Weiss, Moschella, Kwan, and Craven report no conflict of interest. The authors report no discussion of off-label use. Accepted for publication June 3, Acquired reactive perforating collagenosis ARPC is a rare perforating disease of the skin. It is characterized by hyperkeratotic papules with transepidermal elimination of degenerated material including collagen and elastic fibers. The disease presents clinically as umbilicated papules with a central adherent keratotic plug.
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Weiss, Moschella, Kwan, and Craven report no conflict of interest. The authors report no discussion of off-label use. Accepted for publication June 3, Acquired reactive perforating collagenosis ARPC is a rare perforating disease of the skin.
It is characterized by hyperkeratotic papules with transepidermal elimination of degenerated material including collagen and elastic fibers. The disease presents clinically as umbilicated papules with a central adherent keratotic plug. Mucormycosis infection, caused by the molds of the class Zygomycetes and order Mucorales, generally occurs as an opportunistic infection. It presents most frequently in patients with diabetes mellitus, in patients with leukemia receiving chemotherapy, and in those on sustained immunosuppressive therapy.
We describe a patient with type 2 diabetes mellitus and end-stage renal disease requiring hemodialysis in whom extensive cutaneous mucormycosis with secondary spread to the brain, lumbar spine, and breast developed in the setting of ARPC. To our knowledge, this is the first case report of a patient with ARPC who developed extensive cutaneous mucormycosis. Acquired reactive perforating collagenosis ARPC , first described in by Mehregan et al, 1 is a rare perforating disease of the skin characterized by hyperkeratotic papules with transepidermal elimination of degenerated material, including collagen and elastic fibers, that present clinically as umbilicated papules with a central adherent keratotic plug.
Over a 4-month period, a year-old white woman with type 2 diabetes mellitus and end-stage renal disease requiring hemodialysis 3 times weekly developed multiple skin papules followed by night sweats and a lb weight loss. The patient initially presented to the dermatology department at the Lahey Clinic Medical Center in Burlington, Massachusetts, complaining of severe pruritus and several small skin lesions that resembled warts Figure 1.
When the lesions enlarged, she was able to extract the core by the "root," yielding a gelatinous exudate. Having achieved no relief from topical antipruritics over the subsequent month, the patient returned to the dermatology department.
By that time, she had developed large necrotic plaques approximately 8—12 cm in diameter on her right lateral thigh Figure 3 and smaller lesions on her anterolateral tibiofibular areas bilaterally. The infectious disease department was consulted, and the patient was admitted to the hospital for a wedge biopsy, diagnosis, and therapy. On physical examination, the patient was a pale, elderly, afebrile, normotensive obese woman in no apparent distress.
Her cardiopulmonary examination results were normal. The dialysis catheter site in her left anterior chest was not indurated or erythematous, though a left breast mass inferior to the dialysis port was noted. Examination of her skin revealed multiple ulcerated, umbilicated red-brown papules ranging from 4 to 8 mm, a few of which had a retained adherent keratotic plug.
Most lesions had an area of central necrosis and were surrounded by an erythematous ring. The two 8- to cm necrotic ulcerations on the patient's right lateral thigh were acutely tender, were surrounded by a 3- to 4-mm erythematous border, and appeared to extend into the subcutaneous tissue. Initial laboratory results included a leukocyte count of 7. Transaminases, bilirubin, and alkaline phosphatase levels were within reference range.
Blood cultures, both peripheral and from the dialysis catheter port, grew coagulase-negative staphylococci. The dialysis catheter was removed and had more than colonies of coagulase-negative staphylococci; fungal plate culture results were negative.
Pathologic examination of the biopsy of the gangrenous plaque on the right thigh revealed ischemic necrosis of the epidermis, dermis, and subcutis, with hyphal forms throughout the biopsy and within thrombosed vessel walls Figure 4.
Cultures grew mucormycosis, genus Rhizopus. Plain films of the patient's femur and tibia demonstrated no bony destruction. Computed tomography of the chest and abdomen revealed a compression fracture with a probable vertebral lesion and possible paraspinal abscess at the fourth lumbar vertebra. Magnetic resonance imaging of the central nervous system displayed focal areas of subacute hemorrhage that were consistent with a possible infectious process.
Additional focal areas of abnormal enhancement were found within the right centrum semiovale and in the leptomeningeal space, suggestive of infection, infarct, or tumor. Results of the cerebral spinal fluid examination were unremarkable. The small mass noted in her left breast on initial presentation rapidly increased to the size of a tennis ball. The infection in the thighs and tibiofibular areas were so extensive that surgical resection was not performed. The patient died of progressive disease after 3 weeks of hospitalization; permission for postmortem examination was not granted.
This patient presented with 2 unusual illnesses, ARPC and cutaneous mucormycosis, which may have had a cause-and-effect relationship. Both of these diseases are more likely to occur in patients with diabetes mellitus and end-stage renal disease. Perforating collagenosis may present in 2 forms, an inherited autosomal-recessive form and a sporadic acquired form that may be associated with systemic diseases.
As in our patient, most patients with ARPC also have diabetes or renal failure. The constant rubbing results in an epithelial hyperplasia, follicular hyperkeratosis, or prurigolike lesions. Varying clinical and histologic findings are considered to be due to different stages of disease. Cutaneous mucormycosis is a rare, acute, subacute, or chronic infection with only cases listed in the English literature as of The mycelium of the Mucorales pathogen is composed of nonseptate coenocytic hyphae, which have a special affinity for blood vessels.
Mucor fungi may cross fascial planes with little regard for tissue barriers by using proteases, lipases, and mycotoxins that may cause further infarction and tissue necrosis.
Although ubiquitous in the environment, particularly soil, Mucor, like other opportunistic saprophytes, almost never causes disease unless the patient also has underlying immunosuppression, neutropenia, or burns and there is direct cutaneous inoculation of the organism or spores at a site of trauma or impaired skin integrity.
Secondary hematogenous spread to her brain, lumbar spine, and breast most likely occurred. Another site of disseminated infection may have been the dialysis catheter, despite the negative culture results.
Treatment strategies generally require intravenous amphotericin B therapy coupled with surgical excision and extensive debridement of the skin lesions. Current oral azole antifungal agents and caspofungin generally are not useful in treating mucormycosis. To our knowledge, cutaneous mucormycosis has not been previously reported in the setting of ARPC.
Because ARPC is common in patients with diabetes and end-stage renal disease, physicians should be alerted to the possibility and potential complications of fungal, mycobacterial, or bacterial opportunistic superinfection of these skin lesions and the necessity of appropriate diagnostic tests and early management and therapy.
Skip to main content. Coronavirus News Center. Author and Disclosure Information Drs. Stefan C. Weiss, MD; Samuel L. Craven, MD Accepted for publication June 3, CASE REPORT Over a 4-month period, a year-old white woman with type 2 diabetes mellitus and end-stage renal disease requiring hemodialysis 3 times weekly developed multiple skin papules followed by night sweats and a lb weight loss.
Comment This patient presented with 2 unusual illnesses, ARPC and cutaneous mucormycosis, which may have had a cause-and-effect relationship. Menu Menu Presented by. Menu Close.
Cutaneous Mucormycosis Secondary to Acquired Reactive Perforating Collagenosis
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