overlooking an important and potentially curable complicating comorbidity. In this article, we present consensus recommendations regarding when to perform patch testing in the AD patient, best practices, and common pitfalls. Patch testing should be considered in AD patients with dermatitis that fails to improve with topical therapy; with atypical/changing distribution of dermatitis, or pattern suggestive of ACD; with therapy-resistant hand eczema in the working population; with adult- or adolescent-onset AD; and/or before initiating systemic immunosuppressants for the treatment of dermatitis. A suggested patch testing algorithm for AD patients is provided.

Congenital nevi versus metastatic melanoma in a newborn to a mother with malignant melanoma - diagnosis supported by sex chromosome analysis and Imaging Mass Spectrometry.

A 37‐year‐old pregnant woman presented with a 2‐cm irregular reddish nodule on her left upper arm during pregnancy. A biopsy from the lesion showed a 2.2‐mm thick malignant melanoma with intravascular invasion, 25 mitosis/mm2 and no ulceration. Following induction of labor, the patient underwent re‐excision with sentinel lymph node biopsy. This showed no residual melanoma and no lymph node metastasis. The newborn boy had multiple pigmented lesions on the trunk, some of which were large and irregular. Two were biopsied and histologic examination showed dense dermal proliferation of medium sized melanocytes with multiple mitotic figures and no maturation with their descent into the dermis, raising suspicion of transplacental metastases. Examination of the placenta failed to show metastatic lesions. Multiplex polymerase chain reaction (PCR)‐based genotyping, including testing for amelogenin locus for sex chromosome determination, demonstrated the presence of Y chromosome material in the melanocytes of the newborns lesions excluding maternal origin. A diagnosis of congenital nevi was rendered. Subsequently, Imaging Mass Spectrometric analysis of the mothers lesion showed proteomic signature expression indicative of malignant melanoma, whereas the two lesions in the newborn showed changes indicative of nevi. This case demonstrates the utility of genotyping and Mass Spectrometry analysis in this challenging clinical scenario

Perineural granulomas in cutaneous sarcoidosis may be associated with sarcoidosis small-fiber neuropathy

Perineural granulomas in cutaneous sarcoidosis have been rarely reported and their clinical significance has yet to be evaluated. Recently, a 27‐year‐old male presented with multiple pink papules on the flank and lower back, accompanied by a painful, burning sensation. Biopsies revealed well‐defined granulomas, consistent with sarcoidosis, in the dermis and involving small cutaneous nerves. We hypothesized that perineural granulomas may be an under‐recognized feature of cutaneous sarcoidosis and may be responsible for sensory disturbances. We reviewed cases from 29 consecutive patients with cutaneous sarcoidosis. Perineural granulomas were identified in 18/29 (62%) patients and in 22/40 (55%) biopsies. Perineural granulomas were identified in 7/9 biopsies from the proximal upper extremity, 1/3 from the distal upper extremity, 7/12 from the head and the neck, including 4/4 from the nose, 5/9 from the back, 1/2 from the flank and 1/1 from the proximal lower extremity and 0/4 from the distal lower extremity. The anatomical distribution is similar to sarcoidosis small‐fiber neuropathy (SSFN), in which sarcoidosis patients without evident skin lesions experience sensory disturbances of unknown etiology involving the face, the proximal extremities and the trunk. Our results indicate perineural granulomas in cutaneous sarcoidosis are more common than previously appreciated, primarily involve the head, the proximal upper extremities and the back, and may be responsible for neurological manifestations.

The coexistence of lupus erythematosus panniculitis and subcutaneous panniculitis-like T-cell lymphoma in the same patient

CT: computed tomography HPS: hemophagocytic syndrome LEP: lupus erythematosus panniculitis PET: positron emission tomography SPTCL: subcutaneous panniculitis-like T-cell lymphoma INTRODUCTION Lupus erythematosus panniculitis (LEP) most commonly presents as multiple deep subcutaneous indurated nodules or erythematous plaques involving the face and proximal extremities. Lipoatrophy is another characteristic feature on resolution. Histopathologically, LEP shows lymphoplasmacytic lobular panniculitis, often with dermal mucin deposition, hyaline fat necrosis, and lymphoid follicles. There may be epidermal vacuolar change and a perivascular and periadnexal lymphocytic infiltrate with prominent plasma cells. Treatment options include antimalarials, systemic steroids, dapsone, or rituximab. LEPmay be limited to the subcutaneous fat; in such cases, it can be difficult to entirely exclude subcutaneous panniculitis-like T-cell lymphoma (SPTCL). Histopathologic findings of SPTCL showpleomorphic T cells in the subcutaneous adipose tissue in a pattern mimicking panniculitis. Since 2005, SPTCL has been restricted to ab pannicular infiltrates, usually CD8 and CD56 , and has an indolent outcome. These patients can be treated with systemic corticosteroids, bexarotene, interferon, or low-dose chemotherapy with a single agent such as methotrexate. In contrast, pannicular lymphomas with gd phenotype are usually CD8 and CD56 and may also involve the dermis or epidermis. These patients are generally treated with multiagent chemotherapy and stem cell transplantation, but the prognosis is poor. The histopathologic distinction of LEP and SPTCL can be challenging (Table I) because of significant overlap; other clues that can aid in differentiating LEP from SPTCL include the clinical presentation and