Mohs micrographic surgery-induced pemphigus.

BACKGROUND: Pemphigus is an autoimmune blistering disease that presents with flaccid intraepidermal blisters, erosions of the skin and mucous membranes, acantholysis, and in vivo bound and circulating autoantibodies against keratinocyte antigen. Currently a handful of reports incriminate surgical trauma as an initiating factor in this disease. OBJECTIVE: To document pemphigus evolving in a wound after Mohs micrographic surgery. METHODS: Case report. RESULTS: We present a case of pemphigus that started in a Mohs surgical wound after the excision of a squamous cell carcinoma (SCC) from a 49-year-old woman. Biopsy of the preoperative lesion did not reveal pemphigus. Biopsy of the postoperative lesion revealed pemphigus with no residual SCC. CONCLUSION: We suggest that Mohs surgery, like any other skin surgery, may nonspecifically activate pemphigus. This change must be differentiated from postoperative wound infection and other causes of poor wound healing.

Maffucci's syndrome: two case reports with a literature review.

Sixty-three cases of Maffucci's syndrome in the English literature plus two additional cases of our own are reviewed. This syndrome is nonhereditary and is characterized by multiple enchondromas and hemangiomas. It occurs in all races with no sex predominance. The enchondromas and hemangiomas can occur anywhere but are most common in the hands. Long bone involvement is common and leads to progressive skeletal deformity and pathologic fractures. The incidence of malignancies in patients with this syndrome is high. Chondrosarcomas are especially common and occur in 30% of the patients.

Physically induced pemphigus after cosmetic procedures.

BACKGROUND: Pemphigus is a vesiculobullous autoimmune disease characterized by acantholysis due to antibodies against epidermal antigens. CASE REPORTS: We report the first two cases of pemphigus in which lesions appeared in areas traumatized by cosmetic procedures. In one case, the patient had a history of pemphigus under excellent control; pemphigus lesions appeared only in her surgical scars 2 months after a reduction mammoplasty and facelift. The other patient presented with an outbreak of pemphigus 4 weeks after a chemical peel. CONCLUSIONS: Physical agents including infrared and ionizing radiation as well as ultraviolet light are known triggers of pemphigus. We suggest that cosmetic procedures can initiate pemphigus and that this change must be differentiated from postoperative wound infections and other reasons for poor wound healing.

Sweet syndrome in a neonate with aseptic meningitis.

A 7-week-old infant with antecedent otitis media, upper respiratory infection, and aseptic meningitis was diagnosed as having Sweet syndrome. Although this disease usually affects adults, it has been reported in 17 children. This is the youngest reported patient with the disorder to date, and the first in whom the syndrome was associated with aseptic meningitis.

Drug-induced pemphigus related to angiotensin-converting enzyme inhibitors.

We report a case of drug-induced pemphigus caused by an angiotensin-converting enzyme inhibitor, captopril. The cutaneous reaction remitted after withdrawal of captopril therapy. Unique to this case, however, was the substitution of another angiotensin-converting enzyme inhibitor, enalapril, without exacerbation of the pemphigus. To the best of our knowledge, this is the first reported patient with captopril-induced pemphigus in whom no new lesions developed after subsequent treatment with enalapril. A difference in chemical structure between these two drugs, particularly of a sulfur moiety, may help explain why the drug-induced disease did not recur.

Branchial cleft heterotopia of the lower neck.

A cutaneous ciliated structure from the lower neck of a 61-year-old man having seromucous, sebaceous and respiratory-type differentiation is reported. The finding of continuity between endodermal and ectodermal structures in the reported lesion suggests an origin from the branchial apparatus. Many lesions with similar appearance have been labeled bronchogenic though these, in fact, may have branchial origins. The term cutaneous bronchogenic cyst, implying an origin from the tracheobronchial tree, maybe misleading and should be reconsidered for those lesions with respiratory-type differentiation in the lower neck.

Pemphigus and Kaposi's sarcoma.

A 77-year-old woman developed Kaposi's sarcoma (KS) during prednisone therapy for pemphigus foliaceus. There are 7 prior reports in the literature of KS occurring in association with pemphigus. There are multiple reports of KS occurring in patients without pemphigus who are immunosuppressed either by malignancy or by chemotherapy for various disorders. Although the relationship between KS and pemphigus is a real one, KS is more likely related to therapeutically induced immunosuppression in certain predisposed individuals rather than primarily to pemphigus itself.

Pemphigus, myasthenia gravis, and pregnancy.

A 27-year-old woman with active but stable myasthenia gravis developed pemphigus while she was pregnant. Therapeutic abortion performed for personal reasons was followed by clinical and serologic improvement in her immunobullous disease. Both autoimmune conditions, pemphigus and myasthenia gravis, may be adversely affected by pregnancy. As with another immunobullous disease, herpes gestationis (bullous pemphigoid of pregnancy), hormonal factors appear to induce pemphigus. Although myasthenia gravis and pemphigus have been reported during pregnancy, this is the first instance of coexisting myasthenia gravis and pemphigus in a pregnant woman.

Unusual manifestations of secondary syphilis and abnormal humoral immune response to Treponema pallidum antigens in a homosexual man with asymptomatic human immunodeficiency virus infection.

The case presented here involves a 32-year-old homosexual man with human immunodeficiency virus (HIV) seropositivity and unusual manifestations of secondary syphilis. The patient presented with syphilitic keratoderma and chorioretinitis, and his appearance superficially resembled that of a patient with Reiter's syndrome. Although nontreponemal and treponemal tests for syphilis showed reactivity, the patient's humoral immune response to individual polypeptides of Treponema pallidum, measured by Western blot analysis, was markedly abnormal. The possible relationship between asymptomatic HIV infection and an abnormal humoral immune response to a second pathogen, in this case T. pallidum, is discussed. Our case is one of several recent cases of active syphilis reported in individuals with HIV seropositivity.

Congenital smooth-muscle hamartoma.

Congenital smooth-muscle hamartomas are rare, benign tumors of the skin. Since their original description in 1969, 16 case reports have appeared in the literature, with 7 cases reported within the last year. We describe the 17th patient with this lesion and have included a review of the literature. In addition, we describe three morphologic types of smooth muscle cells-the pale cells, dark cells, and intermediate cells-found on electron microscopy, and the features that support our belief that these may reflect different stages of maturity of the smooth-muscle cells. In addition to the smooth muscle, bundles of nerve fibers (both unmyelinated and myelinated) appear to be an intrinsic part of the lesion, as are the prominent vellus hairs. Congenital smooth muscle hamartoma thus appears to be an organoid nevus.

Sclerosing agents in the treatment of telangiectasia. Comparison of the clinical and histologic effects of intravascular polidocanol, sodium tetradecyl sulfate, and hypertonic saline in the dorsal rabbit ear vein model.

A 0.25-mL quantity of 0.25%, 0.5%, and 1.0% polidocanol (Aethoxysclerol [France]), 0.5% sodium tetradecyl sulfate (Sotradecol injection), and 23.4% hypertonic saline was injected into the dorsal marginal rabbit ear vein; clinical and histologic thrombosis resulted that lasted between four and eight days. The lowest concentration of polidocanol (0.25%) demonstrated immediate thrombosis; however, no clinical or histologic changes occurred eight days after injection. With all other agents, histologic fibrosis of the vessel correlating with clinical disappearance occurred after eight days. However, 0.5% polidocanol and sodium tetradecyl sulfate developed recanalization through the initially sclerosed vessel between eight and 14 days, with clinical reappearance of the 0.5% polidocanol-injected vessel at 30 days, after injection. Cutaneous necrosis was noted clinically and histologically in three of ten vessels injected with 1.0% polidocanol and in two of ten vessels injected with hypertonic saline. Clinical and histologic evidence of necrosis occurred with and without extravasation of the sclerosants.

Pyoderma gangrenosum and hairy cell leukemia.

Hairy cell leukemia (HCL) is an uncommon hematologic malignancy in which simultaneous cutaneous disturbances of various types have been reported. We report a case of a 62-year-old man with HCL who developed pyoderma gangrenosum (PG) at the site of incision for splenectomy. PG has been reported previously in a patient with HCL. Since splenectomy is important in the treatment of HCL, it is important for the clinician to know that surgical wound dehiscence may not be due to poor technique nor infection, but rather to pathergy.

Postsclerotherapy hyperpigmentation: a histologic evaluation.

Linear or macular pigmentation occurs in 10-30% of patients following sclerotherapy of vessels between 0.1 and 5 mm in diameter. Its occurrence is related to solution strength, vessel fragility, injection pressure, and the type of solution used. This adverse sequela of treatment has been assumed, by some, to represent post-inflammatory hyperpigmentation (incontinence of melanin pigment), and has been said to occur in individuals with this tendency. Histologic data presented in this paper suggest that this phenomenon does not represent melanocytic alteration, but is secondary to extravasation of red blood cells into the dermis following rupture of fragile vessels with resulting deposition of hemosiderin. Therapy has included bleaching agents (hydroquinones), trichloroacetic acid, and phenolic peeling agents with variable success. Eighty percent of patients who experience this adverse sequela will clear spontaneously within 6-24 months. The remaining patients will have persistence of pigmentation for up to 5 years, with a small number of patients having pigmentation persisting 5 years after therapy.

Cutaneous involvement in localized forms of bullous pemphigoid.

In summary, there is much overlap among the three types of localized cutaneous pemphigoid discussed. They all tend to affect the same age group, in general. They all may remain regional or be associated with a generalized eruption consistent with bullous pemphigoid. These three variants of subepidermal blistering disease also appear to be susceptible to koebnerization. Direct immunofluorescence findings are identical, as well, with linear IgG and C3 at the basement membrane. Indirect immunofluorescence tends to be negative for all three variants, with a tendency to positivity with increased extent and severity of skin involvement. There are differences, though, between them. BPP tends to affect men more than women, unlike BMMP-CP and localized cutaneous nonscarring BP. Localized cutaneous nonscarring BP usually involves the legs, whereas the other two conditions affect the head and neck, with resultant scarring. Histology is similar to that seen in BP, but dermal fibrosis and sclerosis result in skin affected by BMMP-CP and BPP. Localized cutaneous nonscarring BP is more amenable to topical steroid therapy, whereas BMMP-CP and BPP are more persistent and resistant to systemic immunosuppressive therapy. Clinical, histologic, and immunologic similarities among the pemphigoid variants may reflect common antigenic features. On the other hand, clinical, histologic, and immunologic differences may imply that there are more than one bullous pemphigoid antibody directed at a variety of antigenic structures.