Histiocytoid giant cellulitis-like Sweet's syndrome: case report and review of the literature.

BACKGROUND: Histiocytoid Sweet syndrome is an uncommon variant in which the dermal infiltrate is composed of mononuclear cells with a histiocytic appearance that represent immature myeloid cells. Giant cellulitis-like Sweet syndrome is a recently described variant characterized by relapsing widespread giant lesions. PURPOSE: We report a unique patient with histiocytoid giant cellulitis-like Sweet syndrome and review the current literature on histiocytoid Sweet syndrome and giant cellulitis-like Sweet syndrome. MATERIAL AND METHODS: We reviewed PubMed for the following terms and have reviewed the literature: histiocytoid, giant cellulitis-like, and Sweet syndrome. RESULTS: Six individuals, including our patient, have been reported with giant cellulitis-like Sweet syndrome; four had obesity, two had a hematologic malignancy, and one had breast cancer. Histiocytoid Sweet syndrome has been reported in association with autoimmune diseases, infection or inflammation, inflammatory bowel disease, malignancies, medications, and other conditions. CONCLUSIONS: Histiocytoid Sweet syndrome is a rare variant of Sweet syndrome, often associated with malignancy. Giant cellulitis-like Sweet syndrome has been reported in six individuals; four of the patients were obese and three of the patients had an associated cancer. Our patient had histiocytoid giant cellulitis-like Sweet syndrome-associated myelodysplastic syndrome/myeloproliferative disorder. The diagnosis of histiocytoid Sweet syndrome or giant cellulitis-like Sweet syndrome should prompt the clinician to consider additional evaluation for a Sweet syndrome-associated malignancy.

Granuloma Annulare Mimicking Sarcoidosis: Report of Patient With Localized Granuloma Annulare Whose Skin Lesions Show 3 Clinical Morphologies and 2 Histology Patterns.

Granuloma annulare, a benign dermatosis of undetermined etiology, typically presents in a localized or generalized form. It has 3 distinctive histologic patterns: an infiltrative (interstitial) pattern, a palisading granuloma pattern, and an epithelioid nodule (sarcoidal granuloma) pattern. A man whose granuloma annulare skin lesions mimicked sarcoidosis is described. His localized granuloma annulare presented with a total of 3 lesions that each had a distinctive clinical morphology: an annular lesion of individual papules, a dermal nodule, and a linear arrangement of 3 papules. Two of his lesions showed a palisading granuloma histology pattern of granuloma annulare; however, the linear papules on his posterior neck lesion demonstrated noncaseating granulomas consistent with either the epithelioid nodule histology pattern of granuloma annulare or sarcoidal granuloma compatible with sarcoidosis. A comprehensive evaluation excluded the diagnosis of systemic sarcoidosis. Using the PubMed database, an extensive literature search was performed on granuloma annulare, epithelioid nodule, sarcoidal granuloma, and sarcoidosis. The histology patterns of granuloma annulare-emphasizing the history and differentiating features of the epithelioid nodule pattern from cutaneous sarcoidosis-were reviewed. The epithelioid nodule (sarcoidal granuloma) histology pattern of granuloma annulare is uncommon and may mimic the histology changes observed in sarcoidosis skin lesions; the absence of asteroid or other giant cell inclusions and an increase in mucin deposition between the collagen bundles favor the diagnosis of granuloma annulare. In addition, the epithelioid nodule pattern of granuloma annulare can rarely also show other histologic patterns of granuloma annulare in the same biopsy specimen or concurrently present with other clinical lesions of granuloma annulare that demonstrate a palisading granuloma, or possibly an infiltrative, histology pattern. However, the presence of an isolated skin lesion demonstrating sarcoidal granulomas--even when concurrently appearing with other lesions of granuloma annulare showing either an infiltrative or a palisading granuloma histologic pattern--may prompt the clinician to evaluate and exclude the possibility of systemic sarcoidosis.

Crusted scabies of the scalp in a patient with systemic lupus erythematosus.

BACKGROUND: Crusted scabies is a severe, hyperkeratotic, psoriasiform disorder associated with immune suppression. Affected individuals typically present with crusted hyperkeratotic lesions in a variety of locations. This condition can lead to severe complications: institutional outbreaks and secondary bacterial infections associated with sepsis and high mortality. MAIN OBSERVATIONS: A 37-year-old woman with a 12-year history of systemic lupus erythematosus treated with prednisone, methotrexate, and plaquenil presented with a three-week history of a painful scalp rash with adherent yellow scale. Skin biopsy and tissue culture were consistent with a diagnosis of crusted scabies with superficial bacterial infection. The patient was treated with oral ivermectin and permethrin cream, as well as ciprofloxacin for the bacterial infection. At one-week follow-up, the scalp was no longer tender and hyperkeratotic plaques had significantly improved. At one-month follow-up, the affected scalp demonstrated further improvement with decreasing erythema and alopecia with follicular ostia. CONCLUSIONS: Our case highlights the atypical presentation of crusted scabies with primary scalp involvement and need for vigilance in recognizing and appropriately treating this condition to prevent the consequences of longstanding infection. Combination treatment with ivermectin and permethrin is appropriate management for this condition.

Nonpseudomonal ecthyma gangrenosum associated with methicillin-resistant staphylococcus aureus infection: a case report and review of the literature.

Ecthyma gangrenosum (EG) is a skin infection that is classically associated with Pseudomonas aeruginosa septicemia in immunocompromised patients. Other bacterial, viral, and fungal pathogens also have been implicated in EG. Both bacteremic and nonbacteremic forms of EG have been described. We describe a case of EG associated with methicillin-resistant Staphylococcus aureus (MRSA) in a 35-year-old woman with acute lymphoblastic leukemia (ALL) and review the literature.

Mycobacterial infection identified with broad-range PCR amplification and suspension array identification.

The case of a 66-year-old female who developed sudden redness and swelling of her left thumb is presented. A biopsy showed well formed granulomas, mixed inflammation with a predominance of neutrophils, and acid-fast bacilli. After negative culture results, tissue was sent for a broad-range polymerase chain reaction amplification followed by suspension array identification, which classified the pathogenic organism as Mycobacterium avium-intracellulare. The patient was started on clarithromycin, rifabutin and ethambutol leading to clinical improvement. M. avium-intracellulare is present in soil, fresh water, sea water, dairy products and some animal tissues. Primary cutaneous manifestations are uncommon in the immunocompetent host. As cultures may be negative or can take up to three weeks to show growth, the molecular approach described here offers an opportunity for more rapid and specific diagnosis.

Human tumor antigen MUC1 is chemotactic for immature dendritic cells and elicits maturation but does not promote Th1 type immunity.

The immunostimulatory outcome of the interactions of many pathogens with dendritic cells (DCs) has been well characterized. There are many fewer examples of similar interactions between DCs and self-molecules, especially the abnormal self-proteins such as many tumor Ags, and their effects on DC function and the immune response. We show that human epithelial cell Ag MUC1 mucin is recognized in its aberrantly glycosylated form on tumor cells by immature human myeloid DCs as both a chemoattractant (through its polypeptide core) and a maturation and activation signal (through its carbohydrate moieties). On encounter with MUC1, similar to the encounter with LPS, immature DCs increase cell surface expression of CD80, CD86, CD40, and CD83 molecules and the production of IL-6 and TNF-alpha cytokines but fail to make IL-12. When these DCs are cocultured with allogeneic CD4+ T cells, they induce production of IL-13 and IL-5 and lower levels of IL-2, thus failing to induce a type 1 response. Our data suggest that, in vivo in cancer patients, MUC1 attracts immature DCs to the tumor through chemotaxis and subverts their function by negatively affecting their ability to stimulate type 1 helper T cell responses important for tumor rejection.