J Am Acad Dermatol

Nonneoplastic disorders of the eccrine glands.

Wenzel FG. Horn TD.
Department of Dermatology, The Johns Hopkins School of Medicine, Baltimore, Maryland 21287, USA.
Eccrine glands are uniquely susceptible to a variety of pathologic processes. Alteration in the rate of sweat secretion manifests as hypohidrosis and hyperhidrosis. Obstruction of the eccrine duct leads to miliaria. The excretion of drugs into eccrine sweat may be a contributory factor in neutrophilic eccrine hidradenitis (NEH), syringosquamous metaplasia (SSM), coma bulla, and erythema multiforme (EM). Alterations in the electrolyte composition of eccrine sweat can be observed in several systemic diseases, most notably cystic fibrosis. This article summarizes current knowledge of eccrine gland pathophysiology.

Coexistence of lupus erythematosus and porphyria cutanea tarda in fifteen patients.

Year 1998
Gibson GE. McEvoy MT.
Department of Dermatology, Mayo Clinic, Rochester, Minnesota 55905, USA.
BACKGROUND: Lupus erythematosus (LE) and porphyria cutanea tarda (PCT) may have similar clinical presentations, and their coexistence presents special problems in diagnosis and management. OBJECTIVE: The purposes of this study were to describe a patient with discoid LE and PCT and to present a review of 15 patients with coexistent LE (all variants) and PCT. METHODS: The medical records of all patients with coexistent LE and PCT seen between 1976 and 1995 were retrospectively reviewed. RESULTS: Of 6179 cases of LE and 676 cases of porphyria (all variants), there were 15 patients, 6 men and 9 women, with coexistent LE and PCT. The mean age at diagnosis of LE was 42.8 years and of PCT was 48 years. Nine patients had discoid LE; five patients had systemic LE, and one patient had subacute cutaneous LE. The initial diagnosis was LE in eight patients, PCT in five patients, and simultaneous LE and PCT in two patients. Precipitating factors for PCT included alcohol in seven patients, iron overload in one patient, and estrogen in one patient. Treatment of LE with hydroxychloroquine 200 mg daily precipitated PCT in two patients. Patients were treated with phlebotomy or low-dose antimalarials for PCT. Patients with systemic LE received systemic glucocorticoid therapy. Patients with discoid LE and subacute cutaneous LE were treated with topical glucocorticoids. CONCLUSION: The association of LE and PCT poses therapeutic challenges. The preferred treatment for one may exacerbate the other. Use of standard dose antimalarials for LE is inadvisable, and phlebotomy or low-dose antimalarials should be used cautiously in patients with coexistent disease.

Carbamazepine-induced pseudolymphoma with CD-30 positive cells.

Year 1998
Nathan DL. Belsito DV.
Division of Dermatology, University of Kansas Medical Center, Kansas City 66160, USA.
A 44-year-old woman known to be allergic to phenytoin was treated with carbamazepine for 1 month and developed fever, lymphadenopathy, pneumonitis, hepatitis, and a morbilliform eruption. A skin biopsy specimen showed atypical lymphocytes in the dermis that were CD-3+, CD-30+, and L26-. T-cell gene rearrangement studies were negative. A diagnosis of anticonvulsant hypersensitivity syndrome with histologic features of a pseudolymphoma was made and her illness quickly improved after carbamazepine was discontinued. This case was typical of the anticonvulsant hypersensitivity syndrome and demonstrated cross-reactivity among the aromatic anticonvulsants. However, to our knowledge, this represents the first report of a carbamazepine-induced hypersensitivity with histologic features of a cutaneous pseudolymphoma, including CD-30+ cells.

Childhood-onset porphyria cutanea tarda: successful therapy with low-dose hydroxychloroquine (Plaquenil).

Year 1998
Bruce AJ. Ahmed I.
Department of Dermatology, Mayo Clinic and Mayo Foundation, Rochester, Minnesota, USA.
We describe a 4-year-old girl with a spontaneous blistering disorder that was consistent with porphyria cutanea tarda (PCT). There was no familial history of the disease or any obvious causative factors present. Oral hydroxychloroquine (3 mg/kg) was given twice weekly along with vitamin E (200 U/d) as an antioxidant. Within 6 weeks, marked decreased blistering occurred and by 12 weeks no blistering was evident. Despite clinical improvement and tolerance of hydroxychloroquine, urinary uroporphyrin, aspartate aminotransferase, and ferritin levels continued to rise reaching peak levels at 16 weeks of therapy. Near total biochemical remission was observed at 40 weeks and all therapy was discontinued at 60 weeks.

Iatrogenic necrolytic migratory erythema: a case report and review of nonglucagonoma-associated necrolytic migratory erythema.

Year 1998
Mullans EA. Cohen PR.
Department of Dermatology, University of Texas-Houston Medical School, 77030, USA.
Necrolytic migratory erythema is characterized by waves of irregular erythema in which a central bulla develops, and subsequently erodes and becomes crusted. It usually occurs in patients with an alpha-islet cell tumor of the pancreas. However, necrolytic migratory erythema has also been observed in patients without an associated glucagonoma. We describe a woman with iatrogenic necrolytic migratory erythema. She received intravenous glucagon for hypoglycemia associated with an insulin-like growth factor II-secreting hemangiopericytoma. After chemotherapy, she developed necrolytic migratory erythema. The characteristics of the previously reported patients with nonglucagonoma-associated necrolytic migratory erythema are reviewed. In patients with nonglucagonoma-associated necrolytic migratory erythema, the dermatosis-related conditions most commonly observed were celiac disease or malabsorption, cirrhosis, malignancy, and pancreatitis; less common conditions included hepatitis, inflammatory bowel disease, heroin abuse, and odontogenic abscess. Although the pathogenesis of necrolytic migratory erythema remains unknown, hyperglucagonemia appears to have had a causative role in the development of this dermatosis in our patient. Patients who develop necrolytic migratory erythema should be evaluated for the presence of a glucagonoma; if a glucagonoma is ruled out, evaluation for other conditions known to occur with necrolytic migratory erythema, such as liver disease, malabsorptive disorders, and nonislet-cell tumors is warranted.