Woman Seeks Answers After 3 Years of Painful Genital Ulcers

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A woman in her late 40s had painful ulcers in her groin and armpits that had not healed for three years, a group from India reported. JAMA Dermatology.

The woman explained that when the sores first appeared, they were itchy, oozing, red bumps. Over time, they worsened and formed painful, pus-filled, oozing ulcers. but the pain never completely went away. Her scalp was often scaly and sticky, she added, which had not improved with her shampoos and topical steroids.

Keshavamurthy Vinay, M.D., Ph.D., of the Graduate School of Medical Education and Research, Chandigarh, India, said the patient had been urinating very frequently for the past four years and was always thirsty when providing a more detailed medical history. I pointed out that colleague.

A physical examination by the clinician revealed multiple mobile lymph nodes in the neck and groin, which were firm, dull, and nontender. The patient’s liver was enlarged 2 cm below the right rib margin, and the parotid gland area was also enlarged bilaterally and tender on palpation.

The patient’s scalp had yellowish, greasy scales resembling seborrheic dermatitis. The clinician also noticed red, swollen bumps on the skin behind the ears and in the folds under the chest.

The external genitalia are notable for deep ulcers resembling knife-cut ulcers on both the left and right inguinal folds, Vinay and co-authors write, and similar yellowish, pus-draining lesions appear on the labia majora and the labia majora. Found at the junction of the labia minora. The team obtained him a 3.5 mm punch biopsy specimen from around an ulcer in one of his inguinal folds.

“Histopathological examination revealed a dense infiltration of the lower epidermis and upper dermis by atypical Langerhans cells,” Vinay and colleagues reported. “These cells were large and had a characteristic coffee-bean-shaped grooved vesicular nucleus and moderately amphipathic cytoplasm.” Staining was positive for CD1a and langerin. Based on these findings, clinicians diagnosed the patient with Langerhans cell histiocytosis (LCH).

Routine blood tests returned normal results, and enzyme-linked immunosorbent assays for HLA-B51 and Epstein-Barr virus IgM and IgG were negative. PET scans revealed “abundant involvement of fluorodeoxyglucose in the bone marrow, skeletal system (in the form of lytic lesions), lungs, bilateral salivary glands, mucous membranes, and skin of flexures and scalp.”

A female patient had pituitary involvement in the form of diabetes insipidus confirmed on contrast-enhanced MRI that revealed the absence of posterior pituitary bright spots.

Clinicians have reached a final diagnosis of multisystem LCH with low-risk organ dysfunction.

discussion

The clinician who reported this case of LCH, a clonal proliferation disorder of Langerhans cells, noted that the disease rarely occurs in adults. Children It tends to be more commonly affected, especially children between the ages of 1 and 3.

“Apart from the typical cutaneous features such as refractory seborrheic dermatitis, candida intertriginus, and flexural eczematous eruptions observed in this patient, nonhealing ulcers of the perineum and external genitalia were , may be a hallmark of LCH,” Vinay’s group wrote.

Adults with LCH tend to present with multisystem disease, most commonly bone related. Cutaneous manifestations occur in 50% of cases, disease confined to the skin It has been observed to be rare in reported adult LCH cases.

LCH involving skin, bone, lymph nodes, lungs, or pituitary is classified as low-risk disease, and LCH with hematopoietic, liver, or splenic dysfunction is classified as high-risk multisystem LCH.

The case author described several differential diagnoses that were considered. They said the patient’s genital lesions resembled those seen in metastatic Crohn’s disease. Metastatic Crohn’s disease may present with similar fissures, swelling, and deep knife-cut ulcers on the vulva and perineum, separated from the underlying gastrointestinal pathology.

However, in contrast to LCH, the examination would provide histologic evidence of noncaseating granuloma and evidence of gastrointestinal involvement in Crohn’s disease, the authors said.

They also considered Behcet’s disease, which is known to be associated with genital ulcers, but histopathological examination revealed that dermal neutrophilic infiltration, leukocytoclastic vasculitis, and thrombosis were associated with systemic ulceration. It ultimately did not fit the bill as it was shown alongside symptoms of thrombosis and vasculitis, uveitis, and positive pathology. test.

Other conditions that can cause painful genital or perineal ulcers include cutaneous T-cell lymphoma (CTCL), such as extranodal natural killer/T-cell lymphoma and primary anaplastic large cell lymphoma, the authors said. noted and added that the epidermotropic invasion observed in this patient could also occur in CTCL. “Immunohistochemistry for T cell-specific and other markers such as CD56 and CD30 may be required to rule out CTCL in LCH cases with marked epidermotropism,” they wrote. .

Because genital involvement often mimics the manifestations of other multisystem inflammatory diseases, Vinay et al. A histopathological diagnosis was prompted.

“This will allow proper screening for systemic involvement and early initiation of appropriate treatment,” they said.

Disclosure

The case authors reported no conflicts of interest.

Primary information

JAMA Dermatology

Source reference: Anubha D, et al “Non-healing genital ulcers as clues to multisystem disease.” JAMA Dermatol 2023; DOI: 10.1001/jamadermatol.2022.5863.

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