Biomedical subjects
Publications and source records attributed to Diya F Mutasim.
Macular arteritis is a novel form of cutaneous arteritis in which the primary lesion is a hyperpigmented macule. Traditional stigmata of cutaneous vasculitis such as palpable purpura and erythematous nodules are not present. The disease is asymptomatic and appears to follow an indolent course. Systemic involvement has not been observed.
A 75-year-old male presented with an eruption characteristic of digitate dermatosis. The eruption cleared completely after discontinuation of lisinopril (angiotensin converting enzyme inhibitor). Eighteen months later a similar eruption recurred after using valsartan (a competitive inhibitor of angiotensin II receptor). The eruption cleared after discontinuation of valsartan. Histologic findings in both eruptions were consistent with lymphomatoid drug eruption. This case is interesting in that the eruption occurred after the intake of two structurally unrelated drugs that interfere with angiotensin II function. This observation may shed light on the mechanisms that may be operative in provoking the lymphomatoid drug eruption.
Elderly individuals are susceptible to autoimmune bullous dermatoses (in particular, pemphigoid, epidermolysis bullosa acquisita and paraneoplastic pemphigus). Bullous dermatoses are associated with high morbidity and mortality. Bullous dermatoses result from autoimmune responses to one or more components of the basement membrane or desmosomes. Pemphigoid results from autoimmunity to hemidesmosomal proteins present in the basement membrane of stratified squamous epithelia. Patients present with tense blisters in flexural areas of the skin. Mild or moderate bullous pemphigoid may be treated with potent topical corticosteroids while extensive disease usually requires systemic corticosteroids or systemic immunosuppressive agents such as azathioprine. Mucosal pemphigoid affects one or more mucous membranes that are lined by stratified squamous epithelia. The two most commonly involved sites are the eye and the oral cavity. Lesions frequently result in scar formation, which may cause blindness. Patients with severe disease or ocular involvement require aggressive therapy with corticosteroids and cyclophosphamide. Epidermolysis bullosa acquisita results from autoimmunity to type VII collagen in the anchoring fibrils of the basement membrane area. Lesions may either arise on an inflammatory base or be non-inflammatory and result primarily from trauma. The inflammatory type of the disease is more responsive to therapy than the non-inflammatory type. Treatment options include corticosteroids, dapsone, cyclosporin, plasmapheresis and immunoglobulin G. Paraneoplastic pemphigus results from autoimmunity to multiple antigens within the desmosomes. The disorder is associated with neoplasms, especially leukaemia and lymphoma. Patients present with severe stomatitis and polymorphous skin eruption. The mucosal and cutaneous involvement may respond to successful treatment of the underlying neoplasm or may require immunosuppressive therapy.
BACKGROUND: Debates regarding nosology and clonality surround the entity known as cutaneous pseudolymphoma and its questionable transformation to frank cutaneous lymphoma. The relevance of these arguments is important, not only from a diagnostic standpoint, but also for making inferences based upon behavior, prognosis, and treatment. OBJECTIVE: Our goal was to demonstrate further evidence of progression from cutaneous pseudolymphoma to malignant lymphoma while at the same time advocating a comprehensive plan for evaluation, treatment, and followup of these patients. METHODS: A retrospective review was conducted of four patients initially considered to have cutaneous B-cell pseudolymphoma (CBPL) and who were later treated for primary cutaneous B-cell lymphoma (CBCL). A review of the literature of cases suggesting progression to malignant lymphoma from precursor lesions was also performed. RESULTS: Four patients initially diagnosed with CBPL by a combination of histologic, immunophenotypic, and gene rearrangement criteria had a progressive clinical course that, over a range of 17-51 months, evolved into CBCL. All patients had a comprehensive systemic workup to rule out the possibility of extracutaneous disease and were treated with local radiation therapy and close followup. There has been no evidence of extracutaneous disease with an average followup of 14 months. CONCLUSION: The potential for certain cutaneous pseudolymphomas to progress to CBCL is real. The combination of histologic and immunophenotypic criteria, along with the clinical picture, remains the best way to judge the aggressiveness of the lesion. Gene rearrangement studies, whether performed by Southern blot or polymerase chain reaction (PCR), are of limited value and should be used to support the overall clinicopathologic picture. Radiation therapy of these patients should be thought of early in the management plan and is a very successful form of treatment when combined with close followup.
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The elderly are prone to several autoimmune bullous diseases that have significant morbidity, and an accurate diagnosis is essential for proper management. It is important to be aware of the potential adverse effects of the various systemic agents that can be used.
Knuckle pads, first described by Garrod in 1893, 1 are benign, asymptomatic, well- circumscribed, smooth, firm, skin colored papules, nodules, or plaques. They most commonly occur on the dorsal aspect of the proximal interphalangeal joint of the finger, 2 but also may occur on the dorsal aspects of the foot over joints. 3-5 Knuckle pads may be inherited or acquired. 1 While some authors suggest that trauma is not a significant factor, 6 acquired knuckle pads have been associated with repetitive friction and trauma, 2,7 and may resemble athlete's nodules (also referred to as collagenomas). 1 Histologically, knuckle pads are characterized by hyperkeratosis, hypergranulosis, proliferation of fibroblasts and capillaries, and thickened and irregular collagen bundles. 1 Few cases of knuckle pads involving the lower extremities have been reported. 7 Knuckle pads of the feet have been described in association with inherited syndromes, such as acrokeratoelastoidosis Costa, 3 a syndrome of knuckle pads, leukonychia and deafness, 4 and a syndrome of knuckle pads, leukonychia, deafness and keratosis palmoplantaris. 5 We report a case that illustrates an unusual presentation of acquired knuckle pads of the feet secondary to repetitive friction from athletic gear.
Cicatricial pemphigoid is an autoimmune bullous disease that has a predilection for mucous membranes and often results in scarring. We describe a case of cicatricial pemphigoid with severe involvement of the glans penis, which, despite systemic therapy, led to phimosis.
A 42-year-old woman presented with a recurrent eruption over the distal extremities. Lesions formed a few days after initiation of intravenous immunoglobulin (IVIg) therapy and lasted 2 to 3 weeks after therapy completion. Results of a histologic examination revealed parakeratosis, exocytosis, and spongiosis. The clinical and histologic findings are similar to previous reports. The mechanism of this eruption is not known.
A 35-year-old white man with lifelong stable white lesions on the anterior trunk and extremities presented with a pruritic papular eruption limited to the white patches. Results of a histologic examination led to a diagnosis of Grover disease (transient acantholytic dermatosis). To our knowledge, this article is the first to report an association between Grover disease and piebaldism or other depigmented disorders. We review the literature and speculate on the association between these conditions.
Bullous systemic lupus erythematosus (SLE) is a rare skin manifestation of SLE. It shares many features with epidermolysis bullosa acquisita (EBA). We report on a patient with SLE who developed a vesiculobullous eruption followed by findings not typical in bullous SLE, namely milia, mild scarring, and calcinosis. We discuss the relationship between bullous SLE and EBA.
Acute urticaria is characterized by pruritic, erythematous, edematous plaques. We report the case of a 48-year-old man with acute urticaria, whose lesions appeared yellow secondary to an elevated bilirubin level.