PMC5817217_01
Photos of pretreatment right-axillary ulcer (a); cutaneous eruptions of LCH at the retroauricular area (b), scalp (c), and under the breast (d); posttreatment (after 4.5 months of imiquimod) status at the right axilla (e).
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The case described here is a 61-year-old Japanese female who had been treated for diabetes mellitus and a refractory large ulcer (2.0 cm x 2.6 cm) at her right axilla (Figure 1(a)) for nearly a year. Prior to the diagnosis, cutaneous eruptions, such as erythematous papules/vesicles at retroauricular regions, crusted papules at the scalp, and reddish-brown papules at the lower chest under the breasts, were present (Figures 1(b), 1(c), and 1(d)). After the diagnosis of LCH, we chose to treat this patient systematically, because the axillary ulcer was so deep and enlarged (see Figure 1(a)) along with the presence of CNS lesion. Following treatment for 4 months, the relapsed axillary ulcer as well as other cutaneous lesions improved significantly (Figure 1(e)).
Pathology of the biopsied pretreatment axillary ulcer. H&E stain (a; original magnification x200 with magnified photo showing LCH cells with folded coffee bean-like nucleus, x400) and immunostaining of S100-positive (b), CD1a-positive (c), and CD207-positive (d) LCH cells (original magnification: x200).
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Eventually, the ulcer was biopsied, revealing a typical LCH pathology, with dermal infiltrate of morphologically characteristic Langerhans cells extending into the epidermis, which were positive for S100, CD1a, and CD207, with other inflammatory cells (Figure 2).
Clinical course and treatment. Biopsies #1/2 were done on the axillary ulcer and the retroauricular cutaneous lesions and biopsy #3 was done on the relapsed retroauricular cutaneous lesion. Diagnosis of LCH was made after biopsy #1. Pathological findings of #2 and #3 were almost identical to those of #1 shown in Figure 2. Details of the chemotherapy (VBL/AraC/PSL) and imiquimod regimens are described in the text. Symptoms of central diabetes insipidus were controlled by DDAVP.
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Three months later, the axillary ulcer relapsed and cutaneous eruptions reappeared, and diagnosis of LCH was again confirmed by a biopsy (Figure 3).